VENTRICULAR SEPTAL DEFECT

VSD VSD

BY DR. VIKAS, DEPTT. OF BY DR. VIKAS, DEPTT. OF CTVS, PGIMER , CTVS, PGIMER , CHANDIGARH CHANDIGARH

CASE PRESENTATIONCASE PRESENTATION

Natik 2 year m child Natik 2 year m child

Magholi Chopal shimla .Magholi Chopal shimla . History of 1 Recurrent chest History of 1 Recurrent chest

infection .infection .

2 Difficulty in breathing .2 Difficulty in breathing .

3 Failure to thrive.3 Failure to thrive.

h/o fever present.h/o fever present. h/o cough present.no h/o hemoptysis.h/o cough present.no h/o hemoptysis. h/o dyspnea at rest present . No PNDh/o dyspnea at rest present . No PND No h/o wheeze.No h/o wheeze. Ho palpitation presentHo palpitation present No h/o cynosis.Squatting ,or No h/o cynosis.Squatting ,or Syncopal Syncopal

attack. attack. h/o puffiness of face distention of h/o puffiness of face distention of

abdomen present .abdomen present .

Family history - no h/o D M. HT .TB.Family history - no h/o D M. HT .TB. Past history . antinatal normal, Past history . antinatal normal, General examination .General examination . Height 82 CM Weight . 8 KG Height 82 CM Weight . 8 KG

66% GRADE 11 PEM`66% GRADE 11 PEM`

RAO’S INDEX .12 N (.15 to .16) SPO2 RAO’S INDEX .12 N (.15 to .16) SPO2 H R 130 / min R-R 34/min H R 130 / min R-R 34/min Pallor is present Pallor is present No cynosis ,icterus,clubbing , No cynosis ,icterus,clubbing , JVP ,LAP,Edema.JVP ,LAP,Edema.

CARDIOVASCULAR EXAMINATION .CARDIOVASCULAR EXAMINATION . precordium bulging present.precordium bulging present.

Apex beat 5Apex beat 5thth ICS - ICS -

Thrill present Thrill present

PSM GRADE .3PSM GRADE .3

InvestigationInvestigation

.Hb 13.5 g% TLC 4700..Hb 13.5 g% TLC 4700. Glucose . 79 mg% .Glucose . 79 mg% . Na 138 k 4.5. Cl 31 Na 138 k 4.5. Cl 31 S urea 43 S Creatinine .4S urea 43 S Creatinine .4

INTRODUCTIONINTRODUCTION

A A VSDVSD is a defect in the ventricular is a defect in the ventricular septum septum

The ventricular septum consists of an The ventricular septum consists of an inferior muscular and superior inferior muscular and superior membranous portionmembranous portion

The membranous portion -most The membranous portion -most commonly affected in adults and older commonly affected in adults and older childrenchildren

most common congenital cardiac most common congenital cardiac anomalies.anomalies.

3-3.8 per 1000 live births3-3.8 per 1000 live births

Ventricular Septal DefectVentricular Septal Defect

Most common CHD in children Most common CHD in children (25%)(25%)

75-80% of small VSD’s close 75-80% of small VSD’s close spontaneously by late childhoodspontaneously by late childhood

10-15% of large VSD’s close 10-15% of large VSD’s close spontaneouslyspontaneously

60% of defects close before age 3, 60% of defects close before age 3, and 90% before age 8and 90% before age 8

Risk factors for decreased survival Risk factors for decreased survival for unoperated patients include:for unoperated patients include: Cardiomegaly on CXR, Elevated Cardiomegaly on CXR, Elevated

PASP (>50 mmHg), and CV PASP (>50 mmHg), and CV symptoms.symptoms.


Henri Roger was the first man to Henri Roger was the first man to describe a ventricular septal defect, describe a ventricular septal defect, in 1879 he wrote: in 1879 he wrote: ““A developmental defect of the heart A developmental defect of the heart occurs from which cyanosis does not ensue occurs from which cyanosis does not ensue in spite of the fact that a communication in spite of the fact that a communication exists between the cavities of the two exists between the cavities of the two ventricles and in spite of the fact that the ventricles and in spite of the fact that the admixture of venous blood and arterial admixture of venous blood and arterial blood occurs. This congenital defect, which blood occurs. This congenital defect, which is even compatible with long life, is a is even compatible with long life, is a simple one. It comprises a defect in the simple one. It comprises a defect in the interventricular septum”interventricular septum”

Development of IVSDevelopment of IVS

Muscular septum Muscular septum – – primordial IV septumprimordial IV septum

Closure of Closure of interventricular interventricular foramen& foramen& membranous septummembranous septum formation- formation-

Rt & Lt bulbar ridgesRt & Lt bulbar ridges endocardial cushionsendocardial cushions

AnatomyAnatomy

Morphology – The Morphology – The Ventricular SeptumVentricular Septum

1. Membranous2. Outflow3. Trabecular

septum4. Inflow5. Subarterial /

Supracristal

AnatomyAnatomy 4 morphological components of 4 morphological components of

septumseptum MembranousMembranous InletInlet Outlet/InfundibularOutlet/Infundibular Muscular/TrabecularMuscular/Trabecular

AnatomyAnatomy Membranous-70-80%Membranous-70-80%

SmallSmall Located at base, between inlet and Located at base, between inlet and

outletoutlet Perimembranous - Extends to adjacent Perimembranous - Extends to adjacent

SSEseptumSSEseptumMembranous Membranous

AnatomyAnatomy Inlet Inlet

Inlet 5-8%, Inlet 5-8%, AV valve to chordae attachmentsAV valve to chordae attachments

Inlet

AnatomyAnatomy Outlet/Infundibular Outlet/Infundibular

5-7% 5-7% Separates L and R outflow tractsSeparates L and R outflow tracts

Infundibular

AnatomyAnatomy Muscular/Trabecular (5-20%)Muscular/Trabecular (5-20%)

Anterior/Marginal (anterior to septal Anterior/Marginal (anterior to septal band)band)

Midmuscular/Central (posterior to septal Midmuscular/Central (posterior to septal band)band)

Apical (inferior to moderator band)Apical (inferior to moderator band) Posterior (beneath septal leaflet) Posterior (beneath septal leaflet)

Muscular

Types of VSD (kirklin)Types of VSD (kirklin)

1

23

4

Hemodynamic Hemodynamic classification classification

RestrictiveRestrictive- resistance that limits the shunt at the site - resistance that limits the shunt at the site of vsdof vsd

LVSP > RVSPLVSP > RVSP pulm /aortic systolic pressure ratio < 0.3pulm /aortic systolic pressure ratio < 0.3 Qp / Qs<1.4/1Qp / Qs<1.4/1 Moderately restrictive Moderately restrictive - RVSP high, but less than LVSP- RVSP high, but less than LVSP - Qp/Qs 1.4/2.2- Qp/Qs 1.4/2.2 Non restrictive Non restrictive -Shunt not limited at the site of -Shunt not limited at the site of

defectdefect RVSP , LVSP, PA , Aortic systolic RVSP , LVSP, PA , Aortic systolic

pressures pressures equalequal Qp/Qs >2.2Qp/Qs >2.2 Flow determined by PVRFlow determined by PVR

Natural historyNatural history

Spontaneous closure :75-85 % all Spontaneous closure :75-85 % all VSDs:35% perimemb .VSDs:35% perimemb .

80% at age 1month . 60% at 3 80% at age 1month . 60% at 3 month.50%at age 6 month and 25% month.50%at age 6 month and 25% of those at age 12 monthof those at age 12 month..

More frequent in small defects More frequent in small defects Decrease in size with ageDecrease in size with age Inlet & outlet defects donot become smaller /close spontInlet & outlet defects donot become smaller /close spont Large & nonrestrictive defects : 10- 15%Large & nonrestrictive defects : 10- 15%

Endocarditis Endocarditis – risk of endocarditis 4-10% for the first 30 – risk of endocarditis 4-10% for the first 30 years of lifeyears of life

25 yr survival for all pts with a VSD 87%25 yr survival for all pts with a VSD 87%

Mechanisms of closure Mechanisms of closure

Adherence of tricuspid leaflet , or chordal tissue Adherence of tricuspid leaflet , or chordal tissue to the edges of VSD.to the edges of VSD.

Growth & hypertrophy of septum around the Growth & hypertrophy of septum around the defectdefect

By development of subacute bacterial endocarditis By development of subacute bacterial endocarditis adherence of STL tissue to the margins adherence of STL tissue to the margins

(Negative pressure effect exerted by a high (Negative pressure effect exerted by a high velocity stream flowing through the defect )velocity stream flowing through the defect )

Ventricular septal aneurysmVentricular septal aneurysm prolapse of aortic cuspprolapse of aortic cusp intrusion of a sinus of valsalva aneurysmintrusion of a sinus of valsalva aneurysm

Associated LesionsAssociated Lesions

PDA --6% 25% infants with heart PDA --6% 25% infants with heart failure.failure.

Coarctation of the aorta 5%.Coarctation of the aorta 5%. Congenital aortic stenosis 2% .Congenital aortic stenosis 2% . Congenital mitral valve disease Congenital mitral valve disease

2%2%

Special situations IN VSDSpecial situations IN VSD

VSD WITH PDAVSD WITH PDA VSD WITH COARCTARION OF VSD WITH COARCTARION OF

AORTA.AORTA. RIGHT ATRIAL VERSUS RIGHT RIGHT ATRIAL VERSUS RIGHT

VENTRICULAR APPROACH.VENTRICULAR APPROACH. PERCUTANEOUS CLOSURE OF VSD.PERCUTANEOUS CLOSURE OF VSD. VSD WITH PUL RESISTANCE HIGH . VSD WITH PUL RESISTANCE HIGH .

Timing of surgery in VSDTiming of surgery in VSD

<3months <3months - if symptomatic- if symptomatic 3-6 months 3-6 months - symptomatic, - symptomatic,

growth failure, increasing PAH.growth failure, increasing PAH. >6 months >6 months – primarily based on – primarily based on

PAH PAH Wait till 1 yr , if no PAHWait till 1 yr , if no PAH

PathophysiologyPathophysiology Defect size is often compared to aortic Defect size is often compared to aortic

annulusannulus Large: > 75% of annulus size , flow Large: > 75% of annulus size , flow

velocity 1m svelocity 1m s Medium: 33-75% of annulus size flow Medium: 33-75% of annulus size flow

velocity 1 to 4 m svelocity 1 to 4 m s Small: <33% of annulus size flow velocity Small: <33% of annulus size flow velocity

>4 m s>4 m s

PathophysiologyPathophysiology Restrictive VSD is typically small, such that Restrictive VSD is typically small, such that

a significant pressure gradient exists a significant pressure gradient exists between the LV and RV (high velocity), with between the LV and RV (high velocity), with small shunt (Qp/Qs ≤ 1.4 : 1)small shunt (Qp/Qs ≤ 1.4 : 1)

Moderately restrictive VSD Moderately restrictive VSD moderate moderate shunt (Qp/Qs 1.4 to 2.2 : 1) shunt (Qp/Qs 1.4 to 2.2 : 1)

Large / non-restrictive VSD Large / non-restrictive VSD large shunt large shunt (Qp/Qs > 2.2 : 1)(Qp/Qs > 2.2 : 1)

Eisenmenger VSD Eisenmenger VSD irreversible pulmonary irreversible pulmonary HTN and shunt may be zero or reversed (i.e. HTN and shunt may be zero or reversed (i.e. RRL)L)

During systole, blood is shunted from LV to RVDuring systole, blood is shunted from LV to RV passes through the lungs and re enters the LV passes through the lungs and re enters the LV

via the pulmonary veins and LA causes volume via the pulmonary veins and LA causes volume overload on the LV Shunt into the RV elevates overload on the LV Shunt into the RV elevates RV pressure and volume, leading to pulmonary RV pressure and volume, leading to pulmonary hypertension.hypertension.

More noticeable in patients with larger defects More noticeable in patients with larger defects Magnitude of shunt: size, PVRMagnitude of shunt: size, PVR

Small defect: large resistance occurs at the Small defect: large resistance occurs at the defectdefect

Larger defect: resistance offered by the defect Larger defect: resistance offered by the defect minimumminimum

Heath-Edwards Heath-Edwards ClassificationClassification

Grade I: Medial hypertrophyGrade II: Cellular intimalproliferation in an abnormally muscular artery

Grade III: Occlusive changes Grade IV: Dilatation

Grade V: Plexiform lesions

Grade VI: Acute necrotizingarteritis

Enlargement of Enlargement of LA, LV,PALA, LV,PA

Shunt mainly in Shunt mainly in systole, when the systole, when the RV also contractsRV also contracts

Shunted blood Shunted blood goes directly to goes directly to PA PA

Management Management

Observation & follow upObservation & follow up Small VSDsSmall VSDs Medical management Medical management Medium sized vsd Medium sized vsd CCF- treat with diuretics & CCF- treat with diuretics &

digitalis, ACEI digitalis, ACEI failure ppted by LRTI- Treat bothfailure ppted by LRTI- Treat both 2-3 months follow up 2-3 months follow up RV & PA pressures assessedRV & PA pressures assessed Failure to thriveFailure to thrive SurgicalSurgical Large vsd Large vsd

drugsdrugs

digoxin 10-20mcg/kg per daydigoxin 10-20mcg/kg per day

furosemide 1–3 mg/kg per furosemide 1–3 mg/kg per day day

captopril 0.5–2 mg/kg per captopril 0.5–2 mg/kg per dayday

enalapril 0.1mg/kg per dayenalapril 0.1mg/kg per day

Indications of surgical Indications of surgical interventionintervention

Large VSD with pulmonary Large VSD with pulmonary hypertension hypertension

VSD with aortic regurgitation VSD with aortic regurgitation VSD with associated defectsVSD with associated defects Failure of CCF to respond to Failure of CCF to respond to

medications.medications.

ACC/AHA guidelines 2008 for ACC/AHA guidelines 2008 for management of adults with CHDmanagement of adults with CHD

Surgical VSD closureSurgical VSD closure

Closure of vsd indicated when Closure of vsd indicated when

Qp/Qs Qp/Qs 2 or more & clinical e/o 2 or more & clinical e/o LV volume LV volume overloadoverload

When pt has a history of IEWhen pt has a history of IE

III IIaIIaIIa IIbIIbIIb IIIIIIIIIIII IIaIIaIIa IIbIIbIIb IIIIIIIIIIII IIaIIaIIa IIbIIbIIb IIIIIIIIIIIaIIaIIa IIbIIbIIb IIIIIIIII

III IIaIIaIIa IIbIIbIIb IIIIIIIIIIII IIaIIaIIa IIbIIbIIb IIIIIIIIIIII IIaIIaIIa IIbIIbIIb IIIIIIIIIIIaIIaIIa IIbIIbIIb IIIIIIIII

Surgical VSD closureSurgical VSD closure Closure of vsd is reasonable when LClosure of vsd is reasonable when LR shunt R shunt

is present at a Qp/Qs >1.5, with a PA is present at a Qp/Qs >1.5, with a PA pressure <2/3pressure <2/3rdrd of systemic pressure & pulse of systemic pressure & pulse volume recording < 2/3volume recording < 2/3rdrd of SVR of SVR

Closure of vsd is reasonable when LClosure of vsd is reasonable when LR shunt R shunt is present at a Qp/Qs >1.5, in the presence of is present at a Qp/Qs >1.5, in the presence of LV systolic or diastolic failureLV systolic or diastolic failure

Vsd closure not recommended in pts with Vsd closure not recommended in pts with severe irreversible PAHsevere irreversible PAH

III IIaIIaIIaIIbIIbIIbIIIIIIIIIIII IIaIIaIIaIIbIIbIIbIIIIIIIIIIII IIaIIaIIaIIbIIbIIbIIIIIIIIIIIaIIaIIaIIbIIbIIbIIIIIIIII

III IIaIIaIIaIIbIIbIIbIIIIIIIIIIII IIaIIaIIaIIbIIbIIbIIIIIIIIIIII IIaIIaIIaIIbIIbIIbIIIIIIIIIIIaIIaIIaIIbIIbIIbIIIIIIIII

III IIaIIaIIa IIbIIbIIb IIIIIIIIIIII IIaIIaIIa IIbIIbIIb IIIIIIIIIIII IIaIIaIIa IIbIIbIIb IIIIIIIII

PA bandingPA banding

PA banding- palliative procedure , when PA banding- palliative procedure , when additional lesions make repair difficult, additional lesions make repair difficult, patient in severe heart failure.patient in severe heart failure.

Done in multiple VSDsDone in multiple VSDs 30-50% of original diameter is narrowed30-50% of original diameter is narrowed Systolic pressure of 25-30 mmHg Systolic pressure of 25-30 mmHg

beyond the constriction. beyond the constriction.

Technique of operation. Technique of operation. VSDVSD

Transventricular approach for Transventricular approach for closure of conal septal defectclosure of conal septal defect

COMPLICATIONSCOMPLICATIONS AV Dissociation . RBBB.AV Dissociation . RBBB. Ventricular arrhythmias.Ventricular arrhythmias. Heart block. Heart block. Poor hemodynamic state.Poor hemodynamic state. Residual shunting.2%Residual shunting.2% AR/TRAR/TR Cardiac dysfunction. Cardiac dysfunction. Pulmonary hypertension.Pulmonary hypertension. Early hospital death.<1%Early hospital death.<1%

Post op follow upPost op follow up

Every 1-2 yrsEvery 1-2 yrs VSD & mild PAH& repair after 3 yrs of VSD & mild PAH& repair after 3 yrs of

age- age- watch for progressive watch for progressive pulmonary vascular disease.pulmonary vascular disease.

Surgical cureSurgical cure. (. (surviving the early surviving the early postoperative period and being alive late postoperative period and being alive late postoperativelypostoperatively ..

Endocarditis Prophylaxis Endocarditis Prophylaxis for VSDfor VSD Uncomplicated VSD – no Abx for dental or other Uncomplicated VSD – no Abx for dental or other

procedures required .procedures required . Post repair:Post repair:

Abx for 6 months following surgical or Abx for 6 months following surgical or percutaneous repairpercutaneous repair

Indefinite Abx if there is residual shuntIndefinite Abx if there is residual shunt Focus should be on optimal dental hygiene for Focus should be on optimal dental hygiene for

those with CHDthose with CHD

Eisenmenger SyndromeEisenmenger Syndrome

InIn 1897 Victor Eisenmenger published a paper 1897 Victor Eisenmenger published a paper entitled “congenital defects of the ventricular septum”entitled “congenital defects of the ventricular septum”

In 1958, Paul Wood summarized Eisenmenger’s In 1958, Paul Wood summarized Eisenmenger’s accounts:accounts:““The patient was a powerfully built man of 32 who The patient was a powerfully built man of 32 who gave a history of cyanosis and moderate gave a history of cyanosis and moderate breathlessness since infancy. He managed well until breathlessness since infancy. He managed well until January of 1894 when dyspnea increased and edema January of 1894 when dyspnea increased and edema set in. Seven months later he was admitted to the set in. Seven months later he was admitted to the hospital in a state of heart failure……He improved with hospital in a state of heart failure……He improved with rest and digitalis, but collapsed and died suddenly on rest and digitalis, but collapsed and died suddenly on November 13 following a large hemoptysisNovember 13 following a large hemoptysis

Eisenmenger SyndromeEisenmenger Syndrome As disease progresses, more advanced As disease progresses, more advanced

morphologic changes (plexiform lesions, morphologic changes (plexiform lesions, necrotizing arteritis) occur which are necrotizing arteritis) occur which are irreversibleirreversible

As the increased PVR approaches or exceeds As the increased PVR approaches or exceeds the SVR, the shunt is reversedthe SVR, the shunt is reversed

As R to L shunting develops, cyanosis appearsAs R to L shunting develops, cyanosis appears Most patients will develop exertional dyspnea Most patients will develop exertional dyspnea

and impaired exercise toleranceand impaired exercise tolerance

Eisenmenger SyndromeEisenmenger Syndrome Palpitations occur in >50% of patients (A. fib/flutter Palpitations occur in >50% of patients (A. fib/flutter

in 40% and VT in 10%)in 40% and VT in 10%) Hemoptysis in ~20%Hemoptysis in ~20% PE, angina, syncope, endocarditis ~10%PE, angina, syncope, endocarditis ~10% Signs of PHTN (RV heave, palpable PSigns of PHTN (RV heave, palpable P22, and right , and right

sided Ssided S44) are typically present) are typically present Pulmonary ejection click and a soft scratchy SEM Pulmonary ejection click and a soft scratchy SEM

(d(d/t dilated pulmonary trunk) /t dilated pulmonary trunk) High pitched decrescendo diastolic murmur High pitched decrescendo diastolic murmur

(Graham-Steele) audible in most patients (Graham-Steele) audible in most patients Usually no peripheral edema until right heart Usually no peripheral edema until right heart

failure ensuesfailure ensues

Eisenmenger SyndromeEisenmenger SyndromeCXR reveals prominent central pulmonary arteries and decreased vascular markings (pruning) of the peripheral vessels

Eisenmenger SyndromeEisenmenger Syndrome

Large variation in life expectancy in adults with Large variation in life expectancy in adults with Eisenmenger syndromeEisenmenger syndrome

Rate of survival among patients with Rate of survival among patients with Eisenmenger syndrome isEisenmenger syndrome is 80% at 10 years, 77% at 15 years, and 42% at 25 80% at 10 years, 77% at 15 years, and 42% at 25

yearsyears Recent study of 109 adults revealed following as Recent study of 109 adults revealed following as

independent predictors of mortality:independent predictors of mortality: Age at presentationAge at presentation Supraventricular arrhythmiasSupraventricular arrhythmias Poor NYHA functional class (III or IV)Poor NYHA functional class (III or IV)

Eisenmenger Syndrome Eisenmenger Syndrome Pregnancy is discouraged due to high maternal Pregnancy is discouraged due to high maternal

(50%) and fetal (60%) mortality(50%) and fetal (60%) mortality CVA may occur secondary to paradoxical CVA may occur secondary to paradoxical

emboliemboli Also at higher risk for cerebral abscessesAlso at higher risk for cerebral abscesses Patients should avoid intravascular volume Patients should avoid intravascular volume

depletion, heavy exertion, high altitudes, and depletion, heavy exertion, high altitudes, and use of vasodilatorsuse of vasodilators

IV epoprostenol may be beneficial in IV epoprostenol may be beneficial in decreasing PVRdecreasing PVR

Phlebotomy with isovolumic replacement Phlebotomy with isovolumic replacement is recommended for patients with is recommended for patients with moderate to severe symptoms of moderate to severe symptoms of hyperviscosity and an elevated hyperviscosity and an elevated hematocrit >65%hematocrit >65%

Prevention of iron deficiency is important Prevention of iron deficiency is important Supplemental oxygen reduces episodes Supplemental oxygen reduces episodes

of dyspnea (?survival benefit)of dyspnea (?survival benefit) Lung transplantation (with repair of Lung transplantation (with repair of

cardiac defect) or heart/lung cardiac defect) or heart/lung transplantation is an option.transplantation is an option.

Successful closure is associated with excellent Successful closure is associated with excellent survival if ventricular fx is normal. Elevated PAP survival if ventricular fx is normal. Elevated PAP preop may progress, regress, or remain the same preop may progress, regress, or remain the same postoppostop

A. fib may occur, especially if there has been A. fib may occur, especially if there has been longstanding volume overload of the left heart. longstanding volume overload of the left heart. Late VT and sudden death are potential risks, Late VT and sudden death are potential risks, especially in patients repaired late in life. CHB especially in patients repaired late in life. CHB may also occur after surgical repairmay also occur after surgical repair

Pregnancy is well tolerated in women with small Pregnancy is well tolerated in women with small or moderate VSD and in women with repaired VSDor moderate VSD and in women with repaired VSD

Pregnancy is contraindicated in women with Pregnancy is contraindicated in women with Eisenmenger syndrome due to both high maternal Eisenmenger syndrome due to both high maternal ((>50%) and fetal (~60%) mortality>50%) and fetal (~60%) mortality

Follow-up:Follow-up: Patients with following problems benefit from Patients with following problems benefit from

periodic evaluation by cardiologistperiodic evaluation by cardiologist Patch leaks or residual VSDs (which seldom require Patch leaks or residual VSDs (which seldom require

reoperation)reoperation) Elevated PVR at time of surgeryElevated PVR at time of surgery Aortic valve surgeryAortic valve surgery Late repair of moderate or large defectsLate repair of moderate or large defects Significant atrial or ventricular arrhythmiasSignificant atrial or ventricular arrhythmias Associated cardiac lesions (eg RVOTO, AR)Associated cardiac lesions (eg RVOTO, AR)

Endocarditis prophylaxis is recommended for Endocarditis prophylaxis is recommended for 6/12 following VSD closure or for life if residual 6/12 following VSD closure or for life if residual defect persistsdefect persists

VENTRICULAR VENTRICULAR SEPTAL DEFECTSEPTAL DEFECT





AnatomyAnatomy



1. Membranous2. Outflow3. Trabecular

septum4. Inflow5. Subarterial /

Supracristal

AnatomyAnatomy 4 morphological components of 4 morphological components of

septumseptum MembranousMembranous InletInlet Outlet/InfundibularOutlet/Infundibular Muscular/TrabecularMuscular/Trabecular

AnatomyAnatomy Membranous-70-80%Membranous-70-80%

SmallSmall Located at base, between inlet and Located at base, between inlet and

outletoutlet Perimembranous - Extends to adjacent Perimembranous - Extends to adjacent

septumseptumMembranous Membranous

AnatomyAnatomy Inlet Inlet

Inlet 5-8%, Inlet 5-8%, AV valve to chordae attachmentsAV valve to chordae attachments

Inlet

AnatomyAnatomy Outlet/Infundibular Outlet/Infundibular

5-7% 5-7% Separates L and R outflow tractsSeparates L and R outflow tracts

Infundibular

AnatomyAnatomy Muscular/Trabecular (5-20%)Muscular/Trabecular (5-20%)

Anterior/Marginal (anterior to septal Anterior/Marginal (anterior to septal band)band)

Midmuscular/Central (posterior to septal Midmuscular/Central (posterior to septal band)band)

Apical (inferior to moderator band)Apical (inferior to moderator band) Posterior (beneath septal leaflet) Posterior (beneath septal leaflet)

Muscular

soto et alsoto et al

Perimembranous(membranous/Perimembranous(membranous/ infracristal )-70-80%infracristal )-70-80% Muscular- 5-20%Muscular- 5-20% Central- mid muscularCentral- mid muscular ApicalApical Marginal- along RV septal junctionMarginal- along RV septal junction Swiss cheese septum – multiple Swiss cheese septum – multiple

defectsdefects Inlet/ AV canal type-5-8%Inlet/ AV canal type-5-8% Supracrital/ subaortic- 5-7%Supracrital/ subaortic- 5-7%






PathophysiologyPathophysiology Defect size is often compared to aortic Defect size is often compared to aortic

annulusannulus Large: > 50% of annulus sizeLarge: > 50% of annulus size Medium: 25-50% of annulus sizeMedium: 25-50% of annulus size Small: <25% of annulus sizeSmall: <25% of annulus size

Large VSD Large VSD Size equal to the Size equal to the aortic rootaortic root

Equalization of pressures in RV& Equalization of pressures in RV& LVLV

Increased LA pressureIncreased LA pressure opening of opening of foramen ovalforamen oval

Medium sized VSDMedium sized VSDVSD size about half – equal VSD size about half – equal to the size of the aortic orificeto the size of the aortic orifice

When PA & RVSP are > 50% of systemic When PA & RVSP are > 50% of systemic arterial pressure arterial pressure

mm od-large Lod-large L R shunt develops R shunt develops Small VSD in infancy Small VSD in infancy <1/3<1/3rdrd size of aortic root size of aortic root shunt limited by size of the defectshunt limited by size of the defect Shunt entirely during ventricular systoleShunt entirely during ventricular systole LL R shunt <50% LV output R shunt <50% LV output Pulmonary:systemic flow ratio < 2:1Pulmonary:systemic flow ratio < 2:1

PathophysiologyPathophysiology Restrictive VSD is typically small, such that a Restrictive VSD is typically small, such that a

significant pressure gradient exists between significant pressure gradient exists between the LV and RV (high velocity), with small shunt the LV and RV (high velocity), with small shunt (Qp/Qs ≤ 1.4 : 1)(Qp/Qs ≤ 1.4 : 1)

Moderately restrictive VSD Moderately restrictive VSD moderate shunt moderate shunt (Qp/Qs 1.4 to 2.2 : 1) (Qp/Qs 1.4 to 2.2 : 1)

Large / non-restrictive VSD Large / non-restrictive VSD large shunt large shunt (Qp/Qs > 2.2 : 1)(Qp/Qs > 2.2 : 1)

Eisenmenger VSD Eisenmenger VSD irreversible pulmonary irreversible pulmonary HTN and shunt may be zero or reversed (i.e. HTN and shunt may be zero or reversed (i.e. RRL)L)


via the pulmonary veins and LA via the pulmonary veins and LA causes volume overload on the LVcauses volume overload on the LV Shunt into the RV elevates RV pressure and Shunt into the RV elevates RV pressure and

volume, leading to pulmonary hypertension.volume, leading to pulmonary hypertension.

More noticeable in patients with larger defectsMore noticeable in patients with larger defects

pathophysiologypathophysiology

Magnitude of shunt: size, PVRMagnitude of shunt: size, PVR Small defect: large resistance occurs Small defect: large resistance occurs

at the defectat the defect Larger defect: resistance offered by Larger defect: resistance offered by

the defect minimumthe defect minimum

: Shunt depends largely : Shunt depends largely on PVRon PVR

Lower the PVR, greater the LLower the PVR, greater the LR ShuntR Shunt






Spontaneous closure Spontaneous closure :75-85 % all VSDs:75-85 % all VSDs :35% perimemb ( 1:35% perimemb ( 1stst 6/12) 6/12) More frequent in small defects More frequent in small defects Decrease in size with ageDecrease in size with age Inlet & outlet defects donot become smaller /close Inlet & outlet defects donot become smaller /close

spontspont Large & nonrestrictive defects : 10- 15%Large & nonrestrictive defects : 10- 15%

endocarditis endocarditis – risk of endocarditis 4-10% for the first – risk of endocarditis 4-10% for the first 30 years of life30 years of life

High velocity turbulent jet into RVHigh velocity turbulent jet into RV

Mechanisms of closure Mechanisms of closure Growth & hypertrophy of septum around the Growth & hypertrophy of septum around the

defectdefect By development of subacute bacterial By development of subacute bacterial

endocarditisendocarditis adherence of STL tissue to the margins adherence of STL tissue to the margins (Negative pressure effect exerted by a high (Negative pressure effect exerted by a high

velocity stream flowing through the defect )velocity stream flowing through the defect ) Ventricular septal aneurysmVentricular septal aneurysm prolapse of aortic cuspprolapse of aortic cusp intrusion of a sinus of valsalva aneurysmintrusion of a sinus of valsalva aneurysm

CHF CHF Large VSDsLarge VSDs Mod sized VSDs survive into Mod sized VSDs survive into

adulthoodadulthood Increased rt sided flow Increased rt sided flow

pulmonary vascular disease pulmonary vascular disease Eisenmenger’s physiology if left Eisenmenger’s physiology if left untreateduntreated

Risk factors for decreased survivalRisk factors for decreased survival Shortness of breath, fatigue, Shortness of breath, fatigue,

DOE,progressive ARDOE,progressive AR CardiomegalyCardiomegaly PASP >60mm Hg/ >1/2 of systemic PASP >60mm Hg/ >1/2 of systemic

pressurepressure Good prognosticators Good prognosticators Lack of symptomsLack of symptoms normal LV size & functionnormal LV size & function small Lsmall LR shuntR shunt normal pulmonary pressures / normal pulmonary pressures /

resistanceresistance Intact vasodilator response in Intact vasodilator response in

pulmonary pulmonary vasculaturevasculature

genetic factorsgenetic factors Affected father- 2%Affected father- 2% Affected mother – 6%Affected mother – 6% 25 yr survival for all pts with a VSD 25 yr survival for all pts with a VSD

87%87% Mortality increases with the size of Mortality increases with the size of

VSDVSD

History & clinical History & clinical featuresfeatures

HistoryHistory

Incidence unrelated to maternal age, sex, Incidence unrelated to maternal age, sex, birth orderbirth order

3.3% 13.3% 1stst degree relatives of index patients degree relatives of index patients Among 1Among 1stst degree relatives with CHD, 1/3 degree relatives with CHD, 1/3rdrd

have vsdhave vsd 30-60% siblings of index patients have vsd30-60% siblings of index patients have vsd Parents with spontaneously closed vsd can Parents with spontaneously closed vsd can

have offspring with vsdhave offspring with vsd

Small VSD - infancy Small VSD - infancy

Normal wt gain & developmentNormal wt gain & development 2-8 wks – tachycardia & tachypnea 2-8 wks – tachycardia & tachypnea

especially with infection especially with infection 2-4/6 systolic mr, medium 2-4/6 systolic mr, medium

frequencyfrequency

Large VSD - infancyLarge VSD - infancy Infant well in the immediate postnatal period Infant well in the immediate postnatal period Systolic mr LLSB after 1-7 daysSystolic mr LLSB after 1-7 days develop respiratory distress , in 2-8 wksdevelop respiratory distress , in 2-8 wks CardiomegalyCardiomegaly Systolic thrill , along LSBSystolic thrill , along LSB S1 normal/ soft: s2loud narrow splitS1 normal/ soft: s2loud narrow split Systolic mr , 2-3/6 intensity at birth, louder & Systolic mr , 2-3/6 intensity at birth, louder &

harsh as shunt increasesharsh as shunt increases S3 & MDM at apexS3 & MDM at apex If the infant survives - subsequent course with If the infant survives - subsequent course with

persistent dyspnea, sweating, poor feeding, failure to persistent dyspnea, sweating, poor feeding, failure to thrive, LRTIthrive, LRTI

Beyond infancyBeyond infancy

Arterial pulse- brisk ( vigorous ejection Arterial pulse- brisk ( vigorous ejection from a volume overloaded ventricle)from a volume overloaded ventricle)

N pulse in eisenmenger’s - systemic N pulse in eisenmenger’s - systemic stroke volume maintainedstroke volume maintained

Cyanosis & clubbing : eisenmenger’sCyanosis & clubbing : eisenmenger’s JVP – N in small defectsJVP – N in small defects elevated - Mod restr & nonrestrictive elevated - Mod restr & nonrestrictive

vsd with vsd with ccf ccf Precordial bulge ( large shunt 5-6 Precordial bulge ( large shunt 5-6

months)months) Harrison’s sulcusHarrison’s sulcus

CardiomegalyCardiomegaly RV heave in pts with RV vol overloadRV heave in pts with RV vol overload Features of PAHFeatures of PAH Grade 2-5/6 systolic regurgitant Grade 2-5/6 systolic regurgitant

mrLLSBmrLLSB MDM preceeded by S3MDM preceeded by S3 Infundibular vsd: early diastolic Infundibular vsd: early diastolic

decrescendo mr of AR decrescendo mr of AR

Improvement of symptomsImprovement of symptoms

Closing defectClosing defect findings : findings : soft s2soft s2 high frequency & shorter murmurhigh frequency & shorter murmur Increasing PVR Increasing PVR findings : findings : increased RV pulsations increased RV pulsations s2 loud, narrow splits2 loud, narrow split Infundibular hypertrophy Infundibular hypertrophy decreased Ldecreased LR shunt, R shunt, findings : findings : s2 decreases in intensity , s2 decreases in intensity , crescendo-decrescendo systolic murmur crescendo-decrescendo systolic murmur

in the in the ULSB,ULSB, cyanosis (shunt reversal )cyanosis (shunt reversal )

Eisenmenger’sEisenmenger’s apex by RV apex by RV Palpable dilated hypertensive Palpable dilated hypertensive

pulmonary trunkpulmonary trunk Loud pulmonary closure sound Loud pulmonary closure sound Very short or no systolic mr of vsdVery short or no systolic mr of vsd Short pulmonary ejection mr ULSBShort pulmonary ejection mr ULSB EDM of pulmonary regurgitation EDM of pulmonary regurgitation Loud harsh s1 coincident Loud harsh s1 coincident

holosystolic mr of TRholosystolic mr of TR

ECGECG small defects unremarkablesmall defects unremarkable LA enlargement LA enlargement - Mod restrictive, - Mod restrictive,

large Llarge LR shuntsR shunts left axis deviation left axis deviation Inlet vsd /AV septal defect Inlet vsd /AV septal defect 5% moderately restrictive vsds5% moderately restrictive vsds Ventricular septal aneurysms Ventricular septal aneurysms multiple vsds multiple vsds

LV enlargement LV enlargement in larger defectsin larger defects RVHRVH - Mild or moderate elevation of - Mild or moderate elevation of

RV RV pressure (rsR’ in V4R or V1)pressure (rsR’ in V4R or V1) - Large VSD, equal - Large VSD, equal

ventricular ventricular pressures , pressures , elevated elevated PVRPVR

RVH , RAD RVH , RAD - Eisenmenger’s - Eisenmenger’s RBBBRBBB - Surgical repair - Surgical repair

Chest x rayChest x ray Small defects that were mod restrictive at birth – increased LV Small defects that were mod restrictive at birth – increased LV size, dilated pulmonary trunk & its branchessize, dilated pulmonary trunk & its branches

Large shunts – hyperinflated lungs with flat hemi Large shunts – hyperinflated lungs with flat hemi diaphragms diaphragms

LA enlargement best appreciated in the lateral position LA enlargement best appreciated in the lateral position

Increased PVR, decreases LIncreased PVR, decreases LR shunt, decreases heart size, R shunt, decreases heart size, enlargement of pulmonary trunk& its branches persistsenlargement of pulmonary trunk& its branches persists

Nonrestrictive vsd with elevated but variable PVR- enlargement Nonrestrictive vsd with elevated but variable PVR- enlargement of all 4 chambersof all 4 chambers

Eisenmenger’s syndrome- oligemic lung fields, RA,LA, LV normal, Eisenmenger’s syndrome- oligemic lung fields, RA,LA, LV normal, RV occupies the apex RV occupies the apex

Common locations of vsdCommon locations of vsd --2d echo2d echo

Echocardiography Echocardiography

Common locations of vsdCommon locations of vsd --2d echo2d echo

Echocardiography- dopplerEchocardiography- doppler CFM-Direction, timing of flowCFM-Direction, timing of flow IVG (mmHg) = 4v² IVG (mmHg) = 4v² PG = LVSP - RVSP PG = LVSP - RVSP

LVSP - PG jet = RVsp ≈ PaspLVSP - PG jet = RVsp ≈ Pasp RVSP = cuff systolic BP – 4v² RVSP = cuff systolic BP – 4v² PVR = TRV / TVI in RVOT x 10 + 0.16PVR = TRV / TVI in RVOT x 10 + 0.16 High PA pressure, TRV/TVI rvot < 0.2 ; indicates low PVR, elevated High PA pressure, TRV/TVI rvot < 0.2 ; indicates low PVR, elevated

pressure secondary to the flowpressure secondary to the flow

Cardiac catheterizationCardiac catheterization

Hemodynamic assessmentsHemodynamic assessments

cardiac index oximetry cardiac index oximetry quantification of shuntquantification of shunt

To assess pulmonary vascular To assess pulmonary vascular resistanceresistance

Pts with increased PVR, with mod or large Pts with increased PVR, with mod or large LLR shuntR shunt

If PVR is increased, response to 100% If PVR is increased, response to 100% oxygen,NO testedoxygen,NO tested

cineangiographycineangiography

Defect best imaged in Defect best imaged in LAO(70°)cranial (25°)LAO(70°)cranial (25°)

Inlet defect - hepatoclavicular Inlet defect - hepatoclavicular viewview

( 40°LAO,cranial ( 40°LAO,cranial angulation)angulation)

Anterior muscular VSD- RAO viewAnterior muscular VSD- RAO view Aortography - r/o PDA ,coarctation Aortography - r/o PDA ,coarctation

Management Management



drugsdrugs







VSD with aortic regurgitation VSD with aortic regurgitation VSD with associated defectsVSD with associated defects Failure of congestive cardiac Failure of congestive cardiac

failure to respond to medicationsfailure to respond to medications

Timing of surgeryTiming of surgery in VSD in VSD


growth failure, increasing PAHgrowth failure, increasing PAH >6 months >6 months – primarily based on – primarily based on


VSD closure VSD closure

Direct closure of the defectDirect closure of the defect Surgical mortality <1%Surgical mortality <1% Complications – RBBB- direct injury Complications – RBBB- direct injury

to rt bundle, disruption of purkinje to rt bundle, disruption of purkinje fibersfibers

Residual shunt (<5% )Residual shunt (<5% ) Injuries to tricuspid valve & aortic Injuries to tricuspid valve & aortic

valvevalve


PA banding- palliative procedure , when PA banding- palliative procedure , when additional lesions make repair difficultadditional lesions make repair difficult


beyond the constrictionbeyond the constriction RV/PA pressure gradient > 45 RV/PA pressure gradient > 45

associated with hypoxemia associated with hypoxemia


Every 1-2 yrsEvery 1-2 yrs VSD & mild PAH& repair after 3 yrs VSD & mild PAH& repair after 3 yrs

of age- of age- watch for progressive watch for progressive pulmonary vascular pulmonary vascular diseasedisease

long term follow up neededlong term follow up needed

ATRIAL WAVESATRIAL WAVES

a= atrial contractiona= atrial contraction c= contraction of ventricle and closure of tricuspid valvec= contraction of ventricle and closure of tricuspid valve x=x descent x=x descent v=venous filling v=venous filling y= y descent due to opening of tricuspid valvey= y descent due to opening of tricuspid valve

RIGHT HEART PRESSURESRIGHT HEART PRESSURES

Haemodynamic ParametersHaemodynamic Parameters

The following criteria suggest left atrial enlargement/abnormality The following criteria suggest left atrial enlargement/abnormality when correlated with echocardiographic data:when correlated with echocardiographic data:

Negative phase of P in V1>0.04 sec — sensitivity 83 percent; Negative phase of P in V1>0.04 sec — sensitivity 83 percent; specificity 80 percent specificity 80 percent

Negative phase of P in V1>1 mm — sensitivity 60 percent; Negative phase of P in V1>1 mm — sensitivity 60 percent; specificity 93 percent specificity 93 percent

P-terminal force >0.04 mm/sec — sensitivity 69 percent; P-terminal force >0.04 mm/sec — sensitivity 69 percent; specificity 93 percent specificity 93 percent

Notched P, interpeak interval >0.04 sec — sensitivity 15 percent; Notched P, interpeak interval >0.04 sec — sensitivity 15 percent; specificity 100 percent specificity 100 percent

P wave duration >0.11 sec — sensitivity 33 percent; specificity P wave duration >0.11 sec — sensitivity 33 percent; specificity 88 percent 88 percent

P wave/PR duration >1.6 — sensitivity 31 percent; specificity 64 P wave/PR duration >1.6 — sensitivity 31 percent; specificity 64 percentpercent

Special Special situationssituations

VSD with AR VSD with AR Peri membranous VSD with AR - 5-8%Peri membranous VSD with AR - 5-8% Subarterial VSDs – 30%Subarterial VSDs – 30% Sagging or herniation of RCC or RCC+ NCCSagging or herniation of RCC or RCC+ NCC May cause RVOT obstructionMay cause RVOT obstruction Due to morphological abnormality of valve Due to morphological abnormality of valve LV volume – regurgitant volume & shunt LV volume – regurgitant volume & shunt

volumevolume VSD murmur dates from infancyVSD murmur dates from infancy AR murmur appears (5-9 yrs) AR murmur appears (5-9 yrs)

OutlineOutline Morphology, Types & PathophysiologyMorphology, Types & Pathophysiology Natural History and Clinical PresentationNatural History and Clinical Presentation Some Echo examplesSome Echo examples Clinical Scenarios and RecommendationsClinical Scenarios and Recommendations Interventions: Indications, Surgery, Interventions: Indications, Surgery,

PercutaneousPercutaneous Pregnancy and Endocarditis ProphylaxisPregnancy and Endocarditis Prophylaxis Review QuestionsReview Questions

IntroductionIntroduction The most common form of CHD, accounting for The most common form of CHD, accounting for

up to 20-40% of patients diagnosed with CHDup to 20-40% of patients diagnosed with CHD Impact may range from asymptomatic to Impact may range from asymptomatic to

pulmonary HTN, LV volume overload and RVHpulmonary HTN, LV volume overload and RVH Morphology: 4 typesMorphology: 4 types

Membranous – most common type in adults (80%)Membranous – most common type in adults (80%) Muscular – most common type in young childrenMuscular – most common type in young children Complete AV septal (endocardial cushion) defectsComplete AV septal (endocardial cushion) defects Supracristal (subarterial)Supracristal (subarterial)

VSD TypesVSD Types

VSD TypesVSD Types

VSD TypesVSD Types

Natural HistoryNatural History Restrictive: typically does not have Restrictive: typically does not have

hemodynamic impact and may close hemodynamic impact and may close spontaneouslyspontaneously Location Location Location: Subaortic may result in Location Location Location: Subaortic may result in

progressive AIprogressive AI Moderately restrictive: does create LV Moderately restrictive: does create LV

overload and dysfunction along with variable overload and dysfunction along with variable increase in PVRincrease in PVR

Large / non-restrictive: LV volume overload Large / non-restrictive: LV volume overload earlier in life with progressive pulm HTN and earlier in life with progressive pulm HTN and ultimately Eisenmenger syndromeultimately Eisenmenger syndrome

Clinical FeaturesClinical Features Peds:Peds:

MurmurMurmur Dyspnea, CHF, Failure to thriveDyspnea, CHF, Failure to thrive

Adults:Adults: Asymptomatic murmur – harsh, pansystolic, Asymptomatic murmur – harsh, pansystolic,

left sternal borderleft sternal border Mod restrictive – dyspnea, a.fib, displaced Mod restrictive – dyspnea, a.fib, displaced

apex, murmur, S3apex, murmur, S3 Non-restrictive Eisenmenger VSD – central Non-restrictive Eisenmenger VSD – central

cyanosis, clubbing, RV heave, loud P2 cyanosis, clubbing, RV heave, loud P2

Echo Example 1Echo Example 1


tt

Outlet VSD – Para long axis




Supracristal VSD, with pulm outflow tract obstruction






Echo Example 3Echo Example 3 Type: Type: Size:Size:

Membranous

Restrictive




Type:Size: Shunt:

MuscularLarge / Non-restrictiveRL (inc RH pressures)RV dilatedEisenmengers

Clinical Scenarios & Clinical Scenarios & RecommendationsRecommendations Eisenmenger SyndromeEisenmenger Syndrome

SupportiveSupportive Bosentan (Endothelin receptor Bosentan (Endothelin receptor

antagonist) – improves functional antagonist) – improves functional capacity, QOL capacity, QOL

SildenafilSildenafil

Penny DJ, Vick GW. Lancet 2011; 377: 1103-12

InterventionsInterventions Indications for Surgical Closure in adults:Indications for Surgical Closure in adults:

Evidence of LV volume overload (Class I if Qp/Qs >2, Evidence of LV volume overload (Class I if Qp/Qs >2, Class IIa if Qp/Qs > 1.5)Class IIa if Qp/Qs > 1.5)

History of bacterial endocarditis (Class I)History of bacterial endocarditis (Class I) Significant LSignificant LR shunt with PA pressure < 2/3 systemic R shunt with PA pressure < 2/3 systemic

and PVR is < 2/3 SVRand PVR is < 2/3 SVR Surgical ClosureSurgical Closure

Considered the first-line choice of therapy for those Considered the first-line choice of therapy for those with indicationswith indications

Usually involves direct patch closure w cardio-pulm Usually involves direct patch closure w cardio-pulm bypassbypass

Operative mortality < 2% in most centersOperative mortality < 2% in most centers

Long Term Surgical Long Term Surgical OutcomesOutcomes Retrospective review of 46 pts with Retrospective review of 46 pts with

surgical VSD repair at Mayo Clinicsurgical VSD repair at Mayo Clinic

Mongeon et al. JACC Int 2010; 3: 290-7

Interventional OptionsInterventional Options Percutaneous Device ClosurePercutaneous Device Closure

Muscular VSDs can typically be closed Muscular VSDs can typically be closed percutaneouslypercutaneously

Class IIb recommendation in Guidelines (i.e. Class IIb recommendation in Guidelines (i.e. surgery still preferred)surgery still preferred)

No FDA approved devices for No FDA approved devices for perimembranous VSDs, although there are perimembranous VSDs, although there are specific devices for this purpose specific devices for this purpose

Concern re proximity of defect to AV node and Concern re proximity of defect to AV node and high risk of complete AV block requiring high risk of complete AV block requiring pacemakerpacemaker

Pregnancy and VSDsPregnancy and VSDs Pregnancy well tolerated in women Pregnancy well tolerated in women

with small to moderate sized VSDs as with small to moderate sized VSDs as long as there is no pulmonary vascular long as there is no pulmonary vascular involvementinvolvement

Eisenmenger syndrome: Pregnancy Eisenmenger syndrome: Pregnancy contraindicated due to exceptionally contraindicated due to exceptionally high risk of maternal and fetal deathhigh risk of maternal and fetal death

Question 1Question 1 An isolated VSD will generally cause An isolated VSD will generally cause

enlargement of which chamber(s):enlargement of which chamber(s): A: Left atrium, left ventricleA: Left atrium, left ventricle B: Right ventricleB: Right ventricle C: Right ventricle, pulmonary arteryC: Right ventricle, pulmonary artery D: AortaD: Aorta E: Right ventricle, right atriumE: Right ventricle, right atrium

Question 1Question 1An isolated VSD will generally cause An isolated VSD will generally cause

enlargement of which chamber(s):enlargement of which chamber(s): A: Left atrium, left ventricleA: Left atrium, left ventricle B: Right ventricleB: Right ventricle C: Right ventricle, pulmonary arteryC: Right ventricle, pulmonary artery D: AortaD: Aorta E: Right ventricle, right atriumE: Right ventricle, right atrium

Question 2Question 2

Question 2Question 2 The defect shown on the previous The defect shown on the previous

slide is a:slide is a: A: Muscular VSDA: Muscular VSD B: Sinus venosus VSDB: Sinus venosus VSD C: Perimembranous VSDC: Perimembranous VSD D: Inlet VSDD: Inlet VSD E: Supracristal VSDE: Supracristal VSD

Question 2Question 2The defect shown on the previous The defect shown on the previous

slide is a:slide is a: A: Muscular VSDA: Muscular VSD B: Sinus venosus VSDB: Sinus venosus VSD C: Perimembranous VSDC: Perimembranous VSD D: Inlet VSDD: Inlet VSD E: Supracristal VSDE: Supracristal VSD

Question 3Question 3 A common complication of this defect A common complication of this defect

is:is: A: Pulmonary valve endocarditisA: Pulmonary valve endocarditis B: Aortic regurgitationB: Aortic regurgitation C: Aortic dissectionC: Aortic dissection D: Tricuspid regurgitationD: Tricuspid regurgitation E: Right ventricular enlargementE: Right ventricular enlargement

Question 3Question 3A common complication of this defect A common complication of this defect

is:is: A: Pulmonary valve endocarditisA: Pulmonary valve endocarditis B: Aortic regurgitationB: Aortic regurgitation C: Aortic dissectionC: Aortic dissection D: Tricuspid regurgitationD: Tricuspid regurgitation E: Right ventricular enlargementE: Right ventricular enlargement

Question 4Question 4 There is no diastolic flow in this There is no diastolic flow in this

perimembranous VSDperimembranous VSD A: TrueA: True B: FalseB: False

Question 4Question 4There is no diastolic flow in this There is no diastolic flow in this

perimembranous VSDperimembranous VSD A: TrueA: True B: FalseB: False

Question 5Question 5 A restrictive VSD is a simple lesion with a A restrictive VSD is a simple lesion with a

good long term prognosis. However, good long term prognosis. However, complications can occur. All of the complications can occur. All of the following are possible complications of a following are possible complications of a VSD except:VSD except: A: EndocarditisA: Endocarditis B: Aortic regurgitationB: Aortic regurgitation C: Aortic valve prolapseC: Aortic valve prolapse D: Eisenmenger SyndromeD: Eisenmenger Syndrome E: Right sided volume overloadE: Right sided volume overload

Question 5Question 5A restrictive VSD is a simple lesion with a A restrictive VSD is a simple lesion with a

good long term prognosis. However, good long term prognosis. However, complications can occur. All of the complications can occur. All of the following are possible complications of a following are possible complications of a VSD except:VSD except: A: EndocarditisA: Endocarditis B: Aortic regurgitationB: Aortic regurgitation C: Aortic valve prolapseC: Aortic valve prolapse D: Eisenmenger SyndromeD: Eisenmenger Syndrome E: Right sided volume overloadE: Right sided volume overload

Question 6Question 6

Question 6Question 6 The pulmonary artery systolic The pulmonary artery systolic

pressure in this patient with a VSD is:pressure in this patient with a VSD is: A: NormalA: Normal B: Moderately elevatedB: Moderately elevated C: Systemic / Supra-systemicC: Systemic / Supra-systemic

Question 6Question 6The pulmonary artery systolic The pulmonary artery systolic

pressure in this patient with a VSD is:pressure in this patient with a VSD is: A: NormalA: Normal B: Moderately elevatedB: Moderately elevated C: C: Systemic / Supra-systemicSystemic / Supra-systemic

Question 7Question 7 A patient with a VSD undergoes TTE. BP A patient with a VSD undergoes TTE. BP

measured at the time of the study is 125/75 measured at the time of the study is 125/75 (right arm), MAP 92. CW doppler across the (right arm), MAP 92. CW doppler across the VSD gives a peak velocity of 5 m/s. Assuming VSD gives a peak velocity of 5 m/s. Assuming RA pressure of 5, what is the estimated PASP?RA pressure of 5, what is the estimated PASP? A: 20mmHgA: 20mmHg B: 25 mmHgB: 25 mmHg C: 30 mmHg C: 30 mmHg D: 72 mmHgD: 72 mmHg E: 105 mmHgE: 105 mmHg

Question 7Question 7 A patient with a VSD undergoes TTE. BP A patient with a VSD undergoes TTE. BP

measured at the time of the study is 125/75 measured at the time of the study is 125/75 (right arm), MAP 92. CW doppler across the (right arm), MAP 92. CW doppler across the VSD gives a peak velocity of 5 m/s. Assuming VSD gives a peak velocity of 5 m/s. Assuming RA pressure of 5, what is the estimated PASP?RA pressure of 5, what is the estimated PASP? A: 20mmHgA: 20mmHg B: 25 mmHgB: 25 mmHg C: 30 mmHgC: 30 mmHg D: 72 mmHgD: 72 mmHg E: 105 mmHgE: 105 mmHg

VSD HemodynamicsVSD Hemodynamics Peak gradient = 4 x vPeak gradient = 4 x v22 (Simplied Bernoulli (Simplied Bernoulli

equation)equation) VSD gradient = LV systolic pressure – RV VSD gradient = LV systolic pressure – RV

systolic pressuresystolic pressure RVSP = LVSP - VSD gradient RVSP = LVSP - VSD gradient RVSP = cuff systolic BP - VSD gradient (or 4 x RVSP = cuff systolic BP - VSD gradient (or 4 x

vv22))

Assuming no aortic outflow tract obstructionAssuming no aortic outflow tract obstruction PASP = RVSPPASP = RVSP

Assuming no pulmonary outflow tract obstructionAssuming no pulmonary outflow tract obstruction

VENTRICULAR VENTRICULAR SEPTAL DEFECTSEPTAL DEFECT

Dolly mathewDolly mathew





AnatomyAnatomy

A A VSDVSD is a defect in the ventricular septum is a defect in the ventricular septum The ventricular septum consists of an inferior The ventricular septum consists of an inferior

muscular and superior membranous portionmuscular and superior membranous portion The membranous portion -most commonly The membranous portion -most commonly

affected in adults and older childrenaffected in adults and older children most common congenital cardiac anomalies.most common congenital cardiac anomalies. 3-3.8 per 1000 live births3-3.8 per 1000 live births 30-60% of all newborns with a CHD30-60% of all newborns with a CHD

Prospective studies give a prevalence of 2-5 Prospective studies give a prevalence of 2-5 per 100 births of trabecular VSDs that closes per 100 births of trabecular VSDs that closes shortly after birth in 80-90% of the casesshortly after birth in 80-90% of the cases

Location of VSDsLocation of VSDs

Swiss cheese

Muscular

Inlet

outlet

perimembranous

ClassificationClassification

soto et alsoto et al

Perimembranous(membranous/Perimembranous(membranous/ infracristal )-70-80%infracristal )-70-80% Muscular- 5-20%Muscular- 5-20% Central- mid muscularCentral- mid muscular ApicalApical Marginal- along RV septal junctionMarginal- along RV septal junction Swiss cheese septum – multiple Swiss cheese septum – multiple

defectsdefects Inlet/ AV canal type-5-8%Inlet/ AV canal type-5-8% Supracrital/ subaortic- 5-7%Supracrital/ subaortic- 5-7%






Small VSD in infancySmall VSD in infancy

<1/3<1/3rdrd size of aortic root size of aortic root shunt limited by size of the defectshunt limited by size of the defect Shunt entirely during ventricular Shunt entirely during ventricular

systolesystole LL R shunt <50% LV output R shunt <50% LV output Pulmonary:systemic flow ratio < Pulmonary:systemic flow ratio <

2:12:1

Medium sized VSDMedium sized VSD

VSD size about half VSD size about half – equal to the size – equal to the size of the aortic orificeof the aortic orifice

When PA & RVSP When PA & RVSP are > 50% of are > 50% of systemic arterial systemic arterial pressurepressure

mod-large Lmod-large L R R shunt developsshunt develops

p218p218

Large VSD Large VSD Size equal to the Size equal to the

aortic rootaortic root

Equalization of Equalization of pressures in RV& LVpressures in RV& LV

Increased LA Increased LA pressurepressure opening of opening of foramen ovaleforamen ovale

PathophysiologyPathophysiology


via the pulmonary veins and LA via the pulmonary veins and LA causes volume overload on the LVcauses volume overload on the LV Shunt into the RV elevates RV pressure and Shunt into the RV elevates RV pressure and

volume, leading to pulmonary hypertension.volume, leading to pulmonary hypertension.

More noticeable in patients with larger defectsMore noticeable in patients with larger defects

pathophysiologypathophysiology

Magnitude of shunt: size, PVRMagnitude of shunt: size, PVR Small defect: large resistance occurs Small defect: large resistance occurs

at the defectat the defect Larger defect: resistance offered by Larger defect: resistance offered by

the defect minimumthe defect minimum

: Shunt depends largely : Shunt depends largely on PVRon PVR

Lower the PVR, greater the LLower the PVR, greater the LR ShuntR Shunt






Spontaneous closure Spontaneous closure :75-85 % all VSDs:75-85 % all VSDs :35% perimemb ( 1:35% perimemb ( 1stst 6/12) 6/12) More frequent in small defects More frequent in small defects Decrease in size with ageDecrease in size with age Inlet & outlet defects donot become smaller /close Inlet & outlet defects donot become smaller /close

spontspont Large & nonrestrictive defects : 10- 15%Large & nonrestrictive defects : 10- 15%

endocarditis endocarditis – risk of endocarditis 4-10% for the first – risk of endocarditis 4-10% for the first 30 years of life30 years of life

High velocity turbulent jet into RVHigh velocity turbulent jet into RV

CHF CHF Large VSDsLarge VSDs Mod sized VSDs survive into Mod sized VSDs survive into

adulthoodadulthood Increased rt sided flow Increased rt sided flow

pulmonary vascular disease pulmonary vascular disease Eisenmenger’s physiology if left Eisenmenger’s physiology if left untreateduntreated

Risk factors for decreased survivalRisk factors for decreased survival Shortness of breath, fatigue, Shortness of breath, fatigue,

DOE,progressive ARDOE,progressive AR CardiomegalyCardiomegaly PASP >60mm Hg/ >1/2 of systemic PASP >60mm Hg/ >1/2 of systemic

pressurepressure Good prognosticators Good prognosticators Lack of symptomsLack of symptoms normal LV size & functionnormal LV size & function small Lsmall LR shuntR shunt normal pulmonary pressures / normal pulmonary pressures /

resistanceresistance Intact vasodilator response in Intact vasodilator response in

pulmonary pulmonary vasculaturevasculature

genetic factorsgenetic factors Affected father- 2%Affected father- 2% Affected mother – 6%Affected mother – 6% 25 yr survival for all pts with a VSD 25 yr survival for all pts with a VSD

87%87% Mortality increases with the size of Mortality increases with the size of

VSDVSD

VSD closure VSD closure

Direct closure of the defectDirect closure of the defect Surgical mortality <1%Surgical mortality <1% Complications – RBBB- direct injury Complications – RBBB- direct injury

to rt bundle, disruption of purkinje to rt bundle, disruption of purkinje fibersfibers

Residual shunt (<5% )Residual shunt (<5% ) Injuries to tricuspid valve & aortic Injuries to tricuspid valve & aortic

valvevalve

HistoryHistory

Incidence unrelated to maternal age, sex, Incidence unrelated to maternal age, sex, birth orderbirth order

3.3% 13.3% 1stst degree relatives of index patients degree relatives of index patients Among 1Among 1stst degree relatives with CHD, 1/3 degree relatives with CHD, 1/3rdrd

have vsdhave vsd 30-60% siblings of index patients have vsd30-60% siblings of index patients have vsd Parents with spontaneously closed vsd can Parents with spontaneously closed vsd can

have offspring with vsdhave offspring with vsd

Small VSD - infancy Small VSD - infancy

Normal wt gain & developmentNormal wt gain & development 2-8 wks – tachycardia & tachypnea 2-8 wks – tachycardia & tachypnea

especially with infection especially with infection 2-4/6 systolic mr, medium 2-4/6 systolic mr, medium

frequencyfrequency

Large VSD - infancyLarge VSD - infancy Infant well in the immediate postnatal period Infant well in the immediate postnatal period Systolic mr LLSB after 1-7 daysSystolic mr LLSB after 1-7 days develop respiratory distress , in 2-8 wksdevelop respiratory distress , in 2-8 wks CardiomegalyCardiomegaly Systolic thrill , along LSBSystolic thrill , along LSB S1 normal/ soft: s2loud narrow splitS1 normal/ soft: s2loud narrow split Systolic mr , 2-3/6 intensity at birth, louder & Systolic mr , 2-3/6 intensity at birth, louder &

harsh as shunt increasesharsh as shunt increases S3 & MDM at apexS3 & MDM at apex If the infant survives - subsequent course with If the infant survives - subsequent course with

persistent dyspnea, sweating, poor feeding, failure to persistent dyspnea, sweating, poor feeding, failure to thrive, LRTIthrive, LRTI

Beyond infancyBeyond infancy

Arterial pulse- brisk ( vigorous ejection Arterial pulse- brisk ( vigorous ejection from a volume overloaded ventricle)from a volume overloaded ventricle)

N pulse in eisenmenger’s - systemic N pulse in eisenmenger’s - systemic stroke volume maintainedstroke volume maintained

Cyanosis & clubbing : eisenmenger’sCyanosis & clubbing : eisenmenger’s JVP – N in small defectsJVP – N in small defects elevated - Mod restr & nonrestrictive elevated - Mod restr & nonrestrictive

vsd with vsd with ccf ccf Precordial bulge ( large shunt 5-6 Precordial bulge ( large shunt 5-6

months)months) Harrison’s sulcusHarrison’s sulcus

CardiomegalyCardiomegaly RV heave in pts with RV vol overloadRV heave in pts with RV vol overload Features of PAHFeatures of PAH Grade 2-5/6 systolic regurgitant Grade 2-5/6 systolic regurgitant

mrLLSBmrLLSB MDM preceeded by S3MDM preceeded by S3 Infundibular vsd: early diastolic Infundibular vsd: early diastolic

decrescendo mr of AR decrescendo mr of AR

Improvement of symptomsImprovement of symptoms

Closing defectClosing defect findings : findings : soft s2soft s2 high frequency & shorter murmurhigh frequency & shorter murmur Increasing PVR Increasing PVR findings : findings : increased RV pulsations increased RV pulsations s2 loud, narrow splits2 loud, narrow split Infundibular hypertrophy Infundibular hypertrophy decreased Ldecreased LR shunt, R shunt, findings : findings : s2 decreases in intensity , s2 decreases in intensity , crescendo-decrescendo systolic murmur crescendo-decrescendo systolic murmur

in the in the ULSB,ULSB, cyanosis (shunt reversal )cyanosis (shunt reversal )

Eisenmenger’sEisenmenger’s apex by RV apex by RV Palpable dilated hypertensive Palpable dilated hypertensive

pulmonary trunkpulmonary trunk Loud pulmonary closure sound Loud pulmonary closure sound Very short or no systolic mr of vsdVery short or no systolic mr of vsd Short pulmonary ejection mr ULSBShort pulmonary ejection mr ULSB EDM of pulmonary regurgitation EDM of pulmonary regurgitation Loud harsh s1 coincident Loud harsh s1 coincident

holosystolic mr of TRholosystolic mr of TR

ECGECG small defects unremarkablesmall defects unremarkable LA enlargement LA enlargement - Mod restrictive, - Mod restrictive,

large Llarge LR shuntsR shunts left axis deviation left axis deviation Inlet vsd /AV septal defect Inlet vsd /AV septal defect 5% moderately restrictive vsds5% moderately restrictive vsds Ventricular septal aneurysms Ventricular septal aneurysms multiple vsds multiple vsds

LV enlargement LV enlargement in larger defectsin larger defects RVHRVH - Mild or moderate elevation of - Mild or moderate elevation of

RV RV pressure (rsR’ in V4R or V1)pressure (rsR’ in V4R or V1) - Large VSD, equal - Large VSD, equal

ventricular ventricular pressures , pressures , elevated elevated PVRPVR

RVH , RAD RVH , RAD - Eisenmenger’s - Eisenmenger’s RBBBRBBB - Surgical repair - Surgical repair

Chest x rayChest x ray Small defects that were mod restrictive at birth – increased LV Small defects that were mod restrictive at birth – increased LV size, dilated pulmonary trunk & its branchessize, dilated pulmonary trunk & its branches

Large shunts – hyperinflated lungs with flat hemi Large shunts – hyperinflated lungs with flat hemi diaphragms diaphragms

LA enlargement best appreciated in the lateral position LA enlargement best appreciated in the lateral position

Increased PVR, decreases LIncreased PVR, decreases LR shunt, decreases heart size, R shunt, decreases heart size, enlargement of pulmonary trunk& its branches persistsenlargement of pulmonary trunk& its branches persists

Nonrestrictive vsd with elevated but variable PVR- enlargement Nonrestrictive vsd with elevated but variable PVR- enlargement of all 4 chambersof all 4 chambers

Eisenmenger’s syndrome- oligemic lung fields, RA,LA, LV normal, Eisenmenger’s syndrome- oligemic lung fields, RA,LA, LV normal, RV occupies the apex RV occupies the apex

Echocardiography Echocardiography

Echocardiography- dopplerEchocardiography- doppler CFM-Direction, timing of flowCFM-Direction, timing of flow IVG (mmHg) = 4v² IVG (mmHg) = 4v² PG = LVSP - RVSP PG = LVSP - RVSP

LVSP - PG jet = RVsp ≈ PaspLVSP - PG jet = RVsp ≈ Pasp RVSP = cuff systolic BP – 4v² RVSP = cuff systolic BP – 4v² PVR = TRV / TVI in RVOT x 10 + 0.16PVR = TRV / TVI in RVOT x 10 + 0.16 High PA pressure, TRV/TVI rvot < 0.2 ; indicates low PVR, elevated High PA pressure, TRV/TVI rvot < 0.2 ; indicates low PVR, elevated

pressure secondary to the flowpressure secondary to the flow

Cardiac catheterizationCardiac catheterization

Hemodynamic assessmentsHemodynamic assessments cardiac indexcardiac index oximetryoximetry quantification of shuntquantification of shunt To assess pulmonary vascular To assess pulmonary vascular

resistanceresistance Pts with increased PVR, with mod or large Pts with increased PVR, with mod or large

LLR shuntR shunt If PVR is increased, response to 100% If PVR is increased, response to 100%

oxygen,NO testedoxygen,NO tested

cineangiographycineangiography

Defect best imaged in Defect best imaged in LAO(70°)cranial (25°)LAO(70°)cranial (25°)

Inlet defect - hepatoclavicular Inlet defect - hepatoclavicular viewview

( 40°LAO,cranial ( 40°LAO,cranial angulation)angulation)

Anterior muscular VSD- RAO viewAnterior muscular VSD- RAO view Aortography - r/o PDA ,coarctation Aortography - r/o PDA ,coarctation

Other imaging modalitiesOther imaging modalities

Cardiac CT- Cardiac CT- assess VSD anatomy in assess VSD anatomy in suboptimal echo imgessuboptimal echo imges

No information about No information about shunt fractionshunt fraction

MRIMRI delineate vsd location& shunt delineate vsd location& shunt

fraction in complex associated fraction in complex associated lesionslesions

ManagementManagement



drugsdrugs







VSD with aortic regurgitation VSD with aortic regurgitation VSD with associated defectsVSD with associated defects Failure of congestive cardiac Failure of congestive cardiac

failure to respond to medicationsfailure to respond to medications

Timing of surgeryTiming of surgery in VSD in VSD


growth failure, increasing PAHgrowth failure, increasing PAH >6 months >6 months – primarily based on – primarily based on



PA banding- palliative procedure , when PA banding- palliative procedure , when additional lesions make repair difficultadditional lesions make repair difficult


beyond the constrictionbeyond the constriction RV/PA pressure gradient > 45 RV/PA pressure gradient > 45

associated with hypoxemia associated with hypoxemia


Every 1-2 yrsEvery 1-2 yrs VSD & mild PAH& repair after 3 yrs VSD & mild PAH& repair after 3 yrs

of age- of age- watch for progressive watch for progressive pulmonary vascular pulmonary vascular diseasedisease

long term follow up neededlong term follow up needed

Special Special situationssituations

VSD with AR VSD with AR Peri membranous VSD with AR - 5-8%Peri membranous VSD with AR - 5-8% Subarterial VSDs – 30%Subarterial VSDs – 30% Sagging or herniation of RCC or RCC+ NCCSagging or herniation of RCC or RCC+ NCC May cause RVOT obstructionMay cause RVOT obstruction Due to morphological abnormality of valve Due to morphological abnormality of valve LV volume – regurgitant volume & shunt LV volume – regurgitant volume & shunt

volumevolume VSD murmur dates from infancyVSD murmur dates from infancy AR murmur appears (5-9 yrs) AR murmur appears (5-9 yrs)

LV LV RA shunt RA shunt Gerbode defectGerbode defect Shunt begins Shunt begins

inuteroinutero Usually restrictiveUsually restrictive Rightward thoracic Rightward thoracic

position of murmur position of murmur X ray – RA X ray – RA

enlargement enlargement disproportionate to disproportionate to the size of the size of pulmonary trunkpulmonary trunk

Ventricular Septal Ventricular Septal DefectsDefects

Tate Gisslen, MDTate Gisslen, MD

Mentor: Bradley S. Mentor: Bradley S. Marino, MD, MPP, MSCEMarino, MD, MPP, MSCE

May 6, 2011May 6, 2011

PhysiologyPhysiology Blood flow (which way and how Blood flow (which way and how

much) dependent on multiple much) dependent on multiple factorsfactors Small and restrictiveSmall and restrictive

Lesion sizeLesion size Large and non-restrictiveLarge and non-restrictive

Balance between pulmonary and Balance between pulmonary and systemic vascular resistancesystemic vascular resistance

Lesion SizeLesion Size• Restrictive VSDRestrictive VSD

– < 0.5 cm< 0.5 cm2 2 (Smaller than Ao valve orifice area)(Smaller than Ao valve orifice area)– Small L to R shuntSmall L to R shunt– Normal RV outputNormal RV output– 75% spontaneously close < 2yrs75% spontaneously close < 2yrs

• Non-restrictive VSDNon-restrictive VSD– > 1.0 cm> 1.0 cm2 2 (Equal to or greater than to Ao valve (Equal to or greater than to Ao valve

orifice area)orifice area)– Equal RV and LV pressuresEqual RV and LV pressures– Large hemodynamically significant L to R shuntLarge hemodynamically significant L to R shunt– Rarely close spontaneouslyRarely close spontaneously

Vascular ResistanceVascular Resistance• Pulmonary resistance may remain high longer in Pulmonary resistance may remain high longer in

infants with large VSDinfants with large VSD– Minimal L to R shuntMinimal L to R shunt

• Decreasing pulmonary resistance leads to Decreasing pulmonary resistance leads to significant L to R shunt significant L to R shunt – Clinical symptoms of CHFClinical symptoms of CHF

• Persistent L to R shunt leads to hypertrophy of the Persistent L to R shunt leads to hypertrophy of the medial smooth muscle layer of the pulmonary medial smooth muscle layer of the pulmonary arteries which increases PVR and potential R to L arteries which increases PVR and potential R to L shuntingshunting

• Long-standing L to R shunting that results in Long-standing L to R shunting that results in chronically increased PVR may lead to persistent R chronically increased PVR may lead to persistent R to L shunting described as “Eisenmenger to L shunting described as “Eisenmenger Physiology”Physiology”

Clinical Features-Small Clinical Features-Small LesionsLesions Murmur Murmur

4 to 10 days, early with rapid 4 to 10 days, early with rapid decrease in PVRdecrease in PVR

Asymptomatic Asymptomatic normal feeding, growth and normal feeding, growth and

developmentdevelopment

MurmursMurmurs Restrictive VSD - Holosystolic Restrictive VSD - Holosystolic

murmurmurmurcorrelates with continuous correlates with continuous

pressure gradientpressure gradient

Non-restrictive large VSD – no Non-restrictive large VSD – no murmur (no turbulence if no murmur (no turbulence if no gradient) gradient)

Clinical Features-Large Clinical Features-Large LesionsLesions Accentuated precordial activityAccentuated precordial activity

More prominent as LV volume increasesMore prominent as LV volume increases Signs/symptoms of CHFSigns/symptoms of CHF

DiaphoresisDiaphoresis TachypneaTachypnea Fatigue with feedingFatigue with feeding HepatomegalyHepatomegaly RalesRales Duskiness with cryingDuskiness with crying

May develop as early as 2 weeksMay develop as early as 2 weeks Severity increases as PVR decreases Severity increases as PVR decreases

EvaluationEvaluation

Chest RadiographyChest RadiographyCardiomegalyCardiomegalyIncreased pulmonary Increased pulmonary vasculaturevasculature

Pulmonary edemaPulmonary edema

CXR of VSDCXR of VSD

EvaluationEvaluation EKGEKG

Small: normal or LVHSmall: normal or LVHProminent Q, R, and T waves in II, Prominent Q, R, and T waves in II, III, aVF and V6III, aVF and V6

Large: Biventricular hypertrophyLarge: Biventricular hypertrophyRVH- rsR’ in V1, S wave in V6RVH- rsR’ in V1, S wave in V6

EchocardiographyEchocardiography

Assess indication in consultation with Assess indication in consultation with Cardiology Cardiology

Assess location, size, and multiplicityAssess location, size, and multiplicity RV and PA pressureRV and PA pressure Assess for LA and LV dilationAssess for LA and LV dilation Assess LV functionAssess LV function Note relation to great vessels, AV valvesNote relation to great vessels, AV valves

Cardiac CatheterizationCardiac Catheterization

Able to document Able to document Number of defectsNumber of defects Presence of associated defectsPresence of associated defects Magnitude of shunt Magnitude of shunt Estimate PVR Estimate PVR

Not used if information apparent by other Not used if information apparent by other meansmeans Most information available through Most information available through

EchocardiographyEchocardiography

PrevalencePrevalence Most common congenital heart Most common congenital heart

lesionlesion Occurs in 50% of children with Occurs in 50% of children with

heart lesionsheart lesions 15-20% in isolation15-20% in isolation 5-50 per 1000 live births5-50 per 1000 live births 56% female56% female

Chromosomal Disorders Chromosomal Disorders associated with VSDassociated with VSD

Trisomy 21: 40% of T21 will have VSDTrisomy 21: 40% of T21 will have VSD Trisomy 13, 18: 18% of T13, 31% of T18 will have VSDTrisomy 13, 18: 18% of T13, 31% of T18 will have VSD 22q11 deletion:22q11 deletion:

Tetrology of Fallot is most common anomaly Tetrology of Fallot is most common anomaly VSD with or without aortic arch anomaly is second most VSD with or without aortic arch anomaly is second most

common common Holt-Oram (Hand-heart syndrome): TBX5 gene found Holt-Oram (Hand-heart syndrome): TBX5 gene found

on Chromosome 12on Chromosome 12 Recurrence risk for VSD based on parental VSDRecurrence risk for VSD based on parental VSD

Paternal 2%Paternal 2% Maternal 6-10%Maternal 6-10%

Treatment for Small VSDTreatment for Small VSD No medication or surgery if No medication or surgery if

asymptomaticasymptomatic 75-80% close by 2 years75-80% close by 2 years

ObservationObservation

No antibiotic prophylaxis for No antibiotic prophylaxis for proceduresprocedures

CHF TreatmentCHF Treatment High-calorie formulaHigh-calorie formula MedicationMedication

DiureticsDiuretics Furosemide with or without Furosemide with or without

spironolactonespironolactone Afterload reductionAfterload reduction

Enalapril or Captopril Enalapril or Captopril Digoxin (maybe) Digoxin (maybe) Symptoms of CHF improve as L to R Symptoms of CHF improve as L to R

shunt decreasesshunt decreases

Indications for InterventionIndications for Intervention Decompensated CHFDecompensated CHF Compensated CHF with:Compensated CHF with:

Large hemodynamically significant Large hemodynamically significant VSD - L to R shunting with Qp/Qs VSD - L to R shunting with Qp/Qs >> 2:1, even if asymptomatic, ideally 2:1, even if asymptomatic, ideally before 1 yearbefore 1 year

Growth failure, unresponsive to Growth failure, unresponsive to medical therapy is an indication medical therapy is an indication for surgeryfor surgery

Post-InterventionPost-Intervention

Most infants have normal growth Most infants have normal growth and developmentand development

Early closure (< 1 year) associated Early closure (< 1 year) associated with better LV function and with better LV function and regression of hypertrophyregression of hypertrophy

Residual VSD is not commonResidual VSD is not common RBBB is common following surgeryRBBB is common following surgery

Rare complete heart blockRare complete heart block

Part IPart IFetal Circulation, ASD, VSDFetal Circulation, ASD, VSD


Henri Roger was the first man to Henri Roger was the first man to describe a ventricular septal defect, describe a ventricular septal defect, in 1879 he wrote: in 1879 he wrote: ““A developmental defect of the heart A developmental defect of the heart occurs from which cyanosis does not ensue occurs from which cyanosis does not ensue in spite of the fact that a communication in spite of the fact that a communication exists between the cavities of the two exists between the cavities of the two ventricles and in spite of the fact that the ventricles and in spite of the fact that the admixture of venous blood and arterial admixture of venous blood and arterial blood occurs. This congenital defect, which blood occurs. This congenital defect, which is even compatible with long life, is a is even compatible with long life, is a simple one. It comprises a defect in the simple one. It comprises a defect in the interventricular septum”interventricular septum”


Most common CHD in children (25%)Most common CHD in children (25%) Isolated VSD found in only 10% of Isolated VSD found in only 10% of

adults with CHDadults with CHD 75-80% of small VSD’s close 75-80% of small VSD’s close

spontaneously by late childhoodspontaneously by late childhood 10-15% of large VSD’s close 10-15% of large VSD’s close

spontaneouslyspontaneously 60% of defects close before age 3, 60% of defects close before age 3,

and 90% before age 8and 90% before age 8 Risk factors for decreased survival Risk factors for decreased survival

for unoperated patients include:for unoperated patients include: Cardiomegaly on CXR, Elevated PASP Cardiomegaly on CXR, Elevated PASP

(>50 mmHg), and CV symptoms(>50 mmHg), and CV symptoms

Ventricular SeptumVentricular Septum

Partitioning begins as a Partitioning begins as a muscular ridge near muscular ridge near the apexthe apex

Ridge undergoes active Ridge undergoes active growth which forms the growth which forms the muscular septum (inlet, muscular septum (inlet, trabecular, and outlet)trabecular, and outlet)

Concomitantly Concomitantly endocardial cushions endocardial cushions fuse and the two fuse and the two regions meet regions meet

InletTrabecular

Outlet

Ventricular SeptumVentricular Septum

Partitioning begins as a Partitioning begins as a muscular ridge near muscular ridge near the apexthe apex

Ridge undergoes active Ridge undergoes active growth which forms the growth which forms the muscular septum (inlet, muscular septum (inlet, trabecular, and outlet)trabecular, and outlet)

Concomitantly Concomitantly endocardial cushions endocardial cushions fuse and the two fuse and the two regions meet regions meet

InletTrabecular

Outlet

Types of VSD’sTypes of VSD’s

Perimembranous defect (70-80%)Perimembranous defect (70-80%) Less likely to be associated with other defectsLess likely to be associated with other defects Highest rate of spontaneous closureHighest rate of spontaneous closure

Muscular or apical defects (5-20%)Muscular or apical defects (5-20%) Typically occur in isolationTypically occur in isolation High spontaneous closure rates unless multipleHigh spontaneous closure rates unless multiple

AV-Canal type (5-8%)AV-Canal type (5-8%) Rarely close spontaneously, commonly seen in Trisomy Rarely close spontaneously, commonly seen in Trisomy

2121 Usually large & associated with abnormal AV valveUsually large & associated with abnormal AV valve

Supracristal or subaortic defects (5-7%)Supracristal or subaortic defects (5-7%) Often small but need closure due to associated AROften small but need closure due to associated AR

VSDVSD Arterial pulse is often normalArterial pulse is often normal There may be a systolic thrill on palpation of There may be a systolic thrill on palpation of

the precordium (maximal in 3the precordium (maximal in 3rdrd or 4 or 4thth ICS) ICS) Holosystolic, high frequency murmur (grade Holosystolic, high frequency murmur (grade

4-6/6) with small VSD and normal PAP4-6/6) with small VSD and normal PAP Once PAP increases above the systemic Once PAP increases above the systemic

pressures the holosystolic murmur disappearspressures the holosystolic murmur disappears Increase flow across pulmonary valve causes Increase flow across pulmonary valve causes

a SEM a SEM A loud P2 component is heard in this settingA loud P2 component is heard in this setting

ECG in VSDECG in VSD May be normal but often shows LVH and LAEMay be normal but often shows LVH and LAE Presence of RAD represents elevated RVP and PAPPresence of RAD represents elevated RVP and PAP Postoperative RBBB is commonPostoperative RBBB is common

CXR in VSDCXR in VSD Cardiomegaly with LAE and LVE will be seen with large L to R shuntsCardiomegaly with LAE and LVE will be seen with large L to R shunts A large defect associated with a small heart and oligemic lung fields should A large defect associated with a small heart and oligemic lung fields should

raise the suspicion of pulmonary vascular diseaseraise the suspicion of pulmonary vascular disease

VSDVSD Hemodynamic severity grading of isolated VSDs in Hemodynamic severity grading of isolated VSDs in

adults:adults: Small: Qp:Qs <1.4, and pulmonary to aortic systolic Small: Qp:Qs <1.4, and pulmonary to aortic systolic

pressure <0.3pressure <0.3 Moderate: Qp:Qs = 1.4-2.2, and systolic pressure ratio Moderate: Qp:Qs = 1.4-2.2, and systolic pressure ratio

>0.3>0.3 Large: Qp:Qs >2.2, and systolic pressure ratio >0.3Large: Qp:Qs >2.2, and systolic pressure ratio >0.3 Eisenmenger: Qp:Qs <1.5 and systolic pressure ratio >0.9Eisenmenger: Qp:Qs <1.5 and systolic pressure ratio >0.9

Physiologic classification:Physiologic classification: Restrictive: RV pressure < LV pressure in absence of Restrictive: RV pressure < LV pressure in absence of

RVOTORVOTO Nonrestrictive: RV pressure = LV pressure in absence of Nonrestrictive: RV pressure = LV pressure in absence of

RVOTORVOTO

VSDVSD Clinical severity grading:Clinical severity grading:

Small: Causes negligible hemodynamic changes. LV size Small: Causes negligible hemodynamic changes. LV size normal w/o PHTNnormal w/o PHTN

Moderate: Causes LV and LA enlargment, and usually some Moderate: Causes LV and LA enlargment, and usually some PHTN (reversible)PHTN (reversible)

Large: Results in pulmonary vascular obstructive disease and Large: Results in pulmonary vascular obstructive disease and Eisenmenger physiology unless there is coexistent RVOTOEisenmenger physiology unless there is coexistent RVOTO

Pathologic and surgical classification:Pathologic and surgical classification: Perimembranous: bordered by fibrous continuity of an AV Perimembranous: bordered by fibrous continuity of an AV

valve and an arterial valve, usually with inlet or outlet valve and an arterial valve, usually with inlet or outlet extensionextension

Muscular: bordered by muscular rim, usually trabecularMuscular: bordered by muscular rim, usually trabecular Doubly committed: bordered by fibrous continuity of both the Doubly committed: bordered by fibrous continuity of both the

aortic and pulmonary valves aortic and pulmonary valves

VSD RepairVSD Repair

When repair is performed in the first two years of When repair is performed in the first two years of life, asymptomatic adult survival with normal life, asymptomatic adult survival with normal growth and development can be anticipatedgrowth and development can be anticipated

When surgery is undertaken in older children, a When surgery is undertaken in older children, a late postopeartive increase in LV chamber size, late postopeartive increase in LV chamber size, together with decreased systolic function is seentogether with decreased systolic function is seen

Development of late postoperative PHTN is Development of late postoperative PHTN is largely determined by the age at surgery and largely determined by the age at surgery and preoperative PVRpreoperative PVR

Risk of SBE persists and requires prophylaxisRisk of SBE persists and requires prophylaxis

The initial workup at a minimum The initial workup at a minimum should include:should include: A through clinical assessmentA through clinical assessment ECGECG CXRCXR TTE/Doppler evaluationTTE/Doppler evaluation

The diagnostic workup may require:The diagnostic workup may require: OxymetryOxymetry Right heart Cath (PAP and PVR determination, to assess Right heart Cath (PAP and PVR determination, to assess

pulmonary vascular reactivity)pulmonary vascular reactivity) Coronary angio (in high risk pts or in pts >40 y if surgical Coronary angio (in high risk pts or in pts >40 y if surgical

repair is planned)repair is planned) MRI to prove existence of VSD or to assess for other MRI to prove existence of VSD or to assess for other

anomalie if doubt remains after other imaging anomalie if doubt remains after other imaging modalities. Also can calculate Qp:Qsmodalities. Also can calculate Qp:Qs

Oxygen saturation with exercise if there is any Oxygen saturation with exercise if there is any suggestion of PHTN. Do not exercise if there is severe suggestion of PHTN. Do not exercise if there is severe PHTN or resting oxygen Sat is <85%PHTN or resting oxygen Sat is <85%

Open lung Bx should be considered when the reversibility Open lung Bx should be considered when the reversibility of PHTN is uncertain from hemodynamic dataof PHTN is uncertain from hemodynamic data

Indications for intervention: Geade C, Level IVIndications for intervention: Geade C, Level IV Presence of a significant VSD (symptomatic QP/QS = 2/1, Presence of a significant VSD (symptomatic QP/QS = 2/1,

PASP > 50 mmHg), deteriorating ventricular fx due to PASP > 50 mmHg), deteriorating ventricular fx due to volume (LV) or pressure (RV) overloadvolume (LV) or pressure (RV) overload

Significant RVOTO (pk to pk gradient of > 50 mmHg, or Significant RVOTO (pk to pk gradient of > 50 mmHg, or instantaneous gradient >70 mmHg)instantaneous gradient >70 mmHg)

Perimembranous or doubly committed VSD with more Perimembranous or doubly committed VSD with more than mild ARthan mild AR

In presence of severe PHTN (PAP >2.3 SABP, or PVR >2/3 In presence of severe PHTN (PAP >2.3 SABP, or PVR >2/3 SVR), there must be a net L to R shunt of >1.5 or SVR), there must be a net L to R shunt of >1.5 or evidence of PA reactivity when challenged with evidence of PA reactivity when challenged with pulmonary vasodilator, or lung Bx evidence of PA pulmonary vasodilator, or lung Bx evidence of PA changes are potentially reversible (Heath Edwards grade changes are potentially reversible (Heath Edwards grade II-III or less)II-III or less)

Hx of endocarditis especially if recurrent Hx of endocarditis especially if recurrent

Transvenous pacing should be avoided where Transvenous pacing should be avoided where possible in all patients with VSDs because possible in all patients with VSDs because paradoxical emboli may occur. paradoxical emboli may occur.

Patients with isolated VSD with or without Patients with isolated VSD with or without associated lesions RVOTO, AV prolapse, associated lesions RVOTO, AV prolapse, subaortic stenosis, or infective endocarditis) subaortic stenosis, or infective endocarditis) should be repaired by congenital heart surgeons.should be repaired by congenital heart surgeons.

Device closure of VSDs may be performed in the Device closure of VSDs may be performed in the setting of isolated trabecular muscular VSDs but setting of isolated trabecular muscular VSDs but are still considered experimental for are still considered experimental for perimembranous VSDsperimembranous VSDs

Successful closure is associated with excellent Successful closure is associated with excellent survival if ventricular fx is normal. Elevated PAP survival if ventricular fx is normal. Elevated PAP preop may progress, regress, or remain the same preop may progress, regress, or remain the same postoppostop

A. fib may occur, especially if there has been A. fib may occur, especially if there has been longstanding volume overload of the left heart. longstanding volume overload of the left heart. Late VT and sudden death are potential risks, Late VT and sudden death are potential risks, especially in patients repaired late in life. CHB especially in patients repaired late in life. CHB may also occur after surgical repairmay also occur after surgical repair

Pregnancy is well tolerated in women with small Pregnancy is well tolerated in women with small or moderate VSD and in women with repaired VSDor moderate VSD and in women with repaired VSD

Pregnancy is contraindicated in women with Pregnancy is contraindicated in women with Eisenmenger syndrome due to both high maternal Eisenmenger syndrome due to both high maternal ((>50%) and fetal (~60%) mortality>50%) and fetal (~60%) mortality

Follow-up:Follow-up: Patients with following problems benefit from Patients with following problems benefit from

periodic evaluation by cardiologistperiodic evaluation by cardiologist Patch leaks or residual VSDs (which seldom require Patch leaks or residual VSDs (which seldom require

reoperation)reoperation) Elevated PVR at time of surgeryElevated PVR at time of surgery Aortic valve surgeryAortic valve surgery Late repair of moderate or large defectsLate repair of moderate or large defects Significant atrial or ventricular arrhythmiasSignificant atrial or ventricular arrhythmias Associated cardiac lesions (eg RVOTO, AR)Associated cardiac lesions (eg RVOTO, AR)

Endocarditis prophylaxis is recommended for Endocarditis prophylaxis is recommended for 6/12 following VSD closure or for life if residual 6/12 following VSD closure or for life if residual defect persistsdefect persists

ATRIAL WAVESATRIAL WAVES

a= atrial contractiona= atrial contraction c= contraction of ventricle and closure of tricuspid valvec= contraction of ventricle and closure of tricuspid valve x=x descent x=x descent v=venous filling v=venous filling y= y descent due to opening of tricuspid valvey= y descent due to opening of tricuspid valve

RIGHT HEART PRESSURESRIGHT HEART PRESSURES

Haemodynamic ParametersHaemodynamic Parameters

The following criteria suggest left atrial enlargement/abnormality The following criteria suggest left atrial enlargement/abnormality when correlated with echocardiographic data:when correlated with echocardiographic data:

Negative phase of P in V1>0.04 sec — sensitivity 83 percent; Negative phase of P in V1>0.04 sec — sensitivity 83 percent; specificity 80 percent specificity 80 percent

Negative phase of P in V1>1 mm — sensitivity 60 percent; Negative phase of P in V1>1 mm — sensitivity 60 percent; specificity 93 percent specificity 93 percent

P-terminal force >0.04 mm/sec — sensitivity 69 percent; P-terminal force >0.04 mm/sec — sensitivity 69 percent; specificity 93 percent specificity 93 percent

Notched P, interpeak interval >0.04 sec — sensitivity 15 percent; Notched P, interpeak interval >0.04 sec — sensitivity 15 percent; specificity 100 percent specificity 100 percent

P wave duration >0.11 sec — sensitivity 33 percent; specificity P wave duration >0.11 sec — sensitivity 33 percent; specificity 88 percent 88 percent

P wave/PR duration >1.6 — sensitivity 31 percent; specificity 64 P wave/PR duration >1.6 — sensitivity 31 percent; specificity 64 percentpercent

CYANOSIS IN CHILDRENCYANOSIS IN CHILDREN Central cyanosisCentral cyanosis::

Cyanosis of the tongue,mucous membranes Cyanosis of the tongue,mucous membranes and peripheral skin, it is necessary to have and peripheral skin, it is necessary to have >>3g/dl of reduced Hb to have it.3g/dl of reduced Hb to have it.

Peripheral cyanosis:Peripheral cyanosis:

It is visible only in the skin of the It is visible only in the skin of the extremities with normal arterial saturation extremities with normal arterial saturation due to vasomotor instability,ex. cold due to vasomotor instability,ex. cold environment.environment.

CAUSES OF CENTRAL CAUSES OF CENTRAL CYANOSISCYANOSIS

A.A. Congenital Heart DiseaseCongenital Heart Disease

1) 1) Cyanosis with PBF 2) Cyanosis with PBFCyanosis with PBF 2) Cyanosis with PBF

a)a) TOF a) D-TGATOF a) D-TGA b)b) Pulm. Atresia b) DORVPulm. Atresia b) DORV c)c) Tricuspid Atresia c) TAPVCTricuspid Atresia c) TAPVC d)d) Critical PS d) Truncus Critical PS d) Truncus

arteriosusarteriosus

CAUSES OF CENTRAL CYANOSISCAUSES OF CENTRAL CYANOSIS B) B) LUNG DISEASELUNG DISEASE D) D) CNS DEPRESSIONCNS DEPRESSION a)a) RDS a) IVHRDS a) IVH b)b) Pneumonia b) Perinatal asphyxiaPneumonia b) Perinatal asphyxia c)c) Pneumothorax c) Heavy maternal sedationPneumothorax c) Heavy maternal sedation d)d) Pleural effusion Pleural effusion e)e) Diaphragmatic herniaDiaphragmatic hernia f)f) T.E.Fistula T.E.Fistula

C) C) PERSISTENT PULMONARYPERSISTENT PULMONARY E) E) MISCELLANOUSMISCELLANOUS HYPERTENSIONHYPERTENSION a) shock & sepsisa) shock & sepsis b) Hypoglycemiab) Hypoglycemia (PFC syndrome) c) Methemoglobinemia(PFC syndrome) c) Methemoglobinemia d) Neuromuscular conditionsd) Neuromuscular conditions ( Werdnig ( Werdnig –– Hoffman) Hoffman)

RADIOLOGICAL FEATURESRADIOLOGICAL FEATURES CXR may exclude non cardiac causes of cyanosis e.g. RDS. . CXR may exclude non cardiac causes of cyanosis e.g. RDS. .

Meconium aspiration, Diaphgramatic hernia, PneumothoraxMeconium aspiration, Diaphgramatic hernia, PneumothoraxPulmonary Vascular MarkingsPulmonary Vascular Markings

Decreased Increased Decreased Increased

Heart SizeHeart Size Heart SizeHeart Size

NormalNormal Increased Increased IncreasedIncreased( “Boot shaped”) (“ Wall-to-Wall”) ( “Boot shaped”) (“ Wall-to-Wall”) TOF Ebstein (“ egg-on-end”)TOF Ebstein (“ egg-on-end”) D-TGAD-TGA Aortic Arch \ MediastinumAortic Arch \ Mediastinum

Abdominal SitusAbdominal Situs

ECGECG

RV dominace on ECG is normal in the newbornRV dominace on ECG is normal in the newborn..

Left axis deviation with LVH strongly suggests:Left axis deviation with LVH strongly suggests:

Tricuspid Atresia.Tricuspid Atresia.

Left axis deviation in a newborn may also indicateLeft axis deviation in a newborn may also indicate::

AV canalAV canal

( However , AV canal is usually an acyanotic( However , AV canal is usually an acyanotic

form of heart disease).form of heart disease).

Blood gases & response to 100% Blood gases & response to 100%

O2O2

Always try to obtain ABG from RIGHT radial artery.Always try to obtain ABG from RIGHT radial artery.

Low PH: may indicate sepsis, circulatory shock or Low PH: may indicate sepsis, circulatory shock or

severe hypoxiasevere hypoxia

High pCO 2: may indicate CNS or pulmonary disease.High pCO 2: may indicate CNS or pulmonary disease.

Hyperoxia Test: 100% O2 by hood for 10 minutes.Hyperoxia Test: 100% O2 by hood for 10 minutes.

pO2 > 150 torr= pulmonary disease.pO2 > 150 torr= pulmonary disease. pO2 < 100 torr=cyanotic heart diseasepO2 < 100 torr=cyanotic heart disease

Transposition of the great Transposition of the great arteries ( TGA)arteries ( TGA)

? ASD ? VSD? PS? ASD ? VSD? PS PE: PE:

Single accent. S2Single accent. S2 VSD murmurVSD murmur

CXR : CXR : (egg on a string)(egg on a string) Increase CTRIncrease CTR Increase PVMIncrease PVM

ECG:ECG: RVHRVH

TGA ManagementTGA Management Medical:Medical:

PGE1PGE1 O2 (3L/minute)O2 (3L/minute) Correct :Correct :

acidosis ,hypoglycemia. acidosis ,hypoglycemia. electrolyte disturbances.electrolyte disturbances.

Transcatheter :Transcatheter : BASBAS

Surgical:Surgical: Arterial switch (Jatene operation)Arterial switch (Jatene operation) at 7-15 daysat 7-15 days Atrial switch ( Senning operation)Atrial switch ( Senning operation) at 6-9 monthsat 6-9 months

Tetralogy of FallotTetralogy of Fallot ? Degree of PS? Degree of PS Physical examination: Physical examination:

Single accent. S2Single accent. S2 ESMESM

CXR: CTR normalCXR: CTR normal

Decrease PVMDecrease PVM

ECG: RVHECG: RVH

Cyanotic Spells Cyanotic Spells

Spasm of decrease SVR cryingSpasm of decrease SVR crying

RVOTRVOT

Increase R…..L shuntingIncrease R…..L shunting

Increase systemic venous return DecreaseO2 Increase systemic venous return DecreaseO2 Increase CO2 Increase CO2 Decrease pH Decrease pH

TachypneeaTachypneea

Cyanotic SpellsCyanotic Spells Increase systemic vascular resistanceIncrease systemic vascular resistance Squat/Knee chest positionSquat/Knee chest position Ketamine 1-2mg/kg IVKetamine 1-2mg/kg IV Neosynephrine 0.02mg/kg IVNeosynephrine 0.02mg/kg IV TachycardiaTachycardia Propranolol 0.1mg/ Kg IV Propranolol 0.1mg/ Kg IV Release of infundibular spasmRelease of infundibular spasm Irritability Irritability Morphine 0.2mg/ Kg S.C or IMMorphine 0.2mg/ Kg S.C or IM

Hypoxia Hypoxia Oxygen Oxygen Dehydration Dehydration Volume Volume

AcidosisAcidosis NaHco3 1mEq/ Kg IV NaHco3 1mEq/ Kg IV

TOF managementTOF management

Medical :Medical : Correct iron deficiency anemiaCorrect iron deficiency anemia Correct polycythemiaCorrect polycythemia B-BlockerB-Blocker

Surgical:Surgical:

Palliative = Blalock-Taussig shunt Palliative = Blalock-Taussig shunt

for small PA’s for small PA’s Definitive= Total correctionDefinitive= Total correction

Pulmonary Atresia/VSDPulmonary Atresia/VSD

? ? PDA or PDA or Bronchial collaterals Bronchial collaterals

? ? PAs sizePAs size? Confluent or not.? Confluent or not. PE:PE:

Cyanosis at birthCyanosis at birth S2 singleS2 single No murmurNo murmur

CXRCXR: ( Like TOF): ( Like TOF) Normal CTR/ ↓PVMNormal CTR/ ↓PVM

ECGECG:: RAD/RVHRAD/RVH

Pulmonary Atresia/VSD Pulmonary Atresia/VSD ManagementManagement

Medical:Medical: ? ? PDA dependentPDA dependent

PGE1PGE1 Surgical:Surgical:

Palliative=shuntPalliative=shunt Definitive= staged repair Definitive= staged repair

(Unifocalization)(Unifocalization) + RV-PA conduit+ RV-PA conduit

Pulmonary atresia with intact septumPulmonary atresia with intact septum RV size? Coronary sinusoids present or notRV size? Coronary sinusoids present or not.. PE:PE:

Severe cyanosis/ tachypneaSevere cyanosis/ tachypnea S2 singleS2 single No murmurNo murmur

CXR:CXR: RAERAE Decrease PVMDecrease PVM

ECG:ECG: RAE, LVHRAE, LVH

Pulmonary atresia with intact septumPulmonary atresia with intact septum

Medical :Medical : PGE1 – Radiofrequency perforation of PVPGE1 – Radiofrequency perforation of PV..

Surgical:Surgical: Open pulmonary valvotomy+ palliative Blallock-shunt Open pulmonary valvotomy+ palliative Blallock-shunt (in the neonatal period).(in the neonatal period). If good RV size : Biventricular repair (at 1 year of age).If good RV size : Biventricular repair (at 1 year of age).

If small RV size ± sinusoids + RVDCCIf small RV size ± sinusoids + RVDCC : : BT- shunt in neonatal periodBT- shunt in neonatal period Bidirectinol Glenn (at 6 months of age).Bidirectinol Glenn (at 6 months of age). Total Cavo Pulmonary Connection (TCPC) Total Cavo Pulmonary Connection (TCPC) (at 4 years of age)(at 4 years of age)

Ticuspid AtresiaTicuspid Atresia

? PS ? TGA? VSD? PS ? TGA? VSD

PE:PE: Single S2Single S2 VSD murmurVSD murmur ? CHF ? CHF

ECG:ECG: - Superior QRS axis- Superior QRS axis - RAE& LVH- RAE& LVH

Tricuspid Atresia Management Tricuspid Atresia Management

Medical:Medical: Inadequate pulmonary flow:Inadequate pulmonary flow:

PGE1PGE1 BAS ( balloon atrial septostomy )BAS ( balloon atrial septostomy )

Increase pulmonary blood flow (e.g.sizable VSD):Increase pulmonary blood flow (e.g.sizable VSD): Pulmonary artery bandingPulmonary artery banding

SurgicalSurgical:: Palliative shunt:Palliative shunt: Blalock-Taussig shuntBlalock-Taussig shunt Bidirectional GlennBidirectional Glenn Definitive repair:Definitive repair: TCPC ( Total Cavo Pulmonary Connection)TCPC ( Total Cavo Pulmonary Connection)

Total Anomalous Pulmonary Total Anomalous Pulmonary Venous Connection ( TAPVC)Venous Connection ( TAPVC) ? Site ? Obstructed? Site ? Obstructed PE:PE:

( Large ASD)( Large ASD) CHFCHF Wide split S2Wide split S2 ESMESM Diastolic rumble( overflow at TV)Diastolic rumble( overflow at TV)

CXR: CXR: (snow man appearance)(snow man appearance) Increase CTRIncrease CTR Increase PVMIncrease PVM

ECG:ECG: RAERAE RVHRVH

Total Anomalous Pulmonary Total Anomalous Pulmonary Venous Connection ( TAPVC)Venous Connection ( TAPVC)


TAPVC, supracardiac via vertical vein


TAPVC, to coronary sinus

Total Anomalous Pulmonary Total Anomalous Pulmonary Venous Connection Venous Connection ( TAPVC)( TAPVC)

TAPVC, Infradiaphragmatic

TreatmentTreatment

Correct acidosisCorrect acidosis

AntifailureAntifailure

Surgery:Surgery:

Anastomosis of Anastomosis of

Common Pulmonry Vein to the left Common Pulmonry Vein to the left atriumatrium

Persistent Truncus Persistent Truncus ArteriosusArteriosus::

Increase pulm. blood flowIncrease pulm. blood flow Increase pulm. vascular resistanceIncrease pulm. vascular resistance PE:PE:

RV tapRV tap S2 singleS2 single Ejection clickEjection click ± Diastolic murmur± Diastolic murmur

CXR:CXR: Increase CTRIncrease CTR Increae PVMIncreae PVM

ECG:ECG: RVHRVH

Persistent Truncus Persistent Truncus Arteriosus: Arteriosus:

Medical:Medical:

Antifailure therapyAntifailure therapy

Surgical:Surgical:

Wait till PVR dropsWait till PVR drops Total correction at 2-3 monthsTotal correction at 2-3 months

Ebstein Anomaly Ebstein Anomaly ? PS? PS PE:PE:

Mild to severe cyanosisMild to severe cyanosis S2 wide splitS2 wide split TR murmurTR murmur HepatomegalyHepatomegaly

CXR: CXR: Balloon shapeBalloon shape Increase CTRIncrease CTR Decrease PVMDecrease PVM

ECG:ECG: RAERAE ± RBBB± RBBB WPW? SVT?WPW? SVT?

Ebstein AnomalyEbstein Anomaly 2D-Echo-Doppler2D-Echo-Doppler

Ebstein managementEbstein management Medical:Medical:

Mild: nothingMild: nothing CHF: DiureticsCHF: Diuretics Severe cyanosis:Severe cyanosis:

PGE1PGE1 SVT: InderalSVT: Inderal

Surgical:Surgical: Palliative shuntPalliative shunt Definitive repair:Definitive repair: If good RV size: Danielson repairIf good RV size: Danielson repair Carpentier repairCarpentier repair If small RV----- If small RV----- Univentricular repair strategyUniventricular repair strategy

Single VentricleSingle Ventricle

↑↑Pulm. Blood flowPulm. Blood flow:: No PSNo PS

Lt-→ Rt shuntLt-→ Rt shunt

Like large VSDLike large VSD

↓↓Pulm. Blood flow:Pulm. Blood flow: PSPS

Rt-→Lt shuntRt-→Lt shunt

Like FallotLike Fallot

Single Ventricle Single Ventricle Management Management

With ↓pulm. Blood flow:With ↓pulm. Blood flow:

Medical:Medical: PGE1 PGE1

Surgical :Surgical :

- - Blalock shuntBlalock shunt - Bidirectional Glenn- Bidirectional Glenn - Total Cavo Pulmonary Connection- Total Cavo Pulmonary Connection

((TCPCTCPC))

Thank YouThank You

Precordial ActivityPrecordial Activity Visible precordial impulse:Visible precordial impulse:

Prominent feature of:Prominent feature of: D-TGAD-TGA TAPVRTAPVR Vein of Galen Vein of Galen

aneurysmaneurysm

RV impulse not palpable:RV impulse not palpable: Tricuspid atresiaTricuspid atresia Thrill:Thrill: Only cyanotic newborn with a Only cyanotic newborn with a

thrill: Tricuspid atresiathrill: Tricuspid atresia

AUSCULTATIONAUSCULTATION

HEART SOUNDSHEART SOUNDS::

S1 is normally accentuated in newbornsS1 is normally accentuated in newborns

S2 split is normally heard as a slurring S2 split is normally heard as a slurring

rather than a distinct split.rather than a distinct split. S2 is single in many cyanotic lesions S2 is single in many cyanotic lesions

especially in, D-TGA, TOF.especially in, D-TGA, TOF.

S2 is widely split in TAPVR, Critical PSS2 is widely split in TAPVR, Critical PS..

MURMURSMURMURS SYSTOLIC EJECTION MURMURSSYSTOLIC EJECTION MURMURS May be heard in the first hours of life.May be heard in the first hours of life. Usually due to ventricular obstruction e.g. AS., PS., TOFUsually due to ventricular obstruction e.g. AS., PS., TOF DIASTOLIC MURMURS:DIASTOLIC MURMURS: Rarely heard in newbornsRarely heard in newborns Early diastolic murmurs heard in Truncus arteriosus ,Early diastolic murmurs heard in Truncus arteriosus , TOF with absent pulmonary valveTOF with absent pulmonary valve Continuous:Continuous: Continuous murmurs are caused by AV fistulasContinuous murmurs are caused by AV fistulas (not PDA)(not PDA) ABSENT:ABSENT: Silent hearts often characteristic of Tricuspid atresia , Silent hearts often characteristic of Tricuspid atresia ,

Pulmonary atresia & D-TGA.Pulmonary atresia & D-TGA.

EJECTIONS CLICKSEJECTIONS CLICKS Deformity of semilunar valvesDeformity of semilunar valves

1) Pulmonary: PS1) Pulmonary: PS

2) Aortic: Critical AS2) Aortic: Critical AS

CoACoA

Dilatation of great vesselDilatation of great vessel

1) PULMONARY ARTERY: PS1) PULMONARY ARTERY: PS

HLHSHLHS

2) ASC2) ASC. AORTA: . AORTA: Truncus arteriosusTruncus arteriosus

TOFTOF

Right to Left ShuntingRight to Left Shunting(Tetralogy physiology(Tetralogy physiology))

QS > QPQS > QP

Atrial Ventricular Great vessels Pulm microcirc. Atrial Ventricular Great vessels Pulm microcirc.

(ASD) (VSD)(ASD) (VSD)

Tricuspid Sub PS Tricuspid Sub PS

Atresia Critical PSAtresia Critical PS

USEFUL HINTSUSEFUL HINTS Large male baby with rapid, shallow abdominal breathing:Large male baby with rapid, shallow abdominal breathing:

D-TGAD-TGA Upper body blue, lower body pink; seen in : D-GA+PDA.COAUpper body blue, lower body pink; seen in : D-GA+PDA.COA Only cyanotic newborn who has a thrill: Tricuspid atresia.Only cyanotic newborn who has a thrill: Tricuspid atresia. Ejection click is often heard in : Severe PS, HLHSEjection click is often heard in : Severe PS, HLHS Systolic ejection murmurs in first hours of life: TOF, PS, ASSystolic ejection murmurs in first hours of life: TOF, PS, AS Silent heart characteristic of : D-TGA, Pulmonary atresia.Silent heart characteristic of : D-TGA, Pulmonary atresia. Pulse oximetry& ABG should be obtained from the RIGHT Pulse oximetry& ABG should be obtained from the RIGHT

arm.arm. ECG showing LEFT axis deviation: Tricuspid atresiaECG showing LEFT axis deviation: Tricuspid atresia

Ebstein Anomaly Ebstein Anomaly ? PS? PS PE:PE:

Mild to severe cyanosisMild to severe cyanosis S2 wide splitS2 wide split TR murmurTR murmur HepatomegalyHepatomegaly

CXR: CXR: Balloon shapeBalloon shape Increase CTRIncrease CTR Decrease PVMDecrease PVM

ECG:ECG: RAERAE ± RBBB± RBBB WPW? SVT?WPW? SVT?



TAPVC, supracardiac via vertical vein


TAPVC to coronary sinus

Total Anomalous Pulmonary Total Anomalous Pulmonary Venous Connection Venous Connection ( TAPVC)( TAPVC)

TAPVC, Infradiaphragmatic

Persistent Truncus Persistent Truncus ArteriosusArteriosus::

Increase pulm. blood flowIncrease pulm. blood flow Increase pulm. vascular resistanceIncrease pulm. vascular resistance PE:PE:

RV tapRV tap S2 singleS2 single Ejection clickEjection click ± Diastolic murmur± Diastolic murmur

CXR:CXR: Increase CTRIncrease CTR Increae PVMIncreae PVM

ECG:ECG: RVHRVH

Persistent Truncus Persistent Truncus Arteriosus: Arteriosus:

Medical: Medical: Antifailure therapyAntifailure therapy

Surgical: Surgical: Wait till PVR dropsWait till PVR drops Total correction at 2-3 monthsTotal correction at 2-3 months

Total Anomalous Pulmonary Total Anomalous Pulmonary Venous Connection ( TAPVC)Venous Connection ( TAPVC) ? Site ? Obstructed? Site ? Obstructed PE:PE:

( Large ASD)( Large ASD) CHFCHF Widesplit S2Widesplit S2 ESMESM Diastolic rumble( overflow at TV)Diastolic rumble( overflow at TV)

CXR: CXR: (snow man appearance)(snow man appearance) Increase CTRIncrease CTR Increase PVMIncrease PVM

ECG:ECG: RAERAE RVHRVH

TOF absent pulmonary valveTOF absent pulmonary valve PEPE: To & fro murmur: To & fro murmur

Respiratory symptoms( wheezy Respiratory symptoms( wheezy chest)chest)

CXRCXR:: CTR normalCTR normal Dilated PA segmentsDilated PA segments Hyperinflated lungsHyperinflated lungs

ECGECG: RVH: RVH Management: Total correction with Management: Total correction with

reduction pulmonary angioplasty reduction pulmonary angioplasty

TGA ?? coronaries

Double Outlet RVDouble Outlet RV

Double Outlet RV Double Outlet RV ? position of VSD ?PS? position of VSD ?PS

Sub Sub –– aortic VSD+ no PS aortic VSD+ no PS:: like large VSD like large VSD

CHFCHF Subaortic VSD+PSSubaortic VSD+PS::

like Fallotlike Fallot Sub-pulmonic VSD (Tassig-Bing):Sub-pulmonic VSD (Tassig-Bing):

like TGAlike TGA Doubly commited VSDDoubly commited VSD

CYANOSIS IN CHILDRENCYANOSIS IN CHILDREN

Cyanosis depends not only on O2 saturation Cyanosis depends not only on O2 saturation but also on absolute concentration of reduced but also on absolute concentration of reduced Hb.Hb.

Fetal Hb: Neonates have serious reduction in Fetal Hb: Neonates have serious reduction in pO2 before cyanosis is clinically apparent.pO2 before cyanosis is clinically apparent.

DIFFERENTIAL CYANOSISDIFFERENTIAL CYANOSIS:: Upper body PINK, lower body BLUEUpper body PINK, lower body BLUE PRE-ductal COA ( RV supplying Dao via PDA)PRE-ductal COA ( RV supplying Dao via PDA) Upper body Blue, lower PinkUpper body Blue, lower Pink D-TGA with PDA and COA ( LV supplying Dao D-TGA with PDA and COA ( LV supplying Dao

via PDA).via PDA).

Health & Medicine

VENTRICULAR SEPTAL DEFECT