Evaluation Of The Infant or Child with Congenital Heart Disease

8/14/2019 Evaluation Of The Infant or Child with Congenital Heart Disease

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1 | K e n n e t h D . E s p i n o

Evaluation Of The Infant

or Child with

Congenital Heart Disease

Maria Dolores B. Victor, MD., FPCC

HISTORY & PHYSICAL EXAMINATION

COMPREHENSIVE HISTORY

Perinatal Period

Infancy

Older Child

Selected Aspects of History Taking:

Gestational and Natal History

Infections, medications, excessive smoking or alcoholintake during pregnancy

Birth weight

Postnatal (or Past ) History

Weight gain, development, and feeding pattern

Cyanosis, cyanotic spells, and squatting

Tachypnea, dyspnea, puffy eyelids

Frequency of respiratory infection

Exercise intolerance

Selected Aspects of History Taking:

Postnatal HistoryHeart murmurChest painJoint symptomsNeurologic symptomsMedications

Family HistoryHereditary diseasesCHDRheumatic feverSudden unexpected deathDM, HPN, arteriosclerotic heart disease

HISTORY & PHYSICAL EXAMINATION

CLINICAL PRESENTATIONa. Cyanosis

b. Heart Failure

-feeding difficulties

-poor growth

-exercise intolerance

-respiratory distress

c. Chest Pain

d. Extracardiac Congenital Malformation

e. Early Coronary Disease

Physical Examination:

Inspection:

General Appearance and nutritional state

-is the child in distress, well nourished or

undernourished

Chromosomal Abnormalities

-obvious chromosomal abnormalities known to

be associated with certain CHD should benoted


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Color

- cyanotic, pale, or jaundiced

- arterial saturation is usually 85 % or lowerbefore cyanosis is detectable

- Central cyanosis

- peripheral cyanosis

- differential cyanosis manifested by bluelower extremities and pink upper

extremities


Clubbing

-long-standing desaturation usually longerthan 6 months

- appears earliest and most noticeably in

the thumb

Respiratory rate, dyspnea, retraction

-tachypnea, along with tacchycardia is theearliest sign of left-sided heart failure

Physical ExaminationPalpation:

Peripheral pulses

- pulse rate and note any irregularities

- the right and left arm and an arm and aleg should be compared.

Weak leg pulses and strong arm pulsessuggests COA

Bounding pulses are seen PDA, AR

Weak pulses are found in cardiac failure

or circulatory shock

Physical Examination

Chest

-Apical impulse

-Hyperactive precordium

-Substernal thrust: RV enlargement

-Apical heave:lv hypertrophy

-Thrills: palpable equivalent of murmurs

Physical Examination

BP measurement

- The width of cuff should be 40 50% of the

circumference of the arm- Cuffs that are too narrow overestimate the

true BP, cuffs that are too wide underestimate the true pressure

- The pressure in the leg is about 10 mmhghigher than in the arms

AUSCULTATION

Heart Sounds

S2- physiologic split vs widely split

S1 S3

S2 S4


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Ejection Clicks

Murmurs- described as to:

a. Intensity Grades I-VI (systolic)

Grades I-IV (diastolic)

b. Timing- Pansystolic

Diastolic

Continuous

c. Location

d. Radiation

e. Pitch

Friction Rubs

AUSCULTATION CLINICAL PRESENTATION

CYANOSIS

MURMUR

HEART FAILURE

LABORATORY EVALUATION

Hematologic tests

Arterial Blood Gas/ Pulse Oximetry

Chest Roentgenogram

Electrocardiography

Echocardiography

Stress testing

Cardiac Cath and Angiography

LABORATORY EXAMINATION

I. HEMATOLOGIC DATA

Hematocrit anemia

Polycythemia (HCT >65 vol%)

Blood culture

Serum electrolytes

Acute Phase Reactants

ASO titer

Arterial Blood Gases


Il. RADIOLOGIC ASSESSMENT

Chest Roentgenogram

Postero-AnteriorLateral

Cardiac size and shape and location of apex

Pulmonary vascular markings

Associated lung and thoracic anomalies

views


CXR:

1. Boot-shaped heart tetralogy of fallot

2. Egg-shaped heart transposition ofgreat arteries

3. Snowman sign total anomalouspulmonary venous return


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CONGENITAL HEART

DISEASE

EPIDEMIOLOGY & GENETIC BASIS

Prevalence: 0.5-0.8% of live births

2-3/1000 neonates : symptomatic withheart diseases in 1st year of life

Diagnosis established in:

40-50% by 1 week old

50-60% by 1 month old

Relative Frequency of Major CHDs

LESIONS % OF ALL

LESIONS

VSD 25-30

ASD 2, PDA 6-8

Coarctation of Aorta, PS

Tetralogy of Fallot

d-Transposition of Great Arteries

5-7

3-5

Aortic Stenosis 4-7

TAPVR, Tricuspid Atresia,

Single Ventricle, Double Outlet RV

1-2

ETIOLOGYUNKNOWN in most CHDs

Multifactorial (genetic & environmental)

Chromosomal abnormalities

Trisomy 21, 13 & 18

Turner Syndrome

2-4% - associated with knownenvironmental or adverse maternalconditions and teratogenic influences

Etiology:

The cause of most CHD is unknown

Genetic factors- Marfans,Noonans,conotruncal defects (TOF,DORV,TA,PVA)

Chrosomal abnormality- trisomy 18( 90%),trisomy 21 ( 50 % )

Environmental or adverse maternalconditions- DM, SLE, congenital rubella

Drugs- lithium, thalidomide,ethanol

Gender differences

GENETIC COUNSELLING

Probability of cardiac malformationoccurring in subsequent children

A child with CHD ? 2-6% incidence ofCHD for a 2nd pregnancy

2 First-degree relatives with CHD, risk fora subsequent child may reach 20-30%;CHD similar to 1st child


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Fetal circulation

Differs from adult circulation in severalways:

1.In adults, gas exchange occurs in thelungs.

In the fetus, the placenta provides theexchange of gases and nutrients

2.Parallel circulation

3.3 unique features: ductus venosus,foramen ovale, ductus arteriosus

Changes in Circulation after Birth1. Interruption of the umbilical cords results in:

a) An increase in systemic vascular resistance

b) Closure of ductus venosus

2. Lung expansion results in:

a) Reduction of pulmonary vascular resistanceincrease in pulmonary blood flow, fall in PA

pressure

b) Functional closure in foramen ovale-closed by the 3rd mo of life

c) Closure of Patent Ductus Arteriosus

II. Chest Radiograph Findings

Pulmonary vascular markings or

pulmonary blood flow

a. Increased

b. Normal

c. Decreased


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SYSTEMATIC APPROACH

III. Electrocardiogram

a. Right Ventricular

b. Left Ventricular

c. Biventricular

IV. Echocardiography

Hypertrophy

ACYANOTIC CONGENITAL HEARTLESIONS

A. Lesions Resulting in IncreasedVolume Load

B. Lesions Resulting in IncreasedPressure Work

A. LESIONS RESULTING IN INCREASED VOLUME LOAD

1. LEFT-to-RIGHT SHUNTING

ASD

VSD

AVSD

PDA


PATHOPHYSIOLOGY

Communication between the systemic or

pulmonary side of circulation? shunting

of fully oxygenated blood back into the

lungs.


Direction and magnitude of shunt across the

communication depend on:

a. Size of defect

b. Relative systemic and pulmonarypressure and vascular resistance


COMPLICATIONS

a. Heart Failure

b. Infective Endocarditis

c. Eisenmenger Syndrome


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Ventricular Septal Defect

Prevalence: most common form of CHD and accounts for 25% of alldefects.

Pathology:

A VSD may be classified into 4:

1. Perimembranous- most common

2. Outlet defects- accoutn for 5-7%; also called supracristal, conal,

subpulmonary or subarterial

3. Inlet defects- account for 5-8%

4. Trabecular defects-accoutn for 5-20%; may be central, apical,margin

VSD

Clinical manifestations:

History:

1.with small VSD, the patient is asymptomatic

2.Moderate to large VSD, delayed growth and

development, decreased exercise tolerance,repeated pulmonary infections, and CHF arerelatively common

3.With history of cyanosis and decreased level ofactivity may be present

VSDPhysical Examination:

1. Infants with small VSDs are well developed.

2. Infants and children with large VSDs may havepoor weight gain or show signs of CHF

3. A sytolic thrill may be present Precordial andhyperactivity are present with arge VSD.

4. murmur: regurgitant or pansystolic at LLSB

VSDLaboratory Diagnosis

Electrocardiogram

1.With small VSD, the ECG is normal

2.With moderate VSD, LVH and occasionalLAH may seen

3.With large VSD, the ECG shows combinedventricular hypertrophy

VSD

X-ray studies:

Small VSD: normal

Moderate-large VSD:

1. Cardiomegaly of varying degrees andinvolves LV and RV.

2. Pulmonary vascular marking !nereas

VSD

Natural History:

1. Spontaneous closure occurs in 30-50% of pxs withmembranous and muscular VSDs during first 2 years of life.

2. CHF in infants with large VSD, but usually not until 6-8 weeksof age

3. PVOD may begin to develop as early as 6-12 mos of age inpxs with large VSDs.

4. Infundibular stenosis may develop in some infants with largedefects

5. Aortic insufficiency may develop in pxs with supracristal VSD

6. Infective endocarditis rarely occurs


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VSD

Management:

1.Medical

-Treatment of CHF, if it develops, with digoxin and diuretics

-No .Exercise restriction is required in the absence ofpulmonary hypertension

-Maintenance of .Blood oral hygiene and antibiotic

prophylaxis against infective

Endocarditis

VSD

Management:

2. surgical

If growth cannot be improved, the VSDshould be operated on within the first 6months of life

Surgery is NOT indicated for small VSD

Atrial Septal Defect

Prevalence: occurs as an isolated anomaly in 6-8% more common infemales

Pathology:

1. Three types exist- secundum defect, primum defect, sinus venosus

defect. A PFO does not ordinarily produce shunting and is notconsidered an ASD.

2. Ostium secundum is the most common accounting for 50-70 % of allasds . the delefect located at site of fossa ovalis allowing left-to-riflhtshunting of blood from left atrium to right atrium. Anomalouspulmonary venous return is present in 10% of cases.

ASD

3. ostium secundum defects occur in about30% of all ASDs, including those thatoccur as part of AVSD. Isolated ostium

primum ASD occurs in 15% of all ASDs

4. Sinus Venosus Defect-occurs in about10.5 of all ASDs, most commonly loactedat the entry of superior vena cava into RA.


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ASD

Clinical Manifestations:

History: Infants and asymptomatic; subtle failure may be present inyounger children; in older children varying degrees of exerciseintolerance.

PE:

1. Relatively slender body built is typical

2. A widely split and fixed S2

3. A grade 2-3/6 systolic ejection murmur at LUSB secondary to relativePS

*no murmur, only if blood pass to pulmonic valve.

ASD

Laboratory Diagnosis:

A. X-ray studies

1. Cardiomegaly with enlargement of the RA and RV

2.A prominent pulmonary artery segment and increasedpulmonary vascular markings

B. Electrocardiogram

Right axis deviation and mild right ventricular hypertrophyor right bundle branch block

ASDC. Echocardiography

- Diagnostic

- Indirect signs of left to right shunt RVenlargement and RA enlargement, dilatedPA, paradoxical motion ofinterventricutarseptum

D. Cardiac catheterization

ASDNatural History:

1. Spontaneous closure in about 40 % in the first 4 years of life

2. Most children remain active and asymptomatic.

3. If a large defect is untreated.CHF and pulmonary

hypertension develop in their 20s and 30s

4. With or without surgery, atrial arrhythmias may occur

5. Infective endocarditis does not occur in isolated ASDs.

ASD

Treatment:

1. Non-surgical closure

Devices that can be delivered through cardiaccatheterization

2. Surgical closure -indications: QpQs of > 1.5:1

-age: 3 to 4 years of age

-procedure: open heart surgery

-mortality: < 1%

-complications: CVA, arrhythmias


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1

3

2

4

1. Atrial septal defect, primum type.

2. Inlet (type III) ventricular septal defect.

3. Cleft and deformity of tricuspid valve.

4. Cleft and deformity of mitral valve.

Complete Atrio-Ventricular Canal DefectA. LESIONS RESULTING IN INCREASED VOLUME LOAD

2. REGURGITANT LESIONS

Mitral Regurgitation

Ebstein Anomaly

Pulmonary Regurgitation

Aortic Regurgitation

VSD

2 days old

6 months old

7 years old

1

2

Ebstein's Anomaly of the Tricuspid Valve from birth to 7 years of age


3. CARDIOMYOPATHIES

Viral

Metabolic

Genetic Defects

B. LESIONS RESULTING IN INCREASED PRESSURE

WORK

PATHOPHYSIOLOGY:

Obstruction to normal blood flow

1. Obstruction to Ventricular Outflow

Valvular PS

Valvular AS

Coarctation of Aorta

Pulmonary Stenosis

Prevalence:

Pathology

1. PS may be valvular, subvalvular (infundibular )

supravalvular

2. In valvular PS the valve is thickened, with fused orabsent commissures.

3. Dysplastic valves are frequently seen with Noonanssyndrome isolated infundibular PS is rare, usually seen

in TOF

4. Supravalvular PS is occasionally seen with rubellaWilliams syndrome


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PS

History:

1. Children with mild PS are completelyasymptomatic. Exertional dyspnea andeasy fatigability may be present in pts with

moderately severe cases.

2. Newborns with critical PS feed poorly, aretachypneic, and may be cyanotic

PSPhysical Examination:

1. Most patients are acyanotic and well developed.Newborns with critical PS are cyanotic and techypneic.

2. A right ventricular tap and systolic thrill may be present

3. A systolic ejection click is present with valvular PS. TheS2 may split widely, and the P2 may be diminishintensity. An ejection-type systolic murmur is best

audible at ULSB. The louder and longer the murmur, themore severe the stenosis is.

PSECG:

1. Normal in mild cases

2. Right axis deviation and RVH are present Inmoderate. The degree of RVH on ECGcorrelates with severity of PS

3. Right atrial hypertrophy and RVH with strainmay be seen with severe PS

4. Neonates with critical PS may show LVHbecause of a hypoplastic RV

PSStudies:

1. Heart size is usually normal, but the main PAsegment is prominent

2. Pulmonary vascular markings are usuallynormal but may decrease with severe PS.

3. In neonates with critical PS, lung fields areoligemic with varying degree of cardiomegaly.

Echocardiography

PS

Treatment

A. Medical:

1. Restriction of activity not necessary except incases of severe PS

2. Antibiotic prophylaxis against SBE

3. Balloon valvuloplasty is the procedure of choice

4. Newborns with critical PS: prostaglandin infusion;balloon valvuloplasty

PS

B. Surgical:

1. Children with valvular PS in whom balloonvalvuloplasty is unsuccessful.

2. Other types of obstruction (infundibular,

anomalous RV muscle) with significant pressuregradient also require surgery

3. If balloon valvuloplasty is unsuccessful orunavailable, infants with critical PS requiresurgery on an urgent basis


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Aortic Stenosis

Prevalence: accounts for 5%; M > F

Pathology:

1.Stenosis may be valvular, subvalvular. supravalvular

2. Valvular AS may be caused by bicuspid AV, unicuspidAV, or stenosis of tricuspid AV. (Bicuspid AV is the most

common form)

3. Supravalvular AS is an annular constriction above AV;often associated with Williams syndrome

4. Subvalvular, AS may result from simple diaphragm or

from long tunnel-like fibromuscular narrowing of LVoutflow tract

ASHistory:

1. Most children with mild to moderate AS areasymptomatic. Occasionally, exerciseintolerance may be present

2. Exertional chest pain, easy fatigability, orsyncope may occur with severe obstruction

3. Infants with critical AS may develop CHF withinfirst few weeks to months of life.

ASECG: normal in mild cases. LVH with or without strain

pattern may be present in severe cases.

X-ray studies:

1. Heart size is usually normal, but a dilated ascending

aorta or prominent aortic knob may be seen with valvularAS.

2. Significant cardiomegaly does not develop unles CHF

occurs.

3. Newborns with critical AS show generalized

cardiomegaly with pulmonary venous congestion.

AS

Natural history:

1. Chest pain, syncope, and even sudden death may occurwith severe AS

2. Heart failure occurs with severe AS during the newborn

period or later in adult life3. A significant increase in PG frequently occurs with

growth

4. The stenosis may worsen with aging as a result of

calcification

5. SBE occurs in approximately 4%

AS

Management:

Medical:

1. Maintenance of good oral hygiene and antibiotic

prophylaxis2. Children with mod-severe AS should not

perform sustained, strenuous physical activities

3. For critically ill-newboms with CHF, inotropics,diuretics and prostaglandin should be started


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AS

Balloon valvuloplasty- indicated for childrenwith mod-severe AS, and in symptomaticneonates

Surgical treatment- reserved for valves thatare not amenable to balloon therapy

1. Surgical valvotomy

2. Aortic valve replacement

1

1) Valvular Stenosis

with a bicuspid

aortic valve

RV

LA

RA

Severe Aortic Stenosis in an infant

Coarctation of Aorta

Prevalence: occurs in 5-7 % of all lesions; M>F

-May occur at any point; but most common justbelow the origin of left subclavian artery (98%)

- May be a feature of Turner's(30%)

- Associated with bicuspid AV, mitral valveabnormalities, and subaortic stenosis (Shonecomplex)

COA

Asymptomatic children:

1.Normal growth and development

2.Arterial pulses in the leg are either absent orweak and delayed. There is hypertension in thearm

3.A systolic thrill present at parasternal notch,ejection click is frequently audible whichoriginates from bicuspid AV. A short systolicmurmur can be heard at URSB and mid or lower

left sternal border

COA

X-ray studies:

-infants with severe COA, marked cardiomegalyand pulmonary edema are present

-in children, heart size maybe normal or slightlyenlarged, dilatation of ascending aorta, ribnotching in older children

COA

ECG:

-Normal or rightward QRS axis and RVH in most infantswith severe COA

-LVH is seen in older children; maybe normal in 20% ofpatients

Echocardiography:

- Will show the site and extent of COA

- Will demonstrate associated abnormalities like bicuspidAV, MV abnormalities, PDA


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1. Coarctation of the aorta,

distal to the left

subclavian artery

2. Severe aortic stenosis

1

2

Coarctation of the Aorta with severe aortic stenosis in an infantB. LESIONS RESULTING IN INCREASED PRESSURE

WORK

2. Obstruction to Ventricular Inflow

Tricuscupid Stenosis

Mitral StenosisCor Triatriatum

B. LESIONS RESULTING IN INCREASED PRESSURE

WORK

COMPLICATIONS

Heart Failure

Total Circulatory Collapse

Cyanosis

Systemic Hypertension

CYANOTIC CONGENITAL HEART LESIONS

Divided according to Pathophysiology:

PULMONARY BLOOD FLOW (PBF)

a. Decreased PBF

b. Increased PBF

A. DECREASED PBF

TOF

Pulmonary Atresia with Intact Ventricular

Septum

Tricuspid Atresia

TAPVC with Obstruction

A. DECREASED PBF

Included in these LESIONS are:

1. Obstruction to PBF

2. Pathway by which VENOUS BLOODcan shunt from R? L and enter

systemic circulation (PFO, ASD,VSD)


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Tetralogy Of FallotPrevalence: occurs in 5 7 % of all CHD

- Most common cyanotic heart defect seen inchildren beyond infancy

Pathology:

1. Consists of 4 abnormalities: large VSD,right ventricular outflow tract obstruction,

RVH, overriding of the aorta.2. The pulmonary annulus and MPA is often

small.

3. The aortic arch is right-sided in 20 %

TOF

The degree of right ventricular outflowobstruction determines the timing ofonset of symptoms, the severity ofcyanosis, and the degree of RVH

When the obstruction is mild, thepatient may not be visibly cyanotic

( pink TOF )

TOFHistory:1. A heart murmur is usually audible at birth

2. Most patients are symptomatic withcyanosis at birth or shortly thereafter.Dyspnea on exertion,squatting, or hypoxicspells develop later, even in mildly cyanoticinfants

3. Infants with acyanotic TOF maybeasymptomatic or may show signs of CHF

TOFECG: RAD and RVH

CXR: heart size is normal or smaller thannormal, the pulmonary vascularmarkings are decreased

-a concave MPA segment withupturned apex (boot-shaped or coeuren sabot)

-RAE and right aortic arch

TOF

Echo: establishes the diagnosis, extentof aortic override, location and degreeof pulmonary obstruction, size ofproximal branch pulmonary arteries

Angiography: best demonstrates theanatomy of pulmonary arteries

TOFNatural History:

1. Infants with acyanotic TOF graduallybecome cyanotic

2. Polycythemia develops

3. Development of relative iron-deficiencystate

4. Hypoxic spells

5. Growth retardation

6. Brain abscess and cerebrovascularaccidents

7. SBE


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Pulmonary Atresia with Intact

Septum- The pulmonary valve is atretic, ventricular

septum is intact

- No egress of blood from the RV

- RA pressures increases and blood shunts viathe foramen ovale into the LA

- These pts may have sinusoidal channelswithin the RV

- An interatrial communication and PDAare necessary for the pt to survive

PA with Intact Septum

CM:

-severe cyanosis and tachypnea are seenin distressed infants

-S2 is single, heart murmur is usuallyabsent, but soft murmur of PDA or TRmaybe audible

-inadequate interatrial communicationcauses hepatomagaly


ECG: QRS axis 0 90 degrees, RAE,LVH

CXR: heart size maybe normal or large,resulting from RAE. Pulmonary

vascular markings are decreasedEcho: useful in estimating th RV

dimensions, size of TV, sinusoidalchannels which are of prognostic value


Treatment:

-infusion of Prostaglandin

-aortopulmonary shunt

-radiofrequency ablation catheterfollowed by balloon valvuloplasty

-Fontan procedure


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Tricuspid Valve AtresiaPrevalence: accounts for 1 2 % of CHD

Pathology:

1. TV is absent, and the RV is hypoplastic.Associated defects such as ASD, VSD anPDA are necessary for survival

2. TVA is usually classified according to thepresence or absence of PS and TGA. Thegreat arteries are normally related in about70% and transposed in 30%

TVACM:

1. Cyanosis usually evident at birth

2. A systolic thrill is rarely whenassociated with PS

3. S2 is single, a ger 2-3/6 regurgitantsystolic murmur of VSD is noted atLLSB

4. Hepatomegaly may indicate aninadequate interatrial communicationor CHF

TVAECG: LAD , LVHCXR: heart size maybe normal or slightly

increased. Pulmonary vascularitydecreases in most patients, although itmay increase with TVA/TGA

Echo: establishes the diagnosis;absence of tricuspid orifice, small RV,large LV, associated abnormalities

TVATreatment:

- Prostaglandin infusion

- Atrial septostomy

- Aortopulmonary shunt procedure

- Bidirectional Glenn Shunt- Fontan procedure


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A. DECREASED PBF

Degree of cyanosis depend on:

a. Degree of obstruction to PBF:

MILD vs SEVERE

b. When the ductus arteriosus closes

A. DECREASED PBF

COMPLICATIONS

Hypercyanotic, TET, Spells

Shock

Infective Endocarditis

Cerebrovascular accidents

Brain abscess

B. INCREASED PBF

Not associated with obstruction to PBF

Cyanosis caused by either

a. Abnormal ventricular-arterial connectionsb. Total mixing of systemic venous and

pulmonary venous blood within the heart

ABNORMAL VENTRICULO-ARTERIAL

CONNECTIONS

Transposition of Great Arteries (TGA)

Systemic blood? RA? RV? Aorta? Body

Oxygenated blood? pulmonary veins?LA? LV? pulmonary artery? lungs

Survival depends on fetal pathways

(PFO, PDA and VSD)


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TGA-Defects that permit mixing of the twocirculations ( ASD/PFO, VSD, PDA )are necessary for survival.

-Half of patients with tga do not haveassociated defect other than pfo orsmall pda simple tga

-VSD is present in about half of thesepatients

-The clinical presentation varies inrelation to presence or absence of

associated defects

TGA with Intact SeptumCM:1. Cyanosis and tachypnea are always

present

2. Hypoxemia is usually severe

-the condition is a medical emergency; earlydiagnosis and appropriate treatment canavert the development of hypoxemia ofsevere hypoxemia and acidosis, which leadto death

TGA with Intact Septum

PE:

1. Moderate to severe cyanosis, in large,male newborns

2. Infant is tachypneic but withoutretractions unless CHF supervenes

3. S2 is single and loud. Murmurs maybeabsent, or a soft systolic ejectionmurmur

TGA with Intact Septum

Laboratory Studies:

-severe arterial hypoxemia with or withoutacidosis

-hypoglycemia and hypocalcemia

ECG: RAD, RVHCXR: cardiomegaly with increased

pulmonary vascularity

egg-shaped cardiac silhoutte

Echo: diagnostic


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TGA with Intact SeptumTreatment:

1. Prostaglandin to improveoxygenation

2. Maintain normothermia, correction of

acidosis and hypoglycemia3. Atrial septostomy- performed in all

patients in whom delay in operation isnecessary

TGA with Intact SeptumArterial switch operation ( Jatene )

- Surgical treatment of choice

- Usually performed within the first 2weeks of life

- Restores the normal physiologicrelationships of systemic andpulmonary blood flow

- Survival rate is 90 95 %

TOTAL MIXING LESIONS

Common Atrium or Ventricle

Total Abnomalous Pulmonary Venous

Return (TAPVR)

Truncus Arteriosus

Total Anomalous Pulmonary VenousReturn

Prevalence: accounts for 1-2 % of CHD

Pathology:

1. No direct communication exists between

pulmonary veins and the LA2. Defect maybe divided into 4 types:

a. Supracardiac: accounts for 50 %; pulmonaryveins drain into right SVC via the leftvertical vein and innominate vein

TAPVRb. Cardiac: accounts for 20 %; common

pulmonary vein drains into the coronarysinus or directly into the RA

c. Infracardiac: common pulmonary vein

into the portal vein, hepatic vein, orIVC; most commonly associated withobstruction

d. Mixed type


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LV

RV

AO

LA

RPA

VenousConfluence

VenousConfluence

AO

RPA

VerticalVein

VerticalVein

Right

Pulmonary Veins

InnominateVein

2

Total Anomalous Pulmonary Venous Return to the Innominate Vein

TAPVR

CM:A.Without pulmonary venous obstruction

1. CHF with growth retardation and frequentrespiratory infections

2. History of mild cyanosis from birth

3. Precordial bulge with hyperactive RV

4. S2 is widely split and fixed, P2 isaccentuated

5. A gr 2-3/6 systolic ejection murmur atULSB

TAPVRECG: RAE, RVH

CXR:

1. Moderate to marked cardiomegalywith increased pulmonary vascularmarkings

2. snowman sign or figure of eightmaybe seen in supracardiac type

Truncus Arteriosus- Single arterial trunk arises from the

heart and supplies the systemic,pulmonary and coronary circulations

- A VSD is always present and is directlybelow the truncus

- A right aortic arch is present in 30 %

- Di George syndrome present in 33 %

- 4 types


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TA

- Clinical picture varies with age,

depending on level of pulmonary

vascular resistance

- In newborn period: signs of heart failureare usually absent; minimal cyanosis

- In older infants: pulmonary blood flow isincreased and the clinical picture isdominated by heart failure

TA

ECG: CVH is present in 70 %

CXR: cardiomegaly with increasedpulmonary vascularity, right sided archis seen in 30%

Echo: demonstrates a large truncal artery

overriding a large VSD

1

23

Truncus Arteriosus

1. Pulmonary arteries arise from aorta.

2. Truncal valve, occasionally quadracuspid,

stenotic and or insufficient.

Overrides the ventricular septal defect.3. Ventricular septal defect, large

LV

Truncus

TOTAL MIXING LESIONS

Deoxygenated systemic venous blood andoxygenated blood mix completely inheart? oxygen saturation equal inpulmonary artery and aorta

COMPLICATIONS

Cyanosis

Heart failure

OTHER CONGENITAL HEART & VASCULAR

MALFORMATIONS

Anomalies of aortic arch

Anomalous origin of coronary arteries

PULMONARY HYPERTENSION (PH)

1. Primary PH

2. Pulmonary Vascular Disease(Eisenmenger Syndrome)


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*

EISENMENGER SYNDROME

Patients with VSD, blood shunted R? L

2 pulmonary vascular disease

Also seen in patients with ASD, AVSD,

PDA

Present with cyanosis, right heart failure

GENERAL PRINCIPLES OF TREATMENT OF CHD

MILD CHD-

Require NO treatment in most patients,

NO restrictions in physical activity

MODERATE TO SEVERE CHD-

PE modified appropriate to childs physical activitydetermined by EXERCISE TESTING

Prophylaxis vs Bacterial endocarditis

ex. Dental procedure

Instrumentation of urinary tract

Before lower GI manipulation

GENERAL PRINCIPLES OF TREATMENT OF CHD

MONITOR CYANOTIC PATIENTS

Iron deficiency anemia/ polycythemia

Dehydration avoided

High altitudes and sudden change in thermal

environment be avoided

PHLEBOTOMY IN SYMPTOMATIC PATIENT WITH

POLYCYTHEMIA (Hct >65%)

COUNSEL WOMEN ON RISKS ASSOCIATED WITH

CHILDBEARING

PAROXYSMAL HYPERCYANOTIC ATTACKS(Hypoxic, blue or tet spells)

A problem during first 2 years of life

Characterized by:

Hyperpnea and restlessness

Increased cyanosis

Gasping respirations

Syncope/ seizures (severe spells)

Generalized weakness sleep

Occur frequently in the morning on awakening

or after vigorous crying

Disappearance of systolic murmur

Procedures To Be Instituted During Spells:

1. Place infant on the abdomen in a

knee- chest position.2. Administer oxygen

3. Inject Morphine SO4 0.2 mg/k

Congenital Heart Disease

ACYANOTIC CHD

1. Lesions Resulting In Increased

Volume Load

1. ASD

2. VSD

3. AVSD

4. PDA

2. Lesions Resulting In IncreasedPressure Load

A. Obstructions To Ventricular Outflow:

1) Valvular Pulmonic Stenosis

2) Valvular Aortic Stenosis,

3) Coarctation Of The Aorta

B. Obstruction To Ventricular Inflow:

1) Tricuspid Stenosis

2) Mitral Stenosis

3) Cor Triatriatum

CYANOTIC CHD

1. Increased Pulmonary Blood

Flow:

1. Transposition of the great vessels

2. Defects with a common atrium or

ventricle,

3. Total Anomalous Pulmonary Venous

Return

4. Truncus Arteriosus

2. Decresed Pulmonary Blood Flow:

1. Tetralogy of Fallot

2. Tricuspid Atresia

3. Various forms of single ventricle with

Pulmonary Stenosis

THE END!

Documents

Evaluation Of The Infant or Child with Congenital Heart Disease