Congenital Heart Disease

Congenital Heart Disease

Prof. Pavlyshyn H.A.Prof. Pavlyshyn H.A.

Differential cyanosis

1. pink upper, blue lower

CoA (Coarctation of the

aorta), IAA (Interrupted aortic

arch), Pulm Htn

2. blue upper, pink lower

Transposition of the great vessels

d-TGA with pulm Htn

dextro-Transposition of the great

arteries

*indicates serious underlying

cardiac or lung disease*

Recognition of Cyanosis

Patent Ductus Arteriosus

HemodynamicsHemodynamics

• As a result of As a result of higher aortic higher aortic pressure, blood pressure, blood shunts L to R shunts L to R through the through the ductus from ductus from Aorta to PA.Aorta to PA.


Clinical Signs & SymptomsClinical Signs & Symptoms

• tachycardiatachycardia• respiratory problems - respiratory problems -

shortness of breathshortness of breath• Poor growthPoor growth• Differential cyanosis - Differential cyanosis -

cyanosis of the lower extremities cyanosis of the lower extremities but not of the upper body.but not of the upper body.


• ClassicClassic continuous continuous machine-like murmurmachine-like murmur

• It begins soon after onset of the 1st sound, It begins soon after onset of the 1st sound, reaches maximal intensity at the end of reaches maximal intensity at the end of systole, and wanes in late diastole. systole, and wanes in late diastole.

• prominent apical impulseprominent apical impulse enlarged heart, enlarged heart,

• Left subclavicular thrillLeft subclavicular thrill• Bounding pulseBounding pulse• WWidened pulse pressureidened pulse pressure


Ventricular Septal Defect

• During systole some of the blood from the During systole some of the blood from the LV leaks into the RVLV leaks into the RV, , passes through the lungs and reenters the LV via the pulmonary veins passes through the lungs and reenters the LV via the pulmonary veins and LA.and LA.

• Such circuitous route of blood causes Such circuitous route of blood causes volume overload on the LVvolume overload on the LV. . • The LV normally has a much higher systolic pressure (~100 mm Hg) The LV normally has a much higher systolic pressure (~100 mm Hg)

than the RV (~85 mm Hg) and through VSD blood leaks into the RV than the RV (~85 mm Hg) and through VSD blood leaks into the RV and elevates RV pressure and volume, causing and elevates RV pressure and volume, causing Pulm HTNPulm HTN. .

• These changes lead to elevated RV & pulmonary pressures & volume These changes lead to elevated RV & pulmonary pressures & volume hypertrophy of the LA & LV.hypertrophy of the LA & LV.



•• Small - moderate VSD, 3-6mm, are usually Small - moderate VSD, 3-6mm, are usually

asymptomatic.asymptomatic.

Small defects located predominantly in the muscular Small defects located predominantly in the muscular septum with slight hemodynamic impairment septum with slight hemodynamic impairment ((Tolochinov-RogeTolochinov-Roge disease) disease)

•• Moderate – large VSD, almost always have Moderate – large VSD, almost always have

symptoms and will require surgical repair.symptoms and will require surgical repair.

Listen at the back for radiation of murmurs

• Pansystolic/holosystolic murmurPansystolic/holosystolic murmur - loud, harsh, blowing - loud, harsh, blowing heard best over the LLSB, frequently is accompanied by thrill heard best over the LLSB, frequently is accompanied by thrill (depending upon the size of the defect) +/-(depending upon the size of the defect) +/-• mmore prominent with small VSD, may be absent with a very large ore prominent with small VSD, may be absent with a very large VSD.VSD.


І

ІІ

ІІІ

AVR

AVL

AVF

V1

V2

V3

V4

V5

V6

ECG: overload of LV and RV

LA, LV or biventricular hypertrophy. RV hypertrophy predominates when pulmonary vascular resistance is high.

Atrial Septal Defect - ASD

• isis a form of CHD that enables blood flow between the left and a form of CHD that enables blood flow between the left and right atria via the interatrial septumright atria via the interatrial septum (it is possible for blood to (it is possible for blood to

travel from the left side to the right side of the hearttravel from the left side to the right side of the heart).). • Seen in 10% of all CHD.Seen in 10% of all CHD.

There are 3 major types:There are 3 major types:• Secundum ASDSecundum ASD

• Primum ASDPrimum ASD – – low in the septumlow in the septum

•• Sinus VenosusSinus Venosus ASDASD

Sinus Sinus VenosusVenosus

ASD with left-to-right shunt

• In the case of a large ASD (>9mm), may result in left-to-right shunt, blood will shunt from the LA to the RA.

• This extra blood may cause a volume overload of both the right atrium and the right ventricle.

• Ultimately the RV must push out more blood than the LV due to the L-to-R shunt. This condition can result in eventually RV-failure (dilatation and decreased systolic function) and Pulm Htn.

Listen carefully

Systolic ejection murmurSystolic ejection murmur – its medium pitched, seldom – its medium pitched, seldom accompanied by a thrill, and best heard at the LSB (left middle and accompanied by a thrill, and best heard at the LSB (left middle and upper sternal border); upper sternal border);

Short, rumbling mid-diastolic murmurShort, rumbling mid-diastolic murmur produced by the produced by the increased volume of blood flow across the tricuspid valve is often increased volume of blood flow across the tricuspid valve is often audible at the LLSB (lower left sternal border) .audible at the LLSB (lower left sternal border) .

Atrial Septal Defect

Diagnosis Diagnosis •• X-ray chest: X-ray chest:

pulmonary pulmonary vascularity is vascularity is increasedincreased

• ECG: ECG: right-axis right-axis deviation;deviation;

• Echo-CG: Echo-CG: RV is RV is enlarged, defect is enlarged, defect is visualized;visualized;

Coarctation of the Aorta• CoarctationCoarctation- is - is

narrowing of the aorta narrowing of the aorta at varying points at varying points anywhere from the anywhere from the transverse arch to the transverse arch to the iliac bifurcation.iliac bifurcation.

• Male: Female ratio 3:1.Male: Female ratio 3:1.

• Accounts for 7 % of all Accounts for 7 % of all CHD.CHD.

Coarctation of the AortaHemodynamicsHemodynamics

• Obstruction of left Obstruction of left ventricular outflow ventricular outflow LV afterload LV afterload increases increases pressure pressure hypertrophy of the hypertrophy of the LV.LV.

Coarctation of the AortaClinical Signs & Clinical Signs &

SymptomsSymptoms• Sings of low cardiac Sings of low cardiac

output, poor peripheral output, poor peripheral perfusion - LEperfusion - LE hypoperfusion, acidosis, hypoperfusion, acidosis, HF and shock.HF and shock.

• Decreased and delayed Decreased and delayed pulses in lower extremities.pulses in lower extremities.

• Systolic ejection murmur Systolic ejection murmur @ LSB.@ LSB.

• Cardiomegaly, rib Cardiomegaly, rib notching on X-raynotching on X-ray..

Pulmonary Stenosis

• Pulmonary StenosisPulmonary Stenosis is is obstruction in the region of obstruction in the region of either the pulmonary valve either the pulmonary valve or the subpulmonary or the subpulmonary ventricular outflow tract.ventricular outflow tract.

• Accounts for 7-10% of all Accounts for 7-10% of all CHD.CHD.

• Most cases are Most cases are isolated lesionsisolated lesions

Pulmonary Stenosis

HemodynamicsHemodynamics

• RV pressure hypertrophy RV pressure hypertrophy RV failure. RV failure.• RV pressures maybe > systemic pressure.RV pressures maybe > systemic pressure.• Post-stenotic dilation of main PA.Post-stenotic dilation of main PA.• W/intact septum & severe stenosis W/intact septum & severe stenosis R-L shunt through FO R-L shunt through FO

cyanosis. cyanosis.• Cyanosis is indicative of Critical PS.Cyanosis is indicative of Critical PS.

Pulmonary StenosisClinical Signs & SymptomsClinical Signs & Symptoms

• Depends on the severity of obstruction.Depends on the severity of obstruction.• Asymptomatic w/ mild PS < Asymptomatic w/ mild PS <

30mmHg.30mmHg.• Mod-severe: 30-60mmHg, > 60mmHgMod-severe: 30-60mmHg, > 60mmHg• Prominent jugular a-wave Prominent jugular a-wave • RV lift, RV heaveRV lift, RV heave• Split 2Split 2ndnd hrt sound hrt sound• Ejection click, followed by systolic Ejection click, followed by systolic

murmur.murmur.• Heart failure & cyanosis not relieved Heart failure & cyanosis not relieved

by inhaled oxygen seen in severe by inhaled oxygen seen in severe cases.cases.

Right sided obstruction

1. Obstruction of RV outflow (Pulmonary stenosis);

2. VSD;

3. Dextroposition of the aorta with override of the ventricular septum;

4. RV hypertrophy

Tetralogy of Fallot

Assessment Findings with Tetralogy of Fallot

Symptoms are variable depending of Symptoms are variable depending of degree of obstructiondegree of obstruction

• Cyanosis – is variable (isn’t Cyanosis – is variable (isn’t present at the birth, occurs later present at the birth, occurs later in the 1in the 1stst yr of life) yr of life)

• Digital clubbing and hyperpnea Digital clubbing and hyperpnea at rest are directly related to the at rest are directly related to the degree of cyanosisdegree of cyanosis

• TachycardiaTachycardia• Mental retardationMental retardation• Retarded growth and Retarded growth and

developmentdevelopment• RV heaveRV heave• Systolic ejection murmur Systolic ejection murmur is heard is heard

along the left sternal borderalong the left sternal border

Assessment Findings with Tetralogy of Fallot

• Paroxymal dyspneaParoxymal dyspnea

• Severe dyspnea on exertionSevere dyspnea on exertion

• Squatting position for the Squatting position for the relief of dyspnea caused relief of dyspnea caused physical effort, physical effort,

• “ “Blue” spells, “tet” spells, Blue” spells, “tet” spells, paroxysmal hypercyanotic paroxysmal hypercyanotic attacks – infant becomes attacks – infant becomes hyperpnea, restless, cyanosis hyperpnea, restless, cyanosis increases, gasping respirations, increases, gasping respirations, syncope syncope

Hypercyanotic Spells/Blue Spells/Tet Spells

Clinical Manifestations

,Most often occurs in morning after feedings ٭defecation, or crying

Acute cyanosis ٭

Hyperpnea ٭

Inconsolable crying ٭

Hypoxia which leads to acidosis ٭

Chest X-RayChest X-Ray

• Decreased Decreased pulmonary vascular pulmonary vascular markingmarking

• “ “Boot-shaped Boot-shaped heart”heart”

Treatment of the Child with TOF

• Decrease cardiac workloadDecrease cardiac workload

• Prevention of intercurrent infectionPrevention of intercurrent infection

• Prevention of hemoconcentrationPrevention of hemoconcentration

• Surgical repair – palliative or corrective Surgical repair – palliative or corrective surgerysurgery

• Pathophysiology– Cyanosis due to failure of

delivery of pulmonary venous blood to the systemic circulation

– Two parallel circulations with no mixing

– Open atrial septum (fossa ovalis) allows some left-to-right shunt, enhanced by a left-to-right ductus arteriosus shunt

– Presence of ventricular septal defect facilitates mixing

d-Transposition of the Great Arteries

Transposition of the Great Arteries• Aorta from right Aorta from right

ventricle, pulmonary ventricle, pulmonary artery from left ventricle.artery from left ventricle.

• Cyanosis from birth, Cyanosis from birth, hypoxic spells hypoxic spells sometimes present.sometimes present.

• Heart failure often Heart failure often present.present.

• Cardiac enlargement and Cardiac enlargement and diminished pulmonary diminished pulmonary artery segment on x-ray.artery segment on x-ray.

Transposition of the Great Arteries

• Anatomic communication Anatomic communication must exist between pulmonary must exist between pulmonary and systemic circulation, and systemic circulation, VSD, ASD, or PDA.VSD, ASD, or PDA.

• Untreated, the vast majority of Untreated, the vast majority of these infants would not these infants would not survive the neonatal period.survive the neonatal period.

Transposition of the Great Arteries Clinical Manifestations

• Cyanosis, tachypnea Cyanosis, tachypnea are most are most often recognized within the 1st hrs often recognized within the 1st hrs or days of life.or days of life.

• Hypoxemia Hypoxemia is usually moderate is usually moderate to severe, depending on the degree to severe, depending on the degree of atrial level shunting and of atrial level shunting and whether the ductus is partially whether the ductus is partially open or totally closed. open or totally closed.

• Physical findings, other than Physical findings, other than cyanosis, may be remarkably cyanosis, may be remarkably nonspecific. nonspecific.

• Murmurs may be absentMurmurs may be absent, or a , or a soft systolic ejection murmur may soft systolic ejection murmur may be noted at the midleft sternal be noted at the midleft sternal border. border.

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Congenital Heart Disease