Congenital HD

8/8/2019 Congenital HD

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Congenital Heart Disease

Thoracic Conference

Frank Nami, M.D.


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The Heart


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Congenial Heart Disease Obstructive Congenital Heart Lesions

Congenital Heart Lesions that INCREASE

Pulmonary Arterial Blood Flow

Congenital Heart Lesions that DECREASE

Pulmonary Arterial Blood Flow


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Obstructive Congenital Heart

Lesions Impede the forward flow of blood and

increase ventricular afterloads.

Pulmonary Stenosis

Aortic Stenosis

Coarctation of the Aorta


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Pulmonary Stenosis No symptoms in mild or moderately severe

lesions.

Cyanosis and right-sided heart failure in

patients with severe lesions.

High pitched systolic ejection murmur

maximal in second left interspace.

Ejection click often present.


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Pulmonary Stenosis


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Aortic Stenosis Valvular Aortic Stenosis

Subaortic Stenosis

Supravalvular Aortic Stenosis

Asymmetric Septal Hypertrophy (Idiopathic

Hypertrophic Subaortic Stenosis)


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Valvular Aortic Stenosis Most common type, usually asymptomatic

in children.

May cause severe heart failure in infants.

Prominent left ventricular impulse, narrow

pulse pressure.

Harsh systolic murmur and thrill along left

sternal border, systolic ejection click.


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Valvular Aortic Stenosis Predominantly in males

Thickened, fibrotic, malformed aortic

leaflets.

Fused commissures

Bicuspid aortic valve.


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Valvular Aortic Stenosis


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Coarctation of the Aorta Absent or weak femoral pulses.

Systolic pressure higher in upper

extremities than in lower extremities;

diastolic pressures are similar.

Harsh systolic murmur heard in the back.


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Coarctation of the Aorta Males twice as frequently as females.

98% of all coarctations at segment of aorta

adjacent to ductus arteriosus.

Produced by both an external narrowing and

an intraluminal membrane.

Blood flow to the lower body maintained

through collateral vessels.


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Coarctation of the Aorta


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Congenital Heart Lesions that

INCREASE Pulmonary ArterialBlood Flow

Atrial Septal Defect

Complete Atrioventricular Canal

Ventricular Septal Defect

Patent Ductus Arteriosis

Total Anomalous Pulmonary Venous Connection

Truncus Arteriosus


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Atrial Septal Defect Acyanotic; asymptomatic, or dyspnea on

exertion.

Right ventricular lift.

Fixed, widely split second heart sound.


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Atrial Septal Defect Average life expectancy reduced because of

right ventricular failure, dysrhythmias, and

pulmonary vascular disease.

Surgical closure is recommended.


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Complete Atrioventricular Canal Heart failure common in infancy.

Cardiomegaly, blowing pansystolic

murmur, other variable murmurs.

Deficiencies of both atrial and ventricular

septal cushions and abnormalities of both

mitral and tricuspid valves.


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Complete Atrioventricular Canal Partial and complete AV canal defects

frequently accompany Downs syndrome.

Early surgical correction.

Reconstruction of the AV valves andclosure of the septal defects by a single ordouble patch technique.


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Ventricular Septal Defect Asymptomatic if defect is small.

Heart failure with dyspnea, frequent

respiratory infections, and poor growth if

defect is large.

Pansystolic murmur maximal at the left

sternal border.


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Ventricular Septal Defect Often one component of another more

complex congenital heart lesion.

Heart is enlarged and lung fields are

overcirculated.

Many of the defects will close

spontaneously by age 7-8 years.


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Patent Ductus Arteriosis Murmur usually systolic, sometimes

continuous, machinery

Poor feeding, respiratory distress, and

frequent respiratory infections in infants

with heart failure.

Physical exam and echocardiography.


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Patent Ductus Arteriosis Indomethacin, a prostaglandin E1 inhibitor

may close a PDA.

Surgical treatment after one week, by

ligation, clipping, or division.


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Total Anomalous Pulmonary

Venous Connection Pulmonary veins do not make a direct connection

with the left atrium.

Blood reaches the left atrium only through an

atrial septal defect or patent foramen ovale.

Pulmonary congestion, tachypnea, cardiac failure,

and variable cyanosis.


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Total Anomalous Pulmonary

Venous Connection Diagnosis by cardiac catherization or

echocardiography.

Operative repair in all cases.


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Truncus Arteriosus


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Truncus Arteriosus Corrective operation with a valved conduit

between right ventricle and pulmonary

vessels.

Conduit will need to be changed as child

grows but likelihood to develop pulmonaryvascular disease is greatly reduced.


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Congenital Heart Lesions that

DECREASE Pulmonary ArterialBlood Flow

Tetralogy of Fallot

Transposition of the Great Arteries

Tricuspid Atresia

Ebsteins Anomaly


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Tetralogy of Fallot(1) Pulmonary stenosis

(2) VSD of the membranous portion

(3) Overriding aorta

(4) Right ventricular hypertrophy due to

shunting of blood


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Tetralogy of Fallot Addition of an atrial septal defect falls in

the category of Pentalogy of Fallot.

Hypoxic spells and squatting.

Cyanosis and clubbing.


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Tetralogy of Fallot


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Transposition of the Great

Arteries Aorta from right ventricle, pulmonary artery

from left ventricle.

Cyanosis from birth, hypoxic spells

sometimes present.

Heart failure often present.

Cardiac enlargement and diminished

pulmonary artery segment on x-ray.


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Arteries Anatomic communication must exist

between pulmonary and systemic

circulation, VSD, ASD, or PDA.


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Arteries


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Arteries


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Tricuspid Atresia Tricuspid valve is completely absent in

about 2% of newborns with congenital heart

disease.

Blood flows from right atrium to left atriumthrough foramen ovale.

Early cyanosis.


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Tricuspid Atresia Repair consists of shunt from right atrium to

pulmonary artery or rudimentary right

ventricle (Fontan procedure).


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Ebsteins Anomaly Septal and posterior leaflets of the tricuspid

valve are small and deformed, usually

displaced toward the right ventricular apex.

Most patients have an associated ASD or

patent foramen.

Cyanosis and arrhythmias in infancy arecommon.


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Ebsteins Anomaly Right heart failure in half of patients.

Operative repair with tricuspid valve

replacement.


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Congenital Heart Disease

The end, thank you.

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