Congenital Heart Disease

• Patent ductus arteriosus (PDA)
• Transposition of great vessels

Bicuspid aortic valve

• Small atrial septal defect (ASD)
• Small muscular ventricular septal defect (VSD)

the upper body

thoracic aorta blood + ductus arteriosus

Functional DA closure at about 72 hrs and anatomic closure in 4–8 wks

• Localized narrowing of arch usually distal to left subclavian artery in area of DA so not present in fetus
• “3” sign on CXR

bucuspid aortic valve

cerebral berry aneurysms and some patients have Turner's syndrome (XO karyotype) with webbed neck

• Delayed femoral pulses
• Reduced blood pressure in lower extremities
• Findings associated with bicuspid aortic valve
• Pressure difference between upper and lower extremities considered significant if > 20 mm Hg – intervention considered

LV hypertrophy

• Post-Stenotic aortic dilation
• Prominent ascending aorta
• LV enlargement

Most untreated patients die < 50 yo from complications

Tetralogy of Fallot

• RV outflow obstruction – 2° to pulmonic valve or infundibular stenosis
• VSD – usually membranous and large
• RVH – 2° to RV outflow obstruction
• Overriding aorta – across VSD

Degree of right-to-left shunt depends on degree of RV outflow obstruction

• Acyanotic (pink tetralogy) – mild so shunting small
• Cyanotic – more common so shunting large and “blue” blood shunted into systemic circulation

Complications include arrhythmias (usually ventricular, especially if QRS > 0.18 sec when patients are more at risk for sudden death), paradoxical emboli, bacterial endocarditis (prophylaxis a must in these patients), and severe erythrocytosis

• Usually cyanotic
• Possible clubbing
• Prominent ejection murmur at left sternal border
• Soft or absent P₂

• RV hypertrophy
• RA abnormality

• Boot-shaped heart
• Small pulmonary artery
• Normal pulmonary vasculature

• Blalock-Taussig or similar procedure
• Total repair
• Palliative surgery

procedure in infancy to alleviate RV outflow obstruction and improve pulmonary blood flow

includes VSD patch, enlarging RV outflow tract patch, and removing Blalock shunt

may be needed to create a shunt between the systemic and pulmonary circulations – ↑ pulmonary blood flow to ↑ O₂of systemic blood and improve cyanosis

True

• Atrial Septal Defect
• More frequent in women than in men - 3:1

most common and involves fossa ovale and may include mitral valve prolapse (MVP)

involve AV junction, abnormalities in tricuspid and mitral valve leaflets, and high VSD’s

superior septal defect that can go with partially anomolous pulmonary drainage into SVC or RA

Complications include paradoxical emboli as shunt reverses with worsening and one of most common causes of cryptogenic stroke in people < 55 yo, cardiac arrhythmias especially atrial fibrillation (AF), and heart failure due to chronic right heart overload

• True
• Most asymptomatic until adulthood when symptoms may develop due to RV dysfunction causing fatigue, dyspnea, and poor exercise tolerance

• Parasternal RV impulse
• Wildly and fixed split S₂
• Ejection murmur across pulmonic valve

• Right bundle branch block
• Left axis deviation with ostium primum defect

• Large pulmonary artery
• Increased pulmonary markings

• An opening in the septum between the ventricles that allows blood to shunt between the left and right ventricles.
• Common congenital abnormality – 1 in 500 normal births
• About half close spontaneously as the septum grows and hypertrophies during childhood – both membranous and muscular

Smaller defects have a greater left to right gradient and a louder murmur (loud, harsh, holosystolic in 3rd and 4th left sternal interspaces) but may be asymptomatic

pulmonary hypertension due to pulmonary vascular disease from high pressure and volume effects caused by large shunts and reversal of shunt flow as a consequence leading to cyanosis and clubbing

• endocarditis – more common in smaller shunts due to higher velocity of blood damaging tricuspid septal leaflet or RV free wall so must give antibiotic prophylaxis
• CHF – if shunts large and uncorrected

• Hyperdynamic precordium
• Holosystolicleft parasternal mumur, with or without thrill

• LV and RV hypertrophy
• EKG and CXR may not help – depends on size of VSD and sequela

can show size, anatomy, and magnitude of shunt

• About half close spontaneously as the septum grows and hypertrophies during childhood – both membranous and muscular
• Most large ones are surgically corrected early
• Treatment, if needed, is surgical by closing VSD with graft or percutaneously with closure devices

Failure to close results in persistent communication between aorta and PA near origin of left subclavian but most patients asymptomatic if defect small or moderate until middle age

• Loud continuous, rough machinery-like murmur louder in late systole and best heard in 1st and 2nd intercostal spaces at left sternal border (LSB) with thrill
• If large enough, Eisenmenger physiology (↑ PVR and pulmonary HTN) can develop followed by reversal of flow and LV volume overload – will see cyanosis and clubbing of lower extremities with normal upper extremities

can determine chamber size as well as blood flow in PDA

Complications include CHF and endocarditis (prophylaxis a must)

Treatment is surgical with closure of PDA either by ligation or occluder devices – only done if no pulmonary HTN or right to left shunting