ACYANOTIC CONGENITAL HEART LESIONS WITH LEFT-TO-RIGHT SHUNT
ATRIAL SEPTAL DEFECT (ASD)
Most common is ostium secundum ASD, located at mid interatrial septum. Sinus venosus type ASD involves the high atrial septum and may be associated with anomalous pulmonary venous drainage to the right heart. Ostium primum ASDs (e.g., typical of Down syndrome) appear at lower atrial septum, adjacent to atrioventricular (AV) valves.
Usually asymptomatic until third or fourth decades, when exertional dyspnea, fatigue, and palpitations may occur. Onset of symptoms may be associated with development of pulmonary hypertension (see below).
Prominent right ventricular (RV) impulse, wide fixed splitting of S2, systolic murmur from flow across pulmonic valve, diastolic flow rumble across tricuspid valve, prominent jugular venous v wave.
Incomplete RBBB (rSR' in right precordial leads) common. Left axis deviation frequently present with ostium primum defect. Ectopic atrial pacemaker or first degree AV block occur in sinus venosus defects.
Increased pulmonary vascular markings, prominence of right atrium (RA), RV, and main pulmonary artery (LA enlargement not usually present).
RA, RV, and pulmonary artery enlargement; Doppler shows abnormal turbulent transatrial flow. Echo contrast (agitated saline injection into peripheral systemic vein) may visualize transatrial shunt. Transesophageal echo usually diagnostic if transthoracic echo is ambiguous.
TREATMENT Atrial Septal Defect
In the absence of contraindications an ASD with pulmonary-to-systemic flow ratio (PF:SF) >2.0:1.0 should be repaired surgically or by percutaneous transcatheter closure. Surgery is contraindicated with significant pulmonary hypertension and PF:SF <1.2:1.0. Medical management includes antiarrhythmic therapy for associated atrial fibrillation or supraventricular tachycardia (Chap. 132) and standard therapy for symptoms of heart failure (Chap. 133).
VENTRICULAR SEPTAL DEFECT (VSD)
Congenital VSDs may close spontaneously during childhood. Symptoms relate to size of the defect and pulmonary vascular resistance.
CHF may develop in infancy. Adults may be asymptomatic or develop fatigue and reduced exercise tolerance.
Systolic thrill and holosystolic murmur at lower left sternal border, loud P2, S3; diastolic flow murmur across mitral valve.
Normal with small defects. Large shunts result in LA and LV enlargement.
Enlargement of main pulmonary artery, LA, and LV, with increased pulmonary vascular markings.
LA and LV enlargement; defect may be directly visualized. Color Doppler demonstrates flow across the defect.
TREATMENT Ventricular Septal Defect
Fatigue and mild dyspnea are treated with diuretics and afterload reduction (Chap. 133). Surgical closure is indicated if PF:SF >1.5:1 in absence of very high pulmonary vascular resistance.