Snapshot A 15-year-old boy presents to his cardiologist’s office for weeks of dyspnea on exertion, syncopal episodes, and hemoptysis. He has a past medical history significant for a rather large ventricular septal defect. Concerned for Eisenmenger syndrome, his cardiologist sends him for an echocardiogram and preparations are made for surgical intervention. Introduction Clinical definition a congenital defect resulting in an opening in the interventricular septum of the heart Epidemiology demographics most common congenital cardiac defect present at birth risk factors family history Pathogenesis VSD causes a left-to-right shunting in the heart, causing increased pulmonary blood flow and alterations in pulmonary vasculature over time, with severe defects, this eventually results in pulmonary hypertension and eventual Eisenmenger syndrome the left-to-right shunt will lead to increased oxygen tension in the right ventricle Associated conditions Down syndrome DiGeorge syndrome Turner syndrome Prognosis most remain asymptomatic or self-resolve larger defects can lead to left heart overload and heart failure Presentation Symptoms neonates may be asymptomatic at birth but may develop symptoms over weeks shortness of breath excessive fatigue difficulty breathing or sweating during feeding failure to thrive patients may also remain asymptomatic Physical exam high-pitched and harsh holosystolic murmur best heard at the lower left sternal border decreased during Valsalva and amyl nitrate administration increased during handgrip, squatting, and leg raise small defects may be louder due to higher flow across the smaller opening Imaging Radiography indication often an initial test to rule out other pathologies views chest findings cardiomegaly increased vascular markings Echocardiography indications diagnostic test most specific test findings VSD Studies Making the diagnosis based on clinical presentation and echo Differential Atrial septal defect distinguishing factor if symptomatic, mumur will be a wide and fixed split S2 Patent ductus arteriosus distinguishing factor murmur typically is a continuous machine-like murmur and disappears after 1-2 days after birth Treatment Management approach prophylaxis for infective endocarditis is not necessary mild defects can be left untreated medical management is aimed at complications of VSD Medical palivizumab indication in infants with symptomatic VSD for passive immunization against respiratory syncytial virus (RSV) diuretics indication if patients have heart failure pulmonary vasodilators indication if patients present with Eisenmenger syndrome Operative surgical closure of VSD indications symptomatic patients with large defects or heart failure patients with pulmonary hypertension patients with significant aortic insufficiency Complications Increased risk for subacute infective endocarditis Heart failure Eisenmenger syndrome