Snapshot A 20-year-old woman with a history of a childhood heart murmur presents for multiple syncopal episodes. She reports feeling constantly fatigued and has shortness of breath with exertion, even with minor physical activity. Physical exam reveals clubbing of the fingers and 2+ pitting edema bilaterally on the lower extremities. An electrocardiogram reveals right heart hypertrophy. An echocardiogram and right heart catheterization are scheduled. Introduction Clinical definition login to view 1 more bullet Etiology Ventricular septal defect Atrial septal defect Patent ductus arteriosus Pathogenesis login to view 6 more bullets Epidemiology Risk factors login to view 1 more bullet Presentation Symptoms login to view 5 more bullets Physical exam login to view 9 more bullets Imaging Radiography login to view 9 more bullets Doppler echocardiography login to view 5 more bullets Studies Labs login to view 7 more bullets Electrocardiogram (ECG) login to view 2 more bullets Right heart catheterization login to view 4 more bullets Making the diagnosis login to view 1 more bullet Differential Interstitial lung disease causing pulmonary hypertension login to view 2 more bullets Treatment Management approach login to view 1 more bullet Conservative login to view 3 more bullets Medical login to view 13 more bullets Operative login to view 3 more bullets Complications Heart failure End-organ damage from hyperviscosity of erythrocytosis Prognosis Age of onset depends on type and severity of defect Can present as early as childhood Death can result from decompensated cor pulmonale