Gerbode defect misinterpreted as pulmonary hypertension

AbstractSurvival into adulthood is now routine for patients with congenital heart disease (CHD). Adult cardiology practices increasingly encounter these patients, however, are often challenged by the complexity of the cardiac lesions, prior surgical interventions, and associated conditions. We describe a case of a 64-year-old woman with history of CHD and multiple operations, including surgical aortic valve replacement, who presented for routine evaluation. During transthoracic echocardiogram she was believed to have idiopathic severe pulmonary hypertension (tricuspid regurgitation velocity of 4.6m/s) and a ventricular septal defect (VSD). Consequently, she was referred to a pediatric CHD center. On noninvasive evaluation it became clear that a flow jet through a Gerbode defect, from left ventricle to right atrium, had led to the initial misdiagnosis of severe pulmonary hypertension. The patient was reassured that the VSD was small, restrictive, and that the pulmonary pressures were normal.