How to Image Congenital Left Heart Obstruction in Adults.

Abstract

A 28-year-old man with mild dyspnea on exertion presents for evaluation. He has a history of subaortic stenosis (SAS), bicuspid aortic valve (BAV), and coarctation of the aorta (CoA; Figure 1). He underwent repair of his CoA as a neonate with patch aortoplasty. At age 9, he was discovered to have SAS and had surgical resection of a subaortic membrane (Figure 2). His primary care provider referred him for evaluation after noting arterial hypertension and hearing a to-and-fro murmur on examination. Blood pressures suggested a recurrent coarctation with a 40 mm Hg gradient between the right arm and right leg. An echocardiogram demonstrated severe aortic regurgitation (AR; Figure 3) and recurrent SAS with a mean gradient of 31 mm Hg across the left ventricular outflow tract (LVOT). Cardiac magnetic resonance (CMR) confirmed recurrent CoA and estimated the aortic regurgitation fraction to be 30% (Figures 4 and 5). The patient underwent stenting of his CoA (Figure 6). Evaluation 6 months later revealed a mean gradient of 42 mm Hg across the LVOT with unchanged AR. Consequently, the patient underwent surgical aortic valve (AoV) replacement and SAS resection. Figure 1. Aortic coarctation by echocardiography. Echocardiogram in a 2-wk-old neonate with coarctation of the aorta. The 2-dimensional image ( A ) shows a tight stenosis at the isthmus (asterisk) with a posterior shelf (arrow) and mild hypoplasia of the aortic arch. The left common carotid artery (LCC) is dilated. The color Doppler image ( B ) demonstrates flow acceleration at the site of coarctation. Movie in the Data Supplement. AAo indicates ascending aorta; IA, innominate artery; and LSCA, left subclavian artery. Figure 2. Transesophageal echocardiogram of a discrete subaortic membrane. Transesophageal echocardiogram in the long-axis orientation showing a discrete subaortic membrane on the septal surface of the left ventricular (LV) outflow tract, 6 mm from the aortic valve leaflets. A , Two dimensional …