How to Image Congenital Left Heart Obstruction in Adults
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- aortic coarctation
- aortic stenosis, subvalvular
- aortic stenosis, supravalvular
- aortic valve
- heart defects, congenital
- magnetic resonance imaging
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.