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From 1975 through 1993, 178 patients underwent surgical management of hypertrophic obstructive cardiomyopathy. Operations included isolated septal myectomy (n = 95), septal myectomy and coronary artery bypass grafting (n = 41), septal myectomy plus a valve procedure (n = 25), septal myectomy, valve procedure, and coronary artery bypass grafting (n = 14), and mitral valve replacement without septal myectomy (n = 3). Recent myectomy results were monitored with transesophageal echocardiography. After initial myectomy, 32 patients (20%) underwent a second pump run for more extensive myectomy only (n = 22), mitral valve replacement only (n = 5), or both (n = 2). In-hospital mortality was 6% (n = 11) and 4% (n = 6) for patients undergoing septal myectomy or septal myectomy plus coronary artery bypass grafting, respectively. Heart block occurred in 17 patients (10%). Left ventricular outflow tract systolic gradients decreased from a mean of 93 mm Hg to 21 mm Hg after myectomy. Late survival was 86% and 70% at 5 and 10 postoperative years, respectively, and 93% and 79% for patients undergoing septal myectomy alone or septal myectomy plus coronary artery bypass grafting, respectively. Only 3 of 131 in-hospital survivors of septal myectomy or septal myectomy plus coronary artery bypass grafting died late cardiac deaths, for a yearly mortality of 0.6%. However, the 5-year late survival of patients undergoing valve operation plus septal myectomy was 51%, and multivariate testing confirmed the adverse influence on late survival (p = 0.008), as well as adverse influences of increasing age (p = 0.016) and return to cardiopulmonary bypass for mitral valve replacement (p = 0.038). At follow-up 136 patients (94%) had New York Heart Association class I or II symptoms. For patients with hypertrophic obstructive cardiomyopathy, septal myectomy alone or in combination with coronary artery bypass grafting produces effective symptom relief, excellent long-term survival, and a low risk of late cardiac death.

Vascular rings have been well documented to cause respiratory and gastrointestinal symptoms in infants and children. Few reports document symptomatic vascular rings in adults, and most have emphasized dysphagia as the predominant symptom. We present the case of a 36-year-old white male with a double aortic arch and progressive dyspnea on exertion. This led us to review previous reports of vascular rings in adults. Criteria for review consisted of anatomically complete vascular rings of the aortic arch in adults age 18 years or older. We identified 25 prior cases for review and included our recent patient. The most common vascular ring anomalies in our review of adults is double aortic arch (n = 12; 46%) followed by right aortic arch with aberrant left subclavian artery and ligamentum arteriosum (n = 8; 30%). Of 24 patients (66%), 16 were symptomatic. Reported symptoms involving the respiratory tract (n = 10 of 24; 42%) included dyspnea on exertion (n = 5), bronchitis (n = 2), recurrent pneumonia, stridor, and unspecified respiratory ailment (n = 1 each). Dysphagia was less common, occurring in eight patients (33%). Previously proposed mechanisms for respiratory tract symptoms include tracheomalacia, static or dynamic compression of the airways, intravascular volume infusion, and aspiration. We also propose exercise-induced dilatation of the aortic arch and age-dependent changes in thoracic compliance as potential mechanisms of dyspnea.

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