Supravalvar aortic stenosis: clinical and hemodynamic profile, and surgical outcome.

BACKGROUND: Supravalvar aortic stenosis is the rarest of left ventricular outflow obstructions. Data on this rare entity from India are scarce. METHODS AND RESULTS: We retrospectively analyzed the data of 15 patients (13 males, mean age 15.5+/-10.18 years) with a diagnosis of supravalvar aortic stenosis confirmed by cardiac catheterization. Five patients had morphological features of Williams' syndrome. One patient had diffuse while the rest had discrete type of supravalvar aortic stenosis. Five patients did not have any associated lesions. A 9-year-old male had an ascending aortic aneurysm, and 3 patients had associated peripheral pulmonary artery stenosis. One child had a subaortic ventricular septal defect, and another had severe mitral regurgitation. Twelve patients had electrocardiographic evidence of left ventricular hypertrophy. Three patients had mild aortic valvar stenosis while 2 had aortic regurgitation. Six patients had dilated coronary arteries. Two patients with supravalvar aortic gradients of 20 and 40 mmHg were kept on close follow-up. One patient was not willing to undergo surgery while the other is awaiting surgery. Eleven patients underwent surgical correction. Dacron or pericardial patch aortoplasty was done in all the patients. In addition, one patient each underwent pulmonary artery plasty, ventricular septal defect closure, repair of ascending aortic aneurysm, and mitral valve replacement. The patient with diffuse type of supravalvar aortic stenosis underwent augmentation aortoplasty. Two patients died perioperatively. One was lost to follow-up. Two had moderate residual gradients. The rest of the patients were in New York Heart Association functional class I on follow-up of 6.3+/-4.7 years. CONCLUSIONS: Repair of supravalvar aortic stenosis by single sinus aortoplasty is safe and produces good results.

Congenital coronary anomalies of origin and distribution in adults: a coronary arteriographic study.

BACKGROUND: Coronary anomalies should be recognized to avoid problems during coronary intervention and cardiac surgery. METHODS AND RESULTS: We retrospectively reviewed 7400 coronary angiograms to find out the pattern and incidence of coronary anomalies of origin and distribution. We excluded patients with congenital heart diseases, coronary artery fistulae and patients with separate origin of the conus artery. and found 34 cases (0.46%) (22 males), mean age 50.7 +/- 12 years with coronary anomalies. Six cases underwent angiography prior to valve replacement and the rest were part of the evaluation for atherosclerotic coronary artery disease. The most common anomaly was separate origins of the left anterior descending coronary artery and left circumflex coronary artery [n=12 (35.3%)]. The next most common anomalies were origins of the right coronary artery from the left coronary sinus [n=7 (20.6%)] and left circumflex artery from the right sinus [n=6 (20%)]. A single coronary artery was seen in 3 cases (8.8%) which included one case of postmyocardial infarction ventricular septal rupture with triple-vessel disease, and another with two small coronary fistulae. One case each of the following coronary anomalies was found: (i) double right coronary artery, (ii) left anterior descending coronary artery from the right coronary sinus, (iii) all three coronary arteries originating separately from the right sinus, and (iv) left main coronary artery from the right sinus. Of these 34 patients, 11 (32.4%) had significant atherosclerotic disease in the anomalous vessel. CONCLUSIONS: The incidence of primary coronary anomaly seems to be less than that in earlier reports, but the pattern of anomalies appears to be similar.