Four patients with supravalvular aortic stenosis are reported. In three of these patients a certain facial resemblance was striking, and these three patients were also mentally and physically retarded. The cases are very similar to the four cases recently reported in the literature. Supravalvular aortic stenosis in association with mental retardation and a certain facial appearance may constitute a previously unrecognized clinical syndrome.
A systolic thrill and murmur most pronounced in the first right intercostal space and just below the clavicle, with an empty vascular pedicle on the roentgenogram, may be a clue to the clinical diagnosis of supravalvular aortic stenosis.
Supravalvular aortic stenosis is best demonstrated by left ventriculography. A collateral circulation between the coronary arteries and the internal mammary artery has been shown in one patient.
Background Adjunctive therapy for thrombolysis in acute myocardial infarction consists of platelet inhibition with aspirin and thrombin inhibition with heparin. Thrombin inhibition may be improved by the use of hirudin as indicated by experimental and phase II clinical studies. The randomized, double-blind phase III r-Hirudin for Improvement of Thrombolysis study (HIT III) compared a recombinant hirudin (HBW 023) with heparin. The primary end point was the incidence of death or reinfarction.Methods and Results Seven thousand patients with acute myocardial infarction and a duration of symptoms of less than 6 hours were to be randomized to receive intravenous heparin
Background-Percutaneous transluminal coronary angioplasty of the infarct-related artery in stable survivors of acute myocardial infarction is often performed, even in patients without any symptoms or residual ischemia. Despite the lack of randomized studies, it is widely believed that this intervention will improve the clinical outcome of these patients. Methods and Results-Three hundred patients with single vessel disease of the infarct vessel and no or minor angina pectoris in the subacute phase (1 to 6 weeks) after an acute myocardial infarction were randomized to angioplasty (nϭ149) or medical therapy (nϭ151). Primary end point was the survival free of reinfarction, (re)intervention, coronary artery bypass surgery, or readmission for severe angina pectoris at 1 year. The event-free survival at 1 year was 82% in the medical group and 90% in the angioplasty group (Pϭ0.06). This difference was mainly driven by the difference in the need for (re)interventions (20 versus 8, Pϭ0.03). At long-term follow-up (mean, 56 months), survival was 89% and 96% (Pϭ0.02). Survival free of reinfarction, (re)intervention, or coronary artery bypass surgery was 66% and 80% in the medically and interventionally treated patients, respectively (Pϭ0.05). The use of nitrates was significantly lower in the angioplasty group, both at 1 year (38% versus 67%, Pϭ0.001) and at long-term follow-up (36% versus 55%, Pϭ0.006). Conclusions-Percutaneous revascularization of the infarct-related coronary artery in stable patients with single vessel disease improves clinical outcome at long-term follow-up and reduces the use of nitrates. The results of our study should be reproduced in a confirmatory study with a larger sample size before percutaneous coronary intervention in this low-risk patient subgroup, after myocardial infarction can be recommended as routine treatment in clinical practice.
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