An individualized approach based on the morphology of the pulmonary arterial supply permits achievement of a high rate of complete intracardiac repairs, basing pulmonary arterial flow on the intrapericardial pulmonary arteries in the great majority of cases, and has a low rate of reoperation and mortality.
Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) is a rare congenital anomaly, one which requires a high index of suspicion in order to be diagnosed promptly and managed accurately. ALCAPA from the right branch pulmonary artery (RPA) is a small subset of ALCAPA in general, with only a few reported cases in the world literature. We report two cases of ALCAPA from the RPA: the first case, an infant, presented in severe cardiac failure requiring preoperative extracorporeal membrane oxygenation (ECMO) for stabilization; and the second, a neonate, presented with severe aortic coarctation (CoA). Both patients underwent successful complete surgical repair using a modified surgical technique, and are doing well in midterm follow-up. We describe our operative technique and review the literature of this rare congenital anomaly.
Loeys-Dietz syndrome is a newly recognized constellation that presents with aortic aneurysm or dissection similar to Marfan's syndrome. We describe successful surgical treatment in a 2-year-old with the syndrome in whom we performed a valve-sparing replacement of the aortic root because of significant dilation of the aortic root and the ascending aorta.
Mitral stenosis (MS) is the most common valvular heart disease in developing countries where rheumatic fever is common. It is also more common in women. The normal mitral orifice is 4–5 square cm in area and the symptoms do not occur until the orifice area falls to below 2.0 square cm and usually below 1.5 square cm. The orifice area decreases by 0.1–0.3 square cm per year. Rarely, the dilatation of the left atrium may cause the symptoms of dysphagia from esophageal compression. Although cardiovascular dysphagia is rare, it should be considered in the case of mitral stenosis. The etiologies of mitral stenosis can be congenital, acquired, or iatrogenic. This case report presents a patient having dysphagia due to an enlarged left atrium.
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