1/99. osteogenesis imperfecta with mitral insufficiency due to ballooning of the mitral valve. A case report.A further case of osteogenesis imperfecta with valvular heart disease is added to the 12 already reported in the literature. The presence of a dilated mitral annulus and a ballooned mitral leaflet in this case together with the findings reported in the literature leave little doubt as to the relationship between the valvular lesion and the underlying connective tissue disorder.- - - - - - - - - - ranking = 1keywords = heart (Clic here for more details about this article) |
2/99. Left-ventricular inflow obstruction due to a dilated coronary sinus mimicking cor triatriatum.Persistence of the left superior vena cava with drainage to the coronary sinus is a common congenital anomaly. We report an infant with such a malformation associated with marked enlargement of the coronary sinus, which produced partial supramitral obstruction and consequently impairment to the left-ventricular inflow. The patient pre-sented with cardiac failure in infancy and features mimicking cor triatriatum. Surgical relief of the supramitral obstruction resulted in immediate reversal of the pulmonary hypertension, with clinical improvement. This rare entity, only once previously reported, is an unusual cause of pulmonary hypertension in infancy.- - - - - - - - - - ranking = 0.50728411672978keywords = cardiac (Clic here for more details about this article) |
3/99. Right iliac vein agenesis, varicosities, and widespread hemangiomas: report of a rare case.We present a probable variant of the Klippel-Trenaunay syndrome with the clinical features of capillary hemangiomas, varicosities, and agenesis of the right iliac venous system, but without limb hypertrophy. To our knowledge, this is the 1st such case reported in the medical literature.- - - - - - - - - - ranking = 0.45899613811539keywords = hypertrophy (Clic here for more details about this article) |
4/99. Aortic root dilation in apparent Lujan-Fryns syndrome.We present a patient and his maternal uncle who have a subaortic ventricular septal defect and aortic root dilation. They both have physical anomalies, characteristic behaviors, and cognitive disabilities that are consistent with the diagnosis of Lujan-Fryns syndrome (LFS). Although there have been 4 cases reported in the literature with heart findings, ventricular septal defect and aortic root dilation have not been previously reported in LFS. Differentiation between LFS and marfan syndrome (MS) is discussed. The pathophysiology of LFS as a connective tissue disorder is also considered.- - - - - - - - - - ranking = 1keywords = heart (Clic here for more details about this article) |
5/99. Coronary ectasia in familial hypercholesterolemia: histopathologic study regarding matrix metalloproteinases.A 39-year-old male heterozygous familial hypercholesterolemia patient with marked ectasia over the entire coronary artery tree had been treated with several kinds of lipid-lowering single or combined drug therapies using clofibrate, compactin, cholestyramine, probucol, and pravastatin, and LDL-apheresis. During the 19-year follow-up, he suffered myocardial infarction three times and some of the ectatic coronary segments became enlarged, others narrowed, and one of them occluded in spite of the treatment. At the age of 58, he died after a fourth cardiac attack and subsequent cardiogenic shock. The autopsy indicated that his three coronary arteries showed diffuse ectatic changes and the largest lumen diameter of the left anterior descending artery was 25 mm, of the circumflex artery 12 mm, and of the right coronary artery 13 mm. The ectatic artery wall was not thick and the major part of the lumen was occupied by organized thrombi. Microscopic examinations showed that the larger the diameter of the lumen, the more severe the structural damage of the intima and tunica media and the larger the number of infiltrated cells, including lymphocytes, macrophages, and plasma cells. Immunoreactivity against matrix metalloproteinase (MMP)-1, and MMP-2 was observed in smooth muscle cells, macrophages, lymphocytes, and endothelial cells of the vasa vasorum or neovasculature. MMP-9 immunoreactivity was also localized in intimal foamy macrophages and round cells (macrophages and lymphocytes) of the media and adventitia. MMP-1 increased with the lumen diameter of the ectatic arteries. The ratio of immunoreactivity against both MMP-2 and MMP-9 to that against tissue inhibitor of metalloproteinase (TIMP)-2 also increased with the lumen diameter, but it no longer increased when the diameter was over 10 mm. These observations suggest that the MMP-TIMP system appears to play a significant role in the development of coronary ectasia- - - - - - - - - - ranking = 0.50728411672978keywords = cardiac (Clic here for more details about this article) |
6/99. Aorticoright atrial tunnel.Two unusual cases of aorticoright atrial tunnel are described. Both patients were referred to our institution for evaluation of a continuous heart murmur best heard along the right upper sternal border. Ascending aortography showed the tunnel taking its origin from the aortic root and entering the right atrium through a tortuous link. Both patients underwent surgical closure. In addition, a review of similar cases in the literature is presented.- - - - - - - - - - ranking = 1keywords = heart (Clic here for more details about this article) |
7/99. Ortner's syndrome in association with mitral valve prolapse.The case of an 83-year-old woman with a history of hypertension, valvular heart disease, atrial fibrillation, and cardiomegaly is presented. The patient also had progressive hoarseness of her voice and intermittent dysphagia. Ear, nose, and throat examination revealed left vocal cord paralysis. echocardiography revealed severely dilated left (LA) and right atria (RA), moderate mitral regurgitation, severe tricuspid regurgitation, and prolapse of both these valves. A review of literature of Ortner's or cardiovocal syndrome is presented. Ortner's syndrome due to mitral valve prolapse has not been reported previously.- - - - - - - - - - ranking = 15.665927613277keywords = cardiomegaly, heart (Clic here for more details about this article) |
8/99. Idiopathic annular dilation: a rare cause of isolated severe tricuspid regurgitation.The management of patients with severe tricuspid regurgitation (TR) requires the clinician to clarify the mechanism of regurgitation. Primary disorders of the tricuspid valve, either congenital or acquired, may be readily identified by echocardiography. Severe TR most often results from left-sided heart disease and secondary pulmonary hypertension. Cardiomyopathic processes may also cause right ventricular failure and functional TR. We report three patients with severe TR due to idiopathic annular dilation. The tricuspid valves were otherwise normal on surgical inspection, and the pulmonary pressures were not significantly elevated. Each patient was aged over 65 years and had chronic atrial fibrillation with preserved left ventricular systolic function. Surgical treatment was associated with marked clinical improvement. Clinicians should recognize this unusual but treatable cause of right-sided congestive heart failure.- - - - - - - - - - ranking = 2keywords = heart (Clic here for more details about this article) |
9/99. Premature closure of the foramen ovale associated with aortic stenosis, left ventricular dilation with thrombus, and early mortality.Premature foramen ovale (FO) closure has been postulated as a cause of hypoplastic left heart syndrome. We suggest that premature FO closure is also associated with left ventricular (LV) dilation and LV thrombus formation, and that FO closure in patients with aortic stenosis and LV dilation is a secondary event that occurs later in gestation than that seen with the hypoplastic left heart.- - - - - - - - - - ranking = 2keywords = heart (Clic here for more details about this article) |
10/99. cardiac tamponade complicating closure of a median sternotomy.A case of intraoperative cardiac tamponade manifested during closure of a median sternotomy is presented. We postulate that cardiac tamponade was caused by acute dilatation of the cardiac chambers as a result of intraoartic balloon pumping in a patient with aortic and mitral regurgitation. It has been shown experimentally that acute rises in ventricular end-diastolic pressure result in increased intrapericardial pressure and that if a certain point on the pericardial pressure-volume curve is reached, cardiac tamponade will occur. sternotomy closure was accomplished easily as soon as the need for intra-aortic balloon pumping diminished.- - - - - - - - - - ranking = 2.0291364669191keywords = cardiac (Clic here for more details about this article) |
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