451/907. Apical hypertrophic cardiomyopathy with spontaneous post partum coronary artery dissection. Primary coronary dissection is usually a catastrophic event with a fatal outcome; less than 20 cases of long term survival have been reported. A 32-year-old woman with apical hypertrophic cardiomyopathy who survived a spontaneous dissection of the left anterior descending coronary artery and acute anterior wall infarction two days postpartum is described. To the authors' knowledge, the simultaneous occurrence of these two rare conditions has never been previously reported. thallium-dipyridamole imaging was performed to estimate the amount of residual viable myocardium in the infarcted area and to evaluate the hemodynamic significance of the residual luminal narrowing. myocardial perfusion imaging can be useful to decide on surgical versus conservative treatment of these patients. ( info) |
452/907. Progressive systemic sclerosis presenting as recurrent ventricular tachycardia and hypertrophic cardiomyopathy--a case report. A 39 year old male who presented with recurrent ventricular tachycardia developed features of progressive systemic sclerosis during the 4-year course of the illness. The condition was diagnosed during life with skin biopsy. Myocardial involvement was detected by 2-D echocardiography and was confirmed at autopsy. ( info) |
453/907. Infantile hypertrophic cardiomyopathy with QT prolongation. A neonate presented with neonatal myocardial dysfunction and long QT interval with subsequent appearance of hypertrophic cardiomyopathy at one week of age. Maternal glucose metabolism was normal. By 15 months of age the hypertrophy had largely resolved but the long QT persisted. The possible relationship between these conditions is discussed. ( info) |
454/907. Cardiac hypertrophy associated with ACTH therapy for childhood seizure disorder. Hypertrophic cardiomyopathy is a newly recognized, potentially fatal complication of ACTH therapy. We report the clinical, echocardiographic, and pathologic findings of an infant who was treated with ACTH for seizure disorder and subsequently developed severe systemic hypertension. echocardiography revealed marked cardiac hypertrophy with disproportionate septal hypertrophy. Decreasing the dose of ACTH reduced the systemic blood pressure, but the cardiac hypertrophy progressed and the child subsequently died. Children who develop hypertension during ACTH therapy should be considered at risk for hypertrophic cardiomyopathy and should undergo routine echocardiographic evaluation. ( info) |
455/907. Foamy myocardial transformation in a child with a disturbed respiratory chain. A female infant presented with signs of general muscle hypotonia and increasing hypertrophy of the heart muscle. There was a constant lactic acidosis worsened after glucose intake. serum lactate levels were increased up to 6.7 mmol/l. serum alanine levels reached maximum concentrations of 2,800 mumol/l. The patient died after sudden cardiac arrest at 22 months of age. histology of the heart showed the pattern of foamy myocardial transformation with accumulation of mitochondria and an increased glycogen content. Biochemical analysis of the skeletal muscle and the heart demonstrated signs of a disturbed respiratory chain. The content of cytochrome aa3 protein was decreased in skeletal muscle and heart mitochondria. It is suggested that respiratory chain defects may be the cause of foamy myocardial transformation of the heart. ( info) |
456/907. Hypertrophic obstructive cardiomyopathy in neonatal beta-cell adenoma of the pancreas. A case of beta-cell adenoma of the pancreas in a neonate with congestive heart failure due to severe hypertrophic cardiomyopathy is reported. In the course of the illness, he developed myocardial infarction, which was probably caused by the limited coronary reserve of the hypertrophied myocardium. ( info) |
457/907. transplantation of liver, heart, and lungs for primary biliary cirrhosis and primary pulmonary hypertension. liver, heart, and lung replacement was performed in a woman with severe pulmonary hypertension, cardiorespiratory failure, and end-stage primary biliary cirrhosis. The main surgical considerations were the staging of the various parts of the operation in relation to cardiopulmonary bypass and performing the recipient procedures as expeditiously as possible to reduce the bypass time to a minimum. The patient was able to leave hospital on the 46th postoperative day on low doses of immunosuppressive agents, with excellent liver and cardiopulmonary function. This early satisfactory outcome shows the feasibility and potential of the procedure: such combined allografting may be a suitable treatment in carefully selected cases of advanced liver and lung disease from other causes. ( info) |
458/907. Anaesthesia for a patient with Friedreich's ataxia and cardiomyopathy. Friedreich's ataxia is an inherited neuromuscular disorder often associated with significant cardiac disease. We report a case of Friedreich's ataxia in a 13-year-old girl with ulcerative colitis and hypertrophic cardiomyopathy who was successfully managed for subtotal colectomy with general anaesthesia and epidural narcotic. Anaesthetic considerations included the maintenance of fluid volume and stable cardiovascular variables in the intra- and postoperative periods. ( info) |
459/907. Cardiac valve disorders: growing significance in the elderly. Accompanying the aging of the population has been a change in the presentation and the manifestations of valvular heart disease. Calcific aortic stenosis is now the most frequent reason for valvular heart surgery and differs greatly from the stenosis produced by rheumatic fever or a congenital bicuspid valve. Mitral insufficiency is found with increasing frequency and is often due to a calcified mitral valve annulus. mitral valve prolapse, once thought to be a disease found in younger patients, is being diagnosed more and more in the elderly and is a significant cause of mitral regurgitation. It is important for the physician caring for the older patient to be aware of the differing presentations, manifestations, and implications of valvular diseases in the elderly. ( info) |
460/907. Clinical sustained uniform ventricular tachycardia in hypertrophic cardiomyopathy: association with left ventricular apical aneurysm. Of 51 patients with hypertrophic cardiomyopathy who had episodes of ventricular tachycardia detected during ambulatory electrocardiographic monitoring only two had clinical sustained uniform ventricular tachycardia that required medical treatment because of worsening symptoms. In both patients the arrhythmia was associated with the uncommon finding of an apical aneurysm with angiographically normal coronary arteries. ( info) |