Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0264733 (ventricular dilatation)
 2,163 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Severe aortic valve disease is a rare complication of coarctation in adults. Between 1961 and 1990 aortic valve replacement was performed after or combined with the operation of coarctation in 24 adults (4% of entire population operated for coarctation). Bicuspid aortic valves were present in 2/3 of patients. In 10 patients (7/10 with aortic stenosis) coarctation was operated early (mean age 24 years) and aortic valve late (mean age 40 years): in 14 (10/14 with aortic regurgitation, mean age 40 years) aortic valve and coarctation were operated simultaneously (8 patients) or staged within 6 months (6 patients). Additional surgical interventions on the dilated aorta ascendens were performed in 8, mitral valve replacement in 2 and aorto-coronary bypass in 1 patients. Early mortality was 2/24 (8%) and was similar in simultaneously (1/8) and staged (1/14) operated cases: 10 year survival was lower than in an age-matched group of 72 patients with aortic valve disease of similar severity operated during the same period (70% vs 88%, p < 0.01): 6/7 late deaths were cardiac; 5/5 pts with preoperatively severely increased end-diastolic (> 199 ml/m2) and 4/4 with end-systolic (> 90 ml/m2) left ventricular volumes and 2/2 with ejection fraction < 41% died late postoperatively. Severe aortic valve disease arised in 4% of adults with coarctation or after coarctation resection. The results of valve replacement in these patients were less successful due to high late mortality in cases with severe preoperative left ventricular dilatation and/or decrease of ejection fraction. Thus, close surveillance of patients after coarctation surgery, especially those with bicuspid valves, is mandatory for early detection of valvular disease and appropriate timing of valve replacement before left ventricular function begins to deteriorate.
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PMID:[Severe aortic valve diseases and aortic isthmus stenosis in adults. Incidence, clinical aspects and long-term results of surgical treatment]. 944 28

Adult myocardium adapts to changing functional demands by hyper- or hypotrophy while the developing heart reacts by hyper- or hypoplasia. How embryonic myocardial architecture adjusts to experimentally altered loading is not known. We subjected the chick embryonic hearts to mechanically altered loading to study its influence upon ventricular myoarchitecture. Chick embryonic hearts were subjected to conotruncal banding (increased afterload model), or left atrial ligation or clipping, creating a combined model of increased preload in right ventricle and decreased preload in left ventricle. Modifications of myocardial architecture were studied by scanning electron microscopy and histology with morphometry. In the conotruncal banded group, there was a mild to moderate ventricular dilatation, thickening of the compact myocardium and trabeculae, and spiraling of trabecular course in the left ventricle. Right atrioventricular valve morphology was altered from normal muscular flap towards a bicuspid structure. Left atrial ligation or clipping resulted in hypoplasia of the left heart structures with compensatory overdevelopment on the right side. Hypoplastic left ventricle had decreased myocardial volume and showed accelerated trabecular compaction. Increased volume load in the right ventricle was compensated primarily by chamber dilatation with altered trabecular pattern, and by trabecular proliferation and thickening of the compact myocardium at the later stages. A ventricular septal defect was noted in all conotruncal banded, and 25% of left atrial ligated hearts. Increasing pressure load is a main stimulus for embryonic myocardial growth, while increased volume load is compensated primarily by dilatation. Adequate loading is important for normal cardiac morphogenesis and the development of typical myocardial patterns.
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PMID:Remodeling of chick embryonic ventricular myoarchitecture under experimentally changed loading conditions. 997 9

As of 2000, more adults than children are alive with congenital heart disease. Each year, more of these adults with congenital heart disease undergo surgery. Adults with congenital heart disease require lifelong surveillance, follow-up imaging, and clinical decision making by appropriately trained and familiar physicians and extenders. Three common challenges facing adults with congenital heart disease are the neglected patient, weak programmatic infrastructure, and the planning and management of pregnancy in the adult with congenital heart disease. Many challenges must be overcome in order to establish and maintain a comprehensive medical and surgical program for adults with congenital heart disease. Three common and challenging groups of adults with congenital heart disease who may require surgery are adults requiring tricuspid valve surgery for Ebstein's malformation, those requiring reoperation for failing Fontan circulation, and those requiring pulmonary valve replacement (PVR). Surgery for Ebstein's anomaly is now offered to older patients at low risk and with good late outcome. The operation includes tricuspid valve repair or replacement and frequent concomitant procedures such as atrial septal defect closure, arrhythmia surgery (the Maze procedure), and coronary artery bypass grafting. Patients undergoing previous iterations of the Fontan operation are especially prone to arrhythmia and failing circulation. Fontan conversion with arrhythmia surgery and pacemaker therapy was developed by Constantine Mavroudis, Barbara Deal, and Carl Backer to treat these challenging patients, with the first such operation performed in 1994. Fontan conversion involves: (1) conversion of the previously created atriopulmonary connection to a total cavopulmonary connection (TCPC), extracardiac Fontan; (2) arrhythmia surgery, typically with a modified biatrial Maze procedure along with placement of an antitachycardia, dual-chamber pacemaker with steroid-eluting epicardial leads in all patients; and (3) concomitant procedures to treat associated lesions. The need for pulmonary valve replacement is increasing for many adults with congenital heart disease. In the past, chronic pulmonary regurgitation following repair of tetralogy of Fallot was considered benign. Current evidence demonstrates that chronic pulmonary regurgitation causes significant morbidity by producing right ventricular dilatation and dysfunction, exercise intolerance, arrhythmias, and sudden death. Multiple options exist for pulmonary valve replacement including several recent developments such as pulmonary valve replacement with a hand-sewn polytetrafluoroethylene bicuspid valve and percutaneous pulmonary valve replacement. Reoperative cardiac surgery is common in adults with congenital heart disease. Although a history of previous cardiac surgery does not independently confer a significant incremental risk of operative mortality, patients with the greatest number of previous surgeries appear to be a higher risk group. Multi-institutional data about adults with congenital heart disease from The Society of Thoracic Surgeons Congenital Heart Surgery Database can be used to estimate prognosis and council patients and their families. The six manuscripts reviewed in this article have been selected to give a flavor of the state of the art in the domain of caring for adults with congenital heart disease and to provide important information about the long term management of patients undergoing successful pediatric cardiac surgery.
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PMID:Current readings: long-term management of patients undergoing successful pediatric cardiac surgery. 2544 Oct 4