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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The bicuspid aortic valve is the most frequent congenital cardiac malformation; it may be isolated or associated with other congenital heart disease. The present investigation consists of a study of bicuspid aortic valves in 1022 heart specimens belonging to the anatomical collection of the Institute of Pathological Anatomy of the University of Padua. A bicuspid aortic valve was observed in 95 specimens. It occurred as an isolated congenital cardiac defect in 28 cases, seven of which had spontaneous laceration of the aortic valve (aortic dissection). It was associated with other congenital cardiac malformations in 67 out of the remaining 994 specimens (6.7%), 41 of which (61.2%) showed obstruction of the aortic arch. The frequency of bicuspid aortic valve in specimens with complete transposition of great arteries was only 1%. Bicuspid aortic valve was particularly frequent in association with ventricular septal defect and was significantly more frequent in cases with (51.1%) than in cases without (20.5%) aortic arch obstruction (p < 0.001). There was no significant relationship between the occurrence of bicuspid aortic valves and left ventricular outflow tract obstructions or mitral valve malformations. The morphology of the pulmonary valve was also examined. Concurrence of a bicuspid aortic and pulmonary valve was detected in 11 specimens, five of these had trisomy-18. Our findings cast doubt on the assumption that altered fetal blood flow through the aortic valve may be the main factor producing the bicuspid condition. Indeed, they rather support the hypothesis that most bicuspid aortic valves are expressions of a developmental complex that affects the aortic arch and the wall of the ascending aorta as well as the aorta valve.
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PMID:Bicuspid aortic valves in hearts with other congenital heart disease. 861 73

A Rastelli-type operation was successfully performed on two patients with cyanotic congenital heart disease with pulmonary artesia (PA) and severe stenosis or obstruction of the pulmonary bifurcation, following the improvement of a pulmonary artery index (PA index) after palliative systemic pulmonary shunts. It is a point that stenotic or obstructive pulmonary bifurcation was reconstructed by a patch technique. An extracardiac conduit was used a combination of a bicuspid valved pericardial roll and a prosthetic vessel graft. In a 5-year-old male with an extreme type of tetralogy of Fallot associated with PA and patent ductus arteriosus (PDA), the enlargement of stenotic pulmonary bifurcation was attempted using a brimshaped equine pericardial patch in order to get sufficient suture margin and be safely anastomosed with a conduit. In a 6-year-old female with complete transposition of the great arteries, PA, PDA and right aortic arch (RAA), an obstructive pulmonary bifurcation behind the RAA was enlarged using a brimshaped equine pericardial patch. The attachment of the conduit was shifted to the left pulmonary artery to avoid compression of an extracardiac conduit and pulmonary artery behind RAA. Reconstruction of the pulmonary bifurcation using an equine pericardial patch is useful to anastomose an extracardiac conduit and the stenotic or obstructive pulmonary bifurcation.
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PMID:A patch technique for reconstruction of stenotic pulmonary bifurcation in a Rastelli-type operation after palliations for cyanotic congenital heart disease. A report of two cases. 863 27

The incidence of congenital valvular heart disease has not significantly altered in recent decades. Major factors contributing to altered profiles of acquired valvular heart disease in the past few decades include an increased elderly segment of the population and increasing recognition of nonrheumatic forms of valvular heart disease. Mitral valve prolapse, and similar involvement of other valves, together with senile calcific aortic stenosis have emerged as the most common forms of valvular heart disease in developed countries. Body leanness and hypertension are additional etiological factors for senile calcific aortic stenosis. Severe calcification of a congenital bicuspid aortic valve continues to be an important cause of aortic stenosis in the elderly. Idiopathic degeneration of the aortic and mitral valves, apparently a different condition than mitral valve prolapse, has also become recognized. Despite a recent increase in the incidence of acute rheumatic fever in North America, rheumatic heart disease remains an infrequent cause of valvular heart disease in developed nations. Its incidence has diminished in the Middle East, but it is still frequent in underdeveloped countries. Intravenous drug abuse is increasing in importance as a cause of valvular heart disease in urban centers in the United States. Syphilitic heart disease is very rare.
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PMID:Etiology of valvular heart disease. 873 80

The average life expectancy of patients with congenital heart disease has dramatically improved over the past four decades because of advances in medical and surgical therapy, with patients with complex lesions surviving to adolescence and adulthood. Tetralogy of Fallot, transposition of the great arteries, ventricular septal defects, patent ductus, and bicuspid aortic valves in particular are susceptible to infective endocarditis. Most operated patients are left with some form of residua or sequelae, many of which predispose to infective endocarditis. Surgical palliation, such as systemic-to-pulmonary shunts, and reparative surgery, often requiring prosthetic valve or conduit replacement, are major predisposing conditions. Accordingly, recognition, prevention, and treatment strategies for infective endocarditis assume increasing importance in adolescents and adults with congenital heart disease, operated or not.
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PMID:Infective endocarditis in congenital heart disease. 885 32

Echocardiographic (ECHO) findings in 211 consecutive examinations performed at Eldoret Medical Services from August 1992 to May 1994 were analysed. Most patients were referred by physicians from Eldoret and from the surrounding hospitals for assessment of heart failure or a heart murmur. Thirty three echocardiograms were judged as being normal and 177 abnormal. Of the abnormal ECHOs, congenital heart disease accounted for 38.4% while rheumatic heart disease accounted for 40.7%: Of the 68 patients with congenital defects, 21 (30.1%) had ventricular septal defects; 19 (27.9%) persistent ductus arteriosus; five (7.5%) mitral valve; four (5.9%) tetralogy of Fallot; and three (4.4%) atrial septal defects. In addition, there were two (3.0%) patients each with complete atrio-ventricular septal defects, tricuspid atresia, complete transposition of the great arteries, truncus arteriosus, single ventricle and bicuspid aortic valve. Of the 67 patients with uncomplicated rheumatic heart disease, mitral valve involvement alone was seen in 39 (58.2%), mitral and aortic in 26 (38.8%) and aortic valve alone in two (3.0%). Rheumatic heart disease was complicated by bacterial endocarditis in five patients. Nine patients had pericardial effusion. The possible role of colour-flow ECHO as a feasible, non-invasive and, in the long term, cost-effective means of allowing for early detection and timely institution of secondary prophylaxis while rheumatic heart disease is still asymptomatic is highlighted.
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PMID:Echocardiographic findings in Eldoret: retrospective study. 899 59

A large family with congenital heart disease is described. The pattern of inheritance suggests an autosomal dominant trait with high penetration, although the morphologic phenotype is quite variable, including Ebstein's anomaly, cleft mitral leaflet, bicuspid aortic valve, and atrioventricular canal.
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PMID:A large pedigree with valvuloseptal defects. 938 22

Q fever is caused by the rickettsia Coxiella burnetti, an obligate intracellular bacterium acquired by inhalation of infected dust from subclinically infected animals. Q fever may be acute or chronic; the chronic form mostly presents as endocarditis. Immunocompromised states and underlying heart disease are the most important risk factors. Usually the symptoms of Q fever endocarditis are nonspecific and diagnosis is often established very late. New criteria for diagnosis include a single blood culture positive for Coxiella burnetti, positive Q fever serology and characteristic echocardiographic studies. We describe a 49-year-old man with bicuspid aortic valve admitted with fever, weight loss and a new heart murmur. The diagnosis of Q fever endocarditis was established by positive Q fever serology, and an echocardiogram showing vegetations and valvular dysfunction. This case suggests that Q fever endocarditis should be considered in patients with "sterile" endocarditis.
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PMID:[Q fever endocarditis and bicuspid aortic valve]. 941 57

Twenty-four adults (average age 28 years) patients with congenital heart disease were examined; infective endocarditis was diagnosed in all of them by Duke criteria and was confirmed in 18 by surgery and/or pathology. Patients were divided into 2 groups. Group A was made up of 13 patients with left ventricular outflow obstruction, including ten with bicuspid aorta. Group B was made up of 11 patients with shunts (PDA or VSD), either isolated or associated with other abnormalities. The principal alterations associated with the infectious processes were trauma to the endocardium or vascular endothelium from accelerated turbulent flow (jet lesion) and valvular deformities. The principally transesophageal echocardiographic recordings showed infective vegetations on the four cardiac valves, mural endocarditis in both ventricles and right atrium, perivalvular abscesses and fistulae. The echocardiographic information aided in selecting the type of treatment in this group of patients with high intrahospital mortality (25%).
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PMID:[Transthoracic and transesophageal echocardiography in the study of adults with congenital cardiopathy and infectious endocarditis]. 981 Mar 66

Congenital valvular heart disease in childhood is often complex. Conventional echocardiography provides two-dimensional views which require mental reconstruction for three-dimensional assessment. This problem may be solved by the use of three-dimensional (3D) echocardiography which obtains images of valves comparable to those seen at surgery. This was confirmed by 4 cases of congenital valvular heart disease studied by 3D echocardiography: stenotic bicuspid aortic valve disease, parachute mitral valve and two cases of mitral regurgitation in patients with atrioventricular canal. The 3D views of the aortic valve showed the commissural opening after percutaneous balloon valvuloplasty of the bicuspid valve. The surface of the aortic orifice and the surface of the two mitral leaflets were measured from 3D reconstructions. The longitudinal 3D view analysed the extension of the single obstructive mitral papillary muscle of the parachute malformation. The 3D ventricular views allowed assessment of the extension of the cleft and the surface of the three mitral leaflets of the 2 cases of atrioventricular canal. In one of these cases, the results of surgical valvuloplasty were evaluated after 3D reconstruction of the valve. 3D echocardiography is not only a diagnostic tool for congenital heart disease but also a very useful complementary investigation for accurate evaluation of congenital valvular lesions to optimise possible valve repair.
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PMID:[A new anatomical approach to congenital valve diseases by three-dimensional echocardiography]. 1036 74

Echo and Doppler echocardiographic procedures have gained special importance in the diagnostics of congenital diseases in adults. These procedures permit detailed visualization of the pathomorphology of the heart as well as reliable evaluation of the hemodynamic changes. There are differentiated indications for the various procedures, such as transthoracic and transesophageal echocardiography, Doppler and color-Doppler echocardiography, contrast echocardiography and 3-dimensional echocardiography. This article discusses the opposition of the various echo and Doppler echocardiographic procedures with respect to the diagnostics of the most frequent non-operated congenital diseases in adults. The pathomorphology of the various congenital diseases will be summarized and then the important echocardiographic criteria presented which are decisive for the diagnostic procedure. In simple congenital malformation of cardiac valves, such as bicuspid aortic valve (Figure 1: aortic ring abscess), pulmonary valve stenosis (Figure 2), Ebstein's anomaly (Figure 3) or malformations of the mitral valve (Figure 4: cleft in the anterior mitral cusp), the diagnosis can often be made using transthoracic echo and Doppler echocardiography, and the severity of the defect determined. However, the sonographic conditions, especially in adults, are frequently too limited to permit recognition of detailed smaller changes, so that transesophageal examination is required to finally confirm the diagnosis in these patients. In the diagnostics of diseases of the left ventricular outflow tract and the thoracic aorta, such as subvalvular aortic valve stenosis (Figure 5), the sinus of Valsalva aneurysm or the coarctation of the aorta (Figure 6), the left ventricular outflow tract can be evaluated morphologically from a transthoracic procedure and the accelerations of flow can be recorded by continuous wave Doppler. If there is no sclerosis of the fibrous membrane, these can often not be depicted by transthoracic procedures, so that a supplementary transesophageal examination is meaningful. This is required in any case for diseases of the descending thoracic aorta. In the case of congenital lesions, such as atrial septal defects (Figure 7: anomalous pulmonary venous return, Figure 8: 3-dimensional visualization of an atrial septal defect, Figure 9: sinus venosus defect), ventricular septal defect or a patent ductus arteriosus Botalli (Figure 10), color-Doppler and contrast echocardiography have become especially important. Transesophageal examination is also indicated for these congenital diseases for direct depiction of the defect as well as for precise evaluation of the shunt. Moreover, in atrial septal defects, it has been shown that a 3-dimensional echocardiography provides additional advantage with respect to spatial relationship of the defect to the other cardiac structures, as well as presenting dynamic changes during a heart cycle. Extensive knowledge of complex congenital heart disease, such as tetralogy of Fallot (Figure 11), complete transposition of the great arteries, congenitally corrected transposition of the great arteries (Figure 12), the double-outlet right ventricle, truncus arteriosus communis, the cor triatriatum, tricuspid atresia (Figure 13) or the univentricular heart (Figure 14) usually requires performance of a transthoracic echo- and Doppler echocardiographic examination to assess the pathomorphological changes and to examine hemodynamics. In the majority of patients, supplementary transesophageal echocardiography and an echo contrast examination are important. Initial examinations using 3-dimensional echocardiography are very promising in this connection and with respect to the exact spatial presentation of pathoanatomical structures.
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PMID:[Echocardiographic evaluation in unoperated congenital heart disease in adults]. 1044 7


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