Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A cyanotic, tachypneic newborn was diagnosed to have double-outlet right ventricle of the Taussig-Bing type. Cardiac failure did not respond to medical treatment or surgical palliation. Postmortem examination revealed two ventricular septal defects (VSDs), one a malalignment VSD in the membranous septum and adjacent tissue and the other in the anterosuperior part of the muscular septum. The D-malposed aortic root emerged mainly from the right ventricle, with aortic-mitral continuity. The larger posterolateral pulmonary root arose almost entirely from the right ventricle, confluent with the muscular VSD, and unrelated to the mitral valve. Its right ventricular aspect was obstructed by hypertrophied infundibulum. This unique malformation of the heart functioned as a double-outlet right ventricle of Taussig-Bing type. In addition, however, the malformation had elements of tetralogy of Fallot because of the malaligned VSD and hypertrophied conal musculature (although pulmonary flow was excessive), and also of complete transposition of the great arteries because of the arrangements of the two VSDs, which favored aortic flow from right ventricle and pulmonary blood flow from the left ventricle. Thus, a single heart presented similarities to three anatomic and functional entities.
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PMID:Unique variant of Taussig-Bing heart: double-outlet right ventricle with double ventricular septal defects and double overriding of great arteries. 186 33

Double outlet right ventricle (DORV) is a rare cardiac malformation especially in adulthood. We report a woman with DORV who survived to the age of 33 years. She had not been seriously limited, before she visited our hospital for cardiac evaluation at the age of 25 years. She was cyanotic, and had clubbing of fingers and toes and facial edema. Holosystolic murmur and diastolic regurgitant murmur were audible along the left sternal border. Chest X-ray showed cardiomegaly and enlarged pulmonary trunks. Electrocardiography showed right axis deviation and biventricular hypertrophy. Laboratory examination revealed polycythemia (Hb: 22.4 g/dl), increased levels of hepatic enzymes due to congestive liver and marked hypoxemia (Pao2: 40 mmHg), Diagnosis of DORV was made with cardioangiography. Surgical repair was not indicated. Thereafter, she experienced recurrent heart failure which progressed gradually. She died probably of ventricular arrhythmia at the age of 33 years. At autopsy, the heart showed dilatation of the right atrium and ventricle, and hypertrophy of the bilateral ventricles. Both the pulmonary artery without stenosis and the aorta originated completely from the right ventricle, which were almost normally positioned with bilateral conus. Large ventricule septal defect was found at the subaortic portion. Patent ductus arteriosus and preductal aortic coarctation co-existed. To our knowledge, few cases with DORV who survived over 30 years have been reported, and they all manifested pulmonary stenosis. The present case is a very rare DORV that survived over 30 years without the complication of pulmonary stenosis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A rare adult case of double-outlet right ventricle without pulmonary stenosis: an autopsy case]. 228 22

Double-outlet right ventricle with mitral atresia is an uncommon anomaly with a few cases reported in the literature. We present 9 cases of this malformation that have been diagnosed by two-dimensional echocardiography (7 cases), cardiac catheterization (9 cases) and anatomical study (2 cases). We classify them into two groups according to whether or not they have associated pulmonary stenosis. The dominant symptoms were cyanosis and hypoxemia in the first group and cardiac insufficiency signs in the other. The left ventricle was hypoplastic in eight and normal in the one with tricuspid overriding. Six of the cases had ventricular septal defect. The great arteries were in normal relationship in 4 cases, with D-malposition in 3 cases and side-by-side in 2 cases. Rashkind atrial septotomy was performed in 5 patients, but was effective only in two. Palliative surgical treatment was performed on six of them. The actual survival rate is 44%.
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PMID:[Double-outlet right ventricle with mitral atresia. Study of 9 cases]. 231 38

Left ventricular outflow tract obstruction is a concern in the long term after Kawashima intraventricular rerouting for Taussig-Bing anomaly. This study assessed the late results and left ventricular outflow tract dimensions after intraventricular rerouting for Taussig-Bing anomaly in 6 children. Age at operation ranged from 8 months to 7 years. The shortest distance from the tricuspid valve to the pulmonary valve ranged from 8 to 18 mm, which was 79%-139% of the normal aortic annulus diameter. All patients survived the operation, but one died of chronic heart failure 4 months after repair. Three patients had no left ventricular outflow tract obstruction 15 to 31 years after repair, and 2 had significant obstruction at 9 and 14 years. One patient underwent successful revision of the intraventricular baffle; the distance between the tricuspid and pulmonary valves was 16 mm (108% of the normal aortic annulus) although it had been 8 mm (79%) at the initial repair. Late results of intraventricular rerouting were considered favorable.
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PMID:Left ventricular outflow tract after Kawashima intraventricular rerouting. 1791 Oct 61

Tetralogy of Fallot is the most common cyanotic heart disease. Its operative mortality and long-term result are quite good in these days. At the late phase after the correction, pulmonary valve regurgitation associated with right side heart failure, aortic valve regurgitation, arrhythmia and sudden death become major adverse outcomes. Double-outlet right ventricle is a cyanotic heart disease with a wide spectrum of morphology and is divided according to the site of ventricular septal defect: subaortic, subpulmonary, doubly committed and remote type. Its operative methods are completely dependent on its morphology, and vary such as intracardiac tunnel repair, Rastelli type repair, arterial switch procedure and Fontan type repair. Left ventricular outflow tract obstruction is one of the most important problems after the correction. Recent operative strategies for the treatment of tricuspid atresia and single ventricle are quite similar and its final goal is the completion of right heart bypass operation using total cavo-pulmonary connection with staging strategy. Pleural effusion, ascites, protein loosing enteropathy and supraventricular arrhythmia are major adverse outcomes after Fontan type repair, while extracardiac total cavopulmonary connection is expected to reduce the incidence of supraventricular arrhythmia.
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PMID:[Cyanotic heart disease. Part 2]. 2191 80

Double-outlet right ventricle (DORV), a clinically important congenital heart disease, occurs in 1-3% of persons with congenital heart disease. It may occur as an isolated cardiac defect, together with other cardiac lesions, or in association with extracardiac anomalies. Other rare cardiac anomalies include an anomalous muscle bundle (AMB) in the right ventricular outflow tract (RVOT) and an accessory tricuspid valve leaflet. We report a very rare case of concomitant DORV, AMB in the RVOT and accessory tricuspid valve leaflet in a 17-year-old male patient. The patient eventually died from severe decompensated heart failure. To the best of our knowledge, such a case has not been previously reported in the literature.
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PMID:Accessory tricuspid valve leaflet and an anomalous muscle bundle in the right ventricular outflow tract in a patient with double-outlet right ventricle: a rare case report. 2464 94