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The course of the conduction system in dextrocardia.

This is a serial section examination of the conduction system of five hearts of various types of dextrocardia. In one case of dextroversion the conduction system was found to be normal. In one case of mirror-image dextrocardia the conduction system was normal but in the opposite spatial position. In one heart of mixed (discordant) dextrocardia, the sinoatrial node was in correct morphologic position. There were two atrioventricular nodes - a posterior node close to the coronary sinus and an anterior node in the roof of the morphologic right atrium. They formed two separate bundles which joined in the left ventricle to form a "sling". In two cases of presumptive mirror-image dextrocardia, with a common atrium in situs inversus according to the morphology of the atrial appendages, with common atrioventricular orifice (CAVO), the sinoatrial node was situated in wrong positions. Two atrioventricular nodes and bundles again formed a sling. There are various types of slings in discordant dextrocardia.

Atrioventricular Node

[Coronary angiographic findings in dextrocardia (author's transl)].

This paper reports on four different types of dextrocardia in situs solitus and situs inversus. The topography of the atrial and ventricular cavities, of the great vessels and of the coronary arteries is described in detail. The value of the selective coronary arteriography in differentiation between the types of dextrocardia is discussed.

Adult

Transposition of the great arteries, primum atrial septal defect, azygos continuation of the inferior vena cava, bilateral superior venae cavae and dextrocardia with centrally placed liver.

Successful surgical correction of transposition of the great arteries in a nine-year-old girl with dextrocardia, primum atrial septal defect, bilateral venae cavae, and azygos continuation of inferior vena cava is reported. The patient was cooled on cardiopulmonary bypass and the operation performed under circulatory arrest at 19 degrees C. nasopharyngeal temperature. The problems of diagnosis and management are discussed.

Abnormalities, Multiple

Dextrocardia--value of segmental analysis in its categorisation.

Dextrocardia can be defined as a heart in the right chest with the major axis to the right. This definition, however, conveys no information regarding the chamber arrangements and internal anatomy of the heart. Of 40 patients satisfying this definition in the files of the Brompton Hospital, 33 had angiocardiographic data adequate for complete analysis in terms of connections, relations, and morphology of cardiac segments. They form the subject of this report. There were 16 (48%) patients with situs solitus, 11 (33%) with situs inversus, and six (18%) with situs ambiguus. Of the cases of situs ambiguus, four exhibited laevoisomerism and two dextroisomerism. Of the 16 patients with situs solitus, six had two ventricles and 10 had univentricular hearts; two patients had concordant and three discordant ventriculoarterial connections, seven had double outlet ventricle, and four a single outlet heart. Of the 11 patients with situs inversus, nine had two ventricles and two a univentricular heart of right ventricular type; the arterial connection was concordant in two, discordant in two, double outlet in six, and single outlet in one. Of the six patients with situs ambiguus and laevo or dextroisomerism, four had two ventricles, and two univentricular hearts; the arterial connection was concordant in one, double outlet in three, and single outlet in two. Segmental analysis and the use of basic descriptive terms are essential to define the complex anatomy of such hearts.

Angiocardiography

Peters' anomaly: dominant inheritance in one pedigree and dextrocardia in another.

Two case reports are described to illustrate the unusual occurrence of dominant inheritance of Peters' anomaly and the concomitant occurrence of Peters' anomaly with colobomatous microphthalmos and dextrocardia. Studies of additional families are necessary to determine conclusively the pathogenesis, genetic mode of inheritance, ocular and systemic associated malformations, and proper management of this complex entity.

Abnormalities, Multiple

Successful surgical repair of solitus, dextrocardia, atrioventricular discordance, and double outlet right ventricle with l-malposition of the aorta.

The authors report a rare case of situs solitus of viscera and atria, dextrocardia, atrioventricular discordance, atrial and ventricular septal defects, origin of both great arteries from the morphologic right ventricle, pulmonary valvar and subvalvar stenosis and l-malposition of the aorta which has been correctly diagnosed and succesfully operated on. The repair was accomplished by closing the ventricular septal defect through a ventriculotomy in the systemic venous ventricle (morphologic left), and placing a conduit between this ventricle and the pulmonary artery which had been ligated proximally. The conduit was a fresh segment of homograft valved ascending aorta fixed on a Dacron woven tube. The authors discuss: firstly the reasons for performing this type of repair instead of the intraventricular one recommended by the Mayo Clinic group in the past and secondly the peculiar location of the conducting tissue and the method of avoiding it when approaching the ventricular septal defect.

Aorta

Dextrocardia and ventricular septal defect in the Möbius syndrome.

A case of Möbius syndrome is reported with the additional features of dextrocardia and a single ventricle, neither of which have been previously reported in the literature with this syndrome. Correlation of the period in embryogenesis of cardiac partitioning and rotation with the appearance of the cranial nuclei and limb buds would support the proposal that the fourth to the seventh week of gestation is the critical period of embryogenesis, wherein an intrauterine insult could give rise to the Möbius syndrome.

Abnormalities, Multiple

Successful repair of double-outlet right ventricle, complete atrioventricular canal, and atrioventricular discordance associated with dextrocardia and pulmonary stenosis.

The association of complete atrioventricular canal with other complex congenital cardiac anomalies has represented a significant challenge for the cardiac surgion. The combination of double-outlet right ventricle with complete atrioventricular canal has been particularly difficult to correct, with no surgical successes having been reported until recently. This is a report of the first successful repair of double-outlet right ventricle, complete atrioventricular canal, and atrioventricular discordance (ventricular incersion) associated with common atrium, bilateral superior venae cavae, dextrocardia, and pulmonary stenosis. The specialized conduction tissue was identified by intracardiac electrophysiological mapping, and normal sinus rhythm was preserved. Postoperative cardiac catheterization showed excellent hemodynamics. One year postoperatively, the patient was attending school, playing swimming without difficulty, and taking no cardiac medications. This good result lends encouragement for considering total repair for similar patients with the combination of double-outlet right ventricle, complete atrioventricular canal, and other associated congenital cardiac anomalies.

Angiocardiography

Dextrocardia and situs inversus in Sri Lanka.

Transposition of the viscera is an uncommon condition but no figures are available as to its prevalence in a tropical country. 31 cases of dextrocardia were seen among 110,603 persons examined at two chest clinics in Sri Lanka. A further 11 cases were detected by the reading of films taken at a tuberculosis prevalence survey in the general population in which 27,095 persons were examined after random selection. The incidence at the chest clinics was 1 in 3567, while the prevalence in the general population was 1 in 2500, perhaps the highest rate to be reported from any country. The prevalence was higher in the rural than in the urban population. All cases were right handed, implying that there was no transposition of the centres in the brain. In one case the mother had mumps in the first trimester of pregnancy, thus raising the possibility of an aetiological relationship.

Dextrocardia

Double primary cancers in 2 young sibs, leukemia in another, and dextrocardia in a fourth.

Two brothers developed multiple primary neoplasms in childhood; one had glioblastoma and non-Hodgkin's lymphoma at age 11 years, and the other brain tumor and acute leukemia at six years. A third brother died with myelogenous leukemia at thre years, and a fourth with cyanotic congenital heart disease at 11 weeks. Each child also had at least one hamartomatous lesion of the skin. The clinical features suggested von Recklinghausen's neurofibromatosis or other inherited cancer syndrome, but laboratory studies identified no markers of susceptibility to familial neoplasia.

Acute Disease