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At least 19 recordsLinked to original sources

Endocardial cushion defects: an anatomical study of 54 specimens.

An anatomical study of 54 specimens with endocardial cushion defect is described. The material was divided into two groups, according to the morphology and attachments of the anterior component of the anteroseptal mitral leaflet. We suggest that there is no divided or undivided anterior common leaflet. There are always two anterior leaflets, a mitral and a tricuspid one, separated by a commissure; depending on the degree of straddling of the mitral component over the interventricular septum, this will protrude into the interventricular septum or into the anterior papillary muscle of the right ventricle. Thus, the commissure separating the mitral and the tricuspid components will lie over the interventricular septum or will be quite separate in the right ventricle. In the latter case the straddling mitral component gives the false appearance of an undivided anterior common leaflet. The septal tricuspid leaflet was found to be underdeveloped or absent but we were not able to find any clefts or divisions in it. We suggest that this leaflet derives solely from the division of the posteroinferior endocardial cushion into two halves, right and left. The left half later becomes the posterior component of the normal anteroseptal mitral leaflet. Associated anamalies are also reviewed.

Ductus Arteriosus, Patent

Evaluation of intracardiac shunts.

This article will review the qualitative and quantitative methods of radionuclide evaluation of intracardiac shunts. Radionuclide angiocardiography of the cardiopulmonary system has proven adequate, with few exceptions, for qualitative detection of left-to-right and right-to-left intracardiac shunts. Numerous authors have reported on the detection of intracardiac shunts using qualitative methods such as rapid sequential visualization of nuclide angiocardiography using the scintillation camera with Polaroid, 35mm or 70mm film. Recent development of videotape storage systems and computers offers a quantitative technique of permanently recording the nuclide angiocardiogram and thus the capability for replay and analysis of the study. Quantitative analysis of the nuclide angiogram in the form of heart chamber dilution curves or pulmonary dilution curves permits 1) determination of the presence or absence of left-to-right, right-to-left, or bi-directional intracardiac shunts, including shunts which might go undetected using the qualitative techniques, 2) shunt location at the atrial or ventricular level, 3) determination of the functional status of a corrective left-to-right shunt, and 4) shunt quantitation. By quantitative analysis of the isotope dilution curves, it is possible to calculate the size of left-to-right or right-to-left intracardiac shunts (Qp/Qs ratio) to within 10% of the values obtained at cardiac catheterization.

Adolescent

[Autosomal dominant hereditary atrial septal defect with heart conduction defects and mitral valve insufficiency].

An autosomal dominant inherited ASD with conduction defects in one family is described. 6 members of 4 generations were fallen ill. In 4 patients the diagnosis was made clinically, in 2 sisters the diagnosis was confirmed by operation. Moreover, the last 2 patients had a mitral insufficiency--probably congenital. The ecg-findings of 4 patients additionally showed conduction defects in form of AV-and bundle branch blocks.

Adult

Down syndrome with congenital heart malformation.

Two hundred fifty-one patients with Down syndrome and congenital heart disease was based on clinical (41%), catheterization (38%), surgical (11%), or autopsy data (10%). The most common lesions were endocardial cushion defect (43%), ventricular septal defect (32%), secundum atrial septal defect (10%), tetralogy of Fallot (6%), and isolated patent ductus arteriosus (4%). Thirty percent had multiple cardiac defects. The most common associated lesions were patent ductus arteriosus (16%) and pulmonic stenosis (9%). Twenty-five percent of the patients uncerwent cardiac surgery. Motality in the 68 patients undergoing surgery was 26% for open heart procedures and 11% for closed heart surgery. In 32% of nonsurgically treated patients with large left-to-right shunts, irreversible pulmonary vascular disease developed. Improved medical and surgical care have decreased morbidity and mortality in these patients in recent years.

Abnormalities, Multiple

The cause of the hemodynamic disturbances in Ebstein's anomaly based on observations at operation.

Ten adult patients with Ebstein's anomaly had open-heart operation for interruption of a Kent bundle. The 4 patients in Group 1 had arrhythmias. One patient had a patent foramen ovale, which was closed. In 3 patients the Kent pathway was identified and interrupted, but in the other only the pathway's anterograde function was interrupted, leaving the patient subject to supraventricular tachycardia (SVT). The 3 patients in Group 2 had mild to moderate cyanosis and dyspnea during SVT as well as mild impairment during strenuous physical activity. Only interruption of their Kent bundles and closure of the patent foramen ovale were done. The 3 patients in Group 3 had dyspnea and cyanosis on exertion, and 1 had overt right heart failure. In each patient, obstruction between the atrialized right ventricle (RV) and functioning RV by the displaced tricuspid valve (TV) was relieved by valve excision and replacement. Interruption of the Kent bundle was successful in 2 of the 3 patients. Operation for Ebstein's anomaly is indicated when any of the following conditions are present: arrhythmias due to Kent bundles that are refractory to medical management; a defect in the atrial septum that must be closed because of a history of cyanosis or paradoxical emboli, or an arrhythmia that must be corrected by right atriotomy; and obstruction between the atrialized and functioning RV or a small functioning RV, both of which can be corrected by TV replacement.

Adolescent