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Results for “Endocardial Cushion Defects”

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At least 73 records · Page 4Linked to original sources
PubMed1998

[Successful surgical correction for an incomplete endocardial cushion defect in an elderly patient].

A 72-year-old female who presented with symptoms of severe congestive heart failure, was subsequently diagnosed as having an incomplete endocardial cushion defect. A severe left-to-right shunt and mitral and tricuspid valve regurgitation were noted. Closure of the ostium primum defect, mitral valve replacement, and tricuspid valve annuloplasty were performed simultaneously. The postoperative course was uneventful. A review of literature revealed that this is the oldest such surgical patient described in Japan to date. We believe that surgical correction can be effective even in elderly patients.

Aged↗
PubMed1986

Double orifice mitral valve associated with endocardial cushion defect.

Duplication of the mitral valve is a rare congenital cardiac anomaly. We encountered a case of duplication of the mitral valve associated with a partial form of endocardial cushion defect in a 6-year-old girl. The mitral orifice was separated by a fibrous tissue and each orifice provided papillary muscle and complete subvalvular mechanisms except for the cleft region. The fibrous tissue also provided a subvalvular apparatus. The cleft was repaired without complication. The short axis view of the two-dimensional echocardiogram demonstrated two separate holes in the mitral valve, which constituted the most obvious diagnostic sign prior to surgery.

Child↗
PubMed1979

[Morpho-functional assessment of endocardial cushion defect by single crystal and two-dimensional echocardiography (author's transl)].

24 patients with various forms of endocardial cushion defect, ranging in age from 8 months to 22 years, were studied by single crystal (M-mode) and two-dimensional (2-D) echocardiography. In all of them diagnosis was confirmed by cardiac catheterization and angiocardiography, in 8 of them at surgery and in 3 of them at autopsy. From the morphologic point of view, 2-D echocardiography appeared to integrate M-mode recording in the structural definition of atrioventricular valves and in a better differentiation of complete atrioventricular canal. M-mode scanning right atrium-left ventricle, transverse projection by multiscan and long-axis and apical projections by sector-scanner were the most diagnostic projections. From the functional point of view, in patients with complete right bundle branch block (QRS greater than 120 msec), right ventricular isovolumetric contraction time appeared an useful alternative of sysn three patients, in whom these parameters couldn't be used, a qualitative judgement was possible on the basis of the absence of a wave and the presence of a midsystolic closure of pulmonary valve.

Adolescent↗
PubMed1978

Endocardial cushion defect and significant hypoplasia of the left ventricle: a distinct clinical and pathological entity.

We have identified 12 patients with endocardial cushion defect and marked under-development of the left ventricle. Most of these patients had significant obstructive anomalies of the aortic arch. Pathologically, the left ventricle was very small, but without endocardial fibroelastosis, significant subaortic narrowing was evident, resulting from maladherent atrioventricular valve tissue to the left ventricular outflow tract, and the left ventricular posterior leaflet component of the atrioventricular valve was both grossly deficient and dysplastic. In all 12 a large ostium primum atrial communication was present, but the ventricular contribution to the defect was small. The clinical presentation of severe, intractable congestive heart failure in these neonatal patients would suggest the diagnosis of typical hypoplastic left heart syndrome. Important departures in some of these patients included a leftward, superior and counterclockwise frontal vector on the electrocardiogram and a widely split second sound. Selective biplane angiocardiography will reveal the underdeveloped left ventricle, with an elongated, fixed, 'gooseneck' deformity of the left ventricular outflow tract, severe 'mitral' regurgitation, and a small ascending aorta. Selective right ventriculography will opacify the very much larger right ventricle and pulmonary artery. Finally, these cases broaden the concept of double inlet right ventricle and exaggerated displacement of the atrioventricular canal towards the bulbus cordis.

Angiocardiography↗
PubMed1998

[A case report of incomplete endocardial cushion defect with mitral stenosis in an elderly patient].

The following paper describes a mitral valve replacement (SJM 27 mm), the patch closure (EPTFE) of an ostium primum atrial septal defect and tricuspid annuloplasty (De Vega's method) in a 64-year-old female patient with an incomplete endocardial cushion defect and mitral stenosis. Surgery revealed thickened, mitral valve leaflets and the presence of a cleft, findings similar to those observed in case of rheumatic degeneration. Investigation of patient hemodynamics confirmed a diagnosis of Lutembacher syndrome and a lower with left ventricle volume. After surgery, the volume of left ventricle increased and the patients clinical course was uneventful.

Endocardial Cushion Defects↗
PubMed2001

[A case of central nervous system anomalies (agenesis of corpus callosum, colpocephaly, hydrocephalus, congenital dermal sinus) associated with congenital heart disease(double-outlet right ventricle, complete endocardial cushion defect, atrial septal defect, pulmonary arterial stenosis, patent ductus arteriosus)].

A case of central nervous system anomalies(agenesis of corpus callosum, colpocephaly, hydrocephalus, congenital dermal sinus) associated with congenital heart disease(double-outlet right ventricle, complete endocardial cushion defect, atrial septal defect, pulmonary arterial stenosis, patent ductus arteriosus) is reported. Female patient had been already diagnosed as hydrocephalus during pregnancy and ventricular drainage was performed soon after the delivery. Prostaglandin E 1 was also applied for heart disease, but saturation of O2 decreased to 80% on arterial blood gas analysis. Blalock-Taussig operation and ligation of ductus arteriosus was done 41 days after the delivery and ventricle-peritoneal shunt was also made for the progressive hydrocephalus on the same day. Chromosome analysis showed no abnormality. The genesis of this complicated brain and heart anomaly is discussed from the viewpoint of neural crest cell abnormality.

Abnormalities, Multiple↗
PubMed1977

Intraoperative identification of the conduction system in repair of endocardial cushion defect.

Electrophysiological identification intraoperatively of the His-Purkinje system eliminates the complication of complete heart block consequent upon repair of endocardial cushion defect (ECD). Ten patients, 6 with complete ECD and 4 with incomplete ECD, underwent repair of the defect. None of the patients developed heart block. Slight variations were noted in the location of the His bundle in 9 patients, and a major deviation was found in 1 patient with incomplete ECD. The data support the use of intraoperative recordings as a necessary acconpaniment to the operative repair of ECD.

Bundle of His↗
PubMed2004

[Primary pericardial malignant mesothelioma with incomplete endocardial cushion defect; report of a case].

Primary pericardial malignant mesothelioma is rare tumor and its prognosis is quite poor due to its late presentation and difficulty of complete resection. We describe a rare case of primary pericardial malignant mesothelioma in a 46-year-old female with incomplete endocardial cushion defect (ECD). Incidentally, we found 2 masses in the pericardial spaces after pericardiectomy. We have successfully removed these 2 masses en bloc and performed a total repair of incomplete ECD. Pathologically, these masses were a primary pericardial malignant mesothelioma. This patient is doing well 4 months after surgery without any evidence of recurrence despite reported poor prognosis of "primary pericardial malignant mesothelioma".

Adult↗
PubMed1975

Relationships of the tricuspid valve to the membranous ventricular septum in Down's syndrome without endocardial cushion defect: study of 28 specimens, 14 with a ventricular septal defect.

The commissure between the anterior and medial leaflets of the tricuspid valve is commonly absent in Down's syndrome without endocardial cushion defect (19 of 28 specimens). As a result, aneurysm of the membranous ventricular septum may develop (eight of 14 specimens with ventricular septal defect limited to the membranous ventricular septum) and the potential for left ventricle-to-right atrial communication is increased.

Down Syndrome↗
PubMed1999

Oral-facial-digital syndrome with Y-shaped fourth metacarpals and endocardial cushion defect.

We report on a boy with pseudo-cleft of the upper lip, cleft palate, bifid uvula, lobulated tongue, hypoplasia of the epiglottis, both preaxial and central polydactyly of the hands (Y-shaped fourth metacarpals), bilateral preaxial polydactyly of the feet, postaxial polydactyly of the left foot, hearing impairment, and congenital heart disease with endocardial cushion defect. These clinical manifestations resembled oral-facial-digital syndrome type II (OFDS II, Mohr syndrome) or type VI (Váradi syndrome), associated with an atrioventricular canal. Clinical variability of OFDS II has been observed repeatedly. To the best of our knowledge, this is the first reported case of OFDS II with Y-shaped fourth metacarpals. In addition to Y-shaped fourth metacarpals, Mohr syndrome plus atrioventricular canal and hypoplasia of the epiglottis may represent an additional subgroup of OFDS.

Endocardial Cushion Defects↗
PubMed1981

Nonsurgically-acquired complete atrioventricular block in endocardial cushion defect.

Complete atrioventricular heart block (CAVB) most commonly occurs as a complication of cardiac surgery. We report the development of CAVB in an 8-year-old girl with endocardial cushion defect (ECD) who had not undergone a cardiac operation. Although this is the first report of acquired non-surgical CAVB in a child with ECD, we believe that the development of CAVB in patients with unoperated ECD occurs more commonly than is usually realized. An increased awareness of this possibility will allow expedient diagnosis, which is essential to proper treatment. In most cases, the implantation of a permanent ventricular pacemaker will alleviate congestive heart failure or prevent sudden death.

Journal Article↗
PubMed1976

Intracardiac electrography in endocardial cushion defects.

Conduction of the sino-atrial impulse from the high right atrium to the ventricles was studied by intracardiac electrography in 21 unoperated patients, age 3 months to 11 years, with endocardial cushion defects (ECD). The high right atrium-to-low right atrium conduction time was prolonged in 15 of 18 subjects (mean 57 +/- 20 msec). The low right atrium-to-His bundle conduction time (LRA-H) was normal in 16 of 17 subjects (mean 82 +/- 30 msec), prolonged in one. The His-to-ventricle conduction time (H-V) was normal in 16 of 17 subjects (mean 37 +/- 8 msec), equivocally short in one. Nine patients with ECD, age 3 to 21 years, were studied postoperatively. One had an acquired complete atrioventricular block in the His bundle. Two had prolonged LRA-H and two prolonged LRA-H and two prolonged H-V. The surface ECG failed to identify accurately either prolonged atrioventricular conduction or the site of prolongation.

Adolescent↗