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At least 19 recordsLinked to original sources
PubMed1978

Right atrial ultrastructure in congenital heart disease. I. Comparison of ventricular septal defect and endocardial cushion defect.

Ultrastructural studies were performed on portions of the operatively resected right atrium from six patients with a ventricular septal defect and six patients with an endocardial cushion defect. The six patients with a ventricular septal defect had normal right atrial mean pressure and no evidence of right atrial volume overload. Ultrastructurally, the atrial muscle cells in these patients appeared normal and measured 6 to 12 mu in diameter. The six patients with an endocardial cushion defect had elevated right atrial mean pressure and evidence of right atrial volume overload. Ultrastructurally, the atrial muscle cells in these patients were generally larger than 12 mu in diameter. The cells were irregular and had multiple and occasionally widened intercalated discs. In addition, there were degenerative changes in two patients with markedly increased atrial pressure. These changes included extensive loss of contractile elements, aggregation of small irregular mitochondria and proliferation of tubules of the sarcoplasmic reticulum. The structural changes suggest that hypertrophy of the right atrium may be secondary to volume overload of the atrium, whereas degenerative changes may be secondary to increased right atrial pressure.

Basement Membrane↗
PubMed1975

Electrocardiogram of secumdum type atrial septal defect simulating endocardial cushion defect.

Three groups of patients are identified in the series of secumdum atrial septal defect with left axis deviation in an analysis of 910 patients of secundum atrial septal defect with or without other accompanying anomalies: 1) 12 patients with isolated ostium secumdum defect; 2) 5 patients with associated prolapse of posterior leaflet of the mitral valve; 3) 4 patients with associated hypertrophic cardiomyopathy and/or single (left) coronary artery. The findings of these 21 patients with proved secumdum atrial septal defect are analysed. The combination of such anomalies should be considered in the differential diagnosis of primum endocardial cushion defect from the electrocardiographic viewpoints.

Adolescent↗
PubMed1975

Echocardiographic diagnosis of endocardial cushion defects.

Sixteen patients with endocardial cushion defects were studied by echocardiography and ultrasonocardiotomography. Three characteristic findings, common to both complete and partial forms, were observed. They were the ostium primum defect of the interatrial septum, the left ventricular outflow narrowing and the atrioventricular valve abnormalities which are the anatomic bases of endocardial cushion defects. Several additional findings were observed in patients with complete form. Most strikingly, the anterior leaflet echo locating in the left ventricle had the direct connection to that in the right ventricle without the interposition of the interventricular septum by echocardiogram. Ultrasonocardiotomogram, on the other hand, demonstrated beautifully the common anterior leaflet and the high inteventricular septal defect. There were, in addition, the absence of the paradoxical motion of the interventricular septum and the diffuculty in detecting the interatrial septum. The intact interatrial septum continuous with the anterior mitral leaflet was demonstrated in patients with the interventricularseptal defect of atrioventricular canal type, in which there were the typical abnormalities in the atrioventricular valves and the left ventricular outflow narrowing as well. This study indicates that ultrasonic examination may provide a direct, yet noninvasive method in the diagnosis of various types of endocardial cushion defects.

Adolescent↗
PubMed1976

Diagnosis of endocardial cushion defect with cross-sectional and M-mode scanning echocardiography. Differentiation from secundum atrial septal defect.

Twelve cases of endocardial cushion defect were studied before and after operation with ultrasono-cardiotomography (tomography) cross-sectional echocardiography, two-dimensional echocardiography, B-scan echocardiography) and M-mode scan along a horizontal section of the heart. For comparison, 20 healthy subjects, 18 cases of mitral valvular disease, 4 cases of congestive cardiomyopathy, 1 case of partial anomalous pulmonary venous drainage, and 25 cases of atrial septal defect of secundum type were also examined with the same technique. In cases without cardiac malformation, the echo of the anterior mitral valve was usually continuous medially with that of the interatrial septum in the horizontal plane at the level of the membranous septum. This feature was clearly recorded in all cases with right heart enlargement. In ostium secundum atrial septal defect the echo of the anterior mitral valve continued into that of the interatrial septum. An echo interruption was shown, indicating the defect itself to be in the middle part of the interatrial septum. In all the cases of endocardial cushion defect which we examined discontinuity was shown between the echo of the anterior mitral valve and that of the interatrial septum. This discontinuity was interpreted as indicating the defect itself. The mitral valve ring echo was close to the basal end of that of the interventricular septum, possibly reflecting an abnormal attachment of the mitral valve. In all cases, after operation, the echo of the artificial interatrial septum was recorded, continuous with that of the anterior mitral valve. The features of the echocardiographic sweep from the anterior mitral valve to the interatrial septum were thus different in the three groups. These echocardiographic differences are thought to correspond to the anatomical differences between the normal, atrial septal defect of secundum type, and endocardial cushion defect, and are essential features differentiating them from each other.

Adolescent↗
PubMed1991

Surgical options in subaortic stenosis associated with endocardial cushion defects.

Over a 15-year period, 12 patients with endocardial cushion defects undergoing correction had subaortic stenosis requiring operative intervention. Ages ranged from 4 months to 17 years (mean, 7 +/- 6 years) and subaortic gradients from 15 to 100 mm Hg (mean, 60 +/- 25 mm Hg). Subaortic stenosis was due to discrete fibromuscular tissues in 7 patients, mitral valve malattachment in 3, and tunnel outflow in 2. In 2, the subaortic stenosis was clinically significant at the time of endocardial cushion defects repair, whereas in 10 it was noted 2 to 14 years postoperatively (mean, 6.3 +/- 5 years). Surgical relief of subaortic stenosis was accomplished by resection of muscle tissues in 7, apicoaortic conduit insertion in 2, modified Konno procedure (aortic valve preserved) in 2, and lifting of malattached mitral valve from the outflow in 1. There was no early death and one late death (infected conduit). Severe mitral insufficiency developed in the patient who had the mitral valve lifted and necessitated valve replacement. Postoperative echocardiographic gradient in 9 patients ranged from 0 to 36 mm Hg (mean, 10.5 +/- 14 mm Hg). Clinically significant subaortic stenosis has not developed in any patient in 15 years of follow-up (mean, 5 +/- 4 years). We conclude that in subaortic stenosis associated with endocardial cushion defects, resection is effective for discrete obstruction, whereas a modified Konno procedure is preferable for obstruction due to tunnel outflow or mitral valve malattachment.

Aortic Valve Stenosis↗
PubMed1983

Unusual mitral valve abnormalities complicating surgical repair of endocardial cushion defects.

A review of 155 cases of surgically repaired endocardial cushion defects revealed 16 patients (10%) with additional unusual mitral valve abnormalities that complicated the surgical procedure. Eight patients had accessory mitral valve tissue that connected the anterior and posterior leaflets to form a double-orifice valve (Group I). In four (50%), the lesion was associated with intermediate atrioventricular canal and small left ventricle; all four died following repair. In the other four, it was associated with ostium primum defect; all survived and are well. A single papillary muscle in the left ventricle was present in six patients (Group II). Two had intermediate atrioventricular canal and both died postoperatively. The other four had complete endocardial cushion defect and three are well following the operation. Perforation of the valve leaflets was present in two patients with ostium primum (Group III). Both patients are well postoperatively. Modification of the surgical technique is required to effect satisfactory repair. The bridge connecting the posterior and anterior leaflets of the mitral valve should be left undisturbed. Otherwise, severe regurgitation may result. In patients with single papillary muscle and complete atrioventricular canal, repair may be accomplished by borrowing from the tricuspid portion of the anterior leaflet, rotating that part posteriorly, and partially closing the cleft. Small perforations of the mitral leaflet do not require closure and do not result in regurgitation. Echocardiographic and angiographic delineation of these abnormalities and thorough intraoperative exploration are important in avoiding pitfalls at the time of repair.

Child, Preschool↗
PubMed1983

Epicardial and endocardial activation in patients with endocardial cushion defect.

Epicardial and left ventricular endocardial activation were assessed in 5 patients (aged 4 months to 9.5 years) with endocardial cushion defect (ECD) during surgical repair. Epicardial activation was recorded from 40 to 47 sites over the epicardium; left ventricular endocardial activation was measured at 3 sites immediately after institution of cardiopulmonary bypass. Compared with the reported activation sequence in normal hearts, the pattern of excitation in hearts of patients with ECD was abnormal; epicardial excitation began at the left ventricular diaphragmatic surface and spread laterally and anteriorly over the anterobasal left ventricle. It then merged with right ventricular wavefronts ending along the right ventricular anterior atrioventricular groove and outflow tract. Left ventricular endocardial activation also occurred earliest in the diaphragmatic segment of the left ventricle with later wavefronts recorded laterally and anteriorly. This study demonstrates, for the first time in human subjects, correlation between left ventricular epicardial and endocardial activation in patients with ECD. The data indicate that earliest endocardial and epicardial activation occurs at the left ventricular diaphragmatic segments of the heart, and are consistent with the known posterior and inferior displacement of the specialized atrioventricular conduction system in patients with ECD.

Child↗
PubMed1979

Echocardiographic recognition of atrioventricular valve stenosis associated with endocardial cushion defect: pathologic and surgical correlates.

The association of either mitral or tricuspid stenosis with endocardial cushion defects has been well recognized pathologically but is infrequenctly diagnosed clinically. M mode echocardiographic features such as markedly disproportionate ventricular size, abnormal mitral or tricuspid diastolic echoes or failure to define adequately an atrioventricular (A-V) valve are strongly suggestive of associated stenosis of that valve. In this study mitral stenosis in association with endocardial cushion defect was correctly diagnosed in four of five patients. In the fifth patient (the first seen) the condition was easily diagnosed retrospectively. Tricuspid stenosis was similarly diagnosed prospectively in three of four patients. Significant stenosis was not overlooked or misdiagnosed in any patient with an endocardial cushion defect. Significant stenosis of an A-V valve in association with endocardial cushion defect carries a high mortality. It is important to recognize the combined lesion clinically because infants with it are not suitable for total surgical correction. Palliative surgical procedures with aggressive medical therapy probably offer the infant the best chance of surviving until ventricular growth may render corrective surgery feasible.

Aorta↗
PubMed1979

Endocardial cushion defect associated with cor triatriatum sinistrum or supravalve mitral ring.

Clinical and angiographic or autopsy data, or both, on three children with a subdivided left atrium (cor triatriatum) and an associated endocardial cushion defect are reviewed. (One child had ostium primum defect, and two had complete atrioventricular [A-V] canal.) A fourth patient demonstrates the difficulties in differentiating subdivided left atrium from supravalve mitral stenosis in the presence of an endocardial cushion defect. The clinical findings are greatly influenced by the endocardial cushion defect. A pressure gradient between the pulmonary wedge and (left or right) ventricular end-diastolic pressures in patients with an endocardial cushion defect indicates pulmonary venous obstruction and should alert one to the possibility of these combined lesions. The exact diagnosis is made with injections of angiographic contrast medium into the proximal and distal left atrial chambers, to documented the respective relations of the pulmonary veins, left atrial appendage and A-V valves to these atrial chambers. All three patients with an endocardial cushion defect and a subdivided left atrium had an associated patent ductus arteriosus. The common association of subdivided left atrium with intracardiac, pulmonary venous and aortic anomalies is again demonstrated.

Cardiac Catheterization↗
PubMed1988

Skull erosion and bony changes in a child with endocardial cushion defect.

A 9-year-old boy with an endocardial cushion defect who had skull erosion as well as widening of the diploe is described. This was accompanied by thickening of the tables, "hair-on-end" striations in the skull, and cortical thinning and medullary cavity expansion in the femora. The skull erosion is attributed to venous distension and increased blood volume. The finding of skull erosion in cyanotic congenital heart disease has not been reported before.

Bone Diseases↗
PubMed1994

A case report of surgical treatment of a dog with atrioventricular septal defect (incomplete form of endocardial cushion defect).

A 3-month-old female collie was diagnosed as having atrioventricular septal defect with ostium primum atrial septal defect (PASD). The diagnosis was made by echocardiographic observation of the PASD and goose-neck deformity on left ventriculogram. The PASD was treated surgically with a patch graft under cross-circulation cardiopulmonary bypass (CC). The PASD was identified above the ventricular septum after right atriotomy. The patch graft was sutured along the fibrous tissue of the tricuspid annulus on the ventricular side of the PASD to avoid injuring the conduction system. After the operation, cardiac function and renal output were well preserved, but the dog died 33 hr later. At postmortem examination, a mitral cleft was identified.

Animals↗
PubMed1989

[The diagnostic value of M-mode, 2D, and Doppler echocardiography in incomplete endocardial cushion defect].

The diagnostic value of ultrasonography in cases with incomplete endocardial cushion defect which has been verified by subsequent operation was studied. By the M-mode ultrasound examination an enlarged right ventricle, a narrowed outflow tract of left ventricular and an abnormal pattern of mitral value were shown. By the aid of 2-DE its diagnostic accuracy in this disease was almost 100%. Characteristically the ostium primum (atrial septal) defect and anterior mitral cleft were demonstrated. And by pulsed Doppler the signs of mitral regurgitation and the spectral features of atrial shunt could be found. Therefore, it is proposed that ultrasonographic examination could readily take the place of traumatic angiocardiography in the diagnosis of this disease.

Adolescent↗
PubMed1992

[A case of incomplete endocardial cushion defect with mirror-image dextrocardia, IVC defect and azygos connection].

A case of incomplete endocardial cushion defect associated with mirror-image dextrocardia, IVC defect and azygos connection is reported. Intracardiac defect was corrected under moderate hypothermia with cardiopulmonary bypass. Three venous drainage cannulas were necessary to be indwelled into SVC, hepatic vein and right common iliac vein to maintain adequate venous drainage for extra-corporeal circulation. Thus, anomaly of venous system, which is commonly associated with mirror-image dextrocardia, must be recognized correctly and prepared before intracardiac correction.

Azygos Vein↗
PubMed1976

The angiocardiographic appearance of the endocardial cushion defect in selected transposition and malposition complexes.

Although the angiocardiographic features of the endocardial cushion defect (ECD) have been well described in the patient with two normally related ventricles (D-ventricular loop in situs solitus) and normally related great arteries, little attention has been focused on the angiocardiographic appearance of the ECD in patients with complex cardiovascular disease. Because of recent surgical advances in the treatment of single ventricle and double outlet right ventricle complexes, it has become increasingly important to document the status of the atrioventricular valves in these patients. The angiocardiographic features of the ECD are therefore described in two patients, one with D-loop, single left ventricle and outlet chamber, D-transposition of the great arteries, bilateral conus and pulmonary stenosis and the other with dextrocardia, situs inversus, double outlet right ventricle, common atrioventricular canal and pulmonary stenosis. In both patients, the left ventricular outflow tract, despite aortic-mitral discontinuity, is characterized by elongation, scalloping and serration, and the classic appearance of the "gooseneck" deformity is observed on left ventricular angiography. It thus appears that aortic-mitral continuity and two well-formed, normally related ventricles and great arteries are not invariable necessary to the manifestation of the "gooseneck" deformity in the patient with complex cardiovascular disease and associated endocardial cushion defect.

Abnormalities, Multiple↗