[Successful surgical correction of endocardial cushion defect in the aged].
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We have experienced a surgical correction of a 63-year-old female patient with ECD, who had suffered from severe congestive heart failure. Massive left to right shunt and severe mitral and tricuspid regurgitation were noted. The correction consisted of mitral valve replacement, patch closure of the ostium primum defect and annuloplasty of tricuspid valve. Postoperative course was uneventful. Surgical correction should be recommended even in old patient.
A 56-year-old man was evaluated for exertional dyspnoea. Chest X-ray showed mild cardiomegaly and a dilated main pulmonary artery. On echocardiogram he was found to have ostium primum atrial septal defect with moderate tricuspid insufficiency. Cardiac catheterization revealed an oximetry step-up of 14% at low right atrium with angiogram demonstrating a cleft in the mitral valve, an elongated left ventricular outflow tract and ventricular septal defect closed by a septal aneurysm. Coronary angiogram revealed ostial compression of the left main coronary artery with the rest of the coronary artery anatomy being normal.
We have experienced surgical correction of incomplete ECD in a 66-year-old female patient. This is the oldest case in Japanese literatures. Preoperative examination showed mild mitral regurgitation, interatrial shunt (L-R 52%) and moderate pulmonary hypertension (Pp/Ps 0.48). The correction consisted of valvuloplasty of mitral valve and patch closure of ostium primum. The postoperative course was uneventful. The postoperative catheterization showed improved cardiac function.
We have experienced a surgical correction of ECD of a 64-year-old female patient. The preoperative left ventriculogram showed typical goose-neck deformity of the left ventricle and mild mitral regurgitation. The correction consisted of repair of the mitral cleft, and patch closure of the ostium primum. Postoperative course was uneventful. The operation of ECD should be recommended even in an older patient.
A case of DOMV associated with incomplete ECD in a 17-year old girl is presented. Preoperatively, the mitral valve was suspected to have two orifice divided by the bridging tissue with sector echocardiography. DOMV is very rare congenital malformation, and 25 cases of DOMV are reported in Japan. Two of them were diagnosed as DOMV preoperatively with sector echocardiography. Sector echocardiography is useful to diagnose DOMV and to know the shape of it preoperatively.
Successful repair of a 8-month-old girl with polysplenia was reported. The cardiovascular anomalies were TAPVC (II b), incomplete ECD, interruption of inferior vena cava with hemiazygos continuation, bilateral superior vena cava, and left superior vena cava draining into the coronary sinus. Cardiopulmonary bypass was established with ascending aortic perfusion and caval cannulation. A left superior vena cava was directly cannulated after establishing partial bypass. In this case the left pulmonary vein drained into the right atrium near the orifice of the coronary sinus, so the atrial septal flap was made and sutured between the orifice of the left pulmonary vein and the coronary sinus in order to avoid late pulmonary vein obstruction. Then, atrium was separated by an intraatrial baffle which was sutured to the atrial septal flap. Recently, it becomes possible to surgical repair of polysplenia syndrome according to the advancements of the diagnostic methods, cardiopulmonary bypass, and the technique of the open heart surgery.
BACKGROUND: Mitral regurgitation (MR) represents the principal indication for reoperation in patients after repair of atrioventricular septal defects (AVSD). Reports of mitral valvuloplasty (MVP) in such patients are few; the alternative, mitral valve replacement (MVR), necessitates commitment to future valve replacement and long-term anticoagulation. We sought to determine the outcome of those patients who underwent either MVP or MVR between January 1, 1988, and December 31, 1998, for significant MR after repair of AVSD. Furthermore, we sought to identify (a) morphological predictors necessitating MVR, and (b) predictors of future reoperation within the MVP group. METHODS AND RESULTS: Retrospective review of clinical, operative, and echocardiographic data were performed. There were 46 patients identified (37 MVP and 9 MVR). The median age at initial AVSD repair was 0.6 years, and the age at subsequent mitral valve operation was 2.8 years. The early postoperative mortality rate was 2.2%, and survival at 1 and 10 years was 89.9% and 86.6%, respectively. A high rate of complete heart block was noted within the MVR group (37.5%). Freedom from later mitral valve reoperation for both groups was similar. No significant morphological predictors necessitating MVR were found. Predictors of reoperation within the MVP group included the presence of moderate or worse MR in the early postoperative period. In both groups New York Heart Association class, degree of MR, growth, and ventricular volumes improved. CONCLUSIONS: Mitral valve surgery significantly improves clinical status, with a sustained improvement in ventricular chamber size. MR can be successfully managed in patients after repair of AVSD independent of morphological type. Overall survival is acceptable, and further reoperation within the MVP group is influenced by early outcome of repair.
To avoid postoperative mitral valve dysfunction, the common atrioventricular valves of 21 preoperative patients with complete common atrioventricular canal were quantitatively evaluated using two-dimensional echocardiography. The criteria for diagnosing hypoplasia of the left lateral leaflet, which often made complete repair difficult, were also investigated. The patients were 10 boys and 11 girls, who ranged in age from one month to three years and three months. The subcostal short-axis view was used to evaluate the common atrioventricular valves. The sizes of the leaflets and diameters of the ventricles were measured by two-dimensional echocardiography and at surgery. The measurements by both methods were nearly identical, and the correlation coefficient was 0.95. Thus, two-dimensional echocardiography correctly evaluated common atrioventricular valve size. The length of the left lateral leaflet varied from 5 mm to 22 mm. In four patients, it was less than 10 mm, and was under the 99% confidence limit. In all four patients, two papillary muscles were noted in the left ventricle. Two of these patients underwent corrective surgery and died of postoperative mitral valve stenosis. This new technique can prevent postoperative mitral valve dysfunction by estimating preoperatively the appropriate suturing length of the anterior and posterior components of the anterior mitral valve. It is concluded that two-dimensional echocardiography can correctly evaluate the size of common atrioventricular valves, which is often difficult by angiocardiography. Hypoplasia of the left lateral leaflet could be diagnosed when its length was less than 10 mm measured by two-dimensional echocardiography. This technique is more accurate compared to the method which demonstrates the presence of a single papillary muscle in the left ventricle. Corrective surgery for the patients with a hypoplastic left lateral leaflet involves great risks, resulting in postoperative mitral valve stenosis. Preoperative evaluation of a common atrioventricular valve is useful to avoid postoperative mitral valve dysfunction.
The gap junction protein connexin45-deficient (Cx45-KO) mice die shortly after the hearts begin to beat. In addition to the heart defect, they also show defective vascular development which may be closely related with the cardiac phenotype. Therefore, we created mice whose floxed-Cx45 locus could be removed conditionally. We utilized cardiac alpha-actin-Cre transgenic mice to investigate the specific cardiac muscular function of Cx45 in vivo. The resultant conditional mutants were lethal, showing conduction block similar to that of the Cx45-KO mice. Unlike Cx45-KO, development of the endocardial cushion was not disrupted in the conditional mutants. X-gal staining was detected in the embryonic cardiac myocytes as a hallmark of Cre-loxP mediated floxed-Cx45 deletion. These results reconfirm the requirement of Cx45 for developing cardiac myocytes. These also indicate that establishing the first contractions is a crucial task for the early hearts.
Congenital heart defects are the most common type of birth defect and contribute the most to infant mortality due to birth defects. This study examined the relationship between several demographic factors and selected congenital heart defects among the unique multiethnic population in Hawaii during 1986-99, using data from a population-based birth defects registry. Rates were significantly higher in 1993-99 than in 1986-92 for transposition of the great arteries and Ebstein's anomaly, and significantly lower for tetralogy of Fallot. Significantly elevated rates were found with maternal age of > or =35 years for ventricular septal defect, atrial septal defect, endocardial cushion defect, and hypoplastic left heart syndrome. When cases with a known chromosomal abnormality were excluded, elevated rates among the older maternal age group remained for ventricular septal defect, atrial septal defect, and hypoplastic left heart syndrome. Whites had significantly higher rates than one or more of the other racial/ethnic groups for Ebstein's anomaly and coarctation of aorta, and significantly lower rates for tetralogy of Fallot, atrial septal defect, pulmonary valve atresia/stenosis, tricuspid valve atresia/stenosis, and anomalous pulmonary venous return. Significantly higher rates were found among males for transposition of great arteries, aortic valve stenosis, and interrupted aortic arch and, among females, for ventricular septal defect, endocardial cushion defect, and anomalous pulmonary venous return. Some of these differences were consistent with the literature while others were not.
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