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Endocardial cushion defects.

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I M ANDERSON, H M COLES. 1961-03-11. Endocardial cushion defects.. https://doi.org/10.1136/bmj.1.5227.696

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Use of the mitral valve-tricuspid valve distance as a marker of fetal endocardial cushion defects.

OBJECTIVE: The purpose of this study was to compare the mitral valve-tricuspid valve distance in second-trimester fetuses with normal cardiac anatomy versus those fetuses with endocardial cushion defects. STUDY DESIGN: We identified fetuses between 16 and 24 weeks of gestation. The distance between the insertions of the medial leaflets of the mitral and tricuspid valves were obtained. Linear regression curves were generated. RESULTS: The mean mitral valve-tricuspid valve distance for 86 fetuses with normal cardiac anatomy was 2.02 mm, compared with 0.37 mm in 13 fetuses with endocardial cushion defects ( P = .0001). Linear regression curve correlating mitral valve-tricuspid valve distance with gestational age showed a gradual slope (R 2 = 0.28; P < .0001). With a mitral valve-tricuspid valve distance < 5th percentile as a marker for the diagnosis of endocardial cushion defect gave a sensitivity of 69.2%, a specificity of 100%, a positive predictive value of 100%, a negative predictive value of 95.6%, and a false-positive rate of 0% ( P = .0001). CONCLUSION: The mitral valve-tricuspid valve distance is useful clinically in the detection of endocardial cushion defects in second-trimester fetuses.

Endocardial Cushion Defects↗

Surgical repair of double-orifice of the mitral valve in cases with an atrioventricular canal defects.

OBJECTIVE: A Double-orifice in the mitral valve is an uncommon congenital cardiac lesion which occurs as an isolated anomaly or in association with other cardiac malformation. This report deals with our surgical experience of a double-orifice of the mitral valve in cases with an atrioventricular canal defect. PATIENTS AND METHODS: From 1991 through 1999, ten patients were diagnosed to have a double-orifice of the mitral valve at Shizuoka Children's Hospital. Each patient had associated major cardiac malformations, among which atrioventricular canal defect underwent surgical management, with five of these undergoing complete correction with or without previous pulmonary artery banding. Of these 10, the five cases were enrolled in this study. Two of these had a complete type, and the other three had a partial type. The cleft in the left-sided atrioventricular valve was closed partially in four and left untouched in one. Bridging tissue, when present, was left intact. There was no regurgitation from any accessory orifice and no repair for an accessory orifice was needed. RESULT: There was no late death and no replacement of the valve with prosthesis. During follow-up ranging from 1 to 4 years, none of the patients developed severe stenosis or progressive regurgitation in the left-sided atrioventricular valve. CONCLUSION: Meticulous surgical management of a double-orifice in the mitral valve in association with atrioventricular canal defect an achieve an acceptable midterm result without developing severe dysfunction in the left-sided atrioventricular valve.

Endocardial Cushion Defects↗

Early and late results of total correction of congenital cardiac anomalies in infancy.

OBJECTIVE: We evaluated long-term results of surgical correction of congenital cardiac anomalies in infancy. METHODS: We reviewed cases of 856 patients who underwent complete correction of major cardiac anomalies in the first year of life during last 24 years at our institution, and analyzed results. Surgery involved ventricular septal defect (n = 453), tetralogy of Fallot (n = 92), atrioventricular canal defect (n = 93), and complete transposition of the great arteries (n = 218). RESULTS: Operative mortality was 2.2% in ventricular septal defect, 0% in tetralogy of Fallot, 8.6% in atrioventricular canal defect, and 4.1% in complete transposition of the great arteries. Freedom from reoperation at 20 years was 96.5 +/- 2.0% and actuarial survival was 94.2 +/- 1.3% in ventricular septal defect. Freedom from reoperation at 15 years was 90.5 +/- 3.9% in tetralogy of Fallot and 86.6 +/- 4.4% in atrioventricular canal defect. Actuarial survival at 15 years was 97.8 +/- 1.6% in tetralogy of Fallot, 85.7 +/- 3.7% in atrioventricular canal defect, and 89.9 +/- 2.2% in complete transposition of the great arteries. Actuarial survival in complete transposition of the great arteries was significantly better in arterial than in atrial switch operations. CONCLUSIONS: Total correction of ventricular septal defect, tetralogy of Fallot, atrioventricular canal defect, and complete transposition of the great arteries in infancy was conducted safely, but the incidence of reoperation in late follow-up must be reduced.

Endocardial Cushion Defects↗