[Ebstein's anomaly associated with a definite atrial left to right shunt (author's transl)].
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Biomedical subjects
Publications and source records attributed to S Konno.
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The case is reported of a 38-year-old housewife with a common atrium associated with anomalous high insertion of the inferior vena cava, absence of the coronary sinus, and drainage of a left hepatic vein into the left side of common atrium. Surgical repair was performed successfully. The procedure was closure of a nearly total atrial septal defect with a Dacron patch. Embroyologically, the development failure of the sinu-atrial fold appears to be the basic causal factor.
The smallest commercially available prosthetic valve has an external diameter of 17 mm. Therefore, prosthetic valve replacement has been unfeasible in cases in which the diameter of the aortic valve ring is smaller than this size. A new operative method was used on 2 patients with congenital aortic valve lesions associated with hypoplasia of the valve ring. The procedure consists of a longitudinal incision in the aortic septum placed in the midportion of the two coronary ostia, a vertical incision in the outflow tract of the right ventricle to join the septal incision, prosthetic aortic valve replacement, and patch reconstruction of the outflow tracts of both ventricles by means of two layers of a fusiform Dacron patch. A 23-year-old woman with congenital aortic stenosis associated with hypoplattic aortic valve ring underwent prosthetic placement of the aortic valve and is well 1 year after the operation. Our other patient, a 3-month-old infant with congenital aortic regurgitation, died on the first postoperative day because of postperfusion lung. However, her hemodynamic status had been good in the immediate postoperative period.
A case with Ebstein's anomaly associated with large left to right atrial shunt was operated upon. The patient did not demonstrate any tricuspid insufficiency in spite of the downward displacement of the septal and posterior leaflets of tricuspid valve. The surgical closure of the atrial septal defect relieved the impending heart failure. The pulmonary congestion disappeared but the cardiomegaly persisted after the operation.
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