Case Report
A 34-year-old woman was referred to our hospital for inspection before operation for liver transplantation as a donor for her son. She had no symptom and no remarkable medical history. She had a history of successful vaginal delivery. When she was admitted to our hospital, Cardiac auscultation showed a grade 3/6 ejection systolic murmur at the second left sternal border, and the second heart sounds were fixed splitting. Twelve-lead electrocardiography showed sinus rhythm, left axis deviation, incomplete right bundle blanch block pattern (Figure 1A). Two-dimensional echocardiography revealed preserved left ventricular contractility; however, the atrium and right ventricle were dilated. Moreover, there was a defect in the lower part of the atrial septum and ventricular septal defect (VSD) closed with fibrous tissue. Color Doppler echocardiography showed left atrial to right atrial shunt, mild-moderate left atrioventricular valve (LAVV) regurgitation and moderate right atrioventricular valve(RAVV) regurgitation (Figure 1B). The cardiac catheterization showed gooseneck deformity (Figure 1C). Right heart catheterization demonstrated the mean pulmonary artery pressure was 16 mmHg, and mean pulmonary capillary wedge pressure was 10 mmHg. A pulmonary to systemic flow ratio was 2.08.
She was diagnosed as an incomplete atrioventricular septal defect (AVSD). Even though she had no symptoms, we decided her not to be a donor of liver transplantation, and to be undergone a repair of AVSD. After median sternotomy, cardiopulmonary bypass and aortic cross clamping were performed as usual. We observed a defect in the lower part of the atrial septum and a very small VSD surrounded by fibrous tissue and a cleft of LAVV (Figure 2A). It was intermediate type AVSD. At first, the pericardium was sutured to the junction of the RAVV and LAVV annulus and the small VSD was closed by direct suture (Figure 2B). Suturing in the area of the atrioventricular node and coronary sinus was performed very close to the LAVV. Next, the cleft of LAVV was closed with interrupted sutures and edge-to-edge repair for RAVV regurgitation.
Postoperatively, two-dimensional echocardiography demonstrated no leak around pericardial patch. Both RAVV and LAVV regurgitations were trivial (Figure 3). Her postoperative course was uneventful.