Incidentally detected atrioventricular septal defect in an adult
A 34-year-old woman who had planned to be a liver transplantation donor for her son was referred to our hospital for preoperative evaluation. She had no symptoms and had an unremarkable medical history. She had undergone a successful vaginal delivery. Her vital signs were stable upon admission. Cardiac auscultation revealed a grade 3/6 systolic ejection murmur at the second left sternal border and fixed splitting of the second heart sound. Twelve-lead electrocardiography showed sinus rhythm, left axis deviation, and an incomplete right bundle branch block pattern (Figure ). Two-dimensional echocardiography revealed preserved left ventricular contractility, although both atria and right ventricle were dilated. Moreover, there was a defect in the lower part of the atrial septum and a restrictive ventricular septal defect (VSD). Color Doppler echocardiography showed a left-to-right atrial shunt, mild-to-moderate left atrioventricular valve (LAVV) regurgitation, and moderate right atrioventricular valve (RAVV) regurgitation (Figure ). Cardiac catheterization revealed a gooseneck deformity (Figure ). Right-heart catheterization showed a mean pulmonary artery pressure of 16 mmHg and a mean pulmonary capillary wedge pressure of 10 mmHg. The pulmonary-to-systemic flow ratio was 2.08.\nShe was diagnosed with an atrioventricular septal defect. Even though she had no symptoms, she was deemed unfit to be a liver transplant donor, and she was advised to undergo AVSD repair. Median sternotomy, cardiopulmonary bypass, and aortic cross-clamping were performed as usual. We observed a defect in the lower portion of the atrial septum, a small VSD surrounded by fibrous tissue, and a cleft of the LAVV and RAVV (Figure ; white and black arrows indicate a cleft of the LAVV and VSD, respectively). It was diagnosed as an intermediate-type AVSD. We first sutured the pericardium (white arrows) to the junction of the RAVV and LAVV annuli (Figure ). Suturing at the area of the atrioventricular node and coronary sinus was performed very close to the LAVV. Next, the LAVV cleft was closed with interrupted sutures, and edge-to-edge repair was performed to address the RAVV regurgitation.\nPostoperatively, two-dimensional echocardiography showed no leakage around the pericardial patch. Both RAVV and LAVV regurgitations were trivial (Figure ). The patient's postoperative course was uneventful.