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.