日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Some patients with single ventricular physiology reach adulthood without Fontan completion because of risk factors for the procedure. A 40-year-old woman diagnosed with mitral atresia, transposition of the great arteries, ventricular septal defect, and subvalvular pulmonary stenosis had not been considered as a candidate for the Fontan procedure because of pulmonary hypertension. Lung biopsy revealed only mild medial wall thickness in the preacinar small pulmonary artery. Fontan completion following bidirectional Glenn (cavopulmonary anastomosis) procedure, atrioventricular (AV) valve repair, and pulmonary artery banding was successfully performed. Postoperatively, cardiac dysfunction with AV valve regurgitation and cardiac dyssynchrony developed. AV valve replacement and cardiac resynchronization therapy improved her ventricular function. She is doing well 5 years and 3 months postoperatively.