title: Staged Fontan procedure for mitral atresia associated with severe tricuspid regurgitation, pulmonary hypertension, and pulmonary artery distortion.
abstract: Optimal initial palliation and a subsequent staged approach is mandatory for high-risk Fontan candidates. We describe the case of mitral atresia with severe tricuspid regurgitation and pulmonary hypertension successfully managed by repeated palliation from the neonatal period and 2-stage Fontan surgery. A 1-month-old boy diagnosed with mitral atresia and double-outlet right ventricle underwent pulmonary artery banding at 1 month of age, followed by repeated pulmonary artery banding accompanied by tricuspid annuloplasty and atrial septal defect enlargement at 6 months. Because of the presence of pulmonary artery distortion, right ventricular dysfunction, and borderline pulmonary vascular resistance, a hemi-Fontan procedure was conducted with extended pulmonary artery plasty when the boy was 3 years and 8 months old. Cardiac catheterization done 3 months after showed improvement in risk factors, and the final Fontan operation (total cavopulmonary connection) was successfully done in conjunction with repeated tricuspid annuloplasty when the boy was 4 years and 5 months old. The patient remains in excellent clinical condition at the last follow-up 5 years after the final Fontan procedure with sinus rhythm and good ventricular function.
authors: Department of Cardiovascular Surgery, Jikei University School of Medicine, 3-25-8 Nishishinbashi, Minato-ku, Tokyo 105-8461, Japan.
authors: Yoko
authors: Y
authors: Matsumura
authors: Hiromi
authors: H
authors: Kurosawa
authors: Kiyozo
authors: K
authors: Morita
authors: Koji
authors: K
authors: Nomura
authors: Shogo
authors: S
authors: Shimizu
authors: Akira
authors: A
authors: Tatara
mesh_terms: D018729:Fontan Procedure / Q000379:methods*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications*; D007223:Infant; D008297:Male; D008943:Mitral Valve / Q000002:abnormalities* / Q000601:surgery; D011651:Pulmonary Artery / Q000473:pathology* / Q000601:surgery*; D014262:Tricuspid Valve Insufficiency / Q000601:surgery*; D014655:Vascular Resistance