title: Biventricular Repair of Pulmonary Atresia After Fontan Palliation.
abstract: Fontan palliation is used when biventricular repair (BVR) is not possible. Early outcomes are acceptable; however, the long-term sequelae include protein-losing enteropathy, declining functional status, increased pulmonary vascular resistance, heart failure, and hepatic and renal dysfunction. These adverse events are characteristic of persistent venous hypertension and may be avoided if restoring biventricular circulation is possible. Arrhythmias are a common adverse event, particularly in patients with an atriopulmonary connection, which may lead to acute decompensation and early death. We describe a 30-year-old woman who underwent successful BVR for pulmonary atresia with intact ventricular septum and demonstrate that where favorable anatomy exists with a failing Fontan, BVR should be considered.
authors.affiliation: College of Human Medicine, Michigan State University, Grand Rapids, Michigan.
authors.forename: Travis F
authors.initials: TF
authors.lastname: D'Souza
authors.affiliation: Congenital Heart Center, Helen DeVos Children's Hospital of Spectrum Health, Grand Rapids, Michigan.
authors.forename: Bennett P
authors.initials: BP
authors.lastname: Samuel
authors.affiliation: College of Human Medicine, Michigan State University, Grand Rapids, Michigan.
authors.forename: Neal D
authors.initials: ND
authors.lastname: Hillman
authors.affiliation: Congenital Heart Center, Helen DeVos Children's Hospital of Spectrum Health, Grand Rapids, Michigan.
authors.forename: Joseph J
authors.initials: JJ
authors.lastname: Vettukattil
authors.affiliation: Congenital Heart Center, Helen DeVos Children's Hospital of Spectrum Health, Grand Rapids, Michigan. Electronic address: marcus.haw@helendevoschildrens.org.
authors.forename: Marcus P
authors.initials: MP
authors.lastname: Haw
mesh_terms: D000328:Adult; D001705:Bioprosthesis; D017548:Echocardiography, Transesophageal / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects* / Q000379:methods; D019918:Heart Valve Prosthesis Implantation / Q000379:methods*; D006352:Heart Ventricles / Q000473:pathology / Q000601:surgery*; D006801:Humans; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods; D010166:Palliative Care / Q000379:methods; D018633:Pulmonary Atresia / Q000000981:diagnostic imaging / Q000601:surgery*; D011859:Radiography; D020127:Recovery of Function; D012086:Reoperation / Q000379:methods; D018570:Risk Assessment; D056346:Sternotomy / Q000379:methods; D016896:Treatment Outcome; D014262:Tricuspid Valve Insufficiency / Q000175:diagnosis / Q000601:surgery*