title: Surgical treatment for isolated hypoplasia of the right ventricle.
abstract: Four patients with isolated hypoplasia of the right ventricle who underwent intracardiac repair are described. An atrial septal defect (ASD) was successfully closed in 3 patients with mild hypoplasia of the right ventricle. The remaining patients underwent ASD closure and right atrium-to-pulmonary artery connection with a conduit with a bovine pericardial valve because of severe hypoplasia of the right ventricle and tricuspid valve. Postoperative angiocardiogram in the last patient demonstrated two-channel pathways from the right atrium to the pulmonary artery via both the right ventricle and external conduit. At long-term follow-up studies, 3 patients had developed arrhythmias and/or sinus node dysfunction, and one had died, probably because of arrhythmia, 13 years postoperatively. The right ventricular ejection fractions were below normal levels at 11 and 12 years postoperatively in 2 patients who underwent closure of ASD. It was concluded that the mild form of hypoplasia of the right ventricle should be surgically corrected by ASD closure, while right atrium-to-pulmonary artery connection or Fontan's operation should be chosen for cases of severe hypoplasia. Arrhythmias and hypofunction of the right ventricle are long-term problems after intracardiac repair of this anomaly.
authors.affiliation: Department of Thoracic and Cardiovascular Surgery, Tohoku University School of Medicine, Sendai, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Haneda
authors.forename: T
authors.initials: T
authors.lastname: Togo
authors.forename: Y
authors.initials: Y
authors.lastname: Ito
authors.forename: H
authors.initials: H
authors.lastname: Ogata
authors.forename: T
authors.initials: T
authors.lastname: Horiuchi
authors.forename: H
authors.initials: H
authors.lastname: Mohri
mesh_terms: D000293:Adolescent; D000328:Adult; D001145:Arrhythmias, Cardiac / Q000453:epidemiology; D002648:Child; D003490:Cyanosis / Q000151:congenital / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D006352:Heart Ventricles / Q000002:abnormalities / Q000503:physiopathology / Q000601:surgery; D006439:Hemodynamics; D006801:Humans; D008297:Male; D011183:Postoperative Complications / Q000453:epidemiology