title: Biventricular repair of pulmonary atresia with intact ventricular septum and severely hypoplastic right ventricle: a case report of a minimum intervention surgical approach.
abstract: BACKGROUND
  In patients who have pulmonary atresia with an intact ventricular septum and severe right ventricular hypoplasia, biventricular repair is considered to be impossible and multiple interventions are generally required for definitive repair.
  
  
  METHODS
  An initial palliative procedure was performed in a 1-month-old boy to promote right ventricular development by pulmonary valvectomy without disrupting the annulus, and appropriate oxygenation was achieved with a central funnel shunt. The retained annulus caused functional stenosis and prevented unfavorable right ventricular dilatation due to regurgitation. Thirteen years later, without any other intervention, reconstruction of the right ventricular outflow tract was successfully performed for definitive biventricular repair by using a new expanded polytetrafluoroethylene bulging valved conduit with extended longevity.
  
  
  CONCLUSIONS
  The successful outcome in this case suggests that our minimal palliation strategy could be one option for management of these patients.
authors.affiliation: Department of Cardiac Surgery, Nihon University School of Medicine, 30-1 Oyaguchikamimachi, Itabashi-ku, Tokyo, 173-8610, Japan. hatahiroaki@nihon-u.ac.jp.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Hata
authors.affiliation: Department of Pediatric Cardiology, Saitama Medical University International Medical Center, 1397-1 Yamane, Hidaka, 350-1298, Japan.
authors.forename: Naokata
authors.initials: N
authors.lastname: Sumitomo
authors.affiliation: Department of Pediatrics, Nihon University School of Medicine, Tokyo, Japan.
authors.forename: Mamoru
authors.initials: M
authors.lastname: Ayusawa
authors.affiliation: Department of Cardiac Surgery, Nihon University School of Medicine, 30-1 Oyaguchikamimachi, Itabashi-ku, Tokyo, 173-8610, Japan.
authors.forename: Motomi
authors.initials: M
authors.lastname: Shiono
mesh_terms: D000293:Adolescent; D006348:Cardiac Surgical Procedures / Q000379:methods*; D006330:Heart Defects, Congenital / Q000503:physiopathology / Q000601:surgery*; D006352:Heart Ventricles / Q000002:abnormalities* / Q000254:growth & development* / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D019060:Minimally Invasive Surgical Procedures; D010166:Palliative Care; D018633:Pulmonary Atresia / Q000601:surgery*
keywords: Artificial patent foramen ovale; Central shunt; Polytetrafluoroethylene bulging valved conduit; Pulmonary atresia with intact ventricular septum; Reconstruction of the right ventricular outflow tract