title: Percutaneous balloon valvotomy in pulmonary atresia with intact ventricular septum: impact on patient care.
abstract: BACKGROUND
  Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital lesion with high mortality. Therapy was exclusively surgical until recently, when the use of radiofrequency-assisted perforation of the atretic valve was introduced as a treatment option. This study analyzes the outcomes and morphological changes to right heart structures after percutaneous perforation and balloon dilation of the atretic valve.
  
  
  RESULTS
  Between April 1992 and August 2000, 30 patients with PA-IVS underwent attempted percutaneous valve perforation and balloon dilation of the pulmonary valve. Longitudinal echocardiographic measurements of the tricuspid valve diameter, right ventricular length and area were recorded. Z scores were calculated according to published formulas. Perforation was achieved in 27 patients. In 14 patients a modified Blalock-Taussig shunt was performed between 2 and 24 days after valve dilation. There were 3 early and 2 late deaths. Among the survivors (follow-up time of 1 to 87 months), 16 patients had a biventricular circulation, 3 a 1(1/2)-ventricle circulation, and 1 a Fontan operation. Four patients are awaiting further palliation. There was no significant change of the tricuspid valve Z score or right ventricular length Z score with time.
  
  
  CONCLUSIONS
  Percutaneous balloon valvotomy is an effective treatment strategy for patients with PA-IVS provided that there is a patent infundibulum and a lack of a right ventricle-dependent coronary circulation. Despite the observation that right heart growth does not increase with body growth in early follow-up, it appears adequate to maintain a biventricular circulation in many patients.
authors.affiliation: Department of Pediatrics, Division of Cardiology, The Hospital for Sick Children, The University of Toronto School of Medicine, Toronto, ON M5G 1X8, Canada.
authors.forename: Tilman
authors.initials: T
authors.lastname: Humpl
authors.forename: Björn
authors.initials: B
authors.lastname: Söderberg
authors.forename: Brian W
authors.initials: BW
authors.lastname: McCrindle
authors.forename: David G
authors.initials: DG
authors.lastname: Nykanen
authors.forename: Robert M
authors.initials: RM
authors.lastname: Freedom
authors.forename: William G
authors.initials: WG
authors.lastname: Williams
authors.forename: Lee N
authors.initials: LN
authors.lastname: Benson
mesh_terms: D000792:Angiography; D006348:Cardiac Surgical Procedures / Q000401:mortality; D017115:Catheter Ablation / Q000401:mortality; D002404:Catheterization / Q000295:instrumentation / Q000379:methods / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006128:Growth; D006321:Heart / Q000254:growth & development; D006346:Heart Septum / Q000601:surgery*; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007431:Intraoperative Complications; D008297:Male; D005791:Patient Care / Q000706:statistics & numerical data; D011183:Postoperative Complications; D018633:Pulmonary Atresia / Q000175:diagnosis / Q000601:surgery*; D011664:Pulmonary Valve / Q000601:surgery; D016896:Treatment Outcome