title: [Rehabilitation of hypoplastic pulmonary arteries in pulmonary atresia with ventricular septal defect. Medium term results].
abstract: The treatment of pulmonary atresia with ventricular septal defect, hypoplasia of the native pulmonary arteries and major aorto-pulmonary collaterals is controversial with a wide range of options from symptomatic treatment to surgical correction, but usually unifocal surgery. However, the collateral vessels used in the reconstruction are often tortuous and stenosed, resulting in a problematic haemodynamic result. The authors report the results of medico-surgical rehabilitation of the native pulmonary arteries in three stages. Since 1990, patients with an average Nakata index of 27.5 +/- 16.7 mm2/m2 underwent elective surgical connection of the native pulmonary arteries to the right ventricle (1st stage) followed by their rehabilitation with catheter occlusion of the collaterals (2nd stage) with the aim of preparing surgical correction (3rd stage). The first surgical stage at an median age of 5 months (0.1 to 25.2) was complicated by one death. After an average of 2.1 +/- 1 catheterisations, 17 patients underwent surgical correction at an median age of 2.18 (0.6 to 10.3) years with an average Nakata index of 207 +/- 91 mm2/m2. All survived surgery with three late deaths in patients with poor haemodynamic results (right ventricular/left ventricular pressure ratio>80%). After an median follow-up of 9.2 (3.5 to 17) years, the 14 survivors are in NYHA Class I or II with a good haemodynamic result in 10 patients. The authors conclude that despite some late deaths and raised right ventricular and pulmonary artery pressures in a minority of patients, the rehabilitation of the pulmonary arteries is an effective management of this cardiopathy.
authors.affiliation: Cardiologie pédiatrique, Département de cardiologie, Hôpital d'Enfants de La Timone, 264, rue Saint-Pierre, 13385 Marseille cedex 05.
authors.forename: S
authors.initials: S
authors.lastname: Schouvey
authors.forename: A
authors.initials: A
authors.lastname: Dragulescu
authors.forename: O
authors.initials: O
authors.lastname: Ghez
authors.forename: P
authors.initials: P
authors.lastname: Amedro
authors.forename: B
authors.initials: B
authors.lastname: Kreitmann
authors.forename: P
authors.initials: P
authors.lastname: Chetaille
authors.forename: A
authors.initials: A
authors.lastname: Fraisse
authors.forename: D
authors.initials: D
authors.lastname: Metras
mesh_terms: D000800:Angioplasty, Balloon; D021721:Balloon Occlusion; D001794:Blood Pressure / Q000502:physiology; D006328:Cardiac Catheterization; D002648:Child; D002675:Child, Preschool; D003097:Collateral Circulation / Q000502:physiology; D005260:Female; D005500:Follow-Up Studies; D006345:Heart Septal Defects, Ventricular / Q000601:surgery*; D006352:Heart Ventricles / Q000601:surgery; D006801:Humans; D007223:Infant; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000601:surgery; D018633:Pulmonary Atresia / Q000601:surgery*; D012189:Retrospective Studies; D015607:Stents; D015996:Survival Rate; D017725:Ventricular Pressure / Q000502:physiology