Pulmonary atresia with intact ventricular septum: results and predictive factors of surgical treatment

Details

Serval ID
serval:BIB_7E8A67C26E01
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Pulmonary atresia with intact ventricular septum: results and predictive factors of surgical treatment
Journal
Thoracic and Cardiovascular Surgeon
Author(s)
Niederhuser  U., Bauer  E. P., von Segesser  L. K., Carrel  T., Laske  A., Schonbeck  M., Turina  M.
ISSN
0171-6425
Publication state
Published
Issued date
06/1992
Peer-reviewed
Oui
Volume
40
Number
3
Pages
130-4
Notes
Journal Article --- Old month value: Jun
Abstract
Between 1970 and 1989 26 children were operated for pulmonary atresia with intact ventricular septum (PA/IVS). According to the degree of right heart hypoplasia the patients were divided into 3 groups of mild (5), moderate (17) or severe (4) hypoplasia. Palliative operations were performed in 25 children (17 male, 8 female) at a mean age of 10 days: 13 valvotomies (valv.), 5 aortopulmonary shunts, and 7 valv. plus shunt. One patient had total correction as primary procedure. A total of 17 reoperations was necessary in 12 of 26 patients (10 palliations, 7 total corrections). Total corrections were: 2 conduits and 5 patches of the right-ventricular outflow tract (RVOT). Total mortality was 14/26 (54%) children (early 10/26 = 38%, late 4/26 = 16%). After total correction mortality was 3/7 (43%) patients. After a mean follow up of 10.8 years after palliation the 12 survivors are mostly in NYHA class I. Actuarial survival after palliation was 60% after 30 days and 44% after 5 and 10 years. We analyzed 9 clinical and hemodynamic variables by univariate and multivariate analysis to assess the predictive factors of postoperative outcome. Multivariate analysis disclosed the degree of right-ventricular hypoplasia (p = 0.023) as an independent predictor for death whereas the age at palliation only approached significance (p = 0.065). We recommend the following surgical strategy, aiming in the first place at decompressing the right ventricle: in mild hypoplasia valvotomy alone or combined with a shunt for palliation, in moderate hypoplasia shunt plus patch of the RVOT and in severe hypoplasia shunting alone. In case of a restrictive foramen ovale initial balloon septostomy is performed.(ABSTRACT TRUNCATED AT 250 WORDS)
Keywords
Analysis of Variance Cardiac Surgical Procedures/methods Female Heart Defects, Congenital/mortality/*surgery Heart Septum Heart Ventricles/*abnormalities/surgery Humans Infant Infant, Newborn Male Prognosis Pulmonary Artery/*abnormalities/surgery Reoperation Retrospective Studies Survival Analysis Tricuspid Valve/*abnormalities/surgery
Pubmed
Web of science
Create date
14/02/2008 14:19
Last modification date
20/08/2019 14:39
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