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Pulmonary Atresia with Intact Ventricular Septum.
MedLine Citation:
PMID:  12093389     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
The prognosis for patients with pulmonary atresia with intact ventricular septum is poor with and without conventional surgical treatment. Because of this reason, a comprehensive program of medical, transcatheter, and surgical treatment is necessary to improve the long-term outlook of these infants. Algorithms of management plans should be developed based on the presence of right ventricular-dependent coronary circulation as well as size and morphology of the right ventricle. In a tripartite or bipartite right ventricle, transcatheter radiofrequency perforation is preferable. Alternatively, surgical valvotomy may be performed. Augmentation of pulmonary blood flow by prolonged infusion of prostaglandin E(1), stenting the ductus, or a surgical modified Blalock-Taussig shunt may be necessary in some of these patients. In patients with a unipartite or very small right ventricle or a right ventricular-dependent coronary circulation (Tricuspid valve Z score < -2.5), augmentation of pulmonary flow along with atrial septostomy should be undertaken. Follow-up studies to determine the feasibility of biventricular repair should be undertaken and, if feasible, surgical or transcatheter methods may be used to achieve the goals. If not suitable for biventricular repair, one-ventricle (Fontan) or one and one-half ventricular repair should be considered. Comprehensive and well-planned treatment algorithms may help improve survival rate.
Authors:
P. Syamasundar Rao
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Publication Detail:
Type:  JOURNAL ARTICLE    
Journal Detail:
Title:  Current treatment options in cardiovascular medicine     Volume:  4     ISSN:  1534-3189     ISO Abbreviation:  Curr Treat Options Cardiovasc Med     Publication Date:  2002 Aug 
Date Detail:
Created Date:  2002-Jul-2     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9815942     Medline TA:  Curr Treat Options Cardiovasc Med     Country:  -    
Other Details:
Languages:  ENG     Pagination:  321-336     Citation Subset:  -    
Affiliation:
Division of Pediatric Cardiology, University of Texas-Houston Medical School, 6431 Fannin, MSB 3.132, Houston, TX 77030, USA. P.Syamasundar.Rao@uth.tmc.edu
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