Document Detail

Pulmonary atresia with intact ventricular septum. Surgical approach based on ventricular size and coronary anatomy.
MedLine Citation:
PMID:  8412270     Owner:  NLM     Status:  MEDLINE    
Pulmonary atresia with intact ventricular septum has continued to have a high surgical mortality. This may relate to the nonuniformity of the anomaly. We have developed a management algorithm based on the right ventricular size and coronary anatomy. Patients with a well-developed ventricle and normal coronary arteries have undergone right ventricular outflow procedures. The adequacy of their right ventricles is subsequently evaluated; some patients are candidates for a four-chamber repair, whereas others are candidates for a Fontan procedure. Patients with severe hypoplasia of the right ventricle frequently have extensive ventriculo-coronary connections, and for these patients we have developed the technique of right ventricular obliteration. Over the past 7 years, we have treated 20 patients with pulmonary atresia with intact ventricular septum. Fourteen of the 20 patients underwent outflow tract procedures, with no operative mortality. There were two late deaths in this group. Six patients had hypoplasia of the ventricle with ventriculo-coronary connections and underwent right ventricular obliteration. There was one operative death and one late death in the group. Overall, 95% survived the neonatal period, and 80% were still alive at the time this article was written with an average follow-up of 32 months. We conclude that pulmonary atresia with intact ventricular septum can be successfully managed with the use of an algorithm based on ventricular size and coronary anatomy.
R D Mainwaring; J J Lamberti
Publication Detail:
Type:  Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  The Journal of thoracic and cardiovascular surgery     Volume:  106     ISSN:  0022-5223     ISO Abbreviation:  J. Thorac. Cardiovasc. Surg.     Publication Date:  1993 Oct 
Date Detail:
Created Date:  1993-11-17     Completed Date:  1993-11-17     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0376343     Medline TA:  J Thorac Cardiovasc Surg     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  733-8     Citation Subset:  AIM; IM    
Division of Cardiac Surgery, Children's Hospital and Health Center, San Diego, Calif.
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MeSH Terms
Cardiac Surgical Procedures / methods
Constriction, Pathologic
Coronary Angiography
Heart Ventricles / abnormalities*,  surgery*
Hypertrophy, Right Ventricular / surgery
Infant, Newborn
Pulmonary Artery / abnormalities*,  surgery*
Retrospective Studies
Survival Rate
Grant Support

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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