Document Detail

Influence of right heart size on outcome in pulmonary atresia with intact ventricular septum.
MedLine Citation:
PMID:  8222119     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Neonates with pulmonary atresia and intact ventricular septum (PA-IVS) are frequently born with hypoplastic right heart structures that must grow after right ventricular decompression (RVD) procedures for a complete two-ventricle physiology to be achieved. Previous authors have asserted that neonatal right heart size or morphology will predict right heart growth potential. Since 1983, our bias has favored early RVD regardless of initial right heart size. In 1986, we recognized a subset of patients with coronary artery abnormalities associated with poor outcome after RVD and have defined these patients as having a right ventricular-dependent coronary circulation (RVDCC). METHODS AND RESULTS: To assess the influence of right heart size on outcome independent of the presence of RVDCC, we measured echocardiographic right ventricular (RV) dimensions in 37 neonates with adequate studies presenting between 1983 and 1990. Coronary artery anatomy was adequately assessed by angiography in 36. RV volume and tricuspid valve (TV) diameter were significantly smaller in patients with RVDCC than in those without. However, there was no statistically significant association between RV volume or TV diameter and survival among patients with or without RVDCC: Among 29 patients without RVDCC, 23 of 24 (95.8%) who achieved RVD are alive compared with 1 of 5 (20%) who did not achieve RVD (P = .001). Twenty-one of the 23 survivors have a complete two-ventricle physiology with low right atrial pressure. Among 7 patients with RVDCC, 2 patients who underwent RVD died early of left ventricular failure, whereas 4 of 5 who did not undergo RVD have survived single ventricular palliation. CONCLUSIONS: Small right heart size is associated with RVDCC but is not associated with survival in PA-IVS. Patients without RVDCC have improved survival after RVD regardless of neonatal right heart size.
T M Giglia; K J Jenkins; A Matitiau; V S Mandell; S P Sanders; J E Mayer; J E Lock
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Circulation     Volume:  88     ISSN:  0009-7322     ISO Abbreviation:  Circulation     Publication Date:  1993 Nov 
Date Detail:
Created Date:  1993-12-03     Completed Date:  1993-12-03     Revised Date:  2010-03-24    
Medline Journal Info:
Nlm Unique ID:  0147763     Medline TA:  Circulation     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  2248-56     Citation Subset:  AIM; IM    
Department of Cardiology, Children's Hospital, Boston.
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MeSH Terms
Blood Volume
Coronary Circulation
Heart Septal Defects, Ventricular / surgery*,  ultrasonography*
Infant, Newborn
Pulmonary Valve / abnormalities*,  surgery*,  ultrasonography
Survival Analysis
Treatment Outcome
Tricuspid Valve / ultrasonography
Ventricular Function, Right

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