Circulation, Vol 88, 2248-2256, Copyright © 1993 by American Heart Association
TM Giglia, KJ Jenkins, A Matitiau, VS Mandell, SP Sanders, JE Mayer Jr and JE Lock
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.
ARTICLES
Influence of right heart size on outcome in pulmonary atresia with intact ventricular septum
Department of Cardiology, Children's Hospital, Boston.
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