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The Impact of the Left Ventricle on Right Ventricular Function and Clinical Outcomes in Infants with Single-Right Ventricle Anomalies up to 14 Months of Age. J Am Soc Echocardiogr 2018 10;31(10):1151-1157



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Scopus ID

2-s2.0-85049356074   4 Citations


BACKGROUND: Children with single-right ventricle anomalies such as hypoplastic left heart syndrome (HLHS) have left ventricles of variable size and function. The impact of the left ventricle on the performance of the right ventricle and on survival remains unclear. The aim of this study was to identify whether left ventricular (LV) size and function influence right ventricular (RV) function and clinical outcome after staged palliation for single-right ventricle anomalies.

METHODS: In the Single Ventricle Reconstruction trial, echocardiography-derived measures of LV size and function were compared with measures of RV systolic and diastolic function, tricuspid regurgitation, and outcomes (death and/or heart transplantation) at baseline (preoperatively), early after Norwood palliation, before stage 2 palliation, and at 14 months of age.

RESULTS: Of the 522 subjects who met the study inclusion criteria, 381 (73%) had measurable left ventricles. The HLHS subtype of aortic atresia/mitral atresia was significantly less likely to have a measurable left ventricle (41%) compared with the other HLHS subtypes: aortic stenosis/mitral stenosis (100%), aortic atresia/mitral stenosis (96%), and those without HLHS (83%). RV end-diastolic and end-systolic volumes were significantly larger, while diastolic indices suggested better diastolic properties in those subjects with no left ventricles compared with those with measurable left ventricles. However, RV ejection fraction was not different on the basis of LV size and function after staged palliation. Moreover, there was no difference in transplantation-free survival to Norwood discharge, through the interstage period, or at 14 months of age between those subjects who had measurable left ventricles compared with those who did not.

CONCLUSIONS: LV size varies by anatomic subtype in infants with single-right ventricle anomalies. Although indices of RV size and diastolic function were influenced by the presence of a left ventricle, there was no difference in RV systolic function or transplantation-free survival on the basis of LV measures.

Author List

Cohen MS, Dagincourt N, Zak V, Baffa JM, Bartz P, Dragulescu A, Dudlani G, Henderson H, Krawczeski CD, Lai WW, Levine JC, Lewis AB, McCandless RT, Ohye RG, Owens ST, Schwartz SM, Slesnick TC, Taylor CL, Frommelt PC, Pediatric Heart Network Investigators


Peter J. Bartz MD Professor in the Pediatrics department at Medical College of Wisconsin
Peter C. Frommelt MD Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Follow-Up Studies
Heart Ventricles
Hypoplastic Left Heart Syndrome
Infant, Newborn
Norwood Procedures
Palliative Care
Time Factors
Ventricular Function, Right
jenkins-FCD Prod-482 91ad8a360b6da540234915ea01ff80e38bfdb40a