Meryl S Cohen1, Nicholas Dagincourt2, Victor Zak2, Jeanne Marie Baffa3, Peter Bartz4, Andreea Dragulescu5, Gul Dudlani6, Heather Henderson7, Catherine D Krawczeski8, Wyman W Lai9, Jami C Levine10, Alan B Lewis11, Rachel T McCandless12, Richard G Ohye13, Sonal T Owens14, Steven M Schwartz5, Timothy C Slesnick15, Carolyn L Taylor16, Peter C Frommelt4. 1. Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. Electronic address: cohenm@email.chop.edu. 2. New England Research Institutes, Boston, Massachusetts. 3. Division of Cardiology, A.I. DuPont Hospital for Children, Wilmington, Delaware. 4. Division of Cardiology, Medical College of Wisconsin, Milwaukee, Wisconsin. 5. Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, Ontario, Canada. 6. Division of Cardiology, Johns Hopkins All Children's Heart Institute, St. Petersburg, Florida. 7. Division of Pediatric Cardiology, Duke University Medical Center, Raleigh, North Carolina. 8. Division of Cardiology, Cincinnati Children's Hospital, Cincinnati, Ohio. 9. Division of Cardiology, Morgan Stanley Children's Hospital, New York, New York. 10. Department of Cardiology, Children's Hospital, Boston, Boston, Massachusetts. 11. Division of Cardiology, Children's Hospital Los Angeles, Los Angeles, California. 12. Division of Cardiology, Primary Children's Hospital, Salt Lake City, Utah. 13. Division of Cardiac Surgery, University of Michigan Health System, Ann Arbor, Michigan. 14. Division of Pediatric Cardiology, University of Michigan Health System, Ann Arbor, Michigan. 15. Division of Cardiology, Texas Children's Hospital, Houston, Texas. 16. Division of Pediatric Cardiology, Medical University of South Carolina, Charleston, South Carolina.
Abstract
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 influenceright 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. Crown
RCT Entities:
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. Crown
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