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The role of right ventricular function in paediatric idiopathic dilated cardiomyopathy

Published online by Cambridge University Press:  23 August 2012

Abraham Groner ,
Jen Yau ,
Irene D. Lytrivi ,
H. Helen Ko ,
James C. Nielsen ,
Ira A. Parness  and
Shubhika Srivastava
Abraham Groner*
Affiliation:
Division of Pediatric Cardiology, Mount Sinai Medical Center, New York, New York, United States of America
Jen Yau
Affiliation:
Division of Pediatric Cardiology, Mount Sinai Medical Center, New York, New York, United States of America
Irene D. Lytrivi
Affiliation:
Division of Pediatric Cardiology, Mount Sinai Medical Center, New York, New York, United States of America
H. Helen Ko
Affiliation:
Division of Pediatric Cardiology, Mount Sinai Medical Center, New York, New York, United States of America
James C. Nielsen
Affiliation:
Division of Pediatric Cardiology, Mount Sinai Medical Center, New York, New York, United States of America
Ira A. Parness
Affiliation:
Division of Pediatric Cardiology, Mount Sinai Medical Center, New York, New York, United States of America
Shubhika Srivastava
Affiliation:
Division of Pediatric Cardiology, Mount Sinai Medical Center, New York, New York, United States of America
*
Correspondence to: Dr A. Groner, MD, Division of Pediatric Cardiology, Mount Sinai Medical Center, One Gustave L. Levy Place, Box 1201, New York, New York 10029, United States of America. Tel: 212 241 5883; Fax: 212 534 2659; E-mail: agroner@gmail.com
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Abstract

Introduction

The prevalence of right ventricular dysfunction in idiopathic dilated cardiomyopathy is incompletely studied in children. Furthermore, right ventricular function may signal worse outcomes. We evaluated recently published right ventricular function echocardiographic indices in identifying dysfunction in children with idiopathic dilated cardiomyopathy and the impact of right ventricular dysfunction on long-term prognosis.

Methods

A retrospective database review of right ventricular function indices in 30 patients with idiopathic dilated cardiomyopathy was compared with 60 age- and sex-matched controls from January, 2001 until December, 2010. Right ventricular function was assessed by Doppler tissue peak systolic S′, early and late diastolic E′ and A′ waves and isovolumic acceleration at the tricuspid valve annulus; pulsed wave Doppler tricuspid valve inflow E and A waves; right ventricular myocardial performance index; tricuspid annular plane systolic excursion; right ventricular fractional area change.

Results

Right ventricular systolic and diastolic function in idiopathic dilated cardiomyopathy was significantly impaired. All measured indices except for isovolumic acceleration and fractional area change were significantly reduced, with a p-value less than 0.05. There was no right ventricular index predictive of death or transplantation. Patients with poor outcome were significantly more likely to need inotropic support (p-value equal to 0.018), be placed on a ventricular assist device (p equal to 0.005), and have a worse left ventricular ejection fraction z-score (p-value equal to 0.002).

Conclusion

Right ventricular dysfunction is under-recognised in children presenting with idiopathic dilated cardiomyopathy. The need for clinical circulatory support and left ventricular ejection fraction z-score less than minus 8 were primary determinants of outcome, independent of the degree of derangement in right ventricular function.

Keywords

Doppler tissue imagingtricuspid annular plane systolic excursionmyocardial performance index
Type
Original Articles
Information
Cardiology in the Young , Volume 23 , Issue 3 , June 2013 , pp. 409 - 415
Copyright
Copyright © Cambridge University Press 2012 

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