Association of left ventricular dilation at listing for heart transplant with postlisting and early posttransplant mortality in children with dilated cardiomyopathy

Tajinder P. Singh, Lynn A. Sleeper, Steven Lipshultz, Amy Cinar, Charles Canter, Steven A. Webber, Daniel Bernstein, Elfriede Pahl, Jorge A. Alvarez, James D. Wilkinson, Jeffrey Towbin, Steven D. Colan

Research output: Contribution to journalArticle

19 Citations (Scopus)

Abstract

Background-In patients with dilated cardiomyopathy, the magnitude of cardiac remodeling often correlates with the clinical severity of heart failure. We sought to determine whether measures of left ventricular (LV) dilation and systolic dysfunction in children with dilated cardiomyopathy at the time of listing for cardiac transplantation are associated with survival while waiting for and early after transplant. Methods and Results-We analyzed echocardiographic data obtained within 6 months of listing for heart transplant and clinical data from 261 children with dilated cardiomyopathy who were included in both the Pediatric Cardiomyopathy Registry and the Pediatric Heart Transplant Study. Median time to listing after diagnosis was 1.9 months and to transplant after listing was 0.8 months. There were 42 deaths (29 waiting and 13 within 6 months after transplant). We found a significant age-dependent association of LV end-diastolic dimension z score (n=204, 31 deaths) with death controlling for race, transplant status, and medical insurance. The association was strongest for infants younger than 6 months at diagnosis (hazard ratio 1.47, P=0.008) and was not significant in children older than 5 years at diagnosis. A similar interaction was identified between age and LV end-systolic dimension z score (P=0.04). Neither LV function nor mass was associated with death, overall, or in subgroups. Conclusions-The severity of LV dilation at listing for heart transplant is associated with outcome in infants and young children with dilated cardiomyopathy, whereas the severity of LV systolic dysfunction is not. These findings should be considered in risk stratification of these children at listing.

Original languageEnglish (US)
Pages (from-to)591-598
Number of pages8
JournalCirculation: Heart Failure
Volume2
Issue number6
DOIs
StatePublished - Nov 1 2009

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Child Mortality
Dilated Cardiomyopathy
Dilatation
Transplants
Pediatrics
Insurance Coverage
Left Ventricular Dysfunction
Heart Transplantation
Cardiomyopathies
Left Ventricular Function
Registries
Heart Failure
Survival

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

Cite this

Association of left ventricular dilation at listing for heart transplant with postlisting and early posttransplant mortality in children with dilated cardiomyopathy. / Singh, Tajinder P.; Sleeper, Lynn A.; Lipshultz, Steven; Cinar, Amy; Canter, Charles; Webber, Steven A.; Bernstein, Daniel; Pahl, Elfriede; Alvarez, Jorge A.; Wilkinson, James D.; Towbin, Jeffrey; Colan, Steven D.

In: Circulation: Heart Failure, Vol. 2, No. 6, 01.11.2009, p. 591-598.

Research output: Contribution to journalArticle

Singh, TP, Sleeper, LA, Lipshultz, S, Cinar, A, Canter, C, Webber, SA, Bernstein, D, Pahl, E, Alvarez, JA, Wilkinson, JD, Towbin, J & Colan, SD 2009, 'Association of left ventricular dilation at listing for heart transplant with postlisting and early posttransplant mortality in children with dilated cardiomyopathy', Circulation: Heart Failure, vol. 2, no. 6, pp. 591-598. https://doi.org/10.1161/CIRCHEARTFAILURE.108.839001
Singh, Tajinder P. ; Sleeper, Lynn A. ; Lipshultz, Steven ; Cinar, Amy ; Canter, Charles ; Webber, Steven A. ; Bernstein, Daniel ; Pahl, Elfriede ; Alvarez, Jorge A. ; Wilkinson, James D. ; Towbin, Jeffrey ; Colan, Steven D. / Association of left ventricular dilation at listing for heart transplant with postlisting and early posttransplant mortality in children with dilated cardiomyopathy. In: Circulation: Heart Failure. 2009 ; Vol. 2, No. 6. pp. 591-598.
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AU - Singh, Tajinder P.

AU - Sleeper, Lynn A.

AU - Lipshultz, Steven

AU - Cinar, Amy

AU - Canter, Charles

AU - Webber, Steven A.

AU - Bernstein, Daniel

AU - Pahl, Elfriede

AU - Alvarez, Jorge A.

AU - Wilkinson, James D.

AU - Towbin, Jeffrey

AU - Colan, Steven D.

PY - 2009/11/1

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N2 - Background-In patients with dilated cardiomyopathy, the magnitude of cardiac remodeling often correlates with the clinical severity of heart failure. We sought to determine whether measures of left ventricular (LV) dilation and systolic dysfunction in children with dilated cardiomyopathy at the time of listing for cardiac transplantation are associated with survival while waiting for and early after transplant. Methods and Results-We analyzed echocardiographic data obtained within 6 months of listing for heart transplant and clinical data from 261 children with dilated cardiomyopathy who were included in both the Pediatric Cardiomyopathy Registry and the Pediatric Heart Transplant Study. Median time to listing after diagnosis was 1.9 months and to transplant after listing was 0.8 months. There were 42 deaths (29 waiting and 13 within 6 months after transplant). We found a significant age-dependent association of LV end-diastolic dimension z score (n=204, 31 deaths) with death controlling for race, transplant status, and medical insurance. The association was strongest for infants younger than 6 months at diagnosis (hazard ratio 1.47, P=0.008) and was not significant in children older than 5 years at diagnosis. A similar interaction was identified between age and LV end-systolic dimension z score (P=0.04). Neither LV function nor mass was associated with death, overall, or in subgroups. Conclusions-The severity of LV dilation at listing for heart transplant is associated with outcome in infants and young children with dilated cardiomyopathy, whereas the severity of LV systolic dysfunction is not. These findings should be considered in risk stratification of these children at listing.

AB - Background-In patients with dilated cardiomyopathy, the magnitude of cardiac remodeling often correlates with the clinical severity of heart failure. We sought to determine whether measures of left ventricular (LV) dilation and systolic dysfunction in children with dilated cardiomyopathy at the time of listing for cardiac transplantation are associated with survival while waiting for and early after transplant. Methods and Results-We analyzed echocardiographic data obtained within 6 months of listing for heart transplant and clinical data from 261 children with dilated cardiomyopathy who were included in both the Pediatric Cardiomyopathy Registry and the Pediatric Heart Transplant Study. Median time to listing after diagnosis was 1.9 months and to transplant after listing was 0.8 months. There were 42 deaths (29 waiting and 13 within 6 months after transplant). We found a significant age-dependent association of LV end-diastolic dimension z score (n=204, 31 deaths) with death controlling for race, transplant status, and medical insurance. The association was strongest for infants younger than 6 months at diagnosis (hazard ratio 1.47, P=0.008) and was not significant in children older than 5 years at diagnosis. A similar interaction was identified between age and LV end-systolic dimension z score (P=0.04). Neither LV function nor mass was associated with death, overall, or in subgroups. Conclusions-The severity of LV dilation at listing for heart transplant is associated with outcome in infants and young children with dilated cardiomyopathy, whereas the severity of LV systolic dysfunction is not. These findings should be considered in risk stratification of these children at listing.

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