Waitlist and Post-Heart Transplant Outcomes for Children With Nondilated Cardiomyopathy

Background: Although outcomes for pediatric cardiomyopathy (CMP) patients have improved, an understanding of outcomes by CMP phenotype is essential. This study assessed changes in waitlist and post-transplant survival in nondilated cardiomyopathy (DCM) patients over 2 decades, explored ventricular assist device (VAD) utilization in this cohort, and identified risk factors for waitlist and posttransplant mortality in the current era.

Methods: Pediatric patients with a diagnosis of CMP listed for heart transplantation during three eras: Era 1: March 5, 1999 to December 31, 2004; Era 2: January 1, 2005 to December 15, 2011; and Era 3 (current era): December 16, 2011 to February 28, 2018 were included. Multivariable Cox proportional hazards regression was performed to assess waitlist and posttransplant survival.

Results: Compared with patients with DCM, those with hypertrophic and restrictive cardiomyopathy in the current era are less likely to be on VAD (23.4% vs 2.7% vs 4.5%); listed United Network for Organ Sharing Status 1A (75.6% vs 39.8% vs 34.8%), and more likely to have longer waitlist times (P < .01 for all). Only 3.3% hypertrophic and 2.4% restrictive cardiomyopathy patients had VAD implantation, although VAD use did not adversely impact waitlist survival in weighted non-DCM patients. Significant improvements have occurred in waitlist survival of hypertrophic and posttransplant survival of both types of non-DCM patients.

Conclusions: Currently, waitlist and posttransplant survival is similar for all CMP phenotypes. VAD use is low in patients with non-DCM, although this did not increase waitlist mortality in adjusted analysis. Further studies in patients with non-DCM are needed to determine optimal timing and anatomic characteristics most likely to benefit from VAD implantation during the waitlist period.