Outcomes of pediatric patients with hypertrophic cardiomyopathy listed for transplant
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Published on: July 18, 2014
Robert Gajarski1, David C Naftel, Elfriede Pahl
1Division of Pediatric Cardiology, C.S. Mott Children's Hospital, Ann Arbor, Michigan 48109, USA. rjgaj@med.umich.edu
Insights
Pediatric hypertrophic cardiomyopathy (HCM) patients under one year old and listed as UNOS status 1 face the highest mortality risk while awaiting heart transplantation (HTx). Further research is needed to identify optimal timing for HTx in these children.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Transplantation Medicine
Background:
- Pediatric hypertrophic cardiomyopathy (HCM) presents a variable clinical course.
- Indications for heart transplantation (HTx) in children with HCM are not well-defined.
- This study examines outcomes and risk factors for adverse events in pediatric HCM patients listed for HTx.
Purpose of the Study:
- To investigate the outcomes of pediatric patients with HCM listed for heart transplantation.
- To identify risk factors associated with adverse outcomes in this patient population.
- To inform decisions regarding the timing of HTx listing and patient selection.
Main Methods:
- A multicenter, event-driven data registry was utilized.
- Data included patients under 18 years old listed for HTx between January 1993 and December 2007.
- Outcomes and risk factors for mortality were analyzed.
Main Results:
- Of 3,147 children listed for HTx, 77 had HCM. 65% underwent HTx within one year.
- Mortality while awaiting HTx was higher for those listed under one year of age (p=0.0005).
- Risk factors for death post-listing included UNOS status 1 and younger age. 10-year survival post-HTx was 47% for HCM vs. 63% for non-CM.
Conclusions:
- Children with HCM listed at <1 year of age and UNOS status 1 exhibit the highest pre-HTx mortality.
- Additional risk factors require identification to optimize HTx timing and patient selection.
- This research highlights critical factors influencing outcomes in pediatric HCM patients undergoing HTx evaluation.
Background:
The course of pediatric hypertrophic cardiomyopathy (HCM) is variable, and the indications for heart transplantation (HTx) are ill defined. This study investigated outcomes and risk factors for adverse outcomes after listing for HTx.
Methods:
A multicenter, event-driven data registry of patients aged < 18 years listed for HTx between January 1993 and December 2007 was used.
Results:
During the study period, 3,147 children were listed for HTx (mean age, 7.6 +/- 0.8 years). Of l,320 with CM at listing, 77 (6%) had HCM (61% boys; 79% white); 59% were United Network of Organ Sharing (UNOS) status I, 30% were receiving inotropes, 27% were ventilated, and 8% required extracorporeal membrane oxygenation. Arrhythmia had occurred in 27%, and 14% had failure to thrive. Within 1 year, 65% underwent HTx. Overall, 25 patients died after listing: 11 (14%) while waiting and 14 of 49 (29%) after HTx. Pre-HTx survival was lower for those listed at age < 1 year (p = 0.0005). Risk factors for death after listing included UNOS status 1 (p = 0.01) and younger age (relative risk, 2.3; p = 0.001). Late (10-year) survival after HTx for HCM patients was 47% vs 63% for non-CM patients within the database.
Conclusions:
Children with HCM listed for HTx age < 1 year and UNOS status 1 have the highest mortality awaiting HTx. A more rigorous identification of additional risk factors should be performed to better define timing of listing and which patient sub-group may derive optimal benefit from HTx.
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