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Published on: July 18, 2014
Pediatric waitlist and heart transplant outcomes in patients with syndromic anomalies
Sarah J Wilkens1, James Priest1, Beth D Kaufman1
1Lucile Packard Children's Hospital, Stanford University, Palo Alto, CA, USA.
Insights
Systemic anomalies (SA) in pediatric patients awaiting heart transplant (HT) did not impact waitlist mortality or post-transplant outcomes. Heart transplantation is a safe and feasible option for these patients.
Area of Science:
- Pediatric Cardiology
- Transplantation Medicine
- Genetics and Rare Diseases
Background:
- Systemic anomalies (SA) can present complex challenges in pediatric patients.
- The impact of SA on outcomes for pediatric heart transplant (HT) candidates is not well-defined.
Purpose of the Study:
- To investigate the effect of SA on waitlist and post-HT outcomes in pediatric patients.
- To assess the safety and feasibility of HT in children with SA.
Main Methods:
- Retrospective review of pediatric patients listed for HT from 2009-2018.
- Patients with underlying syndromes (chromosomal anomalies, skeletal myopathies, connective tissue disorders, mitochondrial disease, etc.) were identified.
- Waitlist and post-HT outcomes were compared between patients with and without SA.
Main Results:
- 9% of pediatric HT candidates had associated SA.
- Waitlist survival was similar between groups (P=1.0).
- Post-HT outcomes, including survival to discharge (93% vs 95%, P=0.6) and length of stay (18 vs 18 days, P=0.8), were not significantly different.
Conclusions:
- SA in pediatric HT candidates does not increase waitlist mortality or post-transplant morbidity.
- Heart transplantation is a safe and feasible treatment option for pediatric patients with various systemic anomalies.
Purpose:
We sought to determine whether the presence of a systemic SA with potential complicating factors affects waitlist and post-HT outcomes in pediatric patients.
Methods:
This is a single-center retrospective review of pediatric patients listed for HT between January 1, 2009, and July 1, 2018. Patients were selected based on the presence of any underlying syndromes, which included chromosomal anomalies, skeletal myopathies, connective tissue disorders, mitochondrial disease,and other systemic disorders. Waitlist and post-HT outcomes were compared to those without SA.
Results:
A total of 243 patients were listed for HT, of which 21 (9%) patients had associated SA. Of those, 16 (76%) survived to transplant, 3 (14%) died while on the waitlist, 1 (5%) improved and was removed from the waitlist, and 1 (5%) patient is currently listed. Waitlist survival was not different between those with/without an associated syndrome (P = 1.0). Among those who survived to HT, there was no difference in listing days (70 vs 90, P = .8), survival to hospital discharge [14 (93%) vs 150 (95%), P = .6], post-HT intubation days (2 vs 2 days, P = .6), or post-HT hospital length of stay (18 vs 18 days, P = .8). Overall survival during the study period post-HT was not different between groups (P = .8).
Conclusion:
A SA was present in 9% of pediatric patients wait-listed for HT, but was not associated with an increased waitlist mortality or post-HT hospital morbidity or long-term survival. For several anomalies, HT is safe and feasible.
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