Heart and heart–lung transplantation for idiopathic restrictive cardiomyopathy in children
Key result
Pre-transplant prostacyclin links to a ~37% lower transpulmonary gradient in children with restrictive cardiomyopathy.
- P<0.05
- n=18
Why the study?
The outcome of cardiac transplantation for restrictive cardiomyopathy in children and the ability of new strategies to modulate high pulmonary vascular resistance were not well characterized.
What are the outcomes of cardiac transplantation and the effect of pre-transplantation prostacyclin on pulmonary vascular resistance in children with restrictive cardiomyopathy?
Population
18 children with idiopathic restrictive cardiomyopathy referred for transplantation assessment
Comparison
Pre-transplantation prostacyclin treatment vs no prostacyclin treatment
Design
Retrospective case note analysis
Authors
Discussion
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Member takes
Implication
May support prostacyclin to facilitate transplantation in select cases; hypothesis-generating and leaves open need for prospective trials.
Study Design
Type
Observational (n=18)
Multicenter
No
Structured PICO
What are the outcomes of cardiac transplantation and the effect of pre-transplantation prostacyclin on pulmonary vascular resistance in children with restrictive cardiomyopathy?
P
Population
18 children (mean age 6.1 years, 55.6% female) with restrictive cardiomyopathy referred for transplantation assessment.
E
Exposure
Cardiac transplantation (14 orthotopic, 2 heterotopic, 2 heart-lung) and pre-transplantation prostacyclin (in 5 patients for mean 57 days).
O
Outcome
Outcome of cardiac transplantation and reduction in transpulmonary gradient with pre-transplantation prostacyclin.hard clinical
Main Result
Absolute Event Rate: 17% vs 27%
p-value: p=< 0.05
In children with restrictive cardiomyopathy, cardiac transplantation is often required within four years of diagnosis, and pre-transplantation prostacyclin can effectively reduce elevated pulmonary vascular resistance to allow for orthotopic transplantation.
Cite This Study
Fenton et al. (2005) conducted an observational in restrictive cardiomyopathy (n=18). Pre-transplantation prostacyclin vs. Baseline (before treatment) was evaluated on Transpulmonary gradient (p=< 0.05). Pre-transplantation prostacyclin reduced mean transpulmonary gradient from 27 mm Hg to 17 mm Hg (p < 0.05) in children with restrictive cardiomyopathy.