Cardiac transplantation for XXXX : presentation, evaluation, and short-term outcome.

@entity1 @entity296 @entity1854 @entity842
BACKGROUND: Because of the poor prognosis of @entity296 , transplantation has been proposed as the treatment of choice for this disease. METHODS: We reviewed our experience with the presentation, evaluation, and short-term outcome in 8 pediatric @entity1 with @entity296 referred for transplantation. Potential reversibility of elevation in pulmonary vascular resistance was tested before transplantation with @entity1854 and @entity842 , with follow-up cardiac catheterization performed 6 to 12 months after transplantation. RESULTS: The mean age of diagnosis of @entity296 was 6.3 years and the mean interval from diagnosis to referral for transplantation was 3.6 years. Elevation of pulmonary vascular resistance was common and tended to progress with longer follow-up. Three of the 8 @entity1 had pulmonary vascular resistance indices greater than 10 Woods unit/m(2) and transpulmonary gradients greater than 20 mm Hg. The administration of @entity1854 and @entity842 reversed elevated pulmonary resistance and transpulmonary gradients in all @entity1 . @entity842 successfully reversed pulmonary vascular resistance in @entity1 unresponsive to @entity1854 . All @entity1 underwent successful transplantation and follow-up catheterization revealed normal pulmonary hemodynamics in each @entity1 . CONCLUSIONS: @entity296 can be associated with marked elevation in the pulmonary vascular resistance, which may contribute to the poor prognosis in these @entity1 and potentially make cardiac transplantation problematic. Orthotopic cardiac transplantation can be successfully performed in @entity1 who demonstrate reversibility of pulmonary vascular resistance. @entity842 appears to be the best agent to demonstrate reversibility of pulmonary resistance in these @entity1 .
