Cardiac transplantation for pediatric restrictive cardiomyopathy: presentation, evaluation, and short-term outcome

The Journal of Heart and Lung Transplantation : the Official Publication of the International Society for Heart Transplantation
Matthew T KimberlingCharles E Canter

Abstract

Because of the poor prognosis of pediatric restrictive cardiomyopathy, transplantation has been proposed as the treatment of choice for this disease. We reviewed our experience with the presentation, evaluation, and short-term outcome in 8 pediatric patients with restrictive cardiomyopathy referred for transplantation. Potential reversibility of elevation in pulmonary vascular resistance was tested before transplantation with nitroprusside and nitric oxide, with follow-up cardiac catheterization performed 6 to 12 months after transplantation. The mean age of diagnosis of restrictive cardiomyopathy 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 patients had pulmonary vascular resistance indices greater than 10 Woods unit/m(2) and transpulmonary gradients greater than 20 mm Hg. The administration of nitroprusside and nitric oxide reversed elevated pulmonary resistance and transpulmonary gradients in all patients. Nitric oxide successfully reversed pulmonary vascular resistance in patients unresponsive to nitroprusside. All patients underwent successful transplantatio...Continue Reading

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Citations

Dec 12, 2013·Archives of Biochemistry and Biophysics·Shirin AkhterJ-P Jin
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