Abstract | BACKGROUND: METHODS: RESULTS: 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 transplantation and follow-up catheterization revealed normal pulmonary hemodynamics in each patient. CONCLUSIONS: Pediatric restrictive cardiomyopathy can be associated with marked elevation in the pulmonary vascular resistance, which may contribute to the poor prognosis in these patients and potentially make cardiac transplantation problematic. Orthotopic cardiac transplantation can be successfully performed in patients who demonstrate reversibility of pulmonary vascular resistance. Nitric oxide appears to be the best agent to demonstrate reversibility of pulmonary resistance in these patients.
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Authors | Matthew T Kimberling, David T Balzer, Russel Hirsch, Eric Mendeloff, Charles B Huddleston, Charles E Canter |
Journal | The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation
(J Heart Lung Transplant)
Vol. 21
Issue 4
Pg. 455-9
(Apr 2002)
ISSN: 1053-2498 [Print] United States |
PMID | 11927222
(Publication Type: Evaluation Study, Journal Article)
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Topics |
- Cardiomyopathy, Restrictive
(diagnosis, surgery)
- Child
- Child Welfare
- Child, Preschool
- Female
- Follow-Up Studies
- Heart Transplantation
- Humans
- Male
- Missouri
- Treatment Outcome
- Vascular Resistance
(physiology)
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