Elevated pulmonary vascular resistance in a transplantation candidate should be viewed as potentially reversible, and there are several options for therapy. We describe a young patient with congenital restrictive cardiomyopathy and a markedly elevated pulmonary artery pressure. The patient underwent successful orthotopic heart transplantation after pharmacologic lowering of the pulmonary artery pressure with a new drug combination of milrinone and nesiritide. The length of therapy can be extended to 3 days to allow for determining the pulmonary vascular responsiveness.
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