Potential for reversibility of pulmonary vascular obstructive disease in children after cardiac transplantation

Abstract

Patients with pulmonary vascular obstructive disease (PVOD) are usually not considered candidates for orthotopic cardiac transplantation, because the normal donor's right ventricle may be unable to function because of an acute increase in afterload, especially in the postischemic situation of the arrested and transported donar heart. The accepted guideline is that pulmonary vascular resistance (PVR) must be 8 Wood units (mm Hg/liters/min) or lower during maximal medical management. 1,2 Patients whose PVR is between 4 and 8 Wood units are marginal candidates. Combined heart-lung transplantation or heterotopic cardiac transplantation is generally believed to be necessary when the PVR is 8 units. However, among children with a variety of congenital defects, PVOD is often reversible after correction, especially when correction is performed at a young age. This was the case in the patient described herein whose cardiac transplantation was successful despite a PVR of nearly 13 Wood units.