Management of pediatric cardiac transplantation candidates with pulmonary hypertension and high pulmonary vascular resistance

Abstract

Background and objectives. Right ventricular failure is an important cause of mortality and morbidity after orthotopic heart transplantation (OHT). The right ventricle of the donor may fail to accommodate to the high pulmonary vascular resistance (PVR) of the recipient. Pulmonary hypertension (PH) due to chronic heart failure with PVRi > 4 Wood units.m(2), transpulmonary gradient > 15 mmHg adversely affect the outcome of OHT. In this study we aimed to evaluate management strategies in our pediatric cardiac transplantation candidates with PH and high PVR prior to OHT.

Method. Twenty-six cardiac transplantation candidates (age: 10.2 +/- 4.6, 1-17 years) underwent cardiac catheterization for the determination of PVR and pulmonary arterial pressure. They were admitted to the hospital and received 1-3 days of intravenous (IV) vasodilator therapy; 0.5-3 mu g/kg/min nitroglyserin and/or 0.5-3 mu g/kg/min nitroprusside, 5-15 mu g/kg/min dobutamin and/or dopamin to keep systolic blood pressure above 80 mmHg.

Results. Thirteen patients had dilated cardiomyopathy (CMP), 11 had restrictive CMP, one had hypertrophic CMP and one had congenital heart disease (CHD). Nineteen of the 26 patients underwent OHT.

Mean pulmonary arterial pressure of the patients ranged between 11 and 82 mmHg (30.4 +/- 16 mmHg) and PVRi between 0.41-21.4 Wood units.m(2) (5.3 +/- 5.7). Nine patients had PVRi above 4 Wood units.m(2). Six of these patients had IV treatment for longer than three days and some received specific anti-PH treatment. Eventually they underwent a pulmonary vasoreactivity test with IV iloprost and six had PVRi <4 Wood units.m(2). Five of them underwent OHT.

Conclusion. Cardiac transplantation candidates with PH and high PVR should be evaluated after conditioning with vasodilator and inotropic treatment. Specific treatment for PH and vasoreactivity testing may help selected patients reenter the transplantation list.