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DOI: 10.1055/s-0035-1544411
Left Ventricular Assist Device Implantation Reverses Fixed Pulmonary Hypertension in Paediatric Patients with Restrictive Cardiomyopathy Prior to Heart Transplantation
Objective: Fixed pulmonary hypertension in pediatric patients with restrictive cardiomyopathy is a contraindication for heart transplantation due to the high risk of post-transplant right heart failure. We sought to determine whether left ventricular assist device (LVAD) implantation reverses fixed pulmonary hypertension in this high-risk patient group, thereby facilitating staged heart transplantation.
Materials and methods: Two pediatric patients with restrictive cardiomyopathy and fixed pulmonary hypertension (mean age 12.5 ± 0.7 years, female 100%) received a Heartware HVAD® LVAD in preparation for cardiac transplantation. Cardiac catheterisation with vasodynamic testing was performed before LVAD implantation, after 88 ± 45 days of LVAD support to establish eligibility for transplant listing and after cardiac transplantation. Clinical data were monitored.
Results: All patients received optimised heart failure and pulmonary vasodilator therapy, were in NYHA class IV and had fixed pulmonary hypertension (PVR 10.55 ± 11.47 WU, mean PAP 66 ± 39 mm Hg) before LVAD implantation. After 88 ± 45 days of LVAD support pulmonary hypertension improved in all patients (PVR 3.96 ± 3.37 WU, mean PAP 40 ± 25 mm Hg). Subsequent heart transplantation was uneventful in all patients and overall survival 100%. Post transplant vasodynamic testing revealed sustained reversal of pulmonary hypertension (mean PAP 19 ± 1 mm Hg). No complications occurred during LVAD support.
Conclusion: Left ventricular assist device implantation decreases fixed pulmonary hypertension pediatric patients with restrictive cardiomyopathy and thereby facilitates cardiac transplantation.