Abstract
Pulmonary veno-occlusive disease (PVOD) carries a poor prognosis and lung transplantation is the only curative treatment. In PVOD, epoprostenol therapy is controversial, as this condition may be refractory to specific therapy with an increased risk of pulmonary oedema. We retrospectively reviewed clinical, functional and haemodynamic data of 12 patients with PVOD (10 with histological confirmation) treated with continuous intravenous epoprostenol and priority listed for lung transplantation after January 1, 2003. All PVOD patients had severe clinical, functional and haemodynamic impairment at presentation. Epoprostenol was used at low dose ranges with slow dose increases and high dose diuretics. Only one patient developed mild reversible pulmonary oedema. After 3-4 months, improvements were seen in the New York Heart Association functional class (class IV to III in seven patients), cardiac index (1.99+/-0.68 to 2.94+/-0.89 L x min(-1) x m(-2)) and indexed pulmonary vascular resistance (28.4+/-8.4 to 17+/-5.2 Wood units x m(-2); all p<0.01). A nonsignificant improvement in the 6-min walk distance was also observed (+41 m, p = 0.11). Two patients died, one patient was alive on the transplantation waiting list on December 1, 2008 and nine patients were transplanted. Cautious use of continuous intravenous epoprostenol improved clinical and haemodynamic parameters in PVOD patients at 3-4 months without commonly causing pulmonary oedema, and may be a useful bridge to urgent lung transplantation.
Highlights
All Pulmonary veno-occlusive disease (PVOD) patients had severe clinical, functional and haemodynamic impairment at presentation
Baseline characteristics Between January 1, 2003 and December 1, 2008, 12 PVOD patients treated with continuous intravenous epoprostenol and listed for lung transplantation were identified
Eleven patients (91.7%) had a confirmed diagnosis of PVOD: 10 patients had histologically confirmed PVOD on explanted lungs and one patient with characteristics of PVOD developed pulmonary oedema with calcium channel blocker therapy
Summary
All PVOD patients had severe clinical, functional and haemodynamic impairment at presentation. Cautious use of continuous intravenous epoprostenol improved clinical and haemodynamic parameters in PVOD patients at 3–4 months without commonly causing pulmonary oedema, and may be a useful bridge to urgent lung transplantation. PVOD patients have a very poor prognosis and the recommended treatment is lung transplantation [4,5,6,7] In these patients with severe pulmonary vascular disease, the waiting time for transplantation may exceed their expected survival. PULMONARY VASCULAR DISEASE therapeutic class effect but may occur with all specific PAH therapies In this series [6], nearly half of the PVOD patients treated with epoprostenol developed pulmonary oedema. The aim of the present study was to report our experience on the use of epoprostenol in PVOD patients awaiting lung transplantation
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