Goal-oriented therapy in pulmonary veno-occlusive disease: a word of caution.

The evidence for using pulmonary arterial hypertension (PAH) therapies such as prostacyclin derivatives, endothelin receptor antagonists and type-5 phosphodiesterase inhibitors in pulmonary veno-occlusive disease (PVOD) is modest and conflicting [1, 2]. Indeed, despite several reports of clinical deterioration and life-threatening pulmonary oedema in PVOD patients receiving PAH therapies, we have recently shown in the European Respiratory Journal that cautious use of continuous intravenous epoprostenol alone or in combination with endothelin receptor antagonists may be useful as a bridge therapy to lung transplantation [2].