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

To the Editors : 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. The term “goal-oriented therapy” describes a relatively novel therapeutic approach in the field of PAH, which focuses on the question of when a response to therapy can be considered sufficient and when it cannot 3. This question is of fundamental importance in guiding therapeutic decisions: that is, when to maintain a therapy, when to switch from one medication to another and when to combine several medications. As an inadequate response to first-line PAH medical treatments is frequently observed in PVOD patients 1, it is likely that a significant proportion of patients showing insufficient response to first-line PAH therapy could be candidates for sequential add-on combination therapy and that such a strategy might increase the risk of developing pulmonary oedema. This eventuality may be more likely in scleroderma patients, who are prone to present with significant pulmonary venous involvement 4. We report a case of …