Cautious epoprostenol therapy is a safe bridge to lung transplantation in pulmonary veno-occlusive disease

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?min -1 ?m) and indexed pulmonary vascular resistance (28.4¡8.4 to 17¡5.2 Wood units?m -2 ; all p,0.01). A nonsignificant improvement in the 6-min walk distance was also observed (+41 m, p50.11). 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.

Pulmonary veno-occlusive disease misdiagnosed as idiopathic pulmonary arterial hypertension

“…epoprostenol for 3-4 months was found to improve clinical and haemodynamic parameters in 12 patients with PVOD, suggesting that such therapy may be a useful bridge to lung transplantation [9]. Treatment of our patient with bosentan, in combination with high doses of furosemide to reduce lymphatic and venous congestion, may have contributed to improvements in her clinical status and may have slowed disease progression.…”

Pulmonary veno-occlusive disease: advances in clinical management and treatments

“…However, none of these patients developed pulmonary edema during acute testing with NO, nor were any of the clinical, functional or hemodynamic characteristics predictive of the onset of this complication after initiation of specific PAH therapy [8]. In selected cases, there have been reported mild clinical improvement or stabilization with continuous intravenous epoprostenol [10, [80][81][82][83], iloprost therapy [84,85] and oral sildenafil [86,87]. In contrast with idiopathic PAH, the benefits of specific PAH treatments in patients with PVOD remain unclear.…”

“…As the prognosis in PVOD patients is worse than in idiopathic PAH, and due to limited medical therapy, lung transplantation should be considered and discussed early in the course of PVOD or suspected PVOD (HRCT findings, worsening symptoms on treatment or refractoriness to medical therapy, development of worsening hypoxemia or chronic heart failure). In these patients, PAH-specific therapies may serve as a bridge to lung transplantation [5,83]. The survival has been suggested to be broadly similar to that in idiopathic PAH.…”

Ventilation/perfusion lung scan in pulmonary veno-occlusive disease

“…Pulmonary angiography revealed attenuation of peripheral pulmonary arteries without radiological signs of chronic thromboembolic disease. DISCUSSION PVOD is a rare and severe condition with a poor prognosis that requires an early diagnosis because of the need for specific management, including high-dose diuretics, careful management of specific PAH therapy and early referral for lung transplantation [11,13,23,24]. The main risk for these patients is the development of acute pulmonary oedema with the use of PAH-specific drugs (prostacyclin, prostacyclin analogues, endothelin receptor antagonists or phosphodiesterase-5 inhibitors), which may promote fluid extravasation from the capillaries to the alveolus by acting mainly as arteriolar vasodilators against a venular obstruction due to specific remodelling [8,12,13].…”

“…PVOD shares many similarities with idiopathic PAH, in particular, clinical features and haemodynamic characteristics, and therefore it can be difficult to distinguish PVOD from idiopathic PAH [8]. PVOD patients exposed to PAH-specific treatments may develop abrupt and potentially life-threatening deterioration due to severe pulmonary oedema [8,12,13].…”