From the Guidelines
Treatment for pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) should be undertaken only at centers with extensive experience in the management of pulmonary hypertension (PH), and patients should be fully informed about the risks, with lung transplantation being the only curative therapy. The management of PVOD/PCH is challenging due to the high risk of pulmonary edema associated with vasodilator therapy 1.
Key Considerations
- There is no established medical therapy for PVOD/PCH, and vasodilators, especially prostanoids, must be used with great caution due to the high risk of pulmonary edema 1.
- Patients with PVOD/PCH should be referred to a transplant center for evaluation as soon as the diagnosis is established, as lung transplantation is the only curative therapy for these conditions 1.
- Diuretics, such as furosemide and spironolactone, may be needed to manage fluid overload, and supplemental oxygen should be provided to maintain oxygen saturation above 90% 1.
- Anticoagulation with warfarin may be considered in selected patients, but regular monitoring for pulmonary edema is essential when initiating vasodilator therapy 1.
Treatment Approach
- Initial treatment may include phosphodiesterase-5 inhibitors like sildenafil or tadalafil, which are generally safer options, but endothelin receptor antagonists should be used cautiously due to the risk of precipitating pulmonary edema 1.
- Atrial septostomy may be considered but is usually limited by hypoxemia 1.
- The use of investigational therapies, such as angiogenesis inhibitors, is not currently recommended due to limited evidence and potential risks 1.
Monitoring and Follow-up
- Regular monitoring for pulmonary edema is crucial when initiating vasodilator therapy, as these medications can worsen fluid accumulation in the lungs due to the unique vascular pathology of PVOD/PCH 1.
- Patients should be closely followed up by a multidisciplinary team with expertise in PH and lung transplantation to optimize their management and outcomes 1.
From the Research
Treatment Options for PVOD/PCH
The treatment options for Pulmonary Veno-Occlusive Disease (PVOD) and Pulmonary Capillary Hemangiomatosis (PCH) are limited and often challenging.
- Lung transplantation is considered the best treatment option for PVOD 2
- Sequential combination therapy may be used to stabilize patients while they wait for lung transplantation 2
- Vasodilators may not be effective in PVOD and may worsen hemodynamic status 2
- Endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, and prostacyclin analogs are used to treat pulmonary arterial hypertension (PAH), but their effectiveness in PVOD/PCH is not well established 3, 4, 5
- Treatment of PVOD/PCH often requires a multidisciplinary approach and close monitoring of the patient's condition 2
Medications Used in Treatment
Several medications are used to treat PAH, which may also be used in the treatment of PVOD/PCH, including:
- Endothelin receptor antagonists, such as bosentan and sitaxsentan 4
- Phosphodiesterase type 5 inhibitors, such as sildenafil and tadalafil 5
- Prostacyclin analogs, such as epoprostenol 6
- Soluble guanylate cyclase stimulators and prostacyclin-receptor agonists may also be used in the treatment of PAH 6