Appendix
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Pulmonology

Pulmonary Veno-Occlusive Disease

Pulmonary veno-occlusive disease is a rare cause of group 1 pulmonary hypertension. It is characterized by having significant venous and capillary involvement, as opposed to other causes of group 1 pulmonary hypertension, in which the arteries are primarily involved. This disease has significant mortality, and early recognition remains paramount in its management. This activity reviews the evaluation and management of patients with pulmonary veno-occlusive disease and highlights the role of the

v1 · Reviewed Nov 8, 2026 by Automated Clinical Reviewer (Strict Verbatim v1)
  • This disease has significant mortality, and early recognition remains paramount in its management.
  • The prognosis is poor, and life expectancy is two years after symptom onset.
  • Most patients will present with signs of right-sided heart failure, such as leg edema, ascites, and volume overload.
  • Physical findings in these patients may include a split S2, loud P2, crackles, and cyanosis.
  • In the more advanced phase, RV function cannot remain matched to afterload, resulting in RV dilation, myocardial fibrosis, and low RV cardiac output, eventually leading to right-sided heart failure and volume overload from systemic venous congestion.
  • Chest radiographs will typically be performed and can note enlargement of central pulmonary arteries and signs of vascular congestion.
  • Computed tomography of the chest (CT chest) will typically be performed with evidence of elevated pulmonary pressures, such as the increased diameter of pulmonary vessels and possible right ventricular enlargement with bowing of the interventricular septum into the left ventricular lumen.
  • Additionally, there will be signs of pulmonary venous congestion such as mosaicism with centrilobular ground glass opacities, scattered pulmonary nodules, mediastinal lymph node enlargement, and smooth thickening of interlobular septa.
  • Spirometry typically shows normal values, though mild restrictive defects may be seen.
  • DLCO is typically reduced.
  • Echocardiography shows signs of pulmonary hypertension, such as elevated pulmonary arterial systolic pressure (PASP), right ventricular strain/dilation, and right ventricular dysfunction.
  • Ventilation and perfusion scanning (V/Q scanning) is typically performed in the workup of patients with suspected PAH.
  • Right heart catheterization is necessary for the diagnosis of pulmonary hypertension.
  • If a patient develops pulmonary edema during this testing, it can be suggestive of PVOD.
  • Another finding suggesting this diagnosis would be an abnormal rise and fall in PCWP when flushing the distal port.
  • Histology remains the gold standard for making a definite diagnosis.
  • For patients with a family history of PVOD or IPAH, some centers may test for BMPR2 or EIF2AK4 mutations.
  • General and supportive measures include oxygen supplements in hypoxemic patients to prevent worsening PH from hypoxic pulmonary vasoconstriction.
  • Diuretics should be used to optimize volume status.
  • There is no proposed recommendation for anticoagulation in PVOD patients.
  • If vasodilator therapy is considered, it is recommended to start with a single agent at lower doses with close monitoring and the availability of high-dose diuretic therapy.
  • It can be administered as a palliative measure to decrease the rate of decline in patients who will not be transplant candidates or as a bridge to transplant itself.
  • A lung transplant is the only curative option.
  • If suspected, these patients should be referred for lung transplant evaluation early in the course of the disease, as lung transplantation is the only therapy shown to increase life expectancy in patients with PVOD.
Diuretics
Not specified in source
Adult
Not specified in source
Renal
Not specified in source
Duration
Not specified in source
  • There were some reports of the benefit of immunosuppressive agents in patients with idiopathic and heritable PVOD and patients with connective tissue disease-associated PVOD.
  • The complications for these patients are similar to patients with pulmonary arterial hypertension.
  • Elevated pulmonary vascular resistance leads to increased afterload of the right ventricle (RV).
  • In the early phase, RV remains adapted to afterload by increasing contractility without increasing RV chamber dimension.
  • In the more advanced phase, RV function cannot remain matched to afterload, resulting in RV dilation, myocardial fibrosis, and low RV cardiac output, eventually leading to right-sided heart failure and volume overload from systemic venous congestion.
  • Outcomes after heart-lung or lung transplant for pulmonary veno-occlusive disease patients are similar to outcomes of other patients with pulmonary arterial hypertension requiring a transplant.
  • review
    Pulmonary Veno-Occlusive Disease. StatPearls [Internet] — Najam A. Siddiqui, Prangthip Charoenpong — StatPearls Publishing, 2023

Decision support only. Verify against local protocols and current labeling. Source: www.ncbi.nlm.nih.gov