Pulmonary veno-occlusive disease
ICD-10 I28.8 · ICD-11 BB01.01

Treatment of Pulmonary Veno-Occlusive Disease in Idiopathic, Heritable, or Connective Tissue Disease-Associated Forms

Clinical Scenario

This protocol applies to patients with pulmonary veno-occlusive disease (PVOD) presenting in one of the following recognised forms:

Reports have indicated potential benefit from immunosuppressive agents across these PVOD subtypes, underscoring the importance of recognising the underlying aetiology when planning management.

Management Approach

The protocol centres on general and supportive measures aimed at stabilising pulmonary haemodynamics and optimising the patient's clinical status.

The complete protocol specifies the structured approach to supportive interventions and their sequencing — the full regimen is available via the link below.
Instant Access to Structured Evidence-Based Regimens

References

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.

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.

View source ↗