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Published on: November 18, 2018
Pulmonary veno-occlusive disease: a case report
Rogério Teixeira1, Graça Castro, Lina Carvalho
1Serviço de Cardiologia, Hospitais da Universidade de Coimbra, Coimbra, Portugal.
Summary
Pulmonary veno-occlusive disease is a rare cause of pulmonary arterial hypertension. This case highlights the diagnostic challenges and poor prognosis associated with this condition, even with treatment.
Area of Science:
- Cardiology
- Pulmonology
- Pathology
Background:
- Pulmonary arterial hypertension (PAH) requires accurate diagnosis and understanding of underlying causes.
- Pulmonary veno-occlusive disease (PVOD) is a rare and severe form of pulmonary hypertension.
Observation:
- A 22-year-old female presented with symptoms suggestive of PAH.
- Diagnostic workup revealed elevated pulmonary artery pressure with normal pulmonary capillary wedge pressure.
- High-resolution CT and lung biopsy confirmed PAH with associated venous lesions, indicative of PVOD.
Findings:
- The patient showed a poor response to standard medical therapy including oxygen, warfarin, and bosentan.
- Clinical and functional status progressively worsened, leading to acute right heart failure.
- Open lung biopsy confirmed pulmonary veno-occlusive disease.
Implications:
- PVOD presents significant diagnostic challenges, often requiring invasive procedures like lung biopsy.
- Medical management of PVOD has limited efficacy, underscoring the need for early diagnosis and consideration of advanced therapies.
- This case underscores the dismal prognosis associated with PVOD and highlights the importance of recognizing this entity for appropriate patient management and referral.
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