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Pulmonary veno-occlusive disease: an uncommon cause of pulmonary hypertension
  1. Kyle Masters1,
  2. Steven Bennett2
  1. 1Department of Medicine, Madigan Army Medical Center, Tacoma, Washington, USA
  2. 2Department of Pulmonary and Critical Care Medicine, Madigan Army Medical Center, Tacoma, Washington, USA
  1. Correspondence to Dr Kyle Masters, kyle.masters{at}


Pulmonary veno-occlusive disease (PVOD) is a rare and challenging cause of pulmonary hypertension. Clinical presentation is non-specific, including dyspnoea, cough and fatigue. Diagnosis of PVOD is typically based on high clinical suspicion with a definitive diagnosis confirmed by histology. Our case involves a healthy 21-year-old man who developed dyspnoea on exertion at an elevated altitude during deployment to Afghanistan. His work-up included an echocardiogram, a high-resolution CT scan, V/Q scan, pulmonary function tests with diffusion capacity, and a cardiac catheterisation with vasodilator challenge. Initially diagnosed with vasodilator responsive pulmonary arterial hypertension, an oral vasodilator was given with subsequent development of non-cardiogenic pulmonary oedema, thus confirming a clinical diagnosis of PVOD. He was medically stabilised with diuretic therapy, but developed progressive right-ventricular failure. For definitive treatment, he underwent a successful bilateral lung transplant. Explanted lung histology confirmed the diagnosis of PVOD.

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