title: Pulmonary veno-occlusive disease: an uncommon cause of pulmonary hypertension.
abstract: 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.
authors: Department of Medicine, Madigan Army Medical Center, Tacoma, Washington, USA. kyle.masters@us.army.mil
authors: Kyle
authors: K
authors: Masters
authors: Steven
authors: S
authors: Bennett
mesh_terms: D003937:Diagnosis, Differential; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D008168:Lung / Q000473:pathology; D016040:Lung Transplantation; D008297:Male; D011668:Pulmonary Veno-Occlusive Disease / Q000150:complications* / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D011859:Radiography; D055815:Young Adult
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