Abstract
BackgroundPulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension characterized by remodelling of the pulmonary venules. PVOD and pulmonary arterial hypertension share similar clinical presentation. It is important to differentiate between these two conditions as PVOD carries a worse prognosis and life-threatening pulmonary oedema may occur following the initiation of conventional therapy.Case presentationWe are reporting a case of pulmonary hypertension in a middle-aged lady who presented with hemoptysis and features of right heart failure. After extensive work up, no definite aetiology of pulmonary hypertension could be found out. Standard therapy did not cause any symptomatic improvement. After right heart catheterization, we got pre capillary pulmonary hypertension and along with typical findings in HRCT scan of thorax we established PVOD. We did not try lung biopsy as the procedure often lands up with complications. As we could not arrange for lung transplant we eventually lost the patient.ConclusionsHigh suspicion and thorough systematic evaluation helped us to diagnose PVOD in this case. Thus, early diagnosis should be the primary aim in suspected cases of PVOD so that we can prepare for lung transplant at the earliest.
Highlights
Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension characterized by remodelling of the pulmonary venules
We report a case of pulmonary hypertension in a middle-aged lady where we ruled out almost all common aetiologies and reached at the diagnosis of PVOD, though histopathological confirmation was not done
PVOD is often a fatal disease frequently misdiagnosed as idiopathic pulmonary artery hypertension
Summary
PVOD is often a fatal disease frequently misdiagnosed as idiopathic pulmonary artery hypertension. Though surgical lung biopsy is the gold standard for the diagnosis but it is usually avoided due to complications [3]. Heart failure and hemoptysis are the common complications which render high mortality in PVOD in spite of standard therapy of pulmonary hypertension, as evident from our case. Conventional treatment modalities are ineffective in the management of PVOD. Lung transplant is the only definitive management option at present and early identification with referral to a transplant centre is imperative. Though PVOD is a rare cause of PH, it must be kept in the differential when common causes are ruled out
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