| Summary: | Abstract Background Pulmonary hypertension in young children can be due to a myriad of conditions. Few aetiologies of pulmonary hypertension are potentially reversible. An extensive workup for the cause of pulmonary hypertension is a must before attributing it to idiopathic pulmonary hypertension. We describe an uncommon aetiology of pulmonary hypertension in a young boy. Case presentation A 12-year-old child, with past history of tubercular pleural effusion, presented with dyspnoea on exertion and easy fatiguability for 2 years. He was evaluated elsewhere and was being treated as primary pulmonary hypertension with pulmonary vasodilators. The child was revaluated since the clinical features were not completely favouring the diagnosis. On detailed evaluation, a diagnosis of constrictive pericarditis was made. He was referred for pericardiectomy. Conclusions Constrictive pericarditis presenting with severe pulmonary hypertension without congestive symptoms is very rare. In patients presenting with pulmonary hypertension, always look for a reversible cause before labeling them as idiopathic PAH.
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