Cardiopulmonary complications are infrequent in patients with ventriculoatrial shunts (VAS). We report the case of an 18-year-old man with congenital hydrocephalus who developed pulmonary arterial hypertension (PAH) in the long-term follow-up after a VAS procedure. He presented with progressive exertional dyspnea, stabbing chest pain and acral cyanosis.
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