BACKGROUND: The insertion of ventriculoatrial (VA) shunts for the treatment of hydrocephalus is thought to be associated with the development of pulmonary hypertension in adults. OBJECTIVES: It was the aim of this study to describe the frequency and the clinical spectrum of pulmonary hypertension in adults with VA shunts. METHODS: Patients with pulmonary hypertension were retrospectively evaluated from January 1999 to December 2006. RESULTS: Among the 575 patients with pulmonary hypertension, 6 (mean age 42.5 +/- 8.3 years) were identified as having received a VA shunt. Mean pulmonary artery pressure for these patients was 53.3 +/- 14.9 mm Hg. The interval between shunt placement and the diagnosis of pulmonary hypertension was 9-27 years (median 16.5). While ventilation perfusion scans showed multiple bilateral perfusion defects in all patients, chest CT or pulmonary angiography demonstrated pulmonary thromboembolism in only 2 of the 6 patients. These 2 patients subsequently underwent pulmonary endarterectomy. Another patient required heart-lung transplantation because of severe pulmonary hypertension; lung histology showed prominent eccentric medial hypertrophy and intimal proliferation without evidence of thromboembolism. Contrary to earlier reports, outcomes were generally good, with a 100% survival rate for the first 8 years following diagnosis. CONCLUSIONS: Severe pulmonary hypertension can develop in adult patients with VA shunts. Therefore, clinicians should consider pulmonary hypertension as a potential cause for respiratory symptoms in patients who have received VA shunts.
