Development of Pulmonary Hypertension in Adults after Ventriculoatrial Shunt Implantation

Abstract

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.