This report describes a case series of six patients with congenital common atrium who developed pulmonary vascular disease. Three developed early pulmonary vascular disease within their first year of life, while the others became symptomatic at ages 2, 5, and 17. Four of the six underwent surgical repair, and five of the six patients are being treated on targeted pulmonary hypertension therapy. Based on our observations, the clinical course of children with common atrium may differ from patients with a large atrial septal defect. Early monitoring and surgical correction, if necessary, may prevent the onset of severe pulmonary vascular disease.
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http://dx.doi.org/10.1007/s00246-011-9919-6 | DOI Listing |
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