Long-Term Survival in Truncus Arteriosus Communis Type A1 Associated with Ehlers-Danlos Syndrome

Abstract

Long-term survival is rare in patients with truncus arteriosus communis type A1 due to the early appearance of pulmonary artery obstructive changes. A 48-year-old woman with truncus arteriosus communis type A1 diagnosed with chest radiography, two-dimensional echocardio graphy, and cardiac catheterization is presented. The right ventricular pressure was 108 mm Hg , whereas the systolic pulmonary artery pressure was 98 mm Hg. A concomitant diagnosis of the Ehlers-Danlos syndrome was made with clinical findings. The association of Ehlers- Danlos syndrome, in which a collagen synthesis defect is observed in arterial vessels, might have interfered with pulmonary artery intimal proliferation, thus precluding the appearance of advanced pulmonary artery luminal obstructive changes and allowing long-term survival.