Long-term survival in truncus arteriosus communis type A1 associated with Ehlers-Danlos syndrome--a case report.
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 echocardiography, 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.
Carvalho
Gustavo
G
Cardiology Division, Faculdade de Medicina de São José do Rio Preto, Brazil.
Silva
Alexander A
AA
Bestetti
Reinaldo B
RB
Leme-Neto
Antonio C
AC
D003937:Diagnosis, Differential; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000175:diagnosis; D005260:Female; D006801:Humans; D008875:Middle Aged; D014339:Truncus Arteriosus, Persistent / Q000150:complications* / Q000175:diagnosis