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

A Case Report

Gustavo Carvalho, MD

Cardiology Division, Faculdade de Medicina de São José do Rio Preto, São José do Rio Preto, Brazil.

Alexander A. Silva, MD

Cardiology Division, Faculdade de Medicina de São José do Rio Preto, São José do Rio Preto, Brazil.

Reinaldo B. Bestetti, MD, PhD

Cardiology Division, Faculdade de Medicina de São José do Rio Preto, São José do Rio Preto, Brazil.

Antonio C. Leme-Neto, MD

Cardiology Division, Faculdade de Medicina de São José do Rio Preto, São José do Rio Preto, Brazil.

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.

Angiology, Vol. 53, No. 3, 363-365 (2002)
DOI: 10.1177/000331970205300317
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