Persistent Truncus Arteriosus with Survival to the Age of 29 Years

Jang, Hee Cheol; Kim, Dae Su; An, Jeon Ok; Yoon, Sang Jeong; Kim, Young Seung; Jeong, Kyoung Tae; Park, Sun Chang; Kim, Ji Min

Abstract

The Truncus arteriosus is a congenital malformation in which only one great artery arises from the base of the heart and gives origin to the systemic, pulmonary and coronary arteries proximal to the aortic arch. Pulmonary blood flow is governed by the size of the pulmonary arteries and the pulmonary vascular resistance. In infancy, pulmonary blood flow is usually excessive because pulmonary vascular resistance is not greatly increased. Thus, despite an obligatory admixture of systemic and pulmonary venous blood in the common trunk, only minimal cyanosis is present. Rarely pulmonary blood blood flow is restricted by hypoplastic or stenotic pulmonary arteries arising from the truncus. The prognosis in persistent truncus arteriosus is very poor. the median age of survival of the 94 patients with the disease reported up to 1962 was only five weeks. The longest survival reported is the case of the man described by Carr et al who lived to the age of 36 years and 2 months. We report the case of a man with persistent truncus arteriosus who lives to the age of 29 years.