Case report: A young man with a rare grown-up congenital heart disease.

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The persistent truncus arteriosus is an extremely rare condition, moreover in grown-up patients. It accounts for approximately 0.3% of congenital heart disease and is due to the lack of formation of the helical septal trunk that allows the division of the outflow tract into aortic and pulmonary artery. The authors here present a case of a 17-yearold male who was admitted to our hospital’s cardiology department with symptoms of dyspnea and cyanosis. He was transferred from another center with the diagnosis of ventricular septal defect. We decided to perform a transthoracic echocardiography (TTE) that demonstrated a single great vessel connected to the two ventricles, which suggested a truncus arteriosus. The computed tomography (CT) confirmed our diagnosis. The authors report this case, because there are few patients with this condition reaching young hood.

Truncus arteriosus (TA) is a rare, congenital heart defect characterized by a single great artery giving rise to the ascending aorta, pulmonary arteries, and coronary arteries. TA ocurred in 0.21%-0.34% of all cases of congenital heart disease and presents with a right-sided aortic arch or an interrupted aortic arch (IAA) in 18-36% and 11-14% of cases, respectively . TA is classified using the Van Praagh and Collette and Edwards classification systems, depending on the origins of the pulmonary arteries. Collett and Edwards based their system only on the origins of the pulmonary vascular system, while Van Praagh also took into account aortic abnormalities. In this condition, the systemic circulation, pulmonary circulation, and coronary circulation receive a mixture of oxygenated and deoxygenated blood; therefore, cyanosis may be seen in early postnatal life. The presentation includes, as well, congestive cardiac failure within few weeks of life and commonly present with complaints of dyspnea with feeding and failure to thrive.
TA is classified using the Van Praagh and Collette and Edwards classification systems, depending on the origins of the pulmonary arteries. Collett and Edwards based their system only on the origins of the pulmonary vascular system, while Van Praagh also took into account aortic abnormalities.

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