Truncus Arteriosus: Surviving at 46 Years without Intervention

Abstract

The median survival of patients with truncus arteriosus is five weeks of age with rare cases reaching adulthood. The longest reported survivor with balanced ventricles, as the index case has, is 38 years of age. The index case is a 46-year old male with Type 3 truncus arteriosus. His case has never been documented in the English Literature as far as the author is aware.

Truncus arteriosus 5th decade transesophageal and transthoracic echocardiogram features

“…Type 4 truncus arteriosus with multifocal blood supply from bronchial arteries, and naturally occurring pulmonary stenosis protects the lungs from pulmonary hypertension, and is associated with increased longevity, as this index case confirms. [1][2][3][4][5]9,10]. The index case has competent atrioventricular and truncal valves and a single right coronary artery with normal distribution of the right and left coronary vessels.…”

“…The index case has competent atrioventricular and truncal valves and a single right coronary artery with normal distribution of the right and left coronary vessels. The normal coronary artery distribution does not affect his morbidity or mortality, and hence is of no clinical significance [1][2][3][4][5][6][7][8][9][10][11].…”

“…Systemic pulmonary collaterals indicate multifocal blood flow to lung segments and have produced pulmonary hypertension in the segments of lungs supplied by them, which has been documented by Chiaw et al [9]. The index case is the longest balanced biventricular survivor with truncus arteriosus Type 3 without intervention [7,8,10]. The longest documented survivor with Type 4 truncus arteriosus with multifocal pulmonary blood supply and no pulmonary arteries was 54 years of age [11].…”

“…There are seven other truncus arteriosus survivors beyond 30 years of age, excluding the index case. These include Types 1 and 4 truncus arteriosus and four others with no pulmonary stenosis and Eisenmenger syndrome [10][11][12][13][14].…”

“…Persistent truncus arteriosus in the 5th decade is a rare entity. Truncus arteriosus consists of a single great arterial trunk with the systemic, pulmonary, and coronary circulation, with no atretic aortic or pulmonary vessel [1][2][3][4][5][6][7][8][9][10]. The index case was diagnosed in infancy at 5 months of age with truncus arteriosus, after presenting with cyanosis and congestive cardiac failure requiring antifailure therapy, until late adolescence.…”