Common arterial trunk

Common arterial trunk (also known as truncus arteriosus or persistent truncus arteriosus) is a rare congenital heart defect in which a single large blood vessel (the truncus) arises from the heart instead of the normal two separate vessels (the aorta and the pulmonary artery). During normal fetal development, the truncus arteriosus divides into these two great arteries; in this condition, that separation fails to occur. The single trunk overrides a ventricular septal defect (VSD), receiving blood from both the right and left ventricles, resulting in mixing of oxygen-rich and oxygen-poor blood that is then distributed to the body, lungs, and coronary arteries. Infants with common arterial trunk typically present in the neonatal period with signs of congestive heart failure, cyanosis (bluish discoloration of the skin due to low oxygen levels), tachypnea (rapid breathing), poor feeding, failure to thrive, and excessive pulmonary blood flow. A single semilunar (truncal) valve may be abnormal, with regurgitation or stenosis. Without treatment, the condition carries a very high mortality rate in the first year of life due to heart failure and progressive pulmonary vascular disease (Eisenmenger syndrome). The condition is classified into several types (most commonly using the Van Praagh or Collett-Edwards classification) based on the origin of the pulmonary arteries from the trunk. Treatment is surgical and is typically performed in the neonatal period. The primary repair involves separating the pulmonary arteries from the truncal vessel, closing the VSD to direct left ventricular outflow into the reconstructed aorta, and establishing right ventricle-to-pulmonary artery continuity using a conduit (often a homograft). Patients require lifelong cardiac follow-up, as conduit replacement surgeries are commonly needed as the child grows. Common arterial trunk may occur as an isolated defect or in association with genetic conditions, most notably 22q11.2 deletion syndrome (DiGeorge syndrome), which is found in a significant proportion of affected individuals. Other associated anomalies may include interrupted aortic arch, coronary artery anomalies, and truncal valve abnormalities.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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