Abstract
Truncus Arteriosus is a rare congenital anomaly that is characterized by a single great vessel arising from the heart giving rise to both the systemic and pulmonary circulations in a variety of configurations. The embryologic origins involve abnormal neural crest cell migration which is linked to Tbx proteins, which themselves are encoded on the chromosome 22q11 creating a close link to patients with DiGeorge Syndrome. Untreated, Truncus Arteriosus is a fatal condition, which requires early surgical intervention for optimal outcomes. Diagnosis is done by echocardiogram and is focused on describing the anatomy in detail, as well as identifying associated abnormalities. Surgical repair is standardized in general, with numerous details and nuances that are important for executing a safe and efficient repair, especially when additional abnormalities are identified. Postoperative care in the ICU centers around maintaining cardiac output and managing the effects of residual defects, truncal insufficiency, arrhythmias, or pulmonary hypertension. Results in the current era are excellent for straightforward Truncus and less so for those with complex associated anomalies. Late complications are related to truncal insufficiency and reoperation for conduit replacement.
References
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Bibevski, S., Friedland-Little, J., Gaies, M., Ohye, R.G. (2021). Truncus Arteriosus. In: da Cruz, E.M., Ivy, D., Hraska, V., Jaggers, J. (eds) Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care. Springer, London. https://doi.org/10.1007/978-1-4471-4999-6_48-2
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