Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2021 Oct;31(10):1563-1570.
doi: 10.1017/S1047951121002997. Epub 2021 Jul 26.

Total anomalous origin of the coronary arteries from the pulmonary artery: a systematic review

Timothy M Guenther  1   2 , Sarah A Chen  1 , Joshua D Gustafson  2 , Frank F Ing  3 , Julie A Brothers  4 , Gary W Raff  1
Affiliations

Total anomalous origin of the coronary arteries from the pulmonary artery: a systematic review

Timothy M Guenther et al. Cardiol Young. 2021 Oct.

Abstract

Background: Although rare, coronary artery anomalies can have significant clinical implications. Total anomalous origin of the coronary arteries from the pulmonary artery (TCAPA) represents a rare subtype of coronary artery anomaly for which little is known. The aim of this review was to characterise the presentation, utilised diagnostic modalities, associated cardiac lesions, and treatment strategies in patients with TCAPA.

Methods: A systematic review was performed for cases of TCAPA using PubMed, Embase, and Web of Science. Keywords searched included "total anomalous origin of the coronary arteries from the pulmonary artery," "single ostium anomalous coronary artery from the pulmonary artery," and "anomalous origin of both coronary arteries from the pulmonary artery."

Results: Fifty-seven cases of TCAPA were identified in 50 manuscripts. Fifty-eight per cent of patients were male and the median age at presentation was 10 days (mean 1.71 ± 6.6 years, range 0 days-39 years). Most patients were symptomatic at the time of presentation; cyanosis (n = 22) and respiratory distress (n = 14) were the most common symptoms. Cases were most commonly diagnosed at autopsy (n = 26, 45.6%), but operative intervention was pursued in 22 cases (45.6%); aortic re-implantation (n = 14) and a Takeuchi-type repair (n = 7) were the most common routes of repair.

Conclusions: The clinical presentation of patients with TCAPA was found to be variable, likely related to the presence of associated cardiac lesions. TCAPA should be considered in patients with suspected anomalous origin of the left coronary artery from the pulmonary artery for the serious consequences that can occur if not promptly corrected.

Keywords: anomalous origin of a single coronary artery from the pulmonary artery; anomalous origin of both coronary arteries from the pulmonary artery; coronary artery anomalies; total anomalous origin of the coronary arteries from the pulmonary artery.

PubMed Disclaimer

Publication types