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. 2023 Oct 31;23(1):546.
doi: 10.1186/s12887-023-04377-4.

Anomalous right coronary artery originating from the aorta: a series of nine pediatric cases

Jia Na  1 , Xi Chen  1 , Zhen Zhen  1 , Lu Gao  1 , Yue Yuan  2
Affiliations

Anomalous right coronary artery originating from the aorta: a series of nine pediatric cases

Jia Na et al. BMC Pediatr. .

Abstract

Background: To investigate the clinical manifestations, prognosis, and possibly related genes of anomalous right coronary artery originating from the aorta (ARCA-L) in children.

Methods: This case series study included pediatric patients diagnosed with ARCA-L at the Department of Cardiology in Beijing Children's Hospital affiliated to Capital Medical University, between January 2017 and December 2019.

Results: Nine pediatric patients (aged 3 months to 12 years, 4 boys) were included. Two cases presented with cardiac insufficiency as their primary manifestation, while the remaining seven had post-infection or post-exercise symptoms such as chest pain, chest tightness, long exhalation, lack of strength, and dizziness. Six patients displayed varying degrees of ST-T changes on the electrocardiograph, while two patients had a reduced left ventricular ejection fraction (LVEF) of 20-32% according to echocardiography. Multislice computed tomographic angiography confirmed the presence of ARCA-L in all patients. One patient underwent the unroofing technique. The remaining eight received conservative treatment. After a follow-up of 2-64 months, eight children had a good prognosis and survived. One child experienced sudden death due to aggravated heart failure. Whole exome sequencing revealed that one child tested negative, one had mutations in the RYR2 and LDB3 genes, and the remaining four patients had a mutation in the GDF1, LRP6, MEF2A, and KALRN genes, respectively.

Conclusions: ARCA-L in children might have a wide variation in clinical manifestations and a risk of sudden death. The occurrence of the disease might be associated with genetic defects.

Keywords: Anomalous origin of coronary artery; Case series; Clinical manifestations; Pediatric; Prognosis; Whole-exome sequencing.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
(A) ECG of case #2 showing I, II, III, aVF, and V4-V6 lead ST-segment downshifts, III lead T-wave inversion, and aVF lead bidirectional T-wave. (B) ECG of case #3 showing second-degree atrioventricular block, with a fixed conduction ratio of 2:1. The arrows indicate the P-wave with conduction block. (C) ECG of case #8 showing II, III, aVF, and V3-V6 lead T-wave changes
Fig. 2
Fig. 2
(A) MSCTA of case #8 showing the origin of the right coronary artery from the left coronary sinus. (B) MSCTA of case #9 showing the origin of the right coronary artery from the left coronary sinus and traveled between the aorta and pulmonary arteries
See this image and copyright information in PMC

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