Diffuse calcification of the left atrium and ventricle.
Central Message.
AAOCA can be related to coronary ischemia and cardiac sudden death; myocardial calcification arises from a number of etiologies, with previous myocardial infarction being the most common.
A 13-year-old boy was transferred to our institution because of syncope 1 hour prior. His syncope occurred suddenly after physical activity at school. Ventricular fibrillation occurred shortly after admission. He was sent to the intensive care unit following cardiopulmonary resuscitation and defibrillation. Despite the use of mechanical ventilation and inotropes, he still had refractory ventricular fibrillation with unstable hemodynamics 1 day later. Venoarterial extracorporeal membrane oxygenation was then initiated. The bedside echocardiogram (Figure 1, A) indicated anomalous aortic origin of the left coronary artery (AAOCA) from the noncoronary sinus of Valsalva. Hyperechoic changes of the papillary muscles and myocardium were also observed (Figure 1, B). This patient was weaned off extracorporeal membrane oxygenation after 5 days and coronary computed tomographic angiography was performed. The coronary computed tomography angiogram showed AAOCA with an intramural segment (Figure 1, C and D). Massive myocardial calcification of the left atrium and ventricle was also found (Video 1). Therefore, coronary artery unroofing was performed. During the operation, we found that the left coronary artery arose from the noncoronary sinus of Valsalva with an intramural segment (Figure 2, A). The top of the intercoronary commissure was below the intramural segment. Because the length of the interarterial course was limited, we did not perform pulmonary artery translocation. Myocardial calcification of the left heart was significant (Figure 2, B), and biopsy of the left ventricle was performed. The pathology indicated calcification and necrosis of myocites (Figure 2, C). The postoperative recovery was uneventful. He was discharged 12 days after surgery. He was asymptomatic at the 1-month follow-up appointment. The echocardiogram at follow-up showed no signs of narrowing of coronary arteries. The left ventricular ejection fraction was 64%.
Figure 1.
A, Echocardiogram indicated anomalous aortic origin of the left coronary artery from the noncoronary sinus of Valsalva. B, Echocardiogram indicated hyperechoic changes in the papillary muscles (white arrow) and myocardium (black arrow). C and D, Coronary computed tomography angiography indicated anomalous aortic origin of the coronary arteries with an intramural segment. LCA, Left coronary artery.
Figure 2.
A, Anomalous open of the left coronary artery from the noncoronary sinus with an intramural segment (black arrow); (B) Coronary artery unroofing and myocardial biopsy during the operation. Black arrow indicates the noncoronary sinus of Valsalva. White arrow indicates the left coronary sinus of Valsalva and the neo-created ostium of the left coronary artery. Asterisk indicates the myocardial calcification of the left ventricle; (C) Hematoxylin and eosin staining (100×) showed calcification and necrosis of cardiomyocytes.
AAOCA can be related to coronary ischemia and cardiac sudden death, especially in adolescents.1 Timely diagnosis and surgery are necessary. Myocardial calcification arises from a number of etiologies, with previous myocardial infarction being the most common.2 Heart dysfunction may be caused by massive myocardial calcification, which often results in restrictive physiology with or without decreased systolic function.3 IRB approval was not required; the patient's parents provided informed consent for publication.
Conflict of Interest Statement
The authors reported no conflicts of interest.
The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest.
Acknowledgments
The authors thank Dr Xu Zhu and Wenwen Li for providing the clinical images.
Footnotes
Supported by the Chongqing Medical University Program for Youth Innovation in Future Medicine (2022, W0204).
Ethics approval: The Institutional Review Board of Children's Hospital of Chongqing Medical University waived ethical approval of this study because of the nature of case reports.
Informed consent statement: Informed consent was obtained from the parents for publication.
Supplementary Data
Massive myocardial calcification of the left atrium and ventricle. Video available at: https://www.jtcvs.org/article/S2666-2507(24)00173-1/fulltext.
References
- 1.Cipriani A., Dall’Aglio P.B., Mazzotta L., et al. Diagnostic yield of non-invasive testing in patients with anomalous aortic origin of coronary arteries: a multicentric experience. Congeni Heart Dis. 2022;17(4):375–385. [Google Scholar]
- 2.Nance J.W., Jr., Crane G.M., Halushka M.K., Fishman E.K., Zimmerman S.L. Myocardial calcifications: pathophysiology, etiologies, differential diagnoses, and imaging findings. J Cardiovasc Comput Tomogr. 2015;9(1):58–67. doi: 10.1016/j.jcct.2014.10.004. [DOI] [PubMed] [Google Scholar]
- 3.Aras D., Topaloglu S., Demirkan B., Deveci B., Ozeke O., Korkmaz S. Porcelain heart: a case of massive myocardial calcification. Int J Cardiovasc Imaging. 2006;22(1):123–126. doi: 10.1007/s10554-005-9006-2. [DOI] [PubMed] [Google Scholar]
Associated Data
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Supplementary Materials
Massive myocardial calcification of the left atrium and ventricle. Video available at: https://www.jtcvs.org/article/S2666-2507(24)00173-1/fulltext.