Characteristics and long-term outcome for congenital left main coronary artery atresia

Abstract

The prevalence of congenital left main coronary artery atresia is very low. We report the characteristics and long-term outcomes of four children with left main coronary artery atresia. Three patients had heart murmurs due to mitral regurgitation at less than 1 year old. Their myocardial ischaemia worsened on exercise with aging. In the fourth patient, hypertrophic cardiomyopathy and Noonan syndrome were suspected at 1 year old. The development of communicating arteries between the conus branch and the left anterior descending artery was detected at 7 years old. The left main coronary artery atresia was confirmed by a selective coronary angiogram at 15 years old. Congenital left main coronary artery atresia could not be diagnosed by two-dimensional echocardiography; however, the left coronary arteries were small. Two patients underwent coronary artery bypass grafting of the left anterior descending artery using the left internal thoracic artery at 3 years and 6 years old, respectively. Two patients had an angioplasty with a cut back at the orifice of the left coronary artery at 2 years old and 17 years old, respectively. Two patients had no cardiac events without medication for more than 30 years after the operation. We must differentiate the diagnosis of left main coronary artery atresia in the small left coronary arteries with mitral regurgitation during the first year. Coronary artery revascularisation and mitral annuloplasty are needed. The long-term outcome of both coronary artery bypass grafting and angioplasty were good. The degree of mitral regurgitation after surgery may affect the prognosis.

A Noonan‐like pediatric patient with a de novo CBL pathogenic variant and an RNF213 polymorphism p.R4810K presenting with cardiopulmonary arrest due to left main coronary artery ostial atresia

“…However, there are no reports on the association between RNF213 and LMCAOA, and the same applies to CBL and LMCAOA. There are only two reports of LMCAOA complicated with Noonan syndrome (Ramond et al, 2017; Tsuda et al, 2023), and a RIT1 pathogenic variant was identified in one of the cases (Ramond et al, 2017). No other genes are suspected to be associated with LMCAOA to date.…”

Classification, diagnosis and clinical strategy of congenital coronary artery disease in children

“…Furthermore, according to the risk of adverse cardiac events CCAA can be separated into three kinds: benign coronary anomalies (BCA), potentially serious coronary anomalies(PSCA), serious coronary anomalies(SCA); which refers to CCAA-BCA, CCAA-PSCA and CCAA-SCA, in order to help clinicians better understand and identify the related diseases. See Table 1 for abbreviations and Table 2 for details (7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18).…”