TY  - EJOU
AU  - Mahmoud Alsalehi, 
AU  - Aamir Jeewa, 
AU  - Andrea Wan, 
AU  - Juan Contreras, 
AU  - Shi‐Joon Yoo, 
AU  - A. Laks, Jessica 

TI  - A case series of left main coronary artery ostial atresia and a review of the literature
T2  - Structural and Congenital Heart Disease

PY  - 2019
VL  - 14
IS  - 6
SN  - 3071-1738

AB  - Left main coronary artery ostial atresia (LMCAOA) is a rare congenital anomaly of the 
coronary arteries. The published literature regarding the current diagnostic and manage‐
ment recommendations are limited. We present three case series of LMCAOA from our 
institution, including one with a unique association with anomalous origin of left coronary 
artery (LCA) from pulmonary artery. In addition, this report includes a review of 50 pedi‐
atric and 43 adult cases from literature. The majority of the patients were symptomatic. 
Sudden cardiac death occurred in 10% of pediatric patients and 7% of adult patients. 
Almost half of pediatric patients had additional cardiac lesions. At the time of diagnosis, 
82% of patients had abnormal exercise stress test and 73% had abnormal myocardial 
perfusion imaging (MPI). The diagnosis of LMCAOA was suspected by echocardiogra‐
phy in 47% of pediatric patients, while 26% were initially misdiagnosed as anomalous 
origin of LCA from pulmonary artery. Coronary angiography confirmed the diagnosis in 
most cases and 70.5% of pediatric patients had small collaterals, while 80.5% of adult 
patients had large collaterals. Nine pediatric patients had no revascularization surgery 
with five deaths. Revascularization surgery was performed in 39 pediatric patients with 
four deaths. After 2005, there is a gradual shift toward performing coronary osteoplasty 
rather than coronary artery bypass grafting. Eighteen adult patients had revascularization 
surgery and all survived. Fifteen adult patients had no revascularization surgery, of which 
there were five deaths. In patients with LMCAOA, revascularization surgery is currently 
recommended in the presence of symptoms, ischemic changes on electrocardiogram or 
exercise stress test, myocardial perfusion defect on MPI, global left ventricular systolic 
dysfunction on echocardiogram, severe mitral regurgitation, or small‐sized collaterals in 
coronary angiography. Short‐term and mid‐term outcomes are encouraging.
KW  - artery
KW  -  atresia
KW  -  coronary
KW  -  left

DO  - 10.1111/chd.12842