Anomalous Coronary Arteries and Myocardial Bridges: Risk Stratification in Children Using Novel Cardiac Catheterization Techniques

Abstract

The evaluation of the vast majority of children with anomalous aortic origin of a coronary artery (AAOCA) and/or myocardial bridges is performed with non-invasive testing. However, a subset of these patients may benefit from invasive testing for risk stratification. All patients included in the Coronary Anomalies Program (CAP) at Texas Children's Hospital who underwent cardiac catheterization were included. Techniques included selective coronary angiograms (SCA), intravascular ultrasound (IVUS), and fractional flow reserve (FFR) measurements with provocative testing using adenosine and/or dobutamine infusions. Out of the 131 patients followed by the CAP between 12/12-4/16, 8 (6%) patients underwent 9 cath investigations at median age 13.1 (2.6-18.7) years and median weight 49.5 (11.4-142.7) kg. Six patients presented with cardiac signs/symptoms. Four patients had myocardial bridges of the left anterior descending (LAD) coronary artery, 2 patients had isolated AAOCA, and 2 patients had an anomalous left coronary artery (LCA) with an intramyocardial course of the LAD. SCA was performed in all patients. FFR was positive in 4/6 patients: IVUS showed >70% intraluminal narrowing in 3/5 patients. One patient had hemodynamic instability that reversed with catheter removal from the coronary ostium. Based on the catheterization data obtained, findings were reassuring in three patients, surgery was performed in three patients, and two patients are being medically managed/restricted from competitive sports. In our small cohort of patients, we demonstrated that IVUS and FFR can safely be performed in children and may help to risk stratify some patients with AAOCA and myocardial bridges.

Familial clustering of cardiac conditions in patients with anomalous aortic origin of a coronary artery and myocardial bridges

“…The third proband with anomalous right coronary artery had a maternal uncle who suffered sudden cardiac death while playing soccer and was found to have anomalous left coronary artery on autopsy. Moreover, as we previously reported, we also found evidence of myocardial bridge in three siblings whose father suffered sudden death 20 . Among these three siblings, one was asymptomatic with normal myocardial perfusion and two had abnormal myocardial perfusion on stress MRI and underwent surgery.…”

“…Moreover, as we previously reported, we also found evidence of myocardial bridge in three siblings whose father suffered sudden death. 20 Among these three siblings, one was asymptomatic with normal myocardial perfusion and two had abnormal myocardial perfusion on stress MRI and underwent surgery. These siblings demonstrate one of the challenges in screening for myocardial bridges.…”

Diagnosis and Management of Anomalous Coronary Arteries with a Malignant Course

“…[24] As an invasive option, the cross-sectional area of stenosis can be most accurately assessed by using intravascular ultrasound (IVUS) or optical coherence tomography (OTC) to depict the exact proximal anatomy of the anomalous vessel. [25–27] IVUS is highly sensitive and reveals fundamental information on delicate changes of stenosis severity that phasically change during the cardiac cycle and with exercise. Furthermore, fractional flow reserve (FFR) measurement, with the ratio of mean distal (non-ectopic coronary) pressure to mean aortic pressure using adenosine and/or dobutamine and adrenaline, can help to invasively assess the functional severity of stenosis and ischaemia in patients with ACAOS.…”

Cardiac Catheterization in Assessment and Treatment of Kawasaki Disease in Children and Adolescents

“…FFR is the ratio of flow in the coronary artery distal to the stenosis compared to the aortic pressure, with a normal value being 1.0. FFR is measured at baseline, followed by provocative testing with adenosine (140 mcg/kg/min) infusion for 3 minutes [3]. Mean FFR is used for assessment for fixed coronary stenosis.…”