Anomalous origin of the coronary artery coursing between the great vessels presenting with a cardiovascular event (J-CONOMALY Registry)

Abstract

Aims Anomalous origin of the coronary artery (AOCA) with an inter-arterial course (IAC) between the great vessels poses a risk for a life-threatening cardiovascular event. We assessed, in a registry-based study, the clinical features, treatment strategies, and prognoses of life-threatening cardiovascular events ensuant to AOCA. Methods and results Included were 65 AOCA patients (48 men/17 women, aged 41 ± 23 years) from 40 clinical centres who had experienced sudden cardiac arrest (SCA) (n = 30), acute myocardial infarction (AMI) (n = 5), angina (n = 23), or syncope (n = 7). The anomalous vessel was the right coronary artery in 72% of patients and left coronary artery in 28%; the ostium was slit-like in 42%. Coronary luminal narrowing ≥75% was absent in patients with SCA or syncope (86% and 57%, respectively), but occlusion or narrowing was seen in those with AMI (100%) or angina (52%). Age ≤40 years, male sex, sporting activity, absence of prodromal symptoms, acutely angled (≤30°) take-off from the aorta, and absence of luminal narrowing of the IAC segment were associated with SCA in this patient group. Coronary vasospasm was inducible in 12 of 17 patients without coronary narrowing. Management included surgical revascularization (n = 26) percutaneous coronary intervention (n = 9), and medical treatment (n = 26). Four SCA patients died while hospitalized; no others died during the median 5.0 (range, 1.8–7.0)-year follow-up period. Conclusions In patients with AOCA, age ≤40 years, male sex, sporting activity, and an acute take-off angle appear to be risk factors for SCA. Appropriate management can be beneficial. Confirmation in a large-scale study is warranted.

Surgical Therapy for Anomalous Aortic Origin of a Coronary Artery

“…Mainwaring and colleagues 3,12,13) proposed that intramural anatomy is the key risk factor associated with ischemic symptoms and suggested that all intramural coronary arteries should undergo surgical repair. In addition, Nagashima and colleagues 7) reported that age≤40 ages, male sex, participation in sporting activity, absence of prodromal symptoms, acutely angled (≤30°) take-off from the aorta, and absence of luminal narrowing of the inter-arterial course segment were associated with sudden cardiac arrest in patients in AAOCA with inter-arterial course. Take-off angle of the AAOCA from the ascending aorta might be a key factor involved in sudden death (Fig.…”

“…Based on several studies of AAOCA as a frequent cause of sudden cardiac death in young athletes, the main mechanism responsible for ischemic cardiovascular events is presumably incremental mechanical compression of the inter-arterial course by the great vessels or kinking of the sharp angulation at the AAO-CA ostium during exercise [4][5][6][7] . Nagashima and colleagues 7) suggested that the latter mechanism contributes to a greater proportion of ischemic cardiovascular events, based on the findings of their retrospective clinical study. In addition, they noted that coronary spasm might influence cardiovascular events in patients with AAOCA 7) .…”

“…Nagashima and colleagues 7) suggested that the latter mechanism contributes to a greater proportion of ischemic cardiovascular events, based on the findings of their retrospective clinical study. In addition, they noted that coronary spasm might influence cardiovascular events in patients with AAOCA 7) . In middle-aged and elderly patients, regardless of exercise, pulmonary artery dilatation caused by pulmonary hypertension 8,9) and atherosclerotic stenosis might worsen obstruction and cause ischemic cardiac events.…”

“…When a patient has anomalous aortic origin of the right coronary artery and no evidence of ischemia, management is more controversial 10) . Nagashima et al 7) reported that anomalous aortic origin of the right coronary artery was common among patients in whom sudden cardiac arrest occurred (67%). Furthermore, in seven of 16 (44%) patients with anomalous aortic origin of the right coronary artery patients who experienced sudden cardiac arrest, non-invasive stress tests failed to reveal ischemia in the right coronary artery territory, which was consistent with previous autopsy and individual case reports.…”

Anomalous coronary artery from the opposite sinus

“…Follow‐up of patients managed surgically or medically in this registry may further help with evidence‐based protocols . In addition, the Japanese Coronary Anomaly Registry suggested patients with age ≤40 years, male sex, sporting activity, and an acute take‐off angle appear to be risk factors for sudden cardiac death and thus surgical intervention may be appropriate therapy …”