Validation of a Clinical-Significance-Based Classification of Coronary Artery Anomalies

Abstract

The clinical significance and subsequent management of the various types of coronary artery anomalies (CAAs) are different, as is the relationship to sudden death, coronary artery disease, and myocardial ischemia. A practical global classification based on clinical significance has not yet been proposed. This retrospective study was aimed at evaluating the current clinical significance of CAAs and the effectiveness of a clinical-significance-based classification. In a single-center retrospective study at a public hospital, a review of the last 5,100 coronary angiographies was performed in order to select patients with CAAs. The CAAs were classified into 4 categories on the basis of a literature review according to angiographic appearance and clinical significance: benign (class I); relevant-associated with fixed myocardial ischemia (class II); severe-related to sudden death (class III); and critical-associated with superimposed coronary artery disease (CAD) (class IV). Clinical and instrumental records of the selected patients were reviewed as well as the occurrence of cardiovascular events from the date of diagnosis to July 2002. Sixty-two patients (1.2%, women/men 20/42, mean age 65.3 +/-10.6 years) had CAA on coronary angiography. From the above-described classification, 40 patients were categorized in class I (64.5%), 9 in class II (14.5%), 7 in class III (11.3%), and 6 in class IV (9.7%). During the follow-up (mean duration 60.4 +/-12.3 months) most cardiovascular events and death occurred in class III and IV patients (71.3% and 100% respectively). A high correlation was found between significance level and percentage of patients with cardiovascular events (r = 0.99). Actuarial survival at 5 years was 82.9%. CAAs can be practically classified on the basis of clinical presentation and significance. This clinical classification may be useful in managing patients with CAA and personalizing their follow-up and therapeutic options according to their class and case histories.

Normal angiogram in acute coronary syndromes: The underestimated role of alternative substrates of myocardial ischemia

“…Coronary artery anomalies (CAAs) are less rare than in the past due to the aging of the population and the improved quality of life [26]. Although most CAAs are asymptomatic and benign [27], some specific CAA subtypes such as myocardial bridges and origin from the opposite sinus may cause severe myocardial ischemia both episodic and fixed [28]. Pathophysiologic mechanisms of myocardial bridges and origin from the wrong sinus probable include systolic compression and phasic compression within the aortic intramural decourse [29], respectively.…”

Evaluation of anatomy, variation and anomaly of the coronary arteries with coronary computed tomography angiography

“…In the literature, CAAs are classified either anatomically or clinically (19)(20)(21). The most accepted anatomic classification system was proposed by Angelini et al (19) who divided CAAs into anomalies of origination and course, anomalies of intrinsic coronary arterial anatomy and anomalies of coronary termination.…”

“…For example, Yamanaka and Hobbs reviewed 126.595 CCA examinations and only origination and termination anomalies were considered and included as CAA in this large population study (22). Some investigators overrated clinical importance of CAAs and classified them as hemodynamically significant (malignant) and hemodynamically insignificant (benign) (21). Thus, different investigators who used various CAA classification schemes are one of the main factors about different CAA incidence results of CCA studies (20).…”

“…It is reported that 50% of high takeoff RCA have these findings indicating a relatively high risk of acute coronary attack or sudden death (4, 32). Anomalous origin of a coronary artery from the pulmonary artery This is one of the most serious and hemodynamically significant CAA (21). The most common form is the left coronary artery originating from the pulmonary artery, called as ALCAPA syndrome (Fig.…”

“…On the other hand, CAAs are reported to be the most common cause of nontraumatic sudden deaths (61%) in young American military recruits (50). Hemodynamically significant CAAs are an anomalous location of coronary ostium at improper sinus with an interarterial course, an anomalous origin of a coronary artery from the pulmonary artery, a single coronary artery with an interarterial coursing major coronary artery, coronary ostial atresia and a congenital coronary artery fistula (20)(21)(22).…”