Objectives: We attempted to characterize the anatomy, function, clinical consequences, and treatment of right‐sided anomalous coronary artery origin from the opposite side (R‐ACAOS).Background: Anomalous aortic origin of a coronary artery is a source of great uncertainty in cardiology. A recent study by our group found that ACAOS had a high prevalence (0.48%) in a general population of adolescents.Methods: Sixty‐seven consecutive patients were diagnosed with R‐ACAOS according to a new definition: ectopic right coronary artery (RCA) with an intramural proximal course. We used intravascular ultrasonograms of the RCA to quantify congenital stenosis (in patients with potentially serious clinical presentations), and we correlated these measurements with clinical manifestations.Results: All patients had some proximal intramural stenosis (mean 50%, range 16–83% of the cross‐sectional area). Forty‐two patients (62%) underwent stent‐percutaneous coronary intervention (PCI) of R‐ACAOS because of significant symptoms, positive stress tests, and/or significant stenosis. Stent‐PCI was successful in all cases and correlated with improved symptoms at >1‐year follow‐up in 30 patients (71%) who were available for clinical follow‐up. No ACAOS‐related deaths occurred. The instent restenosis rate was 4/30 (13%) at a mean follow‐up time of 5.0 years.Conclusions: This preliminary, but large and unprecedented observational study shows that cases angiographically identified as R‐ACAOS universally feature an intramural aortic course but only occasionally severe stenosis on resting IVUS imaging. Our data suggest that stent‐PCI with IVUS monitoring ameliorates patients’ presenting symptoms.

Improving preparticipation screening of candidates for sports necessitates establishing the prevalence of high-risk cardiovascular conditions (hr-CVC) that predispose young people to sudden cardiac death (SCD). Our accurate, novel protocol chiefly involved the use of cardiac magnetic resonance (CMR) to estimate this prevalence. Middle and high school students from a general United States population were screened by means of questionnaires, resting electrocardiograms, and CMR to determine the prevalence of 3 types of hr-CVC: electrocardiographic abnormalities, cardiomyopathies, and anomalous coronary artery origin from the opposite sinus with intramural coronary course (ACAOS-IM). We examined the range of normal left ventricular size and function in the main study cohort (schoolchildren 11–14 yr old). We defined diagnostic criteria for hr-CVC and compared the cardiac measurements of these younger participants with those of older children whom we examined (age, 15–18 yr). From 5,169 completed diagnostic studies (mean participant age, 13.06 ± 1.78 yr), CMR results revealed 76 previously undiagnosed cases of hr-CVC (1.47% of the total cohort): 11 of dilated cardiomyopathy (14.5%), 3 of nonobstructive hypertrophic cardiomyopathy (3.9%), 23 ACAOS-IM cases (30.3%; 6 left-ACAOS and 17 right-ACAOS), 4 Wolff-Parkinson-White patterns (5.3%), 34 prolonged QT intervals (44.7%), and 1 Brugada pattern (1.3%). Cardiomyopathies were significantly more prevalent in the older children. Of note, we identified 959 cases (18.5%) of left ventricular noncompaction. If our estimate is accurate, only 1.47% of school-age sports participants will need focused secondary evaluations; the rest can probably be reassured about their cardiac health after one 30-minute screening study.

Takotsubo cardiomyopathy (TTC), a persistently obscure dysfunctional condition of the left ventricle, is uniquely transient but nevertheless dangerous. It features variable ventricular patterns and is predominant in women. For 30 years, pathophysiologic investigations have progressed only slowly and with inadequate focus. It was initially proposed that sudden-onset spastic obliteration of coronary flow induced myocardial ischemia with residual stunning and thus TTC. Later, it was generally accepted without proof that, in the presence of pain or emotional stress, the dominant mechanism for TTC onset was a catecholamine surge that had a direct, toxic myocardial effect. We think that the manifestations of TTC are more dynamic and complex than can be assumed from catecholamine effects alone. In addition, after reviewing the recent medical literature and considering our own clinical observations, especially on spasm, we theorize that atherosclerotic coronary artery disease modulates and physically opposes obstruction during spasm. This phenomenon may explain the midventricular variant of TTC and the lower incidence of TTC in men. We continue to recommend and perform acetylcholine testing to reproduce TTC and to confirm our theory that coronary spasm is its initial pathophysiologic factor. An improved understanding of TTC is especially important because of the condition's markedly increased incidence during the ongoing COVID-19 pandemic.