Coronary artery disease in adults with anomalous aortic origin of a coronary artery.

Objectives: This study sought to characterize coronary artery disease (CAD) among adults diagnosed with an anomalous aortic origin of a coronary artery (AAOCA). We hypothesized that coronaries with anomalous origins have more severe CAD stenosis than coronaries with normal origins. Methods: This single-center study of 763 adults with AAOCA consisted of 620 patients from our cardiac catheterization database (1958-2009) and 273 patients from electronic medical records query (2010-2021). Within left main, anterior descending, circumflex, and right coronary arteries, the CAD stenosis severity, assessed by invasive or computer tomography angiography, was modeled with coronary-level variables (presence of an anomalous origin) and patient-level variables (age, sex, comorbidities, and which of the four coronaries was anomalous). Results: Of the 763 patients, 472 (60%) had obstructive CAD, of whom, 142/472 (30%) had obstructive CAD only in the anomalous coronary. Multivariable modeling showed similar CAD stenosis severity between coronaries with anomalous versus normal origins (P = .8). Compared with AAOCA of other coronaries, the anomalous circumflex was diagnosed at older ages (59.7 ± 11.1 vs 54.3 ± 15.8 years, P < .0001) and was associated with increased stenosis in all coronaries (odds ratio, 2.7; 95% confidence interval, 2.2-3.4, P < .0001). Conclusions: Among adults diagnosed with AAOCA, the anomalous origin did not appear to increase the severity of CAD within the anomalous coronary. In contrast to the circumflex, AAOCA of the other vessels may contribute a greater ischemic burden when they present symptomatically at younger ages with less CAD. Future research should investigate the interaction between AAOCA, CAD, and ischemic risk to guide interventions.

Patient-specific fluid-structure simulations of anomalous aortic origin of right coronary arteries.

Objectives: Anomalous aortic origin of the right coronary artery (AAORCA) may cause ischemia and sudden death. However, the specific anatomic indications for surgery are unclear, so dobutamine-stress instantaneous wave-free ratio (iFR) is increasingly used. Meanwhile, advances in fluid-structure interaction (FSI) modeling can simulate the pulsatile hemodynamics and tissue deformation. We sought to evaluate the feasibility of simulating the resting and dobutamine-stress iFR in AAORCA using patient-specific FSI models and to visualize the mechanism of ischemia within the intramural geometry and associated lumen narrowing. Methods: We developed 6 patient-specific FSI models of AAORCA using SimVascular software. Three-dimensional geometries were segmented from coronary computed tomography angiography. Vascular outlets were coupled to lumped-parameter networks that included dynamic compression of the coronary microvasculature and were tuned to each patient's vitals and cardiac output. Results: All cases were interarterial, and 5 of 6 had an intramural course. Measured iFRs ranged from 0.95 to 0.98 at rest and 0.80 to 0.95 under dobutamine stress. After we tuned the distal coronary resistances to achieve a stress flow rate triple that at rest, the simulations adequately matched the measured iFRs (r = 0.85, root-mean-square error = 0.04). The intramural lumen remained narrowed with simulated stress and resulted in lower iFRs without needing external compression from the pulmonary root. Conclusions: Patient-specific FSI modeling of AAORCA is a promising, noninvasive method to assess the iFR reduction caused by intramural geometries and inform surgical intervention. However, the models' sensitivity to distal coronary resistance suggests that quantitative stress-perfusion imaging may augment virtual and invasive iFR studies.

Anomalous Aortic Origin of a Coronary Artery in Adults.

BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden death in youth. However, its significance and optimal management in adults is poorly understood. Our objective is to characterize AAOCA in a large single-center adult cohort based on coronary anatomic variants and surgical management strategies. METHODS: We reviewed imaging, clinic, and operative reports for 645 adults with an encounter diagnosis code of congenital coronary anomaly from July 2015 to July 2017. After excluding other congenital heart defects, we characterized 167 patients with AAOCAs by anatomic variant, symptoms at diagnosis, indication for advanced imaging, and if performed, surgical repair. To describe the anatomic variant, we classified the origin and course by following the atomization scheme developed by the Congenital Heart Surgeon's Society's AAOCA registry. RESULTS: Among adults with AAOCA, the anomalous origin involved the right coronary artery in 57% (96 of 167), left main coronary artery in 23% (39 of 167), left anterior descending in 2% (4 of 167), circumflex in 16% (26 of 167), and multiple coronaries in 1% (2 of 167). Anomalous right coronary arteries were diagnosed at an older median age than anomalous left main coronary arteries (55 vs 51 years, respectively; P = .026). Surgical repair of AAOCA occurred in 22% (36 of 167) of patients. Concomitant cardiac surgical procedures accompanied 36% (13 of 36) of them. No deaths occurred over a median follow-up of 2.5 years. CONCLUSIONS: Most patients in our single-center AAOCA registry were diagnosed in the presence of cardiac symptoms. Concomitant aortic valve disease and coronary atherosclerotic burden complicate both the evaluation and surgical approach to adult AAOCA repair.

Quantitative three-dimensional imaging of live avian embryonic morphogenesis via micro-computed tomography.

Many clinically relevant congenital malformations arise during mid to late embryonic stages. This period is challenging to image quantitatively in live embryos, necessitating the use of multiple specimens with increased experimental variability. Here we establish X-ray and blood-pool computed tomography (CT) contrast agent toxicity and teratogenesis thresholds for 3D Micro-CT imaging of live avian embryos. Day 4 chick embryos micro-injected with Visipaque™ (VP) developed for an additional 6 days without defect. X-ray radiation up to 798 mGy was nontoxic. Peak average contrast of 1,060 HU occurred within 1 hr of imaging at 50 µm resolution. VP-enhanced contrast persisted past 24 hr with delayed accumulation in the allantois. Regional volumes of VP-injected embryos were statistically identical to those of fixed embryos perfused with osmium tetroxide. We further quantified longitudinal volumetric morphogenesis of the allantois over 30 hr. These results demonstrate the safety and efficacy of contrast enhanced quantitative micro-CT imaging for live embryos.