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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. 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. 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. 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. [Display omitted] [ABSTRACT FROM AUTHOR]

Background We present a case series of upper extremity fibromuscular dysplasia (UE FMD) consisting of 22 patients from two tertiary referral centers focusing on clinical presentation, diagnostic findings, and interventional outcomes. FMD is a noninflammatory, nonatherosclerotic arteriopathy that has a predisposition for middle-aged women. Involvement of the UE is thought to be rare. Patients with UE FMD can present with claudication or ischemia, or they can be incidentally diagnosed. The treatment approach is dictated by clinical presentation. Methods Data were collected of patients with UE FMD evaluated at two centers. Demographic data, presenting UE symptoms, UE arteries involved, FMD type, diagnostic method, physical examination findings, management, and outcomes were included. Results Twenty-two patients (29 limbs) were diagnosed with UE FMD. The brachial artery was most commonly involved (89.7% of affected limbs). More than half of limbs (n = 15 of 29 limbs [51.7%]) were asymptomatic, and of those who presented with symptoms, the most common symptoms were ischemic fingers or hand (31% of all affected limbs) and hand or arm claudication (27.6% of all affected limbs). UE FMD was noted on catheter angiography in 58.6% (n = 17 of 29 limbs), duplex ultrasound in 41.4% (n = 12 of 29 limbs), and computed tomography angiography in 27.6% (n = 8 of 29 limbs). Of the symptomatic limbs (n = 14), the majority were treated solely with medical therapy as the first intervention (57.1%). For symptomatic limbs treated with vascular intervention (n = 5), angioplasty was most commonly performed. Only 4 of the 14 limbs (28.6%) had complete symptomatic relief after the initial first intervention, in which 2 limbs were treated with medical therapy, 1 limb underwent angioplasty, and 1 limb had resolution of symptoms despite deferment of any therapy. Of the 10 limbs with residual symptoms after the first intervention, 6 limbs underwent a second intervention: angioplasty in 2 limbs initially treated medically (33.3%), surgical bypass in 2 limbs initially treated with angioplasty, surgical bypass in 1 limb initially treated with medical therapy, and sympathectomy in 1 limb (16.7%) initially treated with angioplasty. Both surgical bypass and angioplasty as secondary interventions resulted in complete symptom relief. Conclusions Presenting symptoms for patients with UE FMD vary in severity from asymptomatic disease to digital ischemia. More than half of symptomatic limbs eventually require at least one invasive intervention for complete relief of symptoms. [ABSTRACT FROM AUTHOR]

Objectives This study sought to evaluate the diagnostic yield of a dedicated computed tomography angiography (CTA) protocol of the chest, abdomen, and pelvis in patients with fibromuscular dysplasia (FMD). Background FMD is an uncommon vascular disease that may result in stenosis, dissection, or aneurysm of nearly all arterial distributions, typically affecting medium-sized arteries. Findings from the United States Registry for Fibromuscular Dysplasia have suggested the potential need to perform screening imaging of the aorta and medium branch vessels. Methods A total of 113 consecutive patients enrolled in our institutional FMD registry who received a tailored CTA protocol at our institution between March 2013 and June 2015 were included in this study. Arterial phase contrast-enhanced images were obtained on a dual-source scanner using high pitch and electrocardiogram trigger. Images were analyzed by 2 readers. Results Abnormalities including beading, aneurysm, dissection, and stenosis/occlusion were noted in aortic, renal, mesenteric, and iliac distributions. The most commonly affected vessels were the renal arteries (n = 76 [67%]), followed by the lower extremity/iliac arteries (n = 37 [32%]). Aortic abnormalities were less frequently encountered (n = 3 [3%]), including 1 case with mild dilation (4.2 cm) of the ascending aorta and 2 cases of dissection involving the descending aorta, 1 with mild dilation (4.4 cm). Incremental findings beyond those known at patient intake were commonly noted, including new areas of arterial beading (n = 55 [49%]), new aneurysms (n = 21 [19%]), and new dissections (n = 3 [3%]). Reformatted images were crucial, affecting final assessment in 56% of cases evaluated by reader 1 and 36% evaluated by reader 2. Conclusions Screening chest, abdomen, and pelvis CTA in patients with FMD showed substantial and incremental diagnostic yield. Reformatted images should routinely be included in imaging analysis. Abnormalities in the aorta were not common, so screening of the thoracic aorta may not be indicated. [ABSTRACT FROM AUTHOR]