NASCI case of the month: a case of anomalous right coronary artery arising from the pulmonary artery.

Coronary artery anomalies are rare but potentially fatal abnormalities with occasional striking imaging findings radiologists should recognize.

[Anomalous origin of right coronary artery from pulmonary artery associated to left main coronary stenosis]. / Anomalie de connexion de l'artère coronaire droite au niveau de l'artère pulmonaire associée à une sténose du tronc commun gauche.

We describe herein a case of a 16 years-old female patient referred to our department for further exploration of a chest pain. Color Doppler echocardiography revealed an abnormal flow at the level of the pulmonary artery. Exercise testing was abnormal leading to further imaging including computed cardiac tomography followed by coronary angiography which showed anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) associated with mid shaft left main stenosis. We discuss this uncommon association and therapeutic options.

Risk Factors for Coronary Artery Complications After Prosthetic Pulmonary Valve Implantation in Patients With Congenital Heart Disease.

BACKGROUND: Coronary artery complications (CACs) in patients who undergoing prosthetic pulmonary valve implantation for congenital heart disease can lead to fetal outcomes. However, the incidence of and risk factors for CACs in these patients remain unknown.Methods and Results: A retrospective cohort study was conducted on patients who underwent cardiac computed tomography or invasive coronary angiography after prosthetic pulmonary valve implantation at Seoul National University Hospital from June 1986 to May 2021. Among 341 patients, 25 (7.3%) were identified with CACs, and 2 of them died. Among the patients with CACs, congenital coronary anomalies and an interarterial course of the coronary artery were identified in 11 (44%) and 18 (72%) patients, respectively. Interarterial and intramural courses of the coronary artery were associated with a 4.4- and 10.6-fold increased risk of CACs, respectively. Among patients with tetralogy of Fallot and pulmonary atresia, the aortic root was rotated further clockwise in patients with coronary artery compression compared to those without it (mean [±SD] 128.0±19.9° vs. 113.5±23.7°; P=0.024). The cut-off rotation angle of the aorta for predicting the occurrence of coronary artery compression was 133°. CONCLUSIONS: Perioperative coronary artery evaluation and prevention of CACs are required in patients undergoing prosthetic pulmonary valve implantation, particularly in those with coronary artery anomalies or severe clockwise rotation of the aortic root.

A Y-incision to enlarge the aortic root for aortic valve stenosis with anomalous aortic origin of the right coronary artery.

BACKGROUND: Anomalous aortic origin of the coronary artery (AAOCA) is a rare congenital heart disease. Therefore, optimal indications for surgery in patients with severe aortic valve stenosis (AS) complicated by AAOCA remain uncertain. CASE PRESENTATION: We report the case of a 57-year-old male patient who underwent aortic valve replacement (AVR) and aortic root enlargement using a Y-incision procedure for severe AS with an anomalous aortic origin of the right coronary artery (AAORCA). Since preoperative single-photon emission computed tomography revealed no ischaemic lesions, an aortic root enlargement with a Y-incision was performed to prevent the potential compression of the prosthetic valve on the AAOCA and prosthesis-patient mismatch. CONCLUSIONS: Preoperative evaluation of the coronary anatomy and myocardial ischaemia using advanced imaging modalities and aortic root enlargement with the Y-incision procedure is an effective strategy for preventing ischaemic complications in cases of severe AS with AAORCA.

Spontaneous Coronary Artery Dissection (SCAD) from an Interventionalist Perspective.

PURPOSE OF REVIEW: Spontaneous coronary artery dissection (SCAD) is an increasingly recognized cause of acute coronary syndrome (ACS), particularly among women < 50 years of age. Here, we aim to review the pathogenesis of SCAD, discuss SCAD as an initial manifestation of systemic arterial disease, and highlight invasive strategies as well as unique challenges in the care of women with SCAD. RECENT FINDINGS: A paradigm shift has occurred in the care of SCAD patients in the past decade as recommendations for conservative management have become widespread. Invasive interventions are reserved for patients with hemodynamic compromise or active ischemia due to increased periprocedural complications and failure rates. Certain patient populations have been identified for larger territory infarcts and proximal disease including patients with known connective tissue disease, premenopausal women, and patients with pregnancy-associated SCAD (P-SCAD). Current recommended management of SCAD is conservative. Despite a growing awareness of SCAD and its known association with systemic arteriopathies in women, evidence-based data remains scarce. Future studies focused on identifying genetic factors, optimal medical therapy after SCAD, and techniques to minimize interventional complications are needed.

Aorta Without Coronary Arteries: Anatomic Variants of a Rare Malformation.

Absence of connection of both coronary arteries to the aorta is an extremely rare congenital malformation. Most cases reported are anatomic variants of anomalous left coronary artery to pulmonary artery, found in isolation or in association with other congenital heart defects. We describe here four cases of patients born without any coronary artery connected to the aorta, including two with an almost complete absence of epicardial coronary arteries, one with single coronary artery to the right pulmonary artery, and one with left ventricular connection of a single coronary artery. Those exceptional coronary malformations have a poor prognosis and are often diagnosed at autopsy. Total absence of epicardial coronary arteries, present in two of our patients and described only once in the literature, leads us to reconsider current knowledge of human coronary artery development.

Non-atherosclerotic acute cardiac syndromes: spontaneous coronary artery dissection and Takotsubo syndrome. Comparison of long-term clinical outcomes.

BACKGROUND: Spontaneous coronary artery dissection (SCAD) and Takotsubo syndrome (TTS) constitute two common causes of nonatherosclerotic acute cardiac syndrome particularly frequent in women. Currently, there is no information comparing long-term clinical outcomes in unselected patients with these conditions. METHODS: We compared the baseline characteristics, in-hospital outcomes, and the 12-month and long-term clinical outcomes of two large prospective registries on SCAD and TTS. RESULTS: A total of 289 SCAD and 150 TTS patients were included; 89% were women. TTS patients were older with a higher prevalence of cardiovascular risk factors. Precipitating triggers were more frequent in TTS patients, while emotional triggers and depressive disorders were more common in the SCAD group. Left ventricular ejection fraction was lower in TTS patients, but SCAD patients showed higher cardiac biomarkers. In-hospital events (43.3% vs. 5.2%, P <0.01) occurred more frequently in TTS patients. TTS patients also presented more frequent major adverse events at 12-month (14.7% vs. 7.1%, HR 5.3, 95% CI: 2.4-11.7, P <0.01) and long-term (median 36 vs. 31 months, P =0.41) follow-up (25.8% vs. 9.6%, HR 4.5, 95% CI: 2.5-8.2, P <0.01). Atrial fibrillation was also more frequent in TTS patients. Moreover, TTS patients presented a higher 12-month and long-term mortality (5.6% vs. 0.7%, P =0.01; and 12.6% vs. 0.7%, P <0.01) mainly driven by noncardiovascular deaths. CONCLUSION: Compared to SCAD, TTS patients are older and present more cardiovascular risk factors but less frequent depressive disorder or emotional triggers. TTS patients have a worse in-hospital, mid-term, and long-term prognosis with higher noncardiac mortality than SCAD patients.

[Anomalous Origin of Right Coronary Artery with Intramural Course, Aortic Valve Stenosis and Coronary Artery Stenosis:Report of a Case].

Anomalous origin of the coronary artery is a rare congenital malformation that can cause myocardial ischemia and arrhythmia in patients with or without atherosclerotic lesions. We present a case of aortic stenosis (AS) and coronary artery stenosis complicated by anomalous origin of the right coronary artery (RCA) from the aortic valve sinus and its intramural course. The patient was a 66-year-old woman who was diagnosed with AS 4 years prior, and scheduled for surgery owing to gradual progression of stenosis. Preoperative coronary angiography revealed an abnormal origin of the RCA, and during the surgery, the RCA was found located within the aortic wall close to the aortotomy. Thus, bypass surgery was performed using a great saphenous vein, to prevent ischemia of the RCA territory. The patient had good intraoperative and postoperative course, and a coronary computed tomography scan clearly showed the reconstructed RCA.

[Coronary Artery Fistula with Aortic Valve Stenosis:Report of a Case].

A coronary artery fistula usually originates in the right coronary artery and often opens into the right ventricle. In approximately 50% of cases with a main pulmonary artery opening, aberrant blood vessels originate from both coronary arteries. Only a few cases of both coronary and bronchial artery-pulmonary artery fistulas have been reported. The patient was an 83-year-old man. Echocardiography showed severe aortic stenosis, while coronary angiography revealed aberrant vessels from both coronary arteries to the pulmonary artery. The right heart catheterization revealed a 26% left-to-right shunt ratio and a pulmonary/body blood flow ratio (Qp/Qs) of 1.36. MDCT scan confirmed that the aberrant vascular plexus originating from both coronary arteries was connected to the bronchial artery. We performed surgery on the patient, replacing the aortic valve and resecting the coronary arteriovenous fistulas. On the 11th postoperative day, the shunt had disappeared, as evidenced by a 1.2% left-toright shunt ratio and a Qp/Qs of the right heart catheterization of 1.02. The patient progressed uneventfully and was discharged on the 25th postoperative day.

Anomalous origin of left main coronary artery from the right sinus of Valsalva.

BACKGROUND: Anomalous coronary arteries are rare congenital variations with cases ranging from asymptomatic to life-threatening. Given the wide variability of coronary anomalies, it is challenging to predict their clinical consequences. Here, we present the 'malignant' variant - interarterial course of the left coronary artery between the aorta and pulmonary trunk - given the highest risk of sudden cardiac death among the various coronary anomalies. CASE PRESENTATION: Our case presents a 22-year-old male presenting to the emergency department after a syncopal episode that occurred while the patient was driving a motor vehicle. Initial Computed Tomography (CT) of the chest performed as part of the trauma work-up revealed a rare case of an anomalous origin of the left main coronary artery (LMCA) originating from a common ostium with the right coronary artery (RCA). The LMCA was found to have a malignant course, as it was positioned between the aorta and pulmonary artery. Given the high risk of sudden cardiac arrest with this congenital variant, the patient underwent coronary artery bypass grafting. CONCLUSION: Anomalous coronary arteries remain the second leading cause of sudden cardiac death in young adult patients. The risk of sudden cardiac death depends on the congenital variant of the anomalous coronary artery as well as the course these vessels take. This case highlights a rare congenital variant featuring both the LMCA and RCA originating from a common ostium, with the LMCA having a malignant course, a variant with the highest risk of sudden cardiac death.

An unusual cause of acute coronary syndrome: thrombosis of right coronary artery to right atrium fistula.

Coronary arterial fistulae are rare, but it is one of the most common coronary artery anomalies. Most of the cases are asymptomatic in younger patients unless it is large and of haemodynamic significance. The incidence of thromboembolic complications usually increases with age. We report a case of a young male in his early 20s presenting with central chest pain. Coronary computed tomographic angiography revealed acute coronary syndrome due to a fistula between right coronary artery and right atrium occluded by thrombus. After discussion with coronary and congenital heart multidisciplinary team, a consensus was agreed that we should manage him conservatively with anticoagulant and antiplatelet therapy and a 3-month follow-up strategy that included repeating cardiac imaging. After a year, his anticoagulation and antiplatelet medication was discontinued.

The Patient Experience After Spontaneous Coronary Artery Dissection.

OBJECTIVE: There is considerable burden of anxiety, depression, and post-traumatic stress disorder in patients with spontaneous coronary artery dissection (SCAD), yet research is limited on the experience and impact of SCAD from the patient perspective. This literature review sought to describe the current state of the literature on the patient experience of SCAD and consequences for patients following a SCAD event from the patient perspective. To better understand how people's experiences of SCAD affect their wellbeing, quality of life, lifestyles, and identity, and what would be useful from the patient perspective, an integrative review was performed. METHODS: An integrative literature review was conducted to understand the experience of SCAD and the post-event implications. Five databases were searched. Search terms included 'spontaneous coronary artery dissection', 'SCAD', 'patient', 'experience', 'perspective', and 'opinion'. English-language, peer-reviewed primary research in people with a diagnosis of SCAD that reflected the patient experience was included. Data indicating the SCAD experience including distress prevalence were extracted into an Excel spreadsheet, and narrative synthesis of included studies followed. FINDINGS: From 325 identified studies, five were included for review, yielding a combined sample of 447 participants. Patients with SCAD reported a lack of information about SCAD and the recovery process, and use of the internet for obtaining information. Patients with SCAD reported challenges in recovery including anxiety associated with fear of recurrence and uncertainty, and a need for greater support. A wide range of negative emotions was reported during and after the SCAD event. Participants reported participating in support groups, with mixed reviews of their appropriateness and effectiveness.