Variant origin of the right coronary artery from the left main coronary artery together with aortic valve stenosis: report of a rare case / Origen variante de la arteria coronaria derecha a partir de la arteria coronaria izquierda junto con estenosis de la valva aórtica: reporte de un caso raro

SUMMARY: Variations in the origin of the right coronary artery have an incidence between 0.09 % and 0.92 %. Herein, we report a rare case of a coronary artery anomaly in which the right coronary artery originates from the left main coronary artery. This variant was found during routine coronarography, combined with an artificial aortic valve. Despite their rare occurrence, some variations in the origins of the coronary arteries can be life threatening and are associated with a higher risk of sudden cardiac death. They can also pose serious technical challenges and predispose to complications during coronary angiographic procedures. Thus, knowledge of such anomalies is paramount for managing the patients correctly.

Las variaciones en el origen de la arteria coronaria derecha tienen una incidencia entre el 0,09 % y el 0,92 %. En este documento, informamos un caso raro de una anomalía de la arteria coronaria en la que la arteria coronaria derecha se originaba en la arteria coronaria izquierda. Esta variante se encontró durante una coronariografía de rutina, combinada con una válvula aórtica artificial. A pesar de su rara aparición, algunas variaciones en los orígenes de las arterias coronarias pueden poner en peligro la vida y se asocian con un mayor riesgo de muerte súbita cardíaca. También pueden plantear serios desafíos técnicos y predisponer a complicaciones durante los procedimientos angiográficos coronarios. Por tanto, el conocimiento de dichas anomalías es fundamental para el manejo correcto de los pacientes.

Analysis on missed diagnosis or misdiagnosis of anomalous origin of left coronary artery from pulmonary artery by echocardiography from one single medical center / 中华心血管病杂志

Objectives: To analyze the reasons of missed diagnosis or misdiagnosis on anomalous origin of left coronary artery from pulmonary artery (ALCAPA) by echocardiography. Methods: This is a retrospective study. Patients with ALCAPA who underwent surgical treatment in Union Hospital, Tongji Medical College, Huazhong University of Science and Technology from August 2008 to December 2021 were included. According to the results of preoperative echocardiography and surgical diagnosis, the patients were divided into confirmed group or missed diagnosis/misdiagnosis group. The results of preoperative echocardiography were collected, and the specific echocardiographic signs were analyzed. According to the experience of the doctors, the echocardiographic signs were divided into four types, namely clear displayed, vague/doubtful displayed, no display and no notice, and the display rate of each sign was calculated (display rate=number of clearly displayed cases/total number of cases×100%). By referring the surgical data, we analyzed and recorded the pathological anatomy and pathophysiological characteristics of the patients, and the rate of missed diagnosis/misdiagnosis of echocardiography in patients with different characteristics was compared. Results: A total of 21 patients were enrolled, including 11 males, aged 1.8 (0.8, 12.3) years (range 1 month to 47 years). Except for one patient with anomalous origin of left anterior descending artery, the others were all originated from the main left coronary artery (LCA). There were 13 cases of ALCAPA in infant and children, and 8 cases of adult ALCAPA. There were 15 cases in the confirmed group (diagnostic accuracy was 71.4% (15/21)), and 6 cases in the missed diagnosis/misdiagnosis group (three cases were misdiagnosed as primary endocardial fibroelastosis, two cases were misdiagnosed as coronary-pulmonary artery fistula; and one case was missed diagnosis). The working years of the physicians in the confirmed group were longer than those in the missed diagnosis/misdiagnosed group ((12.8±5.6) years vs. (8.3±4.7) years, P=0.045). In infants with ALCAPA, the detection rate of LCA-pulmonary shunt (8/10 vs. 0, P=0.035) and coronary collateral circulation (7/10 vs. 0, P=0.042) in confirmed group was higher than that in missed diagnosis/misdiagnosed group. In adult ALCAPA patients, the detection rate of LCA-pulmonary artery shunt was higher in confirmed group than that in missed diagnosis/misdiagnosed group (4/5 vs. 0, P=0.021). The missed diagnosis/misdiagnosis rate of adult type was higher than that of infant type (3/8 vs. 3/13, P=0.410). The rate of missed diagnosis/misdiagnosis was higher in patients with abnormal origin of branches than that of abnormal origin of main trunk (1/1 vs. 5/21, P=0.028). The rate of missed diagnosis/misdiagnosis in patients with LCA running between the main and pulmonary arteries was higher than that distant from the main pulmonary artery septum (4/7 vs. 2/14, P=0.064). The rate of missed diagnosis/misdiagnosis in patients with severe pulmonary hypertension was higher than that in patients without severe pulmonary hypertension (2/3 vs. 4/18, P=0.184). The reasons with an echocardiography missed diagnosis/misdiagnosis rate of≥50% included that (1) the proximal segment of LCA ran between the main and pulmonary arteries; (2) abnormal opening of LCA at the right posterior part of the pulmonary artery; (3) abnormal origin of LCA branches; (4) complicated with severe pulmonary hypertension. Conclusions: Echocardiography physicians' knowledge of ALCAPA and diagnostic vigilance are critical to the accuracy of diagnosis. Attention should be paid to the pediatric cases with no obvious precipitating factors of left ventricular enlargement, regardless of whether the left ventricular function is normal or not, the origin of coronary artery should be routinely explored.

Fístula da Artéria Coronária: Associação entre Padrões de Trajetos, Características Clínicas e Cardiopatias Congênitas / Coronary Artery Fistula: Association between Pathway Patterns, Clinical Features and Congenital Heart Disease

Resumo Fundamento A fístula da artéria coronária (FAC) é uma conexão direta entre uma ou mais artérias coronárias e câmaras cardíacas ou um grande vaso; pode estar associada à cardiopatia congênita. Objetivo Estabelecer os padrões de trajetos de FAC a partir de dados ecocardiográficos e correlacioná-los com aspectos clínicos e cardiopatias congênitas. Métodos Um total de 7.183 prontuários médicos de crianças menores de 5 anos de idade com cardiopatia submetidas a ecodopplercardiograma colorido foram analisados utilizando o teste de correlação de Spearman para associar sinais, sintomas e cardiopatia à FAC, com nível de significância de 5%. Resultados Vinte e seis crianças (0,0036%) apresentaram FAC, nos seguintes trajetos: da artéria coronária direita para o ventrículo direito (26,92%), da artéria coronária esquerda para o ventrículo direito (23,08%), do ramo interventricular anterior para o ventrículo direito (23,08%), da artéria coronária direita para o átrio direito (11,54%), da artéria coronária esquerda para o tronco pulmonar (7,69%) e do ramo interventricular anterior para o tronco pulmonar (7,69%). Em 57,69% dos pacientes, houve uma correlação positiva entre sintomas e a FAC (p = 0,445), relacionada à dispneia ou cianose (53,84%). Em 96,15%, a cardiopatia congênita estava associada à FAC; principalmente, a comunicação interventricular e a comunicação interatrial, em 34,62% dos casos, correlacionaram-se positivamente com a FAC (p = 0,295). O trajeto da FAC foi representado em três dimensões pelo software de modelagem, texturização e animação Cinema 4D R19. Conclusão A FAC é uma entidade anatômica incomum que apresenta quadro clínico compatível com dispneia e cianose e está associada a cardiopatias congênitas, principalmente com a CIV ou a CIA. De acordo com as análises ecocardiográficas, as fístulas na ACD, na ACE ou no RIVA representam aproximadamente um terço dos pacientes, com trajeto prioritário para as câmaras cardíacas direitas.

Abstract Background Coronary artery fistula (CAF) is a direct connection of one or more coronary arteries to cardiac chambers or a large vessel; it may be associated with congenital heart disease. Objective To establish CAF pathway patterns from echocardiographic data and to correlate them with clinical aspects and congenital heart disease. Methods A total of 7.183 medical records of children under the age of five years with cardiac disease submitted to color Doppler echocardiography and Spearman's Correlation test were used to associate signs and symptoms and cardiopathy to CAF with a significance level of 5%. Results Twenty six children (0.0036%) presented CAF: from the right coronary artery (RCA) to the right ventricle (RV) 26.92%, from the left coronary artery (LCA) to the RV 23.08%, from the anterior interventricular branch (AIVB) to RV 23.08%, RCA to right atrium (RA) 11.54%, LCA for pulmonary trunk (PT) 7.69% or AIVB for PT 7.69%. In 57.69% of the patients, there was a positively correlated symptomatology to CAF with p=0.445 related to dyspnea or cyanosis (53.84%); in 96.15%, congenital heart disease associated with CAF, mainly interventricular communication (IVC) or interatrial communication (IAC) in 34.62% positively correlated to CAF with p=0.295. CAF pathway was represented in three dimensions by software modeling, texturing and animation Cinema 4D R19. Conclusion CAF is an uncommon anatomical entity that presents a clinical picture compatible with dyspnea and cyanosis, and this is associated with congenital heart disease, mainly with IVC or IAC. According to echocardiographic analyzes, fistulas in RCA, LCA, or AIVB represent about one-third of the patients, with a priority pathway for right heart chambers.

Infarto agudo al miocardio secundario a disección coronaria espontánea biarterial: reporte de un caso y revisión de literatura / Acute myocardial infarction due to spontaneous two vessel coronary artery dissection

Resumen: La disección coronaria espontánea ha surgido como una causa interesante y poco reconocida de síndrome coronario agudo no aterosclerótico. Ocurre más frecuentemente en mujeres jóvenes, donde de forma importante puede asociarse a anomalías arteriales no coronarias. La clave para un tratamiento oportuno es el diagnóstico precoz que impida su evolución a cuadros más severos. Se presenta el caso de una mujer de 36 años con un síndrome coronario agudo secundario a una disección coronaria espontánea bi-arterial que evolucionó de forma grave a una insuficiencia cardíaca aguda. El objetivo es informar un cuadro poco usual y proporcionar evidencia que respalde el cómo debe enfrentarse.

Abstract: Spontaneous coronary dissection is an important cause of acute non-atherosclerotic coronary syndrome. It occurs more frequently in young women, often without significantly associated coronary arterial anomalies. Early diagnosis is a key to prompt treatment, in order to prevent severe complications. We report the case of a 36-year-old woman with an acute coronary syndrome secondary to spontaneous bi-arterial coronary dissection progressing to severe acute heart failure. A discussion of the management of this condition is included.

Anomalía coronaria sospechada por el signo "RAC" en resonancia magnética cardíaca / Anomalous coronary artery suspected by cardiac magnetic resonance "RAC" sign

Abstract A 63 year-old-male was admitted with a non-ST-segment elevation acute coronary syndrome. Due to poor acoustic windows ventricular function could not be evaluated by echocardiography. Magnetic resonance (CMR) revealed normal biventricular size and function without fibro- sis and portraying a rare finding consisting of a tubular structure originated in the aortic root following a retro aortic course between the aorta and left atrium, traversing through the atrioventricular groove. These characteristics raised the suspicion of an anomalous coronary artery origin. Coronary angiography confirmed the presence of two coronary arteries, the right coronary artery (RCA) and the left circumflex artery (LCx) originating from the right coronary sinus via two separate ostia. The LCx followed a retro aortic course proximally be- fore irrigating the left ventricular lateral wall. From the left coronary sinus, the left anterior descending artery followed its normal course with a thin diagonal vessel that presented an acute plaque. Due to these low-risk findings, medical management was chosen.

Disección coronaria espontánea en paciente trasplantada de corazón / Spontaneous coronary artery dissection in a heart transplant patient: report of one case

Spontaneous coronary artery dissection is an uncommon cause of acute coronary syndrome that predominantly affects women without cardiovascular risk factors. In transplant patients, spontaneous coronary artery dissection is an extremely rare condition, having been described in only three patients, in whom vascular damage secondary to the use of anticalcineurinics is postulated as a probable mechanism. We report a spontaneous coronary dissection in a female who received a heart transplant at 34 years of age. The diagnosis was made in a follow-up coronary angiography three years after transplantation, supplemented with optical coherence tomography. A percutaneous coronary revascularization of the involved artery was performed, with good immediate results and at one year of follow-up.

Challenges in diagnosis and management of spontaneous coronary artery dissection in a young patient

Abstract Spontaneous coronary artery dissection (SCAD) is characterized by tear of the inner layer in the coronary artery, creating a false lumen between the inner and central layer. Its infrequent incidence often leads to delay in diagnosis posing challenges in management. There are currently no guidelines for the treatment of this condition. We describe an adult patient who presented with multiple episodes of ventricular fibrillation, in whom cardiac catheterization showed SCAD, treated by off-pump coronary artery bypass.

Arteria coronaria única y cardiopatía coronaria: origen anómalo de la arteria coronaria derecha desde la descendente anterior: casos clínicos / Anomalous origin of the right coronary artery in the middle third of the anterior descending artery: report of two cases

The anomalous origin of the coronary arteries is uncommon and the origin of the right coronary artery from the middle third of the anterior descending artery is a finding of extreme rarity. We report two patients with coronary atherosclerotic disease and with an acute coronary syndrome, in whom a single left coronary artery was found. The clinical and angiographic characteristics and the treatment of these patients are described.

Anomalías congénitas de arterias coronarias, estudio de aquellas con Importancia hemodinámica / Evaluation of the hemodynamically significant congenital anomalies of coronary arteries

Resumen: Las anomalías de las arterias coronarias son poco frecuentes, con una prevalencia de 0,21- 5,79%. Su presentación clínica es amplia, pudiendo ser asintomáticas o presentarse como isquemia miocárdica y muerte súbita, la que puede estar ligada o no al ejercicio. Existen varias clasificaciones, siendo las más usadas las que las agrupan desde un punto de vista anatómico en relación con el segmento afectado (origen, curso o terminación) y desde el punto de vista funcional (hemodinámicamente significativa y no significativa). Actualmente la Tomografía Computada Cardiaca se considera el estándar de referencia, siendo de suma importancia su adecuada caracterización ya que, de requerir tratamiento, este generalmente es quirúrgico. El objetivo de este trabajo es identificar, caracterizar y clasificar las anomalías congénitas de las arterias coronarias por su importancia hemodinámica mediante la presentación de casos clínicos y revisión de la literatura.

Abstract: Coronary artery anomalies are rare, with a prevalence ranging from 0.21 to 5.79%. Their clinical presentation is variable; being either asymptomatic or presenting as myocardial ischemia and sudden death, which may or may not be linked to exercise. There are several classifications, the most commonly used being those that sort them from an anatomical point of view in relation to the affected segment (origin, course or termination) and from a functional point of view (hemodynamically significant and not significant). Currently, Cardiac Computed Tomography is considered the reference standard allowing an adequate characterization of the anomaly, which is highly relevant since, if treatment is needed, it usually involves surgery. The purpose of this review is to identify, characterize and classify congenital anomalies of the coronary arteries according to their hemodynamic significance through the presentation of clinical cases and review of the literature.