Clinical and cardiac CT characteristics of congenital coronary abnormalities occasionally detected in a middle-aged population: A long-term follow-up study.

INTRODUCTION: Congenital coronary artery anomalies (CCAA) represent one of the most challenging conditions as their clinical presentation may range from sudden cardiac death to a complete subclinical form. The aim of the present study was to evaluate the diagnostic and prognostic role of cardiac CT (CCT) evaluation in patients with CCAA, focusing on anomalies of origin. MATERIAL AND METHODS: The present is a retrospective analysis of a prospective clinical registry including a consecutive cohort of patients who underwent clinically indicated CCT from January 2007 to October 2015 for suspected but unknown coronary artery disease (CAD) and were diagnosed for having a congenital coronary abnormality compared to a control group matched for age, sex and segment stenosis score (SSS). Dedicated analysis of all CCT was performed for the present study and only coronary anomalies of origin were considered and included in the study. Two different composite end-points were identified for the present analysis: major cardiovascular events (MACE) and all-cause of death. RESULTS: Among the 81 patients with CCAAs enrolled the most frequent anomaly was left main artery absence, which was identified in 41 individuals (50.6%). Forty-five subjects (55.5%) have an anomalous origin of the coronary artery from a different sinus of Valsalva and 45 subjects had also an anomalous course with the retro-aortic being the most common (32%). Eleven participants (13.6%) displayed also an intramural segment, while 10 (10.3%) had a slit-like ostial morphology. At multivariate analysis CT identification of ARCA, anomalous inter-arterial course and abnormal ostial morphology were significantly associated with MACE even when adjusted for age and SSS, without any differences in all-cause mortality between the two groups (6.2% vs 2.4% p â€‹= â€‹0.2478). CONCLUSION: The result of the present study is that CCT can be successfully used to define the anatomy and features of CAA. It suggested that in middle-aged patients, the identification of high risk characteristics at CT may have a prognostic value in term of cardiovascular events occurrence at follow-up even if the rate of events strictly linked to CCAA is low.

Origen aórtico anómalo de las arterias coronarias / Abnormal aortic origin of coronary arteries

Resumen Introducción: El origen aórtico anómalo de las arterias coronarias tiene una prevalencia estimada del 0.02-5.7% y están potencialmente involucradas con complicaciones en su evolución natural o aquellos que requieran procedimientos intervencionistas hemodinámico y/o quirúrgicos. Puede asociarse a muerte súbita o dañarse durante intervenciones sobre el anillo mitral, aórtico, pulmonar o el cierre percutáneo de un defecto septal interauricular. Objetivo: identificar estos pacientes por diferentes técnicas de imágenes como el ecocardiograma Doppler color transtorácico (ETT), angiotomografía o angiografía coronaria. Métodos: Las técnicas de imágenes utilizadas para la detección de anomalías coronarias fueron el ETT, angiotomografía coronaria multicorte o angiografía coronaria convencional de acuerdo con lo universalmente aceptado. Resultados: Estudio prospectivo realizado desde enero del 2020 a junio del 2021. Se identificaron 15 pacientes y en 12/15 la sospecha fue por ETT y en los tres restantes por angiotomografía coronaria. La arteria circunfleja fue la más involucrada en forma aislada o asociada a otra anomalía coronaria (12/15 pacientes) y en los tres casos restantes la coronaria anómala tuvo un trayecto interarterial, siendo las arterias coronarias derecha y la descendente anterior las afectadas. Conclusiones: El subdiagnóstico por ETT de las anomalías de las arterias coronarias puede deberse a la dificultad para su visualización, sobre todo en la población adulta. Su detección es crucial, ya que puede generar muerte súbita asociada a isquemia miocárdica y arritmias graves o complicar procedimientos intervencionistas sobre el septum interauricular o sobre los anillos mitral, pulmonar y/o aórtico.

Abstract Introduction: The anomalous aortic origin of the coronary arteries has an estimated prevalence of 0.02-5.7%. It can be associated with sudden death when it has an interarterial or intramural pathway or be damaged during interventions on the mitral, pulmonary and/or aortic annulus or percutaneous closure of an interatrial septal defect. Objective: To identify these patients by imaging techniques such as transthoracic color Doppler echocardiography (TTE), computed tomography (CT) multislice angiography or coronary angiography. Methods: The imaging techniques used for the detection of coronary anomalies were TTE, multislice coronary angiography or coronary angiography according to what is generally accepted. Results: Fifteen patients were identified; in 12 of them the suspicion was due to TTE and in the remaining 3, CT multislice angiography was diagnostic. The circumflex artery was the coronary artery most involved, associated or not with another coronary anomaly (12/15 patients) and in the other three cases, the anomalous coronary artery had an interarterial course, with the right coronary arteries and the anterior descending coronary arteries being involved. Conclusions: The under diagnosis by TTE of coronary artery abnormalities may be due to the difficulty of visualization that is accentuated with age. Their detection is crucial because they can both, lead to sudden death associated with an intramural and/or interarterial pathway and complicate an interventional procedure on the interatrial septum or within the mitral, pulmonary and/or aortic rings.

[Abnormal aortic origin of coronary arteries]. / Origen aórtico anómalo de las arterias coronarias.

Introduction: The anomalous aortic origin of the coronary arteries has an estimated prevalence of 0.02-5.7%. It can be associated with sudden death when it has an interarterial or intramural pathway or be damaged during interventions on the mitral, pulmonary and/or aortic annulus or percutaneous closure of an interatrial septal defect. Objective: To identify these patients by imaging techniques such as transthoracic color Doppler echocardiography (TTE), computed tomography (CT) multislice angiography or coronary angiography. Methods: The imaging techniques used for the detection of coronary anomalies were TTE, multislice coronary angiography or coronary angiography according to what is generally accepted. Results: Fifteen patients were identified; in 12 of them the suspicion was due to TTE and in the remaining 3, CT multislice angiography was diagnostic. The circumflex artery was the coronary artery most involved, associated or not with another coronary anomaly (12/15 patients) and in the other three cases, the anomalous coronary artery had an interarterial course, with the right coronary arteries and the anterior descending coronary arteries being involved. Conclusions: The under diagnosis by TTE of coronary artery abnormalities may be due to the difficulty of visualization that is accentuated with age. Their detection is crucial because they can both, lead to sudden death associated with an intramural and/or interarterial pathway and complicate an interventional procedure on the interatrial septum or within the mitral, pulmonary and/or aortic rings.

Introducción: El origen aórtico anómalo de las arterias coronarias tiene una prevalencia estimada del 0.02-5.7% y están potencialmente involucradas con complicaciones en su evolución natural o aquellos que requieran procedimientos intervencionistas hemodinámico y/o quirúrgicos. Puede asociarse a muerte súbita o dañarse durante intervenciones sobre el anillo mitral, aórtico, pulmonar o el cierre percutáneo de un defecto septal interauricular. Objetivo: Identificar estos pacientes por diferentes técnicas de imágenes como el ecocardiograma Doppler color transtorácico (ETT), angiotomografía o angiografía coronaria. Métodos: Las técnicas de imágenes utilizadas para la detección de anomalías coronarias fueron el ETT, angiotomografía coronaria multicorte o angiografía coronaria convencional de acuerdo con lo universalmente aceptado. Resultados: Estudio prospectivo realizado desde enero del 2020 a junio del 2021. Se identificaron 15 pacientes y en 12/15 la sospecha fue por ETT y en los tres restantes por angiotomografía coronaria. La arteria circunfleja fue la más involucrada en forma aislada o asociada a otra anomalía coronaria (12/15 pacientes) y en los tres casos restantes la coronaria anómala tuvo un trayecto interarterial, siendo las arterias coronarias derecha y la descendente anterior las afectadas. Conclusiones: El subdiagnóstico por ETT de las anomalías de las arterias coronarias puede deberse a la dificultad para su visualización, sobre todo en la población adulta. Su detección es crucial, ya que puede generar muerte súbita asociada a isquemia miocárdica y arritmias graves o complicar procedimientos intervencionistas sobre el septum interauricular o sobre los anillos mitral, pulmonar y/o aórtico.

Longitudinal Cardiac Outcomes of Multisystem Inflammatory Syndrome in Children: A Systematic Review and Meta-Analysis.

There is a paucity of longitudinal data on cardiac outcomes in multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19. We aimed to investigate the longitudinal cardiovascular outcomes in MIS-C. PubMed and EMBASE were searched through May 2022. Observational studies were included, reporting mid-term (≥ 3 months) outcomes in children (aged < 21) with MIS-C. Data were extracted by two researchers. Longitudinal outcomes were synthesized by a one-group meta-analysis using a random-effects model. Eleven studies with a follow-up period (3 months to 1 year) were identified, including 547 MIS-C patients. The mortality was 2.5% (95% CI 1.3-4.9). The majority of left ventricular (LV) systolic dysfunction present in 46.8% (95% CI 32.7-61.3) in the acute phase resolved by 3 months, and the prevalence of LV systolic dysfunction was 1.7% (95% CI 0.5-5.7) and 2.1% (95% CI 0.8-5.4) at 3 month and 6 month follow-up, respectively. Additionally, the persistent LV systolic dysfunction in the small population was mild. However, coronary abnormalities such as coronary artery dilatation or aneurysms, seen in 23.7% (95% CI 17.7-31.1) at baseline, persisted in 4.7% (95% CI 1.5-14.3) at 3 months and 5.2% (95% CI 3.0-8.9) at 6 months. Mitral regurgitation (MR), which was observed in 56.6% (95% CI 27.7-81.6) at baseline, also persisted in 7.5% at 6 months. In conclusion, our study demonstrated largely favorable cardiac outcomes, suggesting resolution of LV systolic dysfunction in the majority of cases. However, coronary abnormalities and MR persisted in a subset of patients at mid-term follow-up.

Intentional Misalignment of a Transcatheter Aortic Valve to Preserve Reaccess to Coronaries of Anomalous Origin.

Achieving patient-specific commissural alignment of transcatheter aortic valves is particularly important to ensure coronary reaccess after transcatheter aortic valve implantation. Nevertheless, in case of uncommon origin of coronary arteries, commissural alignment could be counterproductive. This case shows how alignment techniques could serve to intentionally misalign the neocommissures in this subset of patients. (Level of Difficulty: Intermediate.).

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in adults: Collateral circulation does not preclude direct reimplantation.

BACKGROUND: The adult type of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is characterized by late presentation due to collateral coronary flow. Patients often present with long time recurrent angina or dyspnea. Surgical correction can be a challenge due to the vast collateral coronary circulation. CASE PRESENTATION: A previously healthy 41-year-old male presented in the emergency room referring typical angina and dyspnea. Coronary angiography revealed ALCAPA. Surgical correction was indicated. Under cardiopulmonary bypass, the left main coronary artery ostium was isolated and directly reimplanted in the aorta. The patient was discharged from the hospital without complications and asymptomatic. Control coronary angiography showed enlarged left main coronary artery and regression of the collateral circulation. CONCLUSION: Even with a well-developed collateral circulation, detachment of the left coronary artery and direct implantation in the aorta is feasible, achieving good long-term results.

Cardiac Abnormalities Seen in Pediatric Patients During the SARS-CoV2 Pandemic: An International Experience.

Background During the SARS-CoV2 pandemic, there has been increase in hyperinflammatory presentation in previously healthy children with a variety of cardiac manifestations. Our objective is to describe the cardiac manifestations found in an international cohort of 55 pediatric cases with multi-system inflammatory syndrome (MIS-C) during the SARS-CoV2 pandemic. Methods and Results We reviewed data on previously healthy pediatric patients (≤18 years) with structurally normal hearts who presented at hospitals in the United States, United Kingdom, Spain and Pakistan with MIS-C and had consultation with a pediatric cardiologist. Data collected included demographics, clinical presentation, laboratory values, electrocardiographic abnormalities, echocardiographic findings and initial therapies. A total of 55 patients presented with MIS-C. Thirty-five patients (64%) had evidence of decreased left ventricular function, 17 (31%) had valvulitis, 12 (22%) with pericardial effusion and 11 (20%) with coronary abnormalities. Twenty-seven (49%) required ICU admission and 24 (44%) had evidence of shock. Eleven patients (20%) fulfilled complete Kawasaki disease criteria and had lower NT pro-BNP, D-dimer and ferritin levels compared with those who did not fulfill criteria. Electrophysiologic abnormalities occurred in 6 patients and included complete atrioventricular (AV) block, transient AV block and ventricular tachycardia. Conclusions We describe the first international cohort of pediatric patients with MIS-C during the SARS-CoV2 pandemic with a range of cardiac manifestations. This paper brings awareness and alertness to the global medical community to recognize these children during the pandemic and understand the need for early cardiology evaluation and follow-up.

Transthoracic Echocardiographic Assessment of Coronary Flow in the Diagnosis of Right Ventricular-Dependent Coronary Circulation in Pulmonary Atresia with Intact Ventricular Septum.

Right ventricular-dependent coronary circulation (RVDCC) is associated with pulmonary atresia with intact ventricular septum and is defined by two or more epicardial coronary arteries with atresia or severe stenosis resulting in the dependency of coronary supply by retrograde flow. The hypothesis of this study is that coronary Doppler flow patterns on echocardiography can be used to distinguish patients with RVDCC. Between 2007 and 2016, we reviewed 16 patients with pulmonary atresia or critical pulmonary stenosis. Patients were divided into two groups, those with RVDCC (determined by angiography or pathology evaluation) and those without. Echocardiographic evaluation of the coronary arteries included 2-dimensional measurements and pulse wave Doppler flow pattern in 3 epicardial coronary arteries. Velocity-time integral (VTI) and maximal velocity (Vmax) were measured and compared between the two groups. Three coronary flow patterns were demonstrated: (1) all antegrade flow, (2) antegrade to retrograde VTI flow ratio > 1, and (3) antegrade to retrograde VTI flow ratio ≤ 1. Of the 7 patients with RVDCC, 6 (86%) had evidence of flow pattern 3 in ≥ 2 of the 3 coronary arteries in contrast to 0 (0%) of the non-RVDCC patients (p = 0.001). Higher retrograde Vmax was associated with RVDCC (p < 0.001) and coronary artery dilatation with Z-score ≥ + 3 was also associated with RVDCC (p = 0.02). Echocardiographic evaluation of the coronaries can be useful in identifying RVDCC. More retrograde flow in at least two coronary arteries is strongly suggestive of RVDCC. Dilatation of the coronary arteries is also supportive evidence.

[Sudden cardiac death and anomalous connections of the coronary arteries: What is known and what is unknown?] / Mort subite cardiaque et anomalies de connexion des artères coronaires : connaissances et questions.

Some anomalous connections of the coronary arteries may be associated with a risk of sudden cardiac death. In opposite with others cardiac diseases at risk of sudden cardiac death, the relationship between these congenital abnormalities and the risk of sudden cardiac death are not well understood. A correction of the anomaly is generally indicated after an aborted sudden cardiac death. Primary prevention strategy after the discovery of an anomaly at risk is debated. Even if the absolute risk of sudden death is very low, a pre-participation screening in young athletes may be discussed due to a non-rare incidence.

Coronary artery fistula connecting the left main coronary artery with the superior vena cava.

BACKGROUND: Coronary artery fistulas are rare congenital coronary artery abnormalities, with direct communication between a coronary artery and a cardiac chamber, great vessel or other structure. DESCRIPTION OF CASE: We report a case of a large coronary artery fistula connecting the left main coronary artery with the superior vena cava in a 70-year-old patient undergoing diagnostic coronary angiography for a non-ST-segment elevation myocardial infarction. The patient rejected closure of the fistula and remains asymptomatic on follow-up. CONCLUSION: Angiographic recognition of coronary artery fistulas is important for the appropriate diagnosis and management of patients. Hippokratia 2015; 19 (2):186-188.

[Ectopic connection of the left coronary artery with the contralateral sinus with or without intramural pathway: how and why differentiating them?]. / Connexion ectopique de l'artère coronaire gauche dans le sinus controlatéral avec ou sans passage intramural: comment et pourquoi les différencier?

Among the wide spectrum of congenital abnormalities of coronary arteries, a left coronary artery connected with the contralateral sinus is considered as an anatomical high-risk while associated with a proximal preaortic course. The ectopic connection may be associated with or without intramural pathway regarding the aortic wall. An intramural pathway is a characteristic to be considered because it is able to be involved in myocardial ischemia-related adverse events of which the sudden death. Therefore, making an identification of an intramural pathway is essential for the ectopic connections of the left coronary artery associated with a preaortic course.

Timing of intravenous immunoglobulin treatment and risk of coronary artery abnormalities in children with Kawasaki disease.

BACKGROUND: Kawasaki disease (KD) is a type of febrile self-limiting systemic vasculitis, which affects the coronary arteries (CA) and may cause cardiac ischemia during childhood and adult life. Intravenous immunoglobulin (IVIG) has become the standard therapy for KD. However, it is still uncertain if CA outcome is associated with the timing of IVIG administration with reference to fever onset. METHODS: The present study was designed to identify the risk for development and delay in resolution of CA abnormalities in association with IVIG administration within or after 10 days of KD onset. A retrospective analysis of clinical signs, laboratory data, and prospectively collected echocardiography (ECHO) results of 106 children hospitalized with KD was utilized. RESULTS: IVIG was administered to 86 (81.1%) patients within 10 days, and 20 (18.9%) patients received the first dose of IVIG after 10 days of illness. Among 23 (21.6%) patients who were diagnosed with CA lesions, 18 had a CA abnormality at initial ECHO, whereas they appeared after IVIG therapy in five patients. The risk for CA lesions on initial ECHO was higher among the patients who were admitted after 10 days of disease onset [odds ratio (OR) = 5.3, 95% confidence interval (CI) = 1.7-15.9] but comparable with the post-IVIG treatment group (OR = 3.1, 95% CI = 0.48-19.8). The age <1 year and erythrocyte sedimentation rate (ESR) > 40 mm/hour were associated with non-resolution of CA lesions within 9 weeks of KD onset. Overall, 95.6% of children had resolution of CA abnormalities within 6 months of onset of KD symptoms. CONCLUSION: The results of this study suggest that although IVIG treatment within 10 days is important to minimize development of cardiac pathology, neither occurrence of CA lesions in IVIG-treated children nor the time frame for resolution of established CA abnormalities was associated with the timing of IVIG administration. Age <1 year and high ESR (>40 mm/hour) predict a delay in resolution of CA lesions among children with KD.

[Single coronary ostium: single coronary artery and ectopic coronary artery connected with the contralateral artery. How and why differentiating them?]. / Ostium coronaire unique : artère coronaire unique ou artère coronaire ectopique connectée à l'artère controlatérale. Comment et pourquoi les différencier ?

Among the wide spectrum of congenital abnormalities of coronary arteries, a single coronary artery is often confused with an ectopic coronary artery connected with the contralateral coronary artery. Both abnormalities are characterized by a single coronary ostium, but they differ by the lack or not of an initial ectopic course. The prognosis of anomalous connections of coronary arteries depends mainly on the type of the initial course in relation to other cardiac structures. Therefore, the distinction between a single coronary artery and an ectopic coronary artery connected with the contralateral artery is of importance.

Grande fístula bilateral da artéria coronária: a escolha do tratamento clínico / Gran fístula bilateral de la arteria coronaria: la selección del tratamiento clínico / Large bilateral coronary artery fistula: the choice of clinical treatment

Relatamos o caso de um paciente do sexo feminino, assintomática, com grande fístula bilateral da artéria coronária-artéria pulmonar. O monitoramento clínico foi a opção escolhida para tratamento. Discutimos os pormenores das opções terapêuticas, enfatizando a tendência excessiva de utilizar a abordagem cirúrgica e a grande escassez de acompanhamentos clínicos de longo prazo em pacientes assintomáticos.

We report the case of an asymptomatic female patient, with a large non-complicated bilateral coronary-pulmonary artery fistula. Clinical monitoring was the choice of treatment. We discuss the therapeutic options in depth, emphasizing the excessive tendency of the surgical approach and the great scarcity of reports on long-term clinical follow-up in asymptomatic patients.

Relatamos el caso de un paciente del sexo femenino, asintomática, con gran fístula bilateral de la arteria coronaria-arteria pulmonar. El monitoreo clínico fue la opción elegida para el tratamiento. Discutimos los pormenores de las opciones terapéuticas, enfatizando la tendencia excesiva de utilizar el abordaje quirúrgico y la gran escasez de seguimientos clínicos de largo plazo en pacientes asintomáticos.