Poliarteritis nodosa sistémica / Systemic polyarteritis nodosa

Resumen La poliarteritis nodosa sistémica de inicio en la infancia es una vasculitis caracterizada por inflamación y necrosis fibrinoide de las arterias de mediano y pequeño calibre, con cuadro clínico extraordinariamente variable y dependiente de la localización histológica de la lesión. Su compromiso cardiaco constituye un comportamiento infrecuente en pediatría, por lo que se considera de interés presentar el caso de un escolar de 8 años, determinado por compromiso hemodinámico secundario a derrame pericárdico, disfunción sistólica grave y múltiples dilataciones aneurismáticas, que permite establecer la sospecha de vasculitis de vasos pequeños y medianos. Se realiza un diagnóstico oportuno gracias al cual se evitan complicaciones potencialmente mortales y se instaura un tratamiento con buena respuesta a corto, mediano y largo plazo.

Abstract Systemic polyarteritis nodosa of childhood onset is a vasculitis characterized by inflammation and fibrinoid necrosis in the medium and small-caliber arteries, with an extraordinarily variable clinical picture and dependent on the histological location of the lesion. His cardiac involvement constitutes an infrequent behavior in pediatrics, so it is considered of interest to present the case of an 8-year-old schoolboy, characterized by hemodynamic compromise secondary to pericardial effusion, severe systolic dysfunction, and multiple aneurysmal dilatations that establish the suspicion of vasculitis. small and medium glasses. A timely diagnosis is made avoiding life-threatening complications and allowing to establish a treatment with a good response in the short, medium and long term.

Oblivion: autopsy findings of a 31-year-old man with sudden cardiac arrest, a case report of a sequalae of Kawasaki disease.

A 31-year-old man presented to the hospital after suffering a sudden cardiac arrest. Despite optimal therapy, the patient passed away. His medical history included febrile rash at age 2. At autopsy, there was aneurysmal dilation and severe coronary artery stenosis by atherosclerotic plaques and myocardial fibrosis. These findings were presumed to be due to complications of Kawasaki disease, given the remote history of severe febrile rash as a toddler and the presence of chronic coronary artery injury, recanalization, and thrombosis with ischemic heart disease leading to sudden cardiac collapse and death.

Successful Surgical Treatment of Coronary Aneurysm, Ascending Aortic Aneurysm, and Bicuspid Aorta for a Kawasaki Disease Patient

Abstract Kawasaki disease was first reported in 1967, and it was classified as an autoimmune vasculitis of the small and medium arteries. It is a self-limiting condition that occurs mostly in childhood, but it may involve complications — such as coronary artery aneurysms, myocardial ischemia, and arrhythmias — with significant morbidity and mortality that occur later in life. In this article, we present the association of an ascending aortic aneurysm with bicuspid aortic disease in addition to coronary aneurysm in a 55-year-old patient diagnosed with Kawasaki disease.

Giant Left Anterior Descending Coronary Artery Aneurysm in a Patient with Behçet's Disease.

Coronary artery involvement is quite rare in the course of Behçet's disease. Complications secondary to coronary artery aneurysms, including rupture, dissection, and myocardial ischemia, may be fatal. In young patients without cardiovascular risk factors, systemic inflammatory vasculitis syndromes should be investigated in case of acute coronary syndrome secondary to dilated coronary arteries. In this report, we present our management strategy in a 31-year-old male patient with Bechet's disease.

Giant Left Anterior Descending Coronary Artery Aneurysm in a Patient with Behçet's Disease

ABSTRACT Coronary artery involvement is quite rare in the course of Behçet's disease. Complications secondary to coronary artery aneurysms, including rupture, dissection, and myocardial ischemia, may be fatal. In young patients without cardiovascular risk factors, systemic inflammatory vasculitis syndromes should be investigated in case of acute coronary syndrome secondary to dilated coronary arteries. In this report, we present our management strategy in a 31-year-old male patient with Bechet's disease.

Nonatherosclerotic Giant Right Coronary Artery Aneurysm.

We present an unusual case of a 67-year-old woman with an incidental finding of a cardiac mass on a chest computed tomography. Coronary angiotomography confirmed the diagnosis of right coronary artery aneurysm, with 5.7×5.7 cm. The patient underwent aneurysm resection and coronary bypass surgery, with subsequent histologic study suggestive of arteritis sequelae. Giant coronary artery aneurysms have a high risk of complications and aneurysm exclusion must be beneficial. This is a rare condition that can also be part of a systemic inflammatory disease.

Nonatherosclerotic Giant Right Coronary Artery Aneurysm

Abstract We present an unusual case of a 67-year-old woman with an incidental finding of a cardiac mass on a chest computed tomography. Coronary angiotomography confirmed the diagnosis of right coronary artery aneurysm, with 5.7×5.7 cm. The patient underwent aneurysm resection and coronary bypass surgery, with subsequent histologic study suggestive of arteritis sequelae. Giant coronary artery aneurysms have a high risk of complications and aneurysm exclusion must be beneficial. This is a rare condition that can also be part of a systemic inflammatory disease.

Aneurisma de arteria coronaria derecha y elevación del segmento ST en cara inferior. Reporte de caso y revisión de la literatura / Right Coronary Artery Aneurysm and inferior ST segment elevation. Case Report and Literature Review / Aneurisma de artéria coronária direita e elevação do segmento ST na cara inferior. Reporte de caso e revisão da literatura

Los aneurismas de las arterias coronarias (AAC) son poco comunes, con una incidencia de 0.3-5.3%. Se definen como una dilatación 1.5 veces mayor al diámetro interno de la arteria normal adyacente. La arteria coronaria derecha es la más comúnmente afectada. Su fisiopatología es poco clara, pero se cree que una de sus principales causas es la aterosclerosis. Los AAC son generalmente asintomáticos o pueden estar asociados a isquemia miocárdica. Su tratamiento aún no está bien establecido, debido al desconocimiento general sobre su historia natural, e incluye desde un manejo conservador hasta el tratamiento quirúrgico. A continuación, se reporta el caso de un paciente con infarto agudo de miocardio y elevación del segmento ST, llevado a coronariografía que evidenció aneurisma de la coronaria derecha, que requirió trombectomía, angioplastia y manejo médico vitalicio ambulatorio.

Coronary artery aneurysms (CAA) are a rare entity with an incidence of 0.3-5.3%. They are dilations of 1.5 times larger than normal adjacent artery, with the right coronary artery as the most affected vessel. Its pathophysiology is unclear but atherosclerosis is believed to be the main cause. CAA are generally asymptomatic but can cause coronary ischemia. Its treatment is yet to be established due to general ignorance about its nature. It ranges from conservative management to surgery. In the following text, we report a case of a patient with acute myocardial infarction presenting ST-segment elevation, who showed a right coronary artery aneurysm in coronary angiography. The patient required thrombectomy, angioplasty and subsequent lifetime outpatient medical management.

Os aneurismas das artérias coronárias (AAC) são pouco comuns, com uma incidência de 0.3-5.3%. Se definem como uma dilatação 1.5 vezes maior ao diâmetro interno da artéria normal adjacente. A artéria coronária direita é a mais comumente afetada. Sua fisiopatologia é pouco clara, mas se crê que uma das suas principais causas é a aterosclerose. Os AAC são geralmente assintomáticos ou podem estar associados a isquemia miocárdica. Seu tratamento ainda não está bem estabelecido, devido ao desconhecimento geral sobre sua história natural, e inclui desde um manejo conservador até o tratamento cirúrgico. A continuação, se reporta o caso de um paciente com infarto agudo de miocárdio e elevação do segmento ST, levado a coronariografia que evidenciou aneurisma da coronária direita, que requereu trombectomia, angioplastia e manejo médico vitalício ambulatório.

Successful Surgical Treatment of Coronary Aneurysm, Ascending Aortic Aneurysm, and Bicuspid Aorta for a Kawasaki Disease Patient.

Kawasaki disease was first reported in 1967, and it was classified as an autoimmune vasculitis of the small and medium arteries. It is a self-limiting condition that occurs mostly in childhood, but it may involve complications - such as coronary artery aneurysms, myocardial ischemia, and arrhythmias - with significant morbidity and mortality that occur later in life. In this article, we present the association of an ascending aortic aneurysm with bicuspid aortic disease in addition to coronary aneurysm in a 55-year-old patient diagnosed with Kawasaki disease.

Resolution of coronary arteritis following tuberculosis treatment.

BACKGROUND: Coronary artery aneurysm (CAA) in an uncommon condition usually associated with atherosclerosis, but systemic vasculitides constitute important differential diagnoses. A less recognized cause of CAA, tuberculosis (TB) has also been noted to occur simultaneously in patients with such vascular abnormalities. CASE REPORT: A 60-year-old female presented to the Emergency Department with a non-ST segment elevation myocardial infarction. Angiography demonstrated segmental aneurysms of the left anterior descending coronary artery. Shortly after, she was also diagnosed with cutaneous TB, and treatment was promptly initiated. Reevaluation conducted several months later demonstrated that levels of inflammation markers had significantly decreased. New catheterization of coronary arteries evidenced complete resolution of coronary aneurysm images. CONCLUSION: Due to the clinical and radiologic resolution with only TB treatment, as well as lack of evidence supporting atherosclerotic or vasculitic etiologies, TB can be considered a possible contributor to aneurysm formation in this case. Prospective studies are necessary to reliably demonstrate causality between TB infection and CAA.

[Advanced heart failure due to coronary aneurysms]. / Falla cardiaca avanzada por aneurismas coronarios.

We present the case of a 61 years old man with hypothyroidism, hypertension, type 2 diabetes mellitus, and ischemic cardiopathy, who was admitted with a diagnosis of non-ST elevation myocardial infarction. The coronary angiography describes coronary ectasia with giant aneurysm and slow flow in the right coronary and aneurysm with slow flow anterior descending coronary. The echocardiogram shows contractility disorders with severely decreased left ventricular ejection fraction (20%) despite optimal medical management. This case presents a patient with ischemic heart disease and advanced heart failure secondary to coronary aneurysm, the proposed optimal treatment was a heart transplant given the disease's progression.

Coronary Artery Changes in Patients with Multisystem Inflammatory Syndrome in Children: Los Angeles Experience.

We compared cardiac findings in patients with multisystem inflammatory syndrome in children and Kawasaki disease in the first 6 months of the 2020 coronavirus disease pandemic to patients with Kawasaki disease during 2016-2019. We saw a high rate of coronary aneurysms in 2020, with a similar rate of coronary involvement but greater volume and incidence of cardiac dysfunction compared with previous years.

Oblivion: autopsy findings of a 31-year-old man with sudden cardiac arrest, a case report of a sequalae of Kawasaki disease

ABSTRACT A 31-year-old man presented to the hospital after suffering a sudden cardiac arrest. Despite optimal therapy, the patient passed away. His medical history included febrile rash at age 2. At autopsy, there was aneurysmal dilation and severe coronary artery stenosis by atherosclerotic plaques and myocardial fibrosis. These findings were presumed to be due to complications of Kawasaki disease, given the remote history of severe febrile rash as a toddler and the presence of chronic coronary artery injury, recanalization, and thrombosis with ischemic heart disease leading to sudden cardiac collapse and death.

Adenitis cervical imitando a un absceso cervical bacteriano en un lactante con Enfermedad de Kawasaki incompleta y aneurisma coronario: Reporte de un caso / Cervical adenitis mimicking a bacterial cervical abscess in an infant with incomplete Kawasaki disease and coronary aneurysm: A case repor

Antecedentes: La enfermedad de Kawasaki (EK) es una vasculitis aguda y autolimitada frecuente en menores de cinco años; sin embargo, su diagnóstico es un reto en menores de seis meses. Reporte de caso: Reportamos un lactante de tres meses con fiebre y tumoración cervical dolorosa de tres días de enfermedad. Fue diagnosticado como tumoración cervical abscedada y recibió tratamiento con antibióticos por 10 días. En hospitalización presentó lesiones eritematosas maculares en todo el cuerpo, persistencia de fiebre y elevación de marcadores inflamatorios. Se realizó una ecocardiografía encontrándose dilatación de las arterias coronarias siendo diagnosticado de EK incompleto. Recibió inmunoglobulina humana endovenosa y ácido acetil salicílico. Actualmente está en controles por cardiología con ácido acetil salicílico por persistencia de dilatación de las arterias coronarias. Conclusiones: La adenitis cervical puede imitar a otras infecciones cutáneas como el absceso cervical ocasionado retraso en el diagnóstico de EK produciendo complicaciones como el aneurisma coronario.

Background: Kawasaki disease (KD) is an acute and self-limited vasculitis common in children under five years. The diagnosis is a challenge in children under six months of age. Report case: We report a three-month-old infant with fever and a painful neck tumor of three days of disease. He was diagnosed with an abscessed cervical mass and received antibiotic treatment for ten days. During hospitalization, macular erythematous lesions developed throughout the body, persistence of fever, and elevated inflammatory markers. Echocardiography was performed, finding dilatation of the coronary arteries, being diagnosed with incomplete KD. He received intravenous human immunoglobulin and acetylsalicylic acid. He is currently undergoing cardiology controls with acetylsalicylic acid due to persistent dilatation of the coronary arteries. Conclusions: Cervical adenitis can mimic other skin infections such as cervical abscesses. It can cause a delay in the diagnosis of KD, producing complications such as a coronary aneurysm.

Un caso de aneurisma coronario gigante en enfermedad de Kawasaki / A case of a giant coronary aneurysm in Kawasaki disease

La enfermedad de Kawasaki es una vasculitis que afecta arterias de mediano calibre que ocurre predominantemente en la población pediátrica, de etiología desconocida. Si no es tratada tempranamente existe riesgo de desarrollo de complicaciones tan severas como el desarrollo de aneurismas de arterias coronarias, las cuales pueden estar relacionadas con el desarrollo de trombosis coronaria y riesgo de infarto de miocardio. Se han asociado diversos factores de riesgo de desarrollo de aneurismas coronarios como el tiempo de evolución de la enfermedad o la edad del paciente, la importancia de la identificación de estos factores radica en la posibilidad de la pronta acción sobre aquellos que sean modificables en un intento de limitar el desarrollo de dichas complicaciones coronarias. El tratamiento a largo plazo para esta condición incluye terapia antiagregante y anticoagulante, ambas pueden representar un riesgo en sí mismas para el paciente además de ser limitantes para un paciente pediátrico por el riesgo de sangrado asociado a traumas (propios de la edad pediátrica).

Kawasaki disease is a vasculitis that affects medium-caliber arteries that occurs predominantly in the pediatric population, of unknown etiology. If it is not treated early, there is a risk of developing complications as severe as the development of coronary artery aneurysms, which may be related to the development of coronary thrombosis and the risk of myocardial infarction. Various risk factors have been associated with the development of coronary aneurysms such as the time of evolution of the disease or the age of the patient, the importance of identifying these factors lies in the possibility of prompt action on those that are modifiable in an attempt to limit the development of coronary complications. Long-term treatment for this condition includes antiplatelet and anticoagulant therapy, both may represent a risk in themselves for the patient as well as being limiting for a pediatric patient due to the risk of bleeding associated with trauma (typical of pediatric age).

Case Report: Endothelial Glycocalyx Damage in Critically ill Patients With SARS-CoV-2-Related Multisystem Inflammatory Syndrome (MIS-C).

Endothelial insult and damage is one of the reported consequences of SARS-CoV-2 infection. It has been associated with severe inflammation, thrombotic phenomena and profound hypoxemia in critically ill patients. Endothelial activation leads to a loss of the endothelium's antithrombotic properties which, under normal conditions, are maintained by the endothelial glycocalyx, a carbohydrate-rich layer that covers the luminal surface of endothelial cells. In children, one of the serious forms of SARS-CoV-2 virus disease (COVID-19) is multisystem inflammatory syndrome (MIS-C). This new disease is characterized by a large inflammatory response and frequent cardiovascular, cutaneous and gastrointestinal disorders. We describe the first two cases of critically ill children with MIS-C who evidenced a large inflammatory response associated with elevated plasma and imaging biomarkers of endothelial activation and endothelial glycocalyx degradation. This microcirculation involvement in MIS-C could, at least partially, explain some of the clinical manifestations and laboratory and imaging alterations found in these patients. These findings contribute to a better understanding of this disease and suggest that medications to modulate the inflammatory response and protect or restore the endothelial glycocalyx should be considered in future studies.

Surgical Treatment for a Super-Giant Right Coronary Artery Aneurysm Induced by Kawasaki Disease.

A 32-year-old man diagnosed with Kawasaki disease at the age of three years presented with coronary artery aneurysm (CAA). The aneurysm increased in size, and the patient was referred to our hospital for surgery. Preoperative computed tomography scan showed a super-giant right CAA and giant left CAAs; surgery was performed. The super-giant right CAA was resected, and the ostium of the right coronary artery was closed; then, coronary artery bypass grafting was performed. The left CAAs were not treated surgically because the risk of rupture was low. Here, we describe the successful surgical treatment of a right super-giant CAA.

Slow Flow Phenomenon Impairs the Prognosis of Coronary Artery Ectasia as Well as Coronary Atherosclerosis.

INTRODUCTION: Coronary artery ectasia (CAE) is one of the uncommon cardiovascular disorders and its prognosis is still debated. OBJECTIVE: We aimed to review long-term follow-up data in patients with CAE and to evaluate the prognosis of CAE patients with coronary slow flow phenomenon (CSFP). METHODS: This study had a prospective design and 143 patients with CAE were included. The angiographic and demographic characteristics were reviewed in detail. The patients were categorized, based on concomitant coronary artery stenosis detected by angiography, as CCAE group (n=87, ≥30% luminal stenosis) and ICAE group (n=56, <30% luminal stenosis) and also categorized by the coronary flow as CSFP group (n=51) and normal flow coronary ectasia - NCEA group (n=92). All patients were re-evaluated at 6-month intervals. Followup data, cardiovascular (CV) mortality, hospitalization and major adverse cardiac events (MACE) were collected. The level of statistical significance was set at 5%. RESULTS: Patients were followed up for an average of 56.9±7.4 months. During the follow-up, statistically significant differences were found in hospitalization, CV mortality and MACE between the CCAE and ICAE groups (P=0.038, P=0.003, P=0.001, respectively). The CSFP and NCEA groups were also compared. There was a statistical difference with respect to hospitalization between the CFSP and NCEA groups (P=0.001), but no difference was observed in terms of MACE and CV mortality (P=0.793 and P=0.279). CONCLUSION: CSFP accompanying CAE may be a predictor of hospitalization. Significant atherosclerotic plaques coexisting with CAE may be predictive for MACE.

Slow Flow Phenomenon Impairs the Prognosis of Coronary Artery Ectasia as Well as Coronary Atherosclerosis

Abstract Introduction: Coronary artery ectasia (CAE) is one of the uncommon cardiovascular disorders and its prognosis is still debated. Objective: We aimed to review long-term follow-up data in patients with CAE and to evaluate the prognosis of CAE patients with coronary slow flow phenomenon (CSFP). Methods: This study had a prospective design and 143 patients with CAE were included. The angiographic and demographic characteristics were reviewed in detail. The patients were categorized, based on concomitant coronary artery stenosis detected by angiography, as CCAE group (n=87, ≥30% luminal stenosis) and ICAE group (n=56, <30% luminal stenosis) and also categorized by the coronary flow as CSFP group (n=51) and normal flow coronary ectasia - NCEA group (n=92). All patients were re-evaluated at 6-month intervals. Follow-up data, cardiovascular (CV) mortality, hospitalization and major adverse cardiac events (MACE) were collected. The level of statistical significance was set at 5%. Results: Patients were followed up for an average of 56.9±7.4 months. During the follow-up, statistically significant differences were found in hospitalization, CV mortality and MACE between the CCAE and ICAE groups (P=0.038, P=0.003, P=0.001, respectively). The CSFP and NCEA groups were also compared. There was a statistical difference with respect to hospitalization between the CFSP and NCEA groups (P=0.001), but no difference was observed in terms of MACE and CV mortality (P=0.793 and P=0.279). Conclusion: CSFP accompanying CAE may be a predictor of hospitalization. Significant atherosclerotic plaques coexisting with CAE may be predictive for MACE.