Evaluation of a Kawasaki Disease Risk Model for Predicting Coronary Artery Aneurysms in a Japanese Population: An Analysis of Post RAISE.

OBJECTIVES: To test the performance of the Son risk score, which was created to predict coronary artery abnormalities from baseline variables in North American patients with Kawasaki disease. STUDY DESIGN: The dataset from Post RAISE, the largest prospective cohort study of Japanese patients with Kawasaki disease to date, was used for the present study. With high risk defined as ≥3 points, sensitivity, specificity, positive predictive value, and negative predictive value for coronary artery abnormality development were calculated. To evaluate the effect of each risk factor in the Son score, the OR and 95% CIs were calculated using logistic regression analysis with the presence of coronary artery abnormality at 1 month after disease onset. RESULTS: Post RAISE enrolled 2628 consecutive patients with Kawasaki disease, and 304 patients had a high-risk score, of whom 15.1% showed coronary artery abnormality. At the cutoff ≥3 points, the sensitivity was 37.7%, and the specificity was 87.2%. The maximum z score at baseline ≥2.0 (OR 3.5, 95% CI 2.3-5.2) and age <6 months at disease onset (OR 3.2, 95% CI 1.9-5.4), were significantly associated with coronary artery abnormality development. However, a high concentration of C-reactive protein was not associated with coronary artery abnormality. The area under the receiver operating characteristic curve for the Son score was 0.65 (95% CI 0.59-0.71). CONCLUSIONS: The Son score had insufficient sensitivity and good specificity in a Japanese cohort of patients with Kawasaki disease. Among the variables comprising the Son score, a large baseline z score and young age at disease onset were significant, independent predictors of coronary artery abnormality development.

The Importance of Echocardiogram during the Second Week of Illness in Children with Kawasaki Disease.

OBJECTIVE: To determine the timing of peak coronary artery dilation and the characteristics of patients who present with new-onset coronary artery dilation during the acute phase of Kawasaki disease with an initial normal echocardiogram. STUDY DESIGN: This retrospective study analyzed 231 children hospitalized for Kawasaki disease in Hawai'i over a period of 7 years. Clinical and echocardiographic data were collected to calculate the timing of peak z score, and study subjects were compared based on the timing of coronary dilation. RESULTS: Peak coronary artery dilation was observed on average at 11.5 days from the onset of fever (median 8, IQR 7-13 days). Among study subjects with normal z scores in both coronary arteries during the initial encounter and echocardiogram (n = 164), 16 (10%) developed coronary artery dilation or aneurysm at the second echocardiogram, and 5 (3%) continued to have coronary artery dilation or aneurysm at the convalescent phase. CONCLUSIONS: A repeat echocardiogram during the second week of illness (day 7-14 from fever onset) in patients with normal initial echocardiogram could identify new-onset coronary artery dilation or aneurysm and could be useful in the timely adjustment of antithrombotic or anti-inflammatory therapies.

Inflammatory mediators of coronary artery ectasia / Os mediadores inflamatórios de ectasia coronária

The exact mechanisms underlying coronary artery ectasia (CAE) remain uncertain. This study aims to investigate whether and how inflammatory mediators play a role in the pathogenesis of CAE. The data sources of this study were located by literature searches on MEDLINE, Highwire Press and Google search engine for the year range 2000-2013. The most sensitive of the four types of plasma inflammatory mediators were cell adhesion molecules and systemic inflammatory markers followed by cytokines, while proteolytic substances were the least sensitive indicators of CAE. Hypersensitive C-reaction protein, homocysteine, intercellular adhesion molecule 1, vascular cell adhesion molecule 1, matrix metalloproteinase-9, tissue inhibitor of metalloproteinase-2, vascular endothelial growth factor and neopterin levels were significantly higher in CAE and coronary artery disease (CAD) patients than in controls without CAE. The percentage of granulocytes was higher in CAE, in comparison with individuals with normal coronary arteries. Polymerase chain reaction determination of angiotensin converting enzyme genotypes showed that the DD genotype was more prevalent in CAE patients than in CAD patients, while prevalence of the I allele was higher in CAD than in CAE patients. CAE is more a result of inflammatory processes than of extracellular matrix degradation, as demonstrated by investigations of plasma inflammatory mediators, activation markers and angiotensin converting enzyme genotypes. Contemporary theories are unable to explain CAE's predilection for the right coronary artery or the occurrence of multi-vessel and multi-segment involvement...

Os mecanismos exatos da ectasia de artérias coronárias (EAC) não são completamente compreendidos. Este estudo busca verificar, em detalhes, se e como os mediadores inflamatórios funcionam na pathogenesis de EAC. A fonte de dados do presente estudo veio da recuperação de literatura das investigações relevantes em MEDLINE, na Prensa de Highwire e na ativação de pesquisa do Google, do ano 2000 para 2013. Dos quatro tipos de mediadores inflamatórios do plasma, as moléculas de adesão de célula e os marcadores inflamatórios sistêmicos foram os mais sensíveis, sendo que cytokines foram mais sensíveis e substâncias de protease foram menos sensíveis na indicação da presença de EAC. A proteína C reativa hipersensível, o homocysteine, a molécula de adesão intercelular 1, a molécula de adesão de célula vascular 1, a matriz metalloproteinase-9, o nervo inibidor de tecido de metalloproteinase-2, o fator de crescimento endothelial vascular e os níveis de neopterin foram mais altos nos pacientes com EAC do que nos controles sem EAC. A porcentagem de granulocytes foi mais alta no grupo EAC, comparando-se com os indivíduos com a artéria coronária normal. A determinação de genótipo de enzima do angiotensin-conversão utilizando-se a técnica de reação em cadeia da polimerase revelou que o genótipo DD foi prevalecente na EAC, mas não nos pacientes de DAC, enquanto a presença do alelo I foi maior na DAC do que no EAC. O EAC é mais um resultado do processo inflamatório do que da degradação da matriz extracelular, como evidenciado por investigações dos mediadores inflamatórios de plasma, marcadores de ativação e genótipos de enzima do angiotensin a conversão. A predileção de EAC na artéria coronária direita e nos envolvimentos de multinavio e de multissegmento não é apurada por teorias contemporâneas...

Management of a distal coronary psudoaneurysm using an amplatzer vascular plug II.

Coronary pseudoaneurysms are rare complications of coronary perforation or dissection that can progress to rupture and cardiac tamponade. There is no optimal standard treatment, and their management is often guided by individual criteria including the risk of rupture, location in the coronary tree, size and flow in it. All of them must be taken into account when deciding the best treatment strategy. We report a case in which an Amplatzer Vascular Plug II (AVP II) was used successfully to occlude a distal coronary pseudoaneurysm that developed early after rescue angioplasty in a woman with a myocardial infarction due to spontaneous coronary dissection.

Coronary aneurysms in a child: an unusual presentation of pseudovasculitis.

Abnormalities of the coronary arteries in children are rare and Kawasaki disease is the most common cause of acquired coronary disease in a paediatric population. We report a case of a female child with coronary artery aneurysms and convulsions, who was diagnosed with Kawasaki disease. Due to systemic arterial hypertension and persistence of high inflammatory markers after treatment with high dose glucocorticoid and intravenous immunoglobulin, further investigation was performed and revealed a pheochromocytoma. Surgical removal led to normalization of blood pressure and laboratory parameters. Periodic echocardiography studies revealed progressive reduction of coronary aneurysms, with complete normalisation after 8 months. This is the first case described of coronary aneurysms presenting as a pseudovasculitis syndrome associated with pheochromocytoma.

Marfan's syndrome: pre-pubertal aortic rupture with left coronary artery aneurysms and fistulas / Síndrome de Marfan: ruptura aórtica prepubertal con fístulas y aneurismas de la arteria coronaria izquierda

Aortic dissection and rupture occur in 20-40% of patients with Marfan's syndrome. This occurs predominantly in the third and fourth decade of life, contributing to the increased morbidity and mortality of this specific group of patients. This is the first known documented case report of pre-pubertal left coronary sinus rupture with left coronary artery aneurysms with fistulous communication to both the superior vena cava and right superior pulmonary vein, presenting with a continuous murmur.

La disección y ruptura aórticas ocurren en 20-40% de los pacientes con el síndrome de Marfan. Esto ocurre predominantemente en la tercera y cuarta décadas de la vida, contribuyendo al aumento de la morbilidad y la mortalidad de este grupo específico de pacientes. Éste es el primer reporte de un caso documentado conocido de ruptura prepubertal del seno coronario izquierdo con aneurisma de la arteria coronaria izquierda, y comunicación fistulosa tanto con la vena cava superior como con la vena pulmonar superior derecha, acompañada de un soplo continuo.

Múltiplos aneurismas coronarianos em pacientes com síndrome coronariana aguda / Multiple coronary artery aneurysms in a patient with acute coronary syndrome

Paciente do sexo masculino, 49 anos, foi admitido no Serviço de Emergência com quadro de taquicardia ventricular após síndrome coronariana aguda. A estratificação não invasiva foi realizada, inicialmente, com ecocardiograma transtorácico que evidenciou aneurisma ventricular esquerdo e disfunção sistólica moderada. Na análise segmentar observou-se acinesia anteroapical. Evidenciou-se ainda regurgitação mitral moderada e regurgitação aórtica leve. O paciente foi submetido, a seguir, à cineangiocoronariografia que evidenciou oclusão de artéria descendente anterior, aneurisma em coronária direita, circunflexa, diagonal e trombo organizado em ponta de ventrículo esquerdo. Decidiu-se pelo tratamento clínico.

Male patient, 49 years, was admitted to the ER with signs of ventricular tachycardia after acute coronary syndrome. Non-invasive stratification performed initially with a transthoracic echocardiogram showed a left ventricular aneurysm and moderate systolic dysfunction. The segment analysis showed anteroapical akinesis, as well as moderate mitral regurgitation and mild aortic regurgitation. The patient then underwent coronary angiography that indicated occlusion of the anterior descending artery, aneurysm in the right coronary artery,circumflex, diagonal, and organized thrombus in the left ventricle. Clinical treatment was selected.

Marfan's syndrome: pre-pubertal aortic rupture with left coronary artery aneurysms and fistulas.

Aortic dissection and rupture occur in 20-40% of patients with Marfan's syndrome. This occurs predominantly in the third and fourth decade of life, contributing to the increased morbidity and mortality of this specific group of patients. This is the first known documented case report of prepubertal left coronary sinus rupture with left coronary artery aneurysms with fistulous communication to both the superior vena cava and right superior pulmonary vein, presenting with a continuous murmur

Coronary artery aneurysms in Wegener's granulomatosis.

Atherosclerosis is the main cause of coronary artery aneurysm, however they can be observed in connective tissue diseases such as systemic lupus erythematosus and vasculitis. Kawasaki's disease and polyarteritis nodosa (PAN) are the systemic vasculitis that more often present coronary artery aneurysms. There are descriptions in the literature that small vessel vasculitis such as microscopic polyangiitis and PAN could develop coronary artery aneurysm, which are infrequent in other ANCA-associated vasculitis. Here, we report the case of a 25-year-old man who developed an extensive anterior myocardial infarct. The coronary angiogram showed coronary artery aneurysms, on laboratory ANCA C positivity with elevated levels of anti-proteinase 3 antibodies were present. He was treated with high doses of corticosteroids and cyclophosphamide with resolution of the aneurysms.

Aneurisma gigante de tronco coronario izquierdo en un atleta de 26 años de edad: reporte de caso y revisión de la literatura / Giant left main coronary artery aneurysm in 26-year-old athlete: case report and review

El desarrollo de aneurismas en el tronco coronario izquierdo es una patología rara con una incidencia de 0,1% y cuya etiología puede ser congénita o adquirida. La importancia clínica de los aneurismas coronarios estriba en el riesgo de ruptura coronaria espontánea y de infarto de miocardio por trombosis, embolismo o espasmo. Este reporte describe el caso de un paciente de 26 años de edad, estudiante de licenciatura en educación física, con un aneurisma gigante sacular del tronco coronario izquierdo asociado con estenosis suboclusiva posaneurismática que condicionó la presencia de manifestaciones de isquemia miocárdica de alto riesgo. Presentamos la imaginología y una revisión sobre la etiología, los aspectos clínicos, diagnósticos y terapéuticos de los aneurismas del tronco coronario izquierdo.

The development of the left main coronary artery aneurysms is a rare pathologic process with an incidence of 0.1% and whose etiology can be either congenital or acquired. The clinical importance of the aneurysms of the left main coronary artery is based in the potential risk of spontaneous dissection and rupture and myocardial infarction for thrombosis, embolism or spasm. We report the case of a 26-year-old athlete with agiant aneurysm of the left main coronary artery associated with suboclusive stenosis and clinical manifestations of myocardiali schemia. We present the imagenological characteristics and bibliographic review about the clinical, diagnostic and therapeutic aspects of the left main coronary artery aneurysm.

Aneurismas das artérias coronárias: uma rara manifestação da doneça renal policística autossômica dominante / Coronary aneurysms: a rare manifestation of autosomal dominant polycystic kidney disease

A doença renal policística autossômica dominante (DRPAD), uma das anormalidades genéticas mais comuns, é responsável por 10 por cento dos casos de insuficiência renal crônica terminal. O acometimento extrarrenal da DRPAD manifesta-se por cistos no fígado e pâncreas, aneurismas intra e extracranianos, e anormalidades cardíacas valvares, comprometendo a raiz da aorta e as valvas aórtica e mitral. Neste artigo descrevemos o caso de um paciente com aneurisma das artérias coronárias no contexto de outras manifestações sugestivas de DRPAD.

Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common genetic abnormalities and is responsible for over 10% of end-stage chronic kidney disease cases. Extrarenal involvement of ADPKD is manifested by liver and pancreatic cysts, intra and extracranial aneurysms and valvular heart disease, involving the aortic root and the aortic and mitral valves. In this article we report a case of a patient with large coronary artery aneurysms in the context of other manifestations suggesting ADPKD.

Infarto agudo do miocárdio em adolescente secundário à trombose de aneurisma coronariano / Acute myocardial infarction in adolescent secondary to coronary aneurysm thrombosis

O aneurisma de artéria coronaria é uma doença geralmente descoberta de forma acidental já que a maioria dos pacientes permanece assintomática. Não obstante, raros pacientes podem apresentar complicações locais do aneurisma. Relata-se um caso de trombose de aneurisma de coronária seguido de infarto agudo do miocárdio em um adolescente. São revisadas, também, as modalidades diagnósticas na avaliação desta doença.

Coronary artery aneurysm is a disease usually diagnosed accidentally since most patients remain asymptomatic. Nonetheless, rare patients may havelocal aneurysm complications. We describe a case of coronary artery aneurysm thrombosis followed by acute myocardial infarction in an adolescent. We also review the imaging diagnosis of this disease.

Kawasaki disease: a clinical and epidemiological study of 70 children in Brazil.

This is a prospective study of a cohort of 70 children with Kawasaki disease (KD) admitted from April 2002 to April 2007 to a tertiary hospital in Brasilia, Brazil. Of the 70 children, only 32 (45.7%) were referred during the acute phase of the disease and only 15 (21.4%) were referred with the correct diagnosis. Coronary aneurysms were detected in 13 (18.5%). A high suspicion index is essential in order to detect new cases as KD is probably underdiagnosed in Brazil and other developing countries, and an early diagnosis will dramatically decrease its life-threatening complications.

El tratamiento conservador parece la mejor opción para los aneurismas coronarios sin estenosis / Conservative treatment is apparently the best option in coronary aneurysms without associated stenosis

Los aneurismas coronarios son una patología infrecuente, con una incidencia variable según la población estudiada. La causa más frecuente es la aterosclerosis coronaria, por lo que suele asociarse a estenosis en las coronarias. Realizamos una revisión de todos los estudios angiográficos efectuados en los últimos 10 años en nuestro laboratorio, más de 12 000 pacientes. Trece pacientes mostraron dilataciones aneurismáticas en las coronarias sin relación con estenosis adyacentes. El motivo de ingreso fue un síndrome coronario agudo en la mayoría de los casos, lo que pone de manifiesto la elevada morbilidad asociada de esta patología. Tiene una clara relación con el sexo masculino, sin que hayamos podido encontrar una explicación para esta asociación. A pesar de la ausencia de estenosis coronarias asociadas, creemos que la enfermedad aterosclerótica podría ser su causa etiológica. Debido a la ausencia de grandes registros no se conoce la evolución real de los aneurismas coronarios sin estenosis significativas asociadas y de probable etiología aterosclerótica. En función de nuestra experiencia y de la revisión bibliográfica efectuada, el tratamiento farmacológico conservador es una buena opción en la mayor parte de los pacientes, con buen pronóstico en la evolución a mediano y largo plazo.