Autopsy report of a sudden infant death that was strongly suspicious of Kawasaki disease.

We conducted an autopsy on a 3-month-old boy in whom Kawasaki disease (KD) was strongly suspected based on the autopsy findings. The infant had a fever and was brought to a nearby clinic, where he was prescribed antipyretics and kept under observation. However, 15 days after onset of the fever, he suddenly died in bed. He exhibited no obvious redness of the lips, tongue, or conjunctiva. Membranous desquamation was present on his distal fingers. Vasculitis was observed in the coronary arteries, renal artery, splenic artery, and pulmonary vein. In addition, coronary artery aneurysms were present in the right coronary artery and left anterior descending artery. Thrombotic occlusion was observed in one aneurysm in the right coronary artery, resulting in acute myocardial infarction. The coronary artery wall showed infiltration of numerous macrophages and neutrophils. This case was classified as incomplete KD because the coronary artery aneurysm could not be demonstrated before death and was only recognized at autopsy. Pathologists and forensic scientists need to be aware that there are cases in which KD goes undiagnosed and untreated, leading to coronary artery aneurysm formation and sudden death.

Surgical treatment of a giant right coronary aneurysm.

This case report is a step-by-step description of the surgical treatment of a giant right coronary aneurysm with a maximum diameter of 80 mm in a 57-year-old male.

Right coronary sinus aneurysm repair.

In this case report, we describe the surgical treatment of a right coronary sinus aneurysm. A 69-year-old male patient was screened because of palpitations. He was finally diagnosed with an aneurysm of the sinus of Valsalva of the right coronary cusp. According to current aortic guidelines, surgical reconstruction was proposed. The patient underwent a cardiac operation through a median sternotomy under routine cardiopulmonary bypass. After aortic cross-clamping, the aorta was opened and the connection between the aorta and the aneurysm was clearly visualized, underneath the ostium of the right coronary artery. After excision of the right coronary button and the remaining right coronary sinus wall, this sinus was reconstructed with a Dacron graft, with subsequent coronary reimplantation. The postoperative course was uneventful. The patient was discharged on postoperative day 7. A complete sinus reconstruction was preferred over local patching of the defect because of the proximity of the aneurysm sac to the right coronary artery and the fragile, thin aortic tissue just underneath the coronary ostium.

Acute myocardial infarction associated with multiple giant coronary artery aneurysms: a management dilemma.

A 77-year-old man presented with an acute inferior ST-segment elevation myocardial infarction.

Giant coronary artery aneurysm in the atrial septum.

A man in his 60s with paroxysmal atrial fibrillation was scheduled for a catheter ablation but was admitted to our department after contrast-enhanced CT showed a large homogeneous right atrial mass (52×52 mm) as well as a dilated right coronary artery (RCA). Coronary artery angiography showed a large fistula from the RCA to the mass in the right atrium. A giant coronary artery aneurysm was suspected and a surgical resection was performed. The mass was attached to the atrial septal wall and was palpated in the right atrium with a feeding artery from the RCA. The final diagnosis was an extremely rare case of giant coronary artery aneurysm originating from the RCA. The surgery was successful, and the patient was discharged 30 days later.

Aneurismas coronarios gigantes en lactantes con enfermedad de Kawasaki / Kawasaki giant coronary aneurysms in infants with Kawasaki disease

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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...

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.

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.

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.

Disección espontánea aguda de la arteria coronaria durante el puerperio / Spontaneous coronary artery dissection during the puerperium

La patogénesis de la disección espontánea aguda de las arterias coronarias relacionada con el embarazo aun no ha sido completamente comprendida. Ochenta por ciento ocurren en mujeres. Un tercio de los casos se observan en el embarazo o en el posparto. La mayoría de los casos han ocurrido en el puerperio. Se presenta un caso de paciente de 36 años, 2 gestas 1 para, que cuatro horas después del parto refirió dolor torácico que se irradia a la espalda y brazo izquierdo. La coronariografía demostró un sistema coronario izquierdo normal y una extensa disección de la arteria coronaria derecha. La paciente fue tratada farmacológicamente y posteriormente sometida a una cirugía de bypass del vaso coronario afectado (AU)

The pathogenesis of pregnancy-related spontaneous coronary artery dissection(SCAD) has not yet been fully elucidated. Eighty percent of SCAD cases occur in women. One third of cases are observed during pregnancy or the postpartum. Most cases occur in the puerperium. We present the case of a 26-year-old woman (gesta 2, para 1), who reported chest pain radiating to her back and left arm 4 hours after delivery. Coronary angiography showed anormal left coronary system and extensive dissection of the right coronary artery. The patient was treated pharmacologically and later underwent surgical bypass of the affected coronary vessel (AU)

Enfermedad coronaria aneurism tica severa en un adolescente con angina de esfuerzo secundaria a enfermedad de Kawasaki no diagnosticada previamente / Severe aneurisma I coronary artery disease probably caused by Kawasaki disease. Report of one case

We report a 16 year old male with a history of angina on exertion. A treadmill exercise test was positive for ischemia in concordance with a Thallium-201 scintigraphy showing a septal and infero-posterior reversible myocardial perfusi¢n defect. Coronary angiography disclosed severe aneurysmal coronary artery disease. Bilateral internal mammary coronary artery bypass grafting was successfully performed. Kawasaki disease is the most likely etiology, although not confirmed.

Dissecção espontânea da artériacoronária: resolução angiográfica completa sem colocação de stent / Spontaneous coronary artery dissection: complete angiographic resolution without stenting

Apresenta-se um caso de dissecção espontânea da artéria coronária, emmulher de 49 anos, sem fatores tradicionais de risco cardiovascular. Sãodiscutidos etiologia e tratamento. Foi submetida à intervenção coronarianapercutânea primária na artéria descendente anterior esquerda sem implante de stent e com resolutividade angiográfica completa.