Two-dimensional and three-dimensional echocardiographic assessment of aorticroot diameter in patients with coronary ectasia

Background: Coronary artery ectasia (CAE) is a well-recognized but relatively uncommon finding encountered during diagnostic coronary angiography. It is commonly defined as in appropriate dilation of the coronary arteries exceeding the largest diameter of an adjacent normal vessel more than 1.5-fold. CAE is not an isolated and benign disease but a reflection of a generalized vascular media defect. Objective: The aim of this study was to compare 2D-TTEand 3D-TTE measurements of the aortic root diameter in patients with coronary artery ectasia to assess the presence of aortic root dilatation. Patients and methods: This prospective observational study included 50 consecutive patients came to the Department of Cardiology, Al-Azhar University Hospital, New Damietta for coronary angiography. The study was carried out from November 2017 until December 2018. Injection aortography was used as a gold standard and to assess the presence of ascending aorta dilatation in those patients. Results: The present study shows that there was a good correlation between 3D-TTE and aortography at the levels of aortic annulus, sinuses of Valsalva, sinotubular junction (r =0.98,0.95,0.98) but a rough correlation between 2D-TTE and aortography at these levels (r =0.49,0.48,0.46). The present study shows that there was increase prevalence of aortic root dilatation 13 patients (26%) and ascending aorta dilatation 9patients(18%) in patients with CAE. Conclusions: Accuracy of aortic root measurement by 3DTTE was superior to that by 2DTTE, because the values by 2DTTE were underestimated compared to those measured by 3DTTE and aortography. Increase prevalence of aortic root dilatation and ascending aorta dilatation in patients with coronary artery ectasia. Dilated Ascending aorta was associated with a higher prevalence of aortic root dilatation

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.

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.

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 update.

Kawasaki Disease is rapidly becoming the most common cause of acquired heart disease in children in both the developed and developing world. Its etiology remains a mystery but important progress has been made in characterizing the features of the arterial wall and myocardial pathology and long-term clinical consequences. New treatments aimed at modifying the host immune response are currently under study. The genetic influence on susceptibility and disease outcome is an area of active research.

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.

Idiopathic spontaneous coronary artery dissection presenting as acute myocardial infarction in a young boy.

Spontaneous coronary artery dissection is a rare cause of acute myocardial infarction. It is more commonly seen in the younger age group particularly in women. We report a case of 14-year-old boy that presented with acute myocardial infarction (MI), with left ventricular (LV) dysfunction and was detected to have left main coronary artery dissection on coronary angiography. Myocardial perfusion imaging did not show any evidence of reversible ischemia. Patient was managed conservatively and was marginally improved. This case highlights one of the rare causes of myocardial infarction and the use of myocardial perfusion imaging in selecting treatment options for management of this rare entity.

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.

Difficulties in the diagnosis of Kawasaki disease.

This study aims to highlight the difficulties faced in the clinical diagnosis of Kawasaki Disease (KD) presenting beyond the first week. This is a retrospective study of 25 cases of which only 36% met the criteria for classical and 8% was incomplete KD. Majority (56%) did not meet the criteria for classical KD; at the same time they were not incomplete / atypical cases. Difficulties arise in diagnosis of the cases presenting in the second week, as by that time many of the classical findings disappear or probably have not been present at all. In this scenario high index of suspicion for KD in a child presenting with fever, looking not that sick (Non toxic look) with bulbar conjunctivitis and oral mucositis helped us to reach the diagnosis. We incidentally observed Hyperemia of the Upper Eyelids in 32% of our cases, which might assist in the diagnosis.

Aneurismas coronarios no asociados a estenosis en las arterias coronarias: Tratamiento conservador y evolución en un registro de 6 casos / Coronary aneurysms not associated to stenosis of coronary arteries: conservative treatment and evolution in six patients

Los aneurismas coronarios son una patología relativamente infrecuente, con una incidencia anual del 1-2%. La causa más frecuente es la ateroesclerosis coronaria y en este caso se suelen asociar a estenosis en las arterias coronarias. Revisamos todos los estudios angiográficos realizados en los últimos 7 años y recogimos los casos de 6 pacientes ingresados por síndrome coronario agudo a los que se realizó coronariografía, demostrando dilataciones aneurismáticas en las arterias coronarias sin relación con estenosis proximales o distales. A pesar de la ausencia de estenosis coronarias, pensamos que la enfermedad ateroesclerótica podría ser la causa mediante: lesión del endotelio y la elástica interna con el depósito de lípidos, células musculares lisas, colágeno, macrófagos y linfocitos T, con afectación final de las capas media y adventicia, y neoformación de vasa-vasorum. Se desconoce la evolución de los aneurismas coronarios sin estenosis significativas asociadas y sin otra causa etiológica tratable. A tenor de la escasa literatura publicada al respecto y con nuestra experiencia creemos que el tratamiento médico conservador podría ser una buena opción en estos casos. En el seguimiento a medio-largo plazo no se registraron eventos cardíacos mayores en ningún paciente.

Coronary artery aneurysms are a relatively infrequent finding with an incidence of 1-2% per year. The most frequent cause is atherosclerosis and, in that case, they are always associated to stenosis of coronary arteries. We reviewed the coronary angiographic studies performed in the past seven years and we identified six patients that were admitted with an acute coronary syndrome, whose angiographic studies showed the presence of aneurysms in, at least, one of the coronary arteries. In these patients, we found no relation between aneurysms and distal or proximal stenosis. Although there was no angiographic evidence of classical coronary atherosclerosis, we think that atherosclerotic disease could have been the etiological cause, due to injury of the endothelium and media by deposits of lipids, smooth muscle cells, collagen, macrophages, and T-lymphocytes that would have damaged the adventitia layers of the vessel wall, stimulating "vasa-vasorum" neovascularization. The evolution of coronary aneurysms without associated stenosis in the same coronary artery and without another potentially treatable cause is unknown. Due to the limited literature regarding this issue and, taking into account our experience, we feel that, in these cases, medical treatment might be a good option as we detected no major cardiac events in any patient at mid and long-term follow up.