Aspecto da artéria coronária circunflexa retroaórtica por ecocardiografia transesofágica / Retroaortic circumflex coronary artery appearance by transesophageal echocardiography

O sinal RAC (retroaortic anomalous coronary ou artéria coronária anômala retroaórtica) é um achado desconhecido, descrito ao ecocardiograma transtorácico como uma estrutura tubular ecogênica, localizada na face atrial do sulco atrioventricular. Apresentamos um caso onde o sinal RAC se apresenta na ecocardiografia transesofágica (ETE). O conhecimento do sinal RAC e a avaliação com ETE aumentam a sensibilidade e a especificidade e conferem a oportunidade de avaliar características anatômicas de alto risco, importantes na avaliação do risco de morte súbita.(AU)

Origem anômala da artéria coronária esquerda do seio de Valsalva direito / Anomalous origin of the left coronary artery from the right sinus of Valsalva

Trazemos neste artigo a descrição de uma peça anatômica especificamente preparada para demonstrar uma rara anomalia da origem da artéria coronária esquerda do seio de Valsalva direito, com incidência de 0,15% em pacientes submetidos a cinecoronariografia. Trata-se de um subgrupo de anomalias das artérias coronárias que tem o maior potencial para repercussões clínicas, em especial a morte súbita em jovens. Discutimos, à luz dos conhecimentos atuais, os mecanismos fisiopatológicos, o diagnóstico e as opções de tratamento das variações anatômicas da origem anômala da artéria coronária do seio contralateral.

This article reports an anatomic specimen specifically prepared to demonstrate a rare anomaly of the origin of the left coronary artery from the right sinus of Valsalva, with an incidence of 0.15% in patients undergoing coronary angiography. This is a subgroup of coronary artery anomalies with the greatest potential for clinical repercussions, especially sudden death in young patients. Based on current knowledge, pathophysiologic mechanisms, diagnosis and treatment options of anatomical variations of the anomalous origin of a coronary artery from the contralateral sinus are discussed.

Holt-Oram syndrome: novel TBX5 mutation and associated anomalous right coronary artery.

The Holt-Oram syndrome was confirmed in an asymptomatic 36-year-old man by a novel TBX5-gene mutation (exon 8 acceptor splicing site, c.663-1G greater than A). Computed tomography showed an atrial septal defect and an anomalous right coronary artery crossing between the aorta and pulmonary arteries. Surgery corrected the septal defect and the initial segment of the anomalous vessel was unroofed and enlarged. Anomalous coronary arteries were not previously described in the Holt-Oram syndrome patients and should be added to the list of possible associated cardiac defects.

Cardiopatía y embarazo / Heart disease and pregnancy

Para dar a conocer la experiencia obtenida en el Servicio de Obstetricia del Hospital Universitario de Caracas en el control, evolución, resolución obstétrica y resultados perinatales de la mujer embarazada con cardiopatía confirmada, en el período comprendido entre enero de 2004 y diciembre de 2008, se realizó un estudio retrospectivo, descriptivo con elementos analíticos de 110 embarazos que cumplieron con los criterios de inclusión establecidos. La incidencia fue de una gestante cardiópata por cada 200 nacimientos. El 50,18% de las mujeres se embarazaron entre los 21 y 30 años. El 40% de las pacientes tenían 3 ó más gestas. Las cardiopatías congénitas fueron las más frecuentes (46,53%) seguidas por las adquiridas (28,71%). El 79,31% de las cardiopatías adquiridas fueron de origen reumático, siendo la válvula mitral la más afectada (52,17%). El 87,27% de los embarazos se resolvieron a término, el 12,72% culminaron antes de las 37 semanas de gestación. La vía de nacimiento en el 50% de los casos fue por parto vaginal (19,09% eutócicos y 30,9% asistidos) y el 50% se resolvieron por cesárea. El 80,90% de los recién nacidos pesaron entre 2500 y 3999 gr, el 17,27% pesaron menos de 2500 gr, de los cuales 8 (7,27%) fueron catalogados como prematuros y 11 (10%) como pequeños para edad gestacional. Al 71,81% de las gestantes se le practicó ecocardiograma fetal, esto permitió el diagnóstico prenatal de una cardiopatía congénita. El 69,09% de las pacientes recibieron prevención para endocarditis infecciosa. El 76,36% de las gestantes no presentaron morbilidad, el 23,63% presentó algún tipo de complicación, correspondiendo el 13,63% a descompensación cardiovascular siendo la insuficiencia cardíaca y la endocarditis infecciosa las complicaciones más frecuentes en el embarazo y puerperio. La complicación obstétrica más frecuente fue la bacteriuria asintomática seguida de la diabetes gestacional. No hubo mortalidad materna...

In order to publish the experience obtained in The Obstetric Service of Hospital Universitario de Caracas about evolution, medical care and perinatal outcomes in pregnant women with cardiopathy, was done a retrospective, descriptive study in 110 pregnant women attended between january 2004 and december 2008. The incidence was 1 pregnant women cardiopathy by each 200 newborn; 50,18% were pregnant between 21 and 30 years old. 40% of them have 3 or more pregnancy. Congenital cardiopathy was more frequent (46,53%), followingby rheumatic (mitral valvular cardiopathy) (28,71%). In 87,27% of pregnancy were resolved at term, and 12,72% before 37 weeks. 50% cent of births were by vaginal delivery. 80,90% of the newborn weighed between 2500 at 3999 gr. 17,27% weighed less 2500 gr; eigth of them were catalogized as a preterm and 11 as a small for gestational age. Fetal ecocardiogram was realized in 71,81% of the patients. 69,09% received preventive treatment to infectious endocarditis. 76,36% of pregnant women did no present morbility, and 23,63% presented some cardiac complication, as a cardiac insufficency and infectious endocarditis. The most frequent obstetrical complication was asynptomatic urinary infection and diabetes. There was not maternal death. Two of the new born presented congenital cardiopathy and three of them died. In spite of pregnancy plus cardiopathy are a very high risk condition it is possible to obtainan uncomplicated pregnancy in 80 % of cases.