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.

Aneurisma do Seio de Valsalva direito causando compressão coronariana extrínseca / Aneurisma del Seno de Valsalva derecho causando compresión coronaria extrínseca / Right Sinus of Valsalva Aneurysm causing extrinsic coronary compression

O Aneurisma do Seio de Valsalva (ASV) é um distúrbio cardíaco raro. É mais frequentemente um defeito congênito, mas pode ser adquirido. A doença de Takayasu é uma causa extremamente rara desse distúrbio. A maioria dos casos de ASV não-roto é assintomática. A compressão da artéria coronária esquerda é uma manifestação não usual da doença, que pode causar angina, infarto do miocárdio ou morte. Esse relato de caso descreve um paciente negro de 19 anos, do sexo masculino, apresentando um ASV direito não-roto causado por doença de Takayasu, manifestado através de síndrome coronariana aguda, tratada cirurgicamente com sucesso.

Sinus of Valsalva aneurysm is a rare cardiac disorder. It is more frequently a congenital, but it may also be an acquired condition. Takayasu's disease is an extremely rare cause of this disorder. Most cases of unruptured sinus of Valsalva aneurysm are asymptomatic. Compression of the left coronary artery is an unusual manifestation of the disease that can cause angina, myocardial infarction or death. This report describes a 19-year-old black male with an unruptured right sinus of Valsalva aneurysm caused by Takayasu's disease and manifested by an acute coronary syndrome, successfully treated by surgery.

Aneurisma del Seno de Valsalva (ASV) es un disturbio cardíaco raro. Con mayor frecuencia se trata de un defecto congénito, pero puede ser adquirido. La enfermedad de Takayasu es una causa extremadamente rara de este disturbio. La mayoría de los casos de ASV no roto es asintomática. La compresión de la arteria coronaria izquierda es una manifestación no usual de la enfermedad, que puede causar angina, infarto de miocardio o muerte. Este informe de caso describe un paciente negro, de 19 años, del sexo masculino, presentando un ASV derecho no roto causado por enfermedad de Takayasu, manifestado a través de síndrome coronario agudo, tratado quirúrgicamente con éxito.

Ruptura del seno de valsalva derecho hacia ambas cámaras ventriculares / Ruptured aneurysm of the right coronary sinus of Valsalva towards both right and left ventricles

Se describe un paciente con ruptura de aneurisma del seno Valsalva derecho, diagnosticado por ecocardiografía transtoracica bidimensional y doppler, con historia previa de insuficiencia cardiaca derecha y soplo continuo en precordio.

Rupture of the sinus of valsalva into the pulmonary artery.

Congenital aneurysms of the sinus of Valsalva are rare lesions that can rupture into any cardiac chamber, due to the central position of the aortic root. Rupture into the pulmonary artery, however, is very rare. We encountered an 18-year-old girl with rupture of the right coronary sinus into the pulmonary artery. During surgical correction, the girl was also found to have a small outlet ventricular septal defect, which was obscured by multiple hypertrophied septal trabeculae in the right ventricular outflow tract.

Coexistence of congenital subaortic and sinus of valsalva aneurysms.

We report a rare association of an aneurysm of the sinus of Valsalva and a subaortic aneurysm. Congenital defective union of the aortic wall and ventricular muscle with the fibrous ring is probably the explanation for this condition.

Ruptured aneurysm of sinus of valsalva: clinical manifestations and surgical treatment in eighteen patients

Eighteen patients with ruptured aneurysms of sinus of valsalva were operated in the cardiac surgery department of a large tertiary care teaching hospital between January 1991 and December 1996. Male to Female ratio was 8:1. Age range was 18-40 years with mean of 24. Majority of aneurysms originated from right coronary cusp [67%] and rest from non-coronary cusp [33%]. All right coronary sinus aneurysms opened into right ventricular outflow tract and all non-coronary aneurysms ruptured into right atrium. Associated anomalies were present in eleven cases [61%] comprising VSD in 3 [16.6%], Aortic incompetence in 5 [28%], Sub-aortic membrane in 2 [11%], and Coarctation of Aorta in one [5.5%]. Surgical correction was performed in all patients to close both the defect and the associated lesion. A standard cardio-pulmonary bypass with single aortic and bi-caval cannulation was established. Prolapsing portion of the sinus was excised and repaired simultaneously through aorta and the involved chamber. There were no perioperative deaths. Follow-up ranged from 4 months to 5 years with an average of 3.5. None of the patients required re-operation. We conclude that in patients with ruptured aneurysm of Sinus of Valsalva, dual approach is a better surgical option with minimal operative risk and excellent long term results

Aneurisma del seno de Valsalva con doble rotura a la arteria pulmonar y al ventrículo derecho: Reporte de un caso y revisión de la literatura / Aneurism of the Valsalva sinus with double rupture to the pulmonary artery and to the right ventricle. Report of one case and review of the literature

We report a case of a 21 year old female who presented with congestive cardiac failure. In initial investigations an aneurysm of sinus of Valsalva was found ruptured to the pulmonary artery. It was surgically repaired uneventfully. The patient was discharged home after 10 days. During the follow-up appointments she was found to have reappearing symptoms of congestive cardiac failure. This lead to her readmission and a repeated cardiac catheterization found her to have an aneurysm of sinus of Valsalva ruptured to the right ventricule, which in a second operation was succesfully repaired. We review the bibliography over the last 20 years finding tne unusual presentation of the rupture to the pulmonary artery. We point out the importance of a sharp clinical examination and the preference of cardiac catheterization to make the diagnosis