Anomalous origin of the circumflex coronary artery presenting with ventricular fibrillation cardiac arrest

Abstract

We report a case of an incidental finding of an anomalous left circumflex coronary artery arising from the right pulmonary artery that effectuated a ventricular fibrillation cardiac arrest in a woman aged 34 years. This rarity was detected during routine work-up to delineate the cause of this arrhythmia. Our patient had a background of double-outlet right ventricle and a ventricular septal defect, which was repaired with a Dacron patch and a left ventricle patch over to the aorta at age 14 months. Angiographic study at the time of her presentation showed anomalous origin of the left circumflex artery originating from the right pulmonary artery; this was discussed in multispecialty team meeting and surgical intervention was recommended; eventually, surgery was performed with reimplantation of the anomalous circumflex artery into the ascending aorta. We highlight the importance of early angiographic studies in patients with known congenital heart defects and emphasise the optimal strategy of treatment.

Circumflex Artery Arising From the Pulmonary Artery

“…( 1 ) first described it in adults in 1992, in a patient with no other congenital heart disease (CHD) who reported dyspnea and chest pain at rest. Recently, few additional cases have been described in adults: 2 presented as SCD, 1 with double-outlet right ventricle ( 2 ), and 1 without CHD ( 3 ), and another patient presented with mild exertional chest pain, wall motion abnormalities noted on echocardiography, and aortic coarctation ( 4 ). In contrast, in the larger experience with LCxPA among pediatric patients ( 5 , 6 , 7 ), the presentation was mostly exertional chest pain and dyspnea, and the association with other CHDs was higher.…”

Origem Anômala da Artéria Coronária Circunflexa da Artéria Pulmonar Direita: Diagnóstico por TC Cardíaca

“…As formas mais graves encontradas na literatura incluem isquemia miocárdica, com poucos casos relatados de disfunção miocárdica grave e parada cardíaca secundária a esta anomalia. 1,2 Durante o primeiro mês de vida, a hipertensão pulmonar fisiológica e a hemoglobina fetal fornecem perfusão e oxigenação ao miocárdio; consequentemente, estes indivíduos são assintomáticos. 3 Em crianças maiores e adultos, as pressões relativamente baixas na artéria pulmonar normal criam um gradiente através do qual o sangue flui, direcionado da circulação coronária nativa, através da rede extensa de colaterais, para a artéria anômala e a artéria pulmonar.…”

“…3 Os sintomas e o prognóstico dependem do desenvolvimento de vasos colaterais nas outras duas artérias. 1,2 A nossa paciente permaneceu assintomática durante os primeiros 40 anos da sua vida. Nossa hipótese é que isso seja o resultado da combinação da área relativamente pequena do miocárdio fornecida pela artéria CXE, o grau de colateralização coronária e a falta de desafios cardíacos significativos anteriores.…”

“…Os sintomas estão relacionados à colateralização e à quantidade do miocárdio que ela fornece, sendo que alguns pacientes necessitam de tratamento cirúrgico. 1,2 Relato de Caso Uma mulher de 44 anos com histórico familiar de síndrome de Brugada e nenhum outra histórico médico relevante foi examinada no nosso hospital devido a angina de esforço classe I da Canadian Cardiovascular Society. Ela não apresentava fatores de risco coronariano e não havia histórico familiar de doença arterial coronariana prematura ou doença cardíaca congênita.…”

Anomalous Origin of the Circumflex or Left Anterior Descending Artery From the Pulmonary Artery

“…13 However, in light of the relative rarity of ACxAPA and ALADAPA, a "handful" of case reports have been published. 14 The clinical presentation appears quite variable from sudden cardiac arrest in a child 15 to an asymptomatic murmur in an adult. 16 Although somewhat similar to ALCAPA, ACxAPA and ALADAPA represent unique coronary anomalies with likely different patterns of clinical presentation and utilized treatment strategies.…”