RESUMEN
Atrioventricular (AV) discordance and ventriculoarterial (VA) concordance in the setting of visceral situs inversus are one of the rarest forms of cardiac malformations. To our knowledge, this is the first reported case of prenatal diagnosis of such rare cardiac anatomy in association with double-outlet right ventricle on fetal echocardiography. The physiology of this cardiac anomaly is similar to that of transposition of the great arteries, and the best surgical option is the atrial switch operation.
Asunto(s)
Ventrículo Derecho con Doble Salida/diagnóstico , Situs Inversus/diagnóstico , Adulto , Operación de Switch Arterial/métodos , Ventrículo Derecho con Doble Salida/cirugía , Ecocardiografía , Femenino , Corazón Fetal/diagnóstico por imagen , Atrios Cardíacos/anomalías , Ventrículos Cardíacos/anomalías , Humanos , Recién Nacido , Masculino , Embarazo , Diagnóstico Prenatal , Situs Inversus/cirugía , Transposición de los Grandes Vasos/diagnóstico , Transposición de los Grandes Vasos/cirugía , Resultado del TratamientoRESUMEN
We present the case of an infant with left atrial isomerism with complex pulmonary and systemic venous connections that resulted in physiological parallel circulation in the setting of ventriculoarterial concordance who was surgically treated using the Senning procedure. This case highlights a rare cause for cyanosis due to poor mixing from a parallel circulation and an issue with nomenclature. (Level of Difficulty: Advanced.).
RESUMEN
We present the case of an infant with congenital heart disease which includes a partial atrioventricular canal defect with the absence of the atrial septum (common atrium) and an extremely uncommon course of the right-sided superior vena cava (SVC) including an intra-atrial segment coursing intramurally along the right posterolateral atrial wall, with an intracardiac orifice situated low within the right side of the atrium, close to the atrial orifice of the right hepatic veins. This feature of the anatomy was discovered intraoperatively at the time of surgical repair. Systemic venous anatomy also included interrupted inferior vena cava (IVC) with azygos continuation to a left-sided SVC draining directly into the left side of the atrium. The successful surgical procedure included tunneling of the left-sided SVC to the right side of the common atrium and atrial septation with a patch.
