ABSTRACT
Criss-cross heart is an extremely rare anomaly, characterized by an abnormal rotation of the ventricular mass along its major axis. It may be associated with any malformation of the heart segments and connections. Due to the complex structural changes and rarity of the anomaly, the rotation of ventricular axis is often misdiagnosed. In this paper, two cases of criss-cross heart are reported, with emphasis on diagnostic and surgical techniques used to corrected the main defects. A literature review on the subject is also presented which, although sparse, emphasized on the morphologic, diagnostic and surgical aspects of the anomaly.
Coração entrecruzado (criss-cross heart) é uma anomalia extremamente rara, caracterizada por rotação anormal da massa ventricular ao longo do seu eixo maior e pode estar associada com qualquer malformação dos segmentos e das conexões entre as câmaras cardíacas. Devido às alterações estruturais complexas e à raridade da anomalia, essa anomalia de rotação é muitas vezes mal diagnosticada. Neste trabalho são relatados dois casos de coração entrecruzado com ênfase no diagnóstico morfológico e nas técnicas cirúrgicas utilizadas. Foi também realizada revisão da literatura sobre o assunto, que, embora escassa, foi enfatizada quanto à morfologia, diagnóstico, abordagem cirúrgica e possíveis complicações.
Subject(s)
Child , Female , Humans , Infant, Newborn , Male , Crisscross Heart/pathology , Crisscross Heart/surgery , Diagnosis, Differential , Heart Ventricles/pathology , Medical Illustration , Treatment OutcomeABSTRACT
El corazón con entrecruzamiento de la circulación venosa pulmonar y sistémica a nivel auriculoventricular, denominada criss-cross, puede coexistir en presencia de situs solitus o situs inversus con concordancia o discordancia aurículo-ventricular dependiendo de la rotación cardíaca sobre el eje longitudinal ventricular, a favor o en contra de las manecillas del reloj. Presentamos el caso clínico de un lactante menor masculino con diagnóstico de corazón en criss-cross con concordancia aurículo-ventricular y discordancia ventrículo-arterial que ingresó a nuestro centro con disnea y cianosis. En un primer abordaje se le practica atrioseptostomía quirúrgica, sin embargo, en su evolución intrahospitalaria presenta falla cardíaca global refractaria a tratamiento convencional, por lo cual es llevado a nuevamente a cirugía donde se le realiza intervención de Damus-Kaye-Stansel. En el presente artículo se analizan los detalles de la evaluación ecocardiográfica, el cateterismo cardíaco, la resonancia magnética cardíaca y se plantean las opciones quirúrgicas de esta fascinante entidad patológica.
A heart with cross circulation (criss cross) can coexist in the presence of situs solitus or situs inversus, with atrioventricular concordance or discordance, depending on the cardiac rotation over the ventricular longitudinal axis, clockwise or clock counterwise. This is the presentation of the clinical case of a male lactant, with diagnosis of crisscross heart with atrioventricular concordance and ventriculoarterial discordance, who was admitted to these facilities with dyspnoea and cyanosis. In a first approach, a surgical atrioseptectomy was performed on the patient. However, in his intrahospitalary evolution, the patient had a global heart failure refractory to conventional treatment, causing his admission into the operating room for surgery, where the DamusKayeStensel procedure was performed on him. This article presents an analysis of the details of the echocardiographic evaluation, the cardiac catheterization, and the cardiac magnetic resonance of the clinical case, raising the surgical options for this fascinating pathological entity.