Ortega-Zhindón Diego B, Pérez-Hernández Nonanzit, Rodríguez-Pérez José Manuel, García-Montes José A, Calderón-Colmenero Juan, Rivera-Buendía Frida, Cervantes-Salazar Jorge L
Department of Pediatric Cardiac Surgery and Congenital Heart Disease, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City 14080, Mexico.
Department of Molecular Biology, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City 14080, Mexico.
Diseases. 2023 Nov 20;11(4):170. doi: 10.3390/diseases11040170.
Right atrial isomerism (RAI) is a complex entity with varying diagnostic and treatment outcomes due to its rarity. Treatment options range from palliative to corrective surgeries, resulting in heterogeneous outcomes. The aim of this study was to analyze the results obtained after cardiac surgery in patients with RAI. A retrospective study was conducted, including patients diagnosed with RAI who underwent cardiac surgery. Their follow-up was from 1 January 2010 to 31 March 2020. Demographic characteristics and perioperative conditions were described. Thirty-eight patients were included, the median age was 4 years (IQR 2-9.2) and 57.9% were men. The main diagnoses were atrioventricular canal (63.2%) and pulmonary stenosis (55.3%). The most common surgical procedures were modified Blalock-Taussig shunt (65.8%) and total cavopulmonary connection with an extracardiac conduit fenestrated without cardiopulmonary bypass (15.9%). We did not find any factors associated with negative outcomes in these patients. The overall survival was 86.8%, with a better outcome in those who did not require reintubation (log rank, < 0.01). The survival of RAI was similar to other centers. Individuals with RAI should be evaluated rigorously to determine an adequate repair strategy, considering high morbidity and mortality.
右心房异构（RAI）是一种复杂的病症，因其罕见性而具有不同的诊断和治疗结果。治疗选择范围从姑息性手术到矫正性手术，导致结果各异。本研究的目的是分析RAI患者心脏手术后获得的结果。进行了一项回顾性研究，纳入了诊断为RAI并接受心脏手术的患者。他们的随访时间为2010年1月1日至2020年3月31日。描述了人口统计学特征和围手术期情况。共纳入38例患者，中位年龄为4岁（四分位间距2 - 9.2岁），57.9%为男性。主要诊断为房室管畸形（63.2%）和肺动脉狭窄（55.3%）。最常见的手术操作是改良布莱洛克 - 陶西格分流术（65.8%）和在非体外循环下带开窗的心外管道全腔静脉 - 肺动脉连接术（15.9%）。我们未发现这些患者中与不良结果相关的任何因素。总体生存率为86.8%，无需再次插管的患者预后更好（对数秩检验，<0.01）。RAI患者的生存率与其他中心相似。鉴于高发病率和死亡率，应对RAI患者进行严格评估以确定合适的修复策略。
