[Isomorfismo cardiaco: Una perspectiva multidisciplinaria]. / Atrial isomerism: A multidisciplinary perspective

Atrial isomerism describes complex anatomical findings with defects in the determination of lateralization; being a rare situation, with a prevalence of 1 in every 10.000 to 20.000 live births, with an incidence of up to 4% of all cardiac malformations. The diagnosis can be made in the neonatal age; however, clinical presentation is nonspecific. Depending on the spectrum of malformations, complex and invasive diagnostic tools may be required. Treatment is varied and can range from palliative surgery in view of univentricular physiology to total correction surgery for biventricular repair.

El isomorfismo cardiaco describe hallazgos anatómicos complejos con defectos en la determinación de la lateralización. Es una situación poco frecuente, con prevalencia de 1 en cada 10,000 a 20,000 nacidos vivos, con incidencia hasta del 4% de todas las malformaciones cardiacas. El diagnóstico puede realizarse en la etapa neonatal; sin embargo, el cuadro clínico es inespecífico. De acuerdo con el espectro de malformaciones se pueden necesitar medios diagnósticos complejos e invasivos. El tratamiento es variado y puede ir desde la cirugía paliativa en vista de una fisiología univentricular hasta una cirugía de corrección total para una reparación biventricular.

Cardiac surgery in patients with atrial isomerism: Long-term results and outcomes.

PURPOSE: The aim of the study was to identify, determine, and analyze the clinical and surgical outcomes of patients with atrial isomerism (AI) undergoing cardiac surgery. METHODS: A retrospective study was carried out. Patients with diagnosis of AI undergoing cardiac surgery at the National Institute of Cardiology Ignacio Chávez, from January 1, 2010 and March 31, 2020 were included; demographic characteristics and perioperative conditions of the patients were considered. RESULTS: Sixty-five patients were included, with an average age of 6.4 ± 4.9 years, 50.8% males. Thirty-eight (58.5%) had right atrial isomerism (RAI) and 27 (41.5%) had left atrial isomerism (LAI); univentricular physiology (78.5%) predominated. Atrioventricular septal defect (AVSD) in RAI and septal defects in LAI were identified as the main associated defects. The most common surgical procedures performed were modified Blalock-Taussig shunt (MBTS) (27.6%), MBTS with total anomalous pulmonary venous connection (TAPVC) repair (15.3%) and total cavopulmonary connection (TCPC) with an extracardiac conduit fenestrated (10.8%); 100% RAI required a univentricular approach, while in LAI it was 48.1%. Overall survival was 92.3%, with 100% survival in LAI with biventricular physiology and 86.8% in RAI with univentricular physiology. CONCLUSIONS: The survival of our institution is similar to that of other referral centers, where patients with LAI had a better evolution than RAI; in addition, the univentricular approach was required in all with RAI. Patients with AI must undergo a rigorous evaluation to determine an adequate repair strategy, considering univentricular RAI with a high possibility of morbidity and mortality.