Right atrial isomerism in children older than 3 years

Abstract

BackgroundThere is a high mortality in infants with right atrial isomerism (RAI). However, less is known about outcome in older children with RAI. This study sought to evaluate those patients with RAI who survived older than 3 years of age without surgical intervention.ResultsA total of 33 consecutive patients (20 males) were enrolled in the study, mean age 6 years (range 3–32). None of the patients had surgical intervention for the RAI before age 3. Cardiac abnormalities include altered cardiac position (39 %), atrioventricular valve anomaly (87 %), single or functional single ventricle (55 %), pulmonary/subpulmonary obstruction (97 %), abnormal origin of the aorta (100 %), bilateral superior vena cava (67 %), and anomalous pulmonary venous drainage (66 %). Surgical intervention was performed after 3 years of age in 20 patients (61 %). None of them planned or had biventricular repair performed. 10 patients underwent the total cavopulmonary connection procedure, including four (40 %) who had atrioventricular valve (AVV) repair at the same time [all with common atrioventricular valve (CAVV)]. One patient died the day after the operation. A total of 69 % of patients with a CAVV had moderate or severe regurgitation, while 27 % with a single atrioventricular valve had moderate or severe regurgitation.ConclusionPatients with RAI who have survived to early childhood without surgical intervention have complex cardiac abnormalities. Survival after single stage total cavopulmonary connection is good but AVV repair is common.

Bidirectional Glenn Shunt and Repair of Total Anomalous Pulmonary Venous Connection in a 3 Years Old Child With Heterotaxy Syndrome: A Case Report

“…The cause may be multifactorial including lower prevalence of deteriorating factors such as pulmonary vein obstruction, common atrioventricular valve, systemic outflow stenosis and single superior vena cava in older children. 5 Best results are achieved in cases in whom TAPVC can be repaired at the time of BDG shunt. These, are the cases without pulmonary vein obstruction, which otherwise would necessitate early surgery in neonatal period when BDG shunt is not possible.…”

Isomorfismo cardiaco: Una perspectiva multidisciplinaria

“…El tratamiento de los pacientes con diagnóstico de isomorfismo 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 y esto será en función de la magnitud y gravedad de las anomalías cardiacas y de las lesiones extracardiacas que pueda tener asociadas 1 . Por lo anterior la mortalidad sigue siendo alta en este grupo de pacientes 1 y aún más cuando existe asociación de factores de riesgo como edad neonatal, válvula auriculoventricular única e insuficiente, drenaje venoso pulmonar anómalo y alteración en las ramas de las arterias pulmonares [39][40][41][42] .…”

“…Dependiendo la lateralidad, el paciente podrá presentar alteraciones en el ritmo cardiaco, que van desde un bloqueo auriculoventricular completo hasta taquicardia auricular, ventricular o de la unión 1 , 6 , 7 , 39 . Estas alteraciones se presentan debido a que en los isomorfismos, la topología auricular, así como los nodos y vías de conducción se ven severamente afectadas; presentando en el caso dextromorfismo la duplicación del nodo sinoauricular, mientras que en los casos de levomorfismo el nodo sinoauricular puede presentarse de manera hipoplásica o incluso estar ausente 40 , 41 . Durante el transoperatorio se debe contar en todo momento con las herramientas necesarias para cardioversión eléctrica, desfibrilación o estimulación de marcapasos.…”

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

“…As such, mortality is still high, 1 mainly when associated with risk factors such as neonatal age, atrioventricular valve (AVV) regurgitation, univentricular physiology, and anomalies of systemic and pulmonary venous connection. [13][14][15][16][17][18] The aim of this study is to identify, determine, and analyze the longterm outcomes and mortality, as well as factors associated with morbidity and mortality in patients with AI who underwent cardiac surgery.…”

“…Due to its rare presentation, diagnosis and management include a wide array of possibilities, 8–12 ranging from palliative surgery to corrective surgery. As such, mortality is still high, 1 mainly when associated with risk factors such as neonatal age, atrioventricular valve (AVV) regurgitation, univentricular physiology, and anomalies of systemic and pulmonary venous connection 13–18 …”