Clinical Presentation and Outcome of Right Isomerism

pp. 396-401

Abstract

Background: Right isomerism is one of the most complex forms of congenital heart disease. Recent advances in medical treatment and surgical procedures have allowed addressing the management of these patients. Nevertheless, the prognosis remains uncertain or unsatisfactory.
Objective: The aim of this study was to report the clinical characteristics, management and outcomes of right isomerism in our hospital population.
Methods: This was a retrospective cohort design study conducted at Hospital Nacional de Pediatría “Prof. Dr. Juan P. Garrahan”. Between 1997 and 2011, 72 patients with median follow-up of 5.1 years (1 and 26 years) were identified.
Results: In 91.7% of cases, patients were in the neonatal period, 66 patients with cyanosis and 6 patients with heart failure. The most frequent anatomic lesions were: common AV valve (n=56), pulmonary obstruction (n=67), ventriculoarterial discordance (n=44) and double outlet right ventricle (n=27), common atrium (n=25), bilateral superior vena cava without innominate vein (n=30), total anomalous pulmonary venous return (APVR) (n=43) and asplenia (n=53). Extracardiac lesions were detected in 11 patients. Cardiovascular surgery was indicated in 76.38% of cases: The maximum stage achieved was palliative surgery in 14 patients, Glenn procedure in 17 patients, subpulmonary ventricular bypass (PVBP) in 23 patients and one and a half ventricular correction in 1 patient. Overall mortality was 39.45% (n=28). Mortality for the different palliative procedures was 29%, for the Glenn stage, 29% and for PVBP, 21.76%. In the univariate analysis, a significant association was found between mortality and infradiaphragmatic APVR (p=0.02). Glenn stage mortality was related to bilateral Glenn procedure (p=0.04), whereas no related cause was identified for PVBP. In the single ventricle stages of surgery, 3 patients developed pulmonary vein stenosis, 4 patients developed aortopulmonary collaterals, and 2 patients progressed to AV regurgitation.
Conclusions: The majority of cases presented in the neonatal period and with cyanosis characteristics. In right isomerism, single ventricle physiology is predominant. Extracardiac anomalies were detected in 15% of cases. Mortality of non-surgical and palliative procedure cases was associated with infradiaphragmatic APVR. Glenn stage mortality was related to bilateral procedures. Only one third of patients could reach PVBP. Events in Glenn and PVBP midterm follow-up are frequent.