Effect of Prenatal Laterality Disturbance and Its Accompanying Anomalies on Survival

In this retrospective, observational study of fetuses diagnosed with a laterality disturbance we describe the findings and outcome of fetuses diagnosed between 1980 and 2017 at a tertiary fetal-pediatric cardiology unit. In addition we sought to identify features which impact on outcome. Left atrial isomerism (LAI) was diagnosed in 177 babies and right atrial isomerism (RAI) in 100. Major structural heart disease was present in all cases of RAI and 91% with LAI. Complete heart block (CHB) was present in 40% of LAI. For surviving live-born infants a biventricular circulation was feasible in 3% with RAI and 43% with LAI. The median survival for live-borns with LAI was 13 months (range 0 to 272 months) and for RAI 19 months (range 0.3 to 292 months). The median postnatal survival with CHB was 0.2 months (range 0 to 228 months) compared to 44 months with sinus rhythm (interquartile range 0 to 272 months; p <0.0001). The 5-year survival was 1980 to 1989, RAI 0%, LAI 0%; 1990 to 1999, RAI 62%, LAI 54%; 2000 to 2009, RAI 59%, LAI 53%; 2010 to 2017, RAI 67%, LAI 75% by era. The rate of intrauterine death remained. Risk factors for death/transplantation for RAI were total anomalous pulmonary venous drainage, left heart obstruction (hazard ratios 2.7, p = 0.048; 5.8, p = 0.03) and for LAI: CHB, anomalous pulmonary venous drainage and right heart obstruction (hazard ratios 11.5, 6.2, 3.8, respectively (p = 0.008, p = 0.003, p <0.001)). In conclusion, laterality disturbances represent a complex form of congenital heart disease and although survival is improved, it remains poor especially in the presence of anomalous pulmonary venous drainage, stenotic and/or atretic valves, and CHB.