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Have heterotaxy syndrome outcomes improved?


The jury has been out on whether long-term outcomes have improved for children with heterotaxy syndrome. A new investigation offers some answers.

The long-term effects of heterotaxy syndrome are little understood and previous studies offer conflicting results. An investigation in Pediatrics wanted to describe the long-term outcomes and how outcomes may have changed over time.1

The investigators included chidren with heterotaxy syndrome who been born between 1985 and 2014 who had required any cardiac care, excluding initial palliation, at a hospital. The patients were split into 4 birth eras and survival over time was compared. For patients who underwent surgery, the link between mortality and surgical pathway, univentricular versus biventricular repair, was examined following adjusting for baseline cofounders.

A total of 264 patients with 118 had asplenia and 146 had polysplenia syndrome. Overall the mortality was 40.2% after a median follow-up of 10.2 years. Following multivariable analysis, coarctation, univentricular circulation, asplenia phenotype, mild atrioventricular valve regurgitation, and pulmonary vein stenosis were linked with mortality. However, birth era was not associated with mortality. For patients who had surgery, univentricular repair was linked to mortality following adjustment.

The researchers concluded that outcomes remain poor for patients with heterotaxy syndrome and little improvement has been seen since the early 1990s. They also found that totally anomalous pulmonary venous connection and univentricular circulation were linked to a worse prognosis.


1. Banka P, Adar A, Schaetzle B, Sleeper L, Emani S, Geva T. Changes in prognosis of heterotaxy syndrome over time. Pediatrics. 2020:146(1):e20193345. doi:10.1542/peds.2019-3345

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