Patients with heterotaxy and complex congenital heart disease (CHD) undergo cardiac surgery with high mortality and morbidity. Recent studies have revealed an association between heterotaxy, CHD, and primary ciliary dyskinesia (PCD).
We undertook a retrospective review of patients undergoing cardiac surgery at Childrens National Medical Center between 2004 to 2008 to explore the hypothesis that postoperative complications associated with heterotaxy might be due to PCD. Reviewed were post-surgical outcomes of 87 patients with CHD and heterotaxy defined as any disruption in normal left-right asymmetry. As controls, 634 cardiac surgical patients with CHD, but without laterality defects, were selected. Surgical risks and complexities were similar between the two groups with both having median RACHS-1 scores of 3.0.
Our results showed the mean length of post-operative hospital stay (17 vs. 11 days) and mechanical ventilation (11 vs. 4 days) were significantly increased in heterotaxy compared to control patients. Also elevated were rates of tracheostomies (7%vs2%: Odds Ratio 4.6), ECMO support (13%vs5%: Odds Ratio 2.8), prolonged ventilatory course (23%vs12%: Odds Ratio 2.1) and post-surgical deaths (16%vs5%: Odds Ratio 3.9). All with p<0.01.
In conclusion, our findings show heterotaxy patients had more complicated post-surgical courses with increased mortality despite being matched with controls for RACHS-1 surgical complexity score. This was associated with an increased risk for respiratory complications, consistent with the possible association of ciliary dysfunction or PCD with heterotaxy. Further studies are needed to explore the underlying causes for the increased surgical risks and respiratory complications for heterotaxy patients undergoing cardiac surgery.