Shared best practices can reduce mortality risk in complex cardiac surgery for children with heterotaxy syndrome
Even with the many advances in surgical repair of complex congenital heart malformations, total anomalous pulmonary venous connection (TAPVC) repair in patients with heterotaxy syndrome carries a high mortality risk, particularly with functionally univentricular physiology.
Some of the most complex heart defects occur in children born with heterotaxy syndrome, in which abdominal organs form on the opposite side of the body. The cardiac lesions that result, which are almost always multiple, can vary widely in severity and potential outcome. An analysis led by David Morales, MD, and colleagues, published April 23, 2015 in the Annals of Thoracic Surgery is the first to provide national-level data on the mortality risks that certain patient populations face when receiving TAPVC repairs.
The study examined 261 operations for TAPVC repair in 258 patients from 65 medical centers. Overall, mortality at discharge was 38 percent. Heterotaxy patients undergoing TAPVC repair and requiring postcardiotomy ECMO had still higher operative mortality rates.
The findings suggest that early outcomes of TAPVC repair are significantly worse when patients with heterotaxy are in high-risk subgroups such as those who have functionally univentricular physiology.
“It is clear that TAPVC heterotaxy patients are very rare and present with extremely complicated anatomy and physiology that varies with patients, and therefore their care has to be individualized,” Morales says. “However, there are centers that have achieved higher levels of success treating these patients such as ours. The key next steps will be to understand and share best patient management practices, so that through education they can be spread throughout the country to improve outcomes for all of these children.”
