Swanson SK, Schumacher KR, Ohye RG, Zampi JD.
J Thorac Cardiovasc Surg. 2020 Sep 1:S0022-5223(20)32473-9. doi: 10.1016/j.jtcvs.2020.08.082. Online ahead of print.
Take Home Points:
- Cardiac surgery can be performed safely in the setting of Trisomy 13 and Trisomy 18.
- T13 and T18 patients undergoing cardiac operations have higher rates of airway complications and require longer ICU stays than matched controls.
- While operative interventions are likely to be successful, guidance for parents of these patients should include a discussion of postoperative respiratory support.
Commentary from Dr. Barry Deatrick (Baltimore, MD, USA), section editor of Congenital Heart Surgery Journal Watch: Trisomy 13 (Patau syndrome) and Trisomy 18 (Edwards syndrome) are both associated with high rates of congenital heart diseases, such as VSD, ASD and PDA. More importantly, however, these chromosomal abnormalities are associated with a severely impaired long-term survival, with a median survival of less than 2 weeks. As approximately 5-10% of patients survive over 1 year, it is perhaps not surprising that offering surgical treatment for cardiac malformations remains somewhat controversial. However, as reports of long-term survival increase, more centers offer treatments, such as cardiac surgery, aimed at long-term survival. Although multiple have established that these patients can safely undergo surgery, fewer reports have described the postoperative courses of these patients, and the specific comorbidities which complicate postoperative recovery. Because respiratory complications have been noted to be major complicators of recovery in previous series, this review was performed with a focus on identification and management of respiratory complications. The authors performed a retrospective case-control study of all Trisomy 13 and Trisomy 18 (T 13/18) patients in a single center over 20 years undergoing cardiac surgery. A 3:1 diagnosis-matched control group was identified, and individuals were further matched by age at surgery and then date of surgery.
In this study, 14 patients with T 13/18 were identified, and underwent surgical operations at a median age of 93 days. Trisomy 18 was more common than trisomy 13. The indications for operation included VSD, coarctation, tetralogy of Fallot and complete AVSD. 64% of T 13/18 patients were admitted preoperatively , whereas only 13% of controls were. Preoperatively, 3 of the T13/18 patients had airway evaluation by specialist physicians. Patients with T13/18 were more likely to require preoperative respiratory support (71% vs 14%, P=0.001), including intubation and ventilation as well as noninvasive ventilatory assistance.
Operatively, there were no significant differences in cardiopulmonary bypass time, cross clamp time, or circulatory arrest time. Postoperatively, 13 of the 14 T 13/18 patients experienced at least one complication, And all of these patients experienced at least one respiratory complication. Further, 58% of these patients had at least one non respiratory complication. In addition to having higher rates of respiratory support preoperatively, T 13/18 patients also had high rates of post-operative respiratory complications, including prolonged mechanical ventilation prolonged noninvasive ventilation, reintubation , tracheostomy, and respiratory infections. Some other notable complications, including infection (tracheitis and sepsis), need for gastrostomy tube placement, and increased need for vasoactive and inotropic support.
The authors conclude that although survival is good, hospital and ICU length of stay is longer in T13/18 patients than in matched controls. This retrospective study demonstrates that those increases are at least correlated with an increased prevalence of respiratory and airway anomalies in these patients. While not recommending against surgical repair for these patients, they suggest that awareness of these airway issue should be included in prenatal counseling, preoperative planning, and managing expectations for these patients.