Thoracic Organ Transplantation Options for Eisenmenger Syndrome Treatment

Thoracic organ transplantation remains a viable treatment option in patients with Eisenmenger Syndrome (ES), although these patients are considered higher risk than other potential transplant recipients, according to study results published in CHEST.

Thoracic transplantation is often considered an effective therapy for patients with ES who have refractory right ventricular failure despite optimal therapy for pulmonary arterial hypertension. Therefore, researchers compared the outcomes of bilateral lung transplant (BLT) with cardiac defect repair vs combined heart-lung transplant (HLT) to determine the most effective thoracic transplant strategy in patients with ES. 

By searching the United Network for Organ Sharing (UNOS) database, researchers identified 442 adults with ES who underwent thoracic transplantation from 1987 to 2018 (316 HLT and 126 BLT). After BLT, overall survival at 1, 5, and 10 years posttransplant was 63.1%, 38.5%, and 30.2%, respectively, whereas after HLT overall survival was 68.0%, 47.3%, and 30.5%, respectively (P =.6).

When the researchers stratified the survival analysis by type of defect, patients with an atrial septal defect (ASD) had better survival after BLT than after HLT (88.3% vs 63.2% 1 year posttransplant, P <.01; 71.1% vs 49.8% 3 years posttransplant, P <.01, 37.4% vs 29.9% 10 years posttransplant, P=.08). Patients with a ventricular septal defect (VSD) exhibited better survival after HLT than after BLT (78.2% vs 49.6% 1 year posttransplant, P <.01; 55.6% vs 34.3% 5 years posttransplant, P <.01; 35.7% vs 26.5% 10 years posttransplant, P =.03). The most common cause of mortality in patients with VSD undergoing BLT was cardiac ventricular failure.

“This study suggests that the best transplant option for patients with VSD remains HLT which prevents subsequent development of ventricular failure,” the researchers wrote. “Considering the recent major change in thoracic organ allocation and its unpredictable effect on ES patients, these data may be useful in considering exceptions for these patients at the time of listing.”

Reference

Sertic F, Han J, Diagne D, et al. Not all septal defects are equal. Outcomes of bilateral lung transplant with cardiac defect repair vs. combined heart-lung transplant in patients with Eisenmenger’s Syndrome in the United States [published online June 18, 2020]. CHEST. doi:10.1016/j.chest.2020.05.597