Abstract

Abstract Background Eisenmenger's syndrome (ES) is the end-stage of a longstanding intracardiac shunt, commonly an atrial septal defect (ASD) or ventricular septal defect (VSD). Transplantation is the final option to address the pulmonary hypertension and right heart failure that results. Both combined heart-lung (HLTx) and bilateral sequential lung transplantation (BSLT) have been performed for ES. Overall, BSLT are performed more commonly than HLTx because of donor organ availability. We postulated that ES patients would have higher waitlist mortality and longer time to transplant compared to patients listed for other indications, and BSLT patients would have reduced waitlist mortality and reduced time to transplant compared to HLTx based on availability. Aim This study compared waitlist outcomes between ES ASD, ES VSD and all patients listed for BSLT or HLTx. Methods We analysed the Organ Procurement and Transplant Network (OPTN) database for all patients listed for BSLT or HLTx between October 1, 1987 and March 31, 2018. Mood's median test was used for all tests of significance. Results Table 1 summarises the results of the analysis. A total of 56,698 patients were on the waitlist for BSLT for all aetiologies, which included 176 ES ASD patients and 156 ES VSD patients. A total of 3231 patients were on the waitlist for HLTx for all aetiologies, which included 89 ES ASD and 176 ES VSD patients. Median time to transplant was significantly longer for all ES patients on the waitlist for BSLT or HLTx compare to patients listed for other indications. Comparing time to transplant for BSLT to HLTx for either ES ASD or VSD did not reach statistical significance. Waitlist mortality was substantially higher for all ES patients regardless whether they were listed for BSLT or HLTx compared to patients listed for other indications. Table 1 ES ASD ES VSD All patients BSLT HLTx BSLT HLTx BSLT HLTx Total number of patients listed 176 89 156 176 56698 3231 Transplanted 58 (33.0%) 38 (42.7%) 41 (25.8%) 78 (44.3%) 36441 (64.3%) 1229 (38.0%) Median time to transplant in days 330 (24 to 2723)* 431 (11 to 4527)$ 517 (10 to 2345)# 292 (16 to 2399)& 99 (0 to 6592) 174 (0 to 3606) p=0.54 p=0.08 *p≤0.001, #p=≤0.0001 $p=0.000, &p=0.003 Wait list mortality 27 (15.3%) 25 (28.1%) 23 (14.5%) 46 (26.1%) 2366 (4.2%) 371 (11.5%) Conclusion Our analysis identified longer waitlist time and higher mortality for all ES patients listed for BSLT or HLTx in the United States. These findings indicate current listing methods and prognostication place the ES population at disadvantage for thoracic transplantation.

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