Analysis of 181 Transplants for Pediatric or Congenital Heart Disease: Impact of Congenital Heart Disease versus Acquired Heart Disease

M. S. Bleiweis¹, F. Fricker¹, D. Gupta¹, B. Pietra¹, J. Coppola¹, G. J.. Peek¹, Y. Stukov¹, O. M. Sharaf², M. Purlee¹, C. Brown¹, L. Kugler¹, D. Neal¹, J. P. Jacobs^1
1University of Florida, Gainesville, Florida ²University of Florida College of Medicine, Gainesville, Florida

Purpose: We reviewed our management strategy and outcome data for all 181 patients with pediatric and/or congenital heart disease undergoing cardiac transplantation at University of Florida from 01/01/2011-03/01/2022 and compared the outcomes of those with congenital heart disease (CHD) to those with acquired heart disease (AHD).

Methods: Patient characteristics were assessed stratified by CHD versus AHD. Continuous variables are presented as mean(SD); median[interquartile range](range); categorical variables are presented as N(%). P-values are the results of Fisher's exact tests (categorical variables) or Wilcoxon rank sum tests (continuous variables).

Univariate associations with long-term survival were assessed with a Cox proportional hazards model with the factor as the only predictor of survival; hazard ratios (HR) and P-values are reported.

The impact of CHD versus AHD on survival was estimated with a series of multivariable models, each controlling for one of the factors shown in univariate analysis to be associated with long-term survival.

Kaplan-Meier methods were used to estimate survival for patients stratified by CHD versus AHD status; log-rank tests were used to compare groups.

Results: CHD was present in 112/181=61.9%. AHD was present in 69/181=38.1%.

Pretransplant differences included:
• Fewer CHD patients were supported with pretransplant VAD: 27/69=39.1% versus 24/112=21.4%, p=0.017
• More patients with CHD had prior cardiac surgery: 35/69=50.7% versus 95/112=84.8%, p< 0.0001
• CHD patients had a higher number of prior cardiac surgeries: 1.4(1.9);1[0-2](0-8) versus 2.7(2.1);2 [1-4](0-12), p< 0.0001
• More CHD patients had univentricular circulation: 0/67=0% versus 89/112=79.5%, p< 0.0001
• More CHD patients had pretransplant liver dysfunction 4/67=5.8% versus 19/112=17.0%, p=0.037

Intraoperative differences included:
• CHD patients had longer bypass time: 108(40.3);101[81,125](59,300) versus 148(55.2);137[113,174](72,526), p<.0001
• CHD patients had longer cross clamp time: 66.3(14.7);65 [55,75](44,106) versus 85.2(22.2);84 [72,100](15,152), p<.0001
• CHD transplants had lower minimum temperature: 31.6 (3.1);32[31,34](20,36) versus 27.4 (4.3);28 [24,31](19,34), p<.0001

Post-transplant differences included:
• More CHD patients required ECMO post-transplant 1/69=1.5% versus 5/112=4.5%, p=0.409
• Post-transplant length of stay was longer in CHD patients: 29.7(48.2);18[15,26](0,343) versus 34.5(26.8);27 [19,40](3,158), p=0 .0001

In multivariate analysis, CHD remained a risk factor after controlling for all variables found to be significant in univariable modelling.

Table 1 shows Kaplan-Meier survival estimates with 95% Confidence Intervals.

Table and Figure 1 compare longitudinal survival (CHD versus AHD) and document significantly worse survival in CHD (log-rank p=0.002).

Conclusion: Our single-institution analysis of 181 patients undergoing cardiac transplantation for pediatric and/or congenital heart disease over 11.25 years reveals an overall Kaplan-Meier 5-year survival (95% Confidence Interval) of 85.8% (80.0%-92.1%).

Kaplan-Meier 5-year survival is 98.5% (95.6%-99.9%) in acquired heart disease and 77.4% (68.4%-87.5%) in congenital heart disease, p<.0001. Longitudinal survival is worse in those with CHD even after controlling for multiple risk factors.

The presence of congenital heart disease is a risk factor for survival after cardiac transplantation. Efforts must be made to understand and mitigate this risk.

Identify the source of the funding for this research project: NONE