Incidence and Outcomes of Advanced Heart Failure in Adults With Congenital Heart Disease

Abstract

Background: There are limited data about the stage D heart failure (advanced HF) in adults with congenital heart disease. Our study objectives were (1) to determine the incidence of new-onset advanced HF in patients and the relationship between advanced HF and all-cause mortality and (2) to determine the relationship between therapies for advanced HF and all-cause mortality.

Methods: Retrospective cohort study of adults with congenital heart disease at Mayo Clinic (2003-2019). We defined advanced HF using the European Society of Cardiology diagnostic criteria for advanced HF. Therapies received by the patients with advanced HF were classified into 3 mutually exclusive groups (treatment pathways): (1) conventional cardiac intervention, (2) transplant listing, and (3) palliative care.

Results: Of 5309 patients without advanced HF at baseline assessment, 432 (8%) developed advanced HF during follow-up (1.1%/y), and the incidence of advanced HF was higher in patients with severe or complex congenital heart disease. Onset of advanced HF was associated with 6-fold increase in the risk of mortality. Conventional cardiac intervention was associated with significantly higher risk of mortality as compared to transplant listing. The longer the interval from the initial onset of advanced HF to transplant evaluation, the lower the odds of being listed for transplant.

Conclusions: Based on these data, we postulate that early identification of patients with advanced HF, and a timely referral for transplant evaluation (instead of conventional cardiac intervention) may offer the best chance of survival for these critically ill patients. Further studies are required to validate this postulation.

Keywords: adults; heart disease; heart failure; incidence; mortality.

Figure 1.

Cumulative incidence of advanced heart failure (HF) stratified based on congenital heart disease (CHD) severity and anatomic/physiological classification. A shows CHD severity, and B shows anatomic/physiological classification. The P values were derived from log-rank test comparing the different strata. Cox regression model showing risk factors associated with all-cause mortality among the 5309 patients that without advanced HF at the time baseline assessment (C). Only the covariates with statistically significant association (P<0.05) with all-cause mortality are shown. For the complete list of covariates in the Cox model, please see Table 4. HR indicates hazard ratio; RV, right ventricle; and SV, single ventricle.

Figure 2.

Flowchart showing the different treatment pathways among the 432 patients with advanced heart failure (HF) during follow-up. Of the 203 that were discharged home after conventional cardiac intervention (CCI), 62 patients were subsequently referred for transplant evaluation because of persistent or worsening HF symptoms, and all 62 patients were declined for transplant listing.

Figure 3.

Cox regression model showing the relationship between the different treatment pathways and all-cause mortality among the 432 patients with advanced heart failure (HF). A, Logistic regression model showing the relationship between interval from advanced HF diagnosis (the interval from initial diagnosis of advanced HF to the initial presentation to the transplant clinic) and subsequent transplant listing among the 167 patients that underwent transplant evaluation (B). Only the covariates with statistically significant association (P<0.05) and outcome are shown. For the complete list of covariates in the Cox and logistic regression models, please see Table S4. HR indicates hazard ratio; OR, odds ratio; q6m, every 6 mo; RV, right ventricle; and SV, single ventricle.