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Abstract Background Adults with congenital heart disease (ACHD) represent a rapidly expanding population with evolving mortality patterns. Despite improved survival, excess mortality persists. Objective To evaluate the incidence, causes, and predictors of mortality in a contemporary ACHD cohort. Methods We performed a retrospective cohort study of adults (≥18 years) first evaluated at Mayo Clinic from 2002–2023. Baseline clinical, imaging, and electrocardiographic data were analyzed. Vital status was determined using institutional records and the Accurint national mortality database. Kaplan-Meier analysis and Cox proportional hazard models were used to evaluate mortality and identify independent predictors of mortality Results A total of 7,678 ACHD patients were included, with median age of 36.8 years and median follow-up of 11.4 years. During 78,768 patient-years of follow-up, 1,116 patients died (median age at death 57.2 years), corresponding to an annual mortality rate of 1.4%. The cumulative rate of all-cause mortality at 5, 10, 15, and 20 years was 6.9%, 12.0%, 19.0%, and 26.2%, respectively. When stratified by CHD complexity, the annual death rate in patients with severe CHD (2.4%/year) was twice that of patients with moderate or mild CHD (both 1.2%/year). Older age and ACHD subtypes, specifically, cyanotic heart disease (HR 3.9, 95% CI 2.9–5.3) and Fontan physiology (HR 3.2, 95% CI 2.3–4.4), were strongly associated with increased mortality. Additional independent predictors included male sex, ventricular dysfunction, advanced NYHA class, prior heart failure hospitalization, hypertension, smoking, coronary artery disease, renal dysfunction, and abnormal hemoglobin levels. Cardiovascular causes accounted for 57.7% of deaths with known etiology, predominantly heart failure (48.9%) and sudden cardiac death (21.9%), while non-cardiovascular causes were driven mainly by infection and malignancy. Conclusions In this large contemporary ACHD cohort, mortality was driven by ventricular dysfunction, heart failure, and systemic end-organ involvement in addition to the underlying congenital anatomy. Both cardiovascular and non-cardiovascular causes contributed significantly to mortality. These findings underscore the need for comprehensive multidisciplinary ACHD care focused on early recognition of cardiac functional decline, management of acquired comorbidities, and end-organ dysfunction.

Background: Patients with repaired Tetralogy of Fallot (TOF) undergoing pulmonary valve replacement (PVR) are at increased risk of major adverse ventricular arrhythmias and related events (MAREs). Perioperative electrophysiological study (EPS) has been used for risk stratification and guiding future management, but these studies are limited by small sample size. Objectives: To perform a systematic review of outcomes of pre-PVR EPS in TOF patients with PVR. Methods: A comprehensive search of PubMed, Embase, Scopus and Google scholar databases was performed per PRISMA 2020 guidelines. Studies on patients with repaired TOF undergoing PVR with perioperative EPS were included. Data were extracted on study design, sample size, EPS outcomes and MAREs during follow-up. Results: A total of 1,528 patients (age: 35.5±3.8 y; 57% male; LVEF: 57.1±1.5%, RVEF: 42.6±1.5%) were included from 12 studies. EPS was performed in 1212/1528 (79.3%), with sustained VT inducibility in 404 (33.3%)-monomorphic VT in 258 (63.8%), while 808 (66.6%) were non- inducible. 8/12 studies reported the type of PVR (transcatheter-36.8%). 174/293 (59.3%) inducible patients had an ablation (9 studies), while ICD (6 studies) was implanted in 70/263 (26.6%). Follow-up EPS (6 studies) showed 35/230 (15.2%) remained inducible. Overall,113 (9.3%) patients experienced MAREs over a median follow-up ranging from 6 months to 6.7 years. Sustained VT (11/12 studies) occurred in 70/1092 (6.4%), appropriate ICD therapy (7/12 studies) in 13/622 (2.1%) and sudden cardiac death (10/12 studies) in 30/906 (3.3%). Five studies provided MARE data stratified by VT inducibility. 12/164 (7.3%) inducible patients experienced MAREs, compared to 2/318 (0.6%) non-inducible patients. Reported risk factors for MAREs included older age at initial repair and PVR, QRS duration (>180ms), LVEF <45%, RVEF <35%, history of syncope, prior palliative shunts and atrial arrhythmias. Some studies identified MRI-derived scar burden, pulmonary annulus size, and RV/LV volume ratios as additional predictors. Conclusion: TOF patients remain at high risk of VA after PVR with 1/10 patients experiencing MAREs. People with inducible VT had a 12-fold greater risk than people who were non-inducible. EPS identifies a subset of TOF patients at higher arrhythmic risk during PVR and supports tailored ablation and ICD strategies. Prospective multicentre studies are needed to optimize integration of EPS and clinical risk models.