Arrhythmias in congenital heart disease: A nationwide cohort study.

BACKGROUND: As more patients with congenital heart disease (CHD) survive into adulthood, the population of adults with CHD is expanding. This trend is accompanied by an increasing incidence of complications, including arrhythmias. However, the long-term risk of arrhythmias remains sparsely investigated. METHODS: In this observational cohort study, all Danish patients with CHD born from 1977 to 2024 were identified using registries and followed from date of birth until the occurrence of arrhythmia, emigration, death, or end of follow-up (March 2024). The risk of arrhythmias was assessed among patients with CHD and compared to age- and sex-matched controls from the background population. RESULTS: A total of 45,820 patients with CHD (50.9% men) were identified and matched with 183,280 controls from the background population. During a median follow-up of 21.5 years, 2.6% of patients with CHD and 0.2% of controls developed arrhythmias-corresponding to incidence rates (IR) of 1.2 (95% CI 1.2-1.3) and 0.1 (95% CI 0.1-0.1) per 1,000 PY, respectively, and a hazard ratio (HR) of 16.4 (95% CI 14.4-18.7). The most common arrhythmias in patients with CHD were advanced atrioventricular block (IR 0.4 [95% CI 0.4-0.4] per 1,000 PY) and atrial flutter/fibrillation (IR 0.5 [95% CI 0.5-0.6] per 1,000 PY). Patients with malformations of the heart chambers, transposition of the great arteries, tetralogy of Fallot, and atrioventricular septal defect were at the highest risk of arrhythmias. Moreover, the risk of arrhythmias among those with ASD was not negligible. In patients with CHD, arrhythmia was associated with a significantly higher risk of death (HR of 6.9 [95% CI 5.9-8.1]). CONCLUSIONS: Patients with CHD are at significantly higher risk of arrhythmias than the background population, and those with complex CHD are at particularly high risk. In patients with CHD, arrhythmia is associated with an increased risk of death. Additional studies are warranted to investigate how we can improve the diagnosis and management of arrhythmias in CHD.

Pulmonary Valve Replacement in Tetralogy of Fallot: Procedural Volume and Durability of Bioprosthetic Pulmonary Valves.

BACKGROUND: Robust data on changes in pulmonary valve replacement (PVR) procedural volume and predictors of bioprosthetic pulmonary valve (BPV) durability in patients with tetralogy of Fallot (TOF) are scarce. OBJECTIVES: This study sought to assess temporal trends in PVR procedural volume and BPV durability in a nationwide, retrospective TOF cohort. METHODS: Data were obtained from patient records. Robust linear regression was used to assess temporal trends in PVR procedural volume. Piecewise exponential additive mixed models were used to estimate BPV durability, defined as the time from implantation to redo PVR with death as a competing risk, and to assess risk factors for reduced durability. RESULTS: In total, 546 PVR were performed in 384 patients from 1976 to 2021. The annual number of PVR increased from 0.4 to 6.0 per million population (P < 0.001). In the last decade, the transcatheter PVR volume increased by 20% annually (P < 0.001), whereas the surgical PVR volume did not change significantly. The median BPV durability was 17 years (Q1: 10-Q3: 10 years-not applicable). There was no significant difference in the durability of different BPV after adjustment for confounders. Age at PVR (HR: 0.78 per 10 years from <1 year; 95% CI: 0.63-0.96; P = 0.02) and true inner valve diameter (9-17 mm vs 18-22 mm HR: 0.40; 95% CI: 0.22-0.73; P = 0.003 and 18-22 mm vs 23-30 mm HR: 0.59; 95% CI: 0.25-1.39; P = 0.23) were associated with reduced BPV durability in multivariate models. CONCLUSIONS: The PVR procedural volume has increased over time, with a greater increment in transcatheter than surgical PVR during the last decade. Younger patient age at PVR and a smaller true inner valve diameter predicted reduced BPV durability.

Incidence, predictors, and prognostic impact of rehospitalization after transcatheter aortic valve implantation.

AIMS: Despite rehospitalization being common after transcatheter aortic valve implantation (TAVI), an in-depth analysis on this topic is missing. This study sought to report on the incidence, predictors, and prognostic impact of rehospitalization within one year following TAVI. METHODS AND RESULTS: All consecutive patients treated with TAVI between 2016 and 2020 in East Denmark were included. Medical records of all patients were reviewed to validate rehospitalizations up to 1 year after discharge from the index admission. The study population consisted of 1,397 patients, of whom 615 (44%) had an unplanned rehospitalization within the first year post-TAVI. The rehospitalization incidence rate was 3-fold higher in the early period (within 30 days) compared with the late period (30 days to 1 year; 2.5 vs. 0.8 per patient-year, respectively; p < 0.001). Predictors of early unplanned rehospitalization were procedure-related complications and prior stroke, whereas late unplanned rehospitalization was associated with pre-existing comorbidities. Predictors of HF rehospitalization included ischemic heart disease, the extent of cardiac damage, atrial fibrillation, and NYHA class at baseline. HF rehospitalization within 30 days and 1-year post-TAVI was associated with a markedly increased 1-year and 5-year mortality risk (hazard ratio (HR) of 4.3 and 3.2 for 1-year mortality and HR of 3.2 and 2.9 for 5-year mortality, respectively; p < 0.001). CONCLUSIONS: Rehospitalization after TAVI is frequent in real-world practice. Early rehospitalization is mostly procedure-related whereas late rehospitalization is related to pre-existing comorbidities. HF rehospitalization is associated with poor long-term survival and could be validated as a prognostically relevant endpoint for TAVI trials.

Temporal changes in the surgical management of patients with tetralogy of Fallot in Denmark: a nationwide cohort study.

OBJECTIVES: To assess temporal changes in the surgical management of patients with tetralogy of Fallot including the timing of interventions, surgical techniques, reinterventions and survival in a nationwide cohort. METHODS: Patients with tetralogy of Fallot in Denmark were divided into 3 eras based on their year of birth: early (1977-1991), intermediate (1992-2006) and late (2007-2021). RESULTS: The cohort consisted of 745 patients. Median follow-up was 21.2 years (13.7-30.5). There was a temporal trend towards less shunt palliation (-0.3% per year, 95% CI -0.05 to -0.1). Median age at intracardiac repair was 2.9 years (1.8-5.0), 0.8 years (0.5-1.3) and 0.5 years (0.4-0.7) (P < 0.001) in the early, intermediate and late era, respectively. There was a temporal trend towards less valve-sparing repair (-0.7% per year, 95% CI -0.5 to -1.0) and more repair with transannular patches (0.7% per year, 95% CI 0.5-1.0). Survival at 10 years was 79% (64-76), 90% (87-93) and 95% (92-98) (P < 0.001) and pulmonary valve replacement within the first 10 years after intracardiac repair was performed in 3% (1-6), 12% (8-16) and 21% (13-29) (P < 0.001) in the early, intermediate and late era, respectively. CONCLUSIONS: There was a temporal trend towards less shunt palliation and intracardiac repair at a younger age with more use of transannular patches. While survival throughout childhood and adolescence has improved, more patients undergo pulmonary valve replacement during the first 10 years after intracardiac repair.

Incidence of Infective Endocarditis Among Patients With Tetralogy of Fallot.

Background Patients with tetralogy of Fallot (ToF) are considered at high risk of infective endocarditis (IE) as a result of altered hemodynamics and multiple invasive procedures, including pulmonary valve replacement (PVR). Data on the long-term risk of IE are sparse. Methods and Results In this observational cohort study, all patients with ToF born from 1977 to 2018 were identified using Danish nationwide registries and followed from date of birth until occurrence of first-time IE, emigration, death, or end of study (December 31, 2018). The comparative risk of IE among patients with ToF versus age- and sex-matched controls from the background population was assessed. Because of rules on anonymity, exact numbers cannot be reported if the number of patients is <4. A total of 1164 patients with ToF were identified and matched with 4656 controls. Among patients with ToF, 851 (73.1%) underwent early surgical intracardiac repair and 276 (23.7%) underwent PVR during follow-up. During a median follow-up of 20.3 years, 41 (3.5%) patients with ToF (comprising 24 [8.7%] with PVR and 17 [1.9%] without PVR) and <4 (<0.8%) controls were admitted with IE. The incidence rates of IE per 10 000 person-years were 22.4 (95% CI, 16.5-30.4) and 0.1 (95% CI, 0.01-0.7) among patients and controls, respectively. Moreover, PVR was associated with a further increased incidence of IE among patients with ToF (incidence rates per 10 000 person-years with and without PVR were 46.7 [95% CI, 25.1-86.6] and 2.8 [95% CI 2.0-4.0], respectively). Conclusions Patients with ToF are associated with a substantially higher incidence of IE than the background population. In particular, PVR was associated with an increased incidence of IE. With an increasing life expectancy of these patients, intensified awareness, preventive measures, and surveillance of this patient group are decisive.

Infective endocarditis in right ventricular outflow tract conduits: a register-based comparison of homografts, Contegra grafts and Melody transcatheter valves.

OBJECTIVES: The aim was to investigate the incidence of infective endocarditis (IE) in right ventricle-to-pulmonary artery conduits implanted at a Danish tertiary centre. METHODS: Cases of IE in patients with homografts, Contegra grafts and Melody transcatheter valves were evaluated retrospectively with regard to the likeliness of the diagnosis using the modified Duke criteria and the likeliness of conduit involvement. Incidence rates for IE were calculated 1 and 5 years after valve implantation for all 3 conduits, and separately for Melody subgroups depending on which conduit served as landing zone. Cox regression with time-dependent covariates was used to model the impact of the conduit type on the incidence of IE. RESULTS: Annualized incidence rates of IE in homografts, Contegra grafts and Melody valves were 0.40% (0.40 cases per 100 patient-years), 0.97% and 6.96% 1 year and 0.27%, 1.12% and 2.89% 5 years after valve implantation. Hazard ratios (HRs) were 3.20 [95% confidence interval (CI) 0.91-11.17, P = 0.069] for Contegra grafts and 11.89 (95% CI 2.91-48.48, P < 0.001) for Melody valves compared to homografts. CONCLUSIONS: Bovine pulmonary conduits were more prone to endocarditis, with Melody valves being the most frequently infected. HRs for the risk of suffering from endocarditis were substantially higher for Melody valves and Contegra grafts compared to homografts, although this finding was only statistically significant for Melody valves and not for Contegra grafts.