Burden and Impact of Arrhythmias in Repaired Tetralogy of Fallot.

PURPOSE OF REVIEW: Arrhythmias are a leading cause of morbidity and mortality following repair of tetralogy of Fallot (TOF). This review will highlight current understanding of arrhythmia pathogenesis in this patient population and review novel therapeutic options. RECENT FINDINGS: Risk factors for developing ventricular arrhythmias in repaired TOF have thus far been better defined than for atrial arrhythmias. Growing understanding of the pathophysiology of arrhythmias, development of risk stratification models, and novel techniques such as electrophysiologic ultrahigh-density mapping should help to better identify patients that benefit from advanced therapies such as ablation and implantable cardioverter defibrillators. Atrial and ventricular arrhythmias are common in TOF patients. Methods of risk stratification and therapeutic approaches are rapidly evolving, leading to ever improving clinical outcomes in this patient population.

Hybrid transcatheter pulmonary valve replacement with a SAPIEN S3 valve after pulmonary artery banding via left lateral thoracotomy.

For many patients with repaired congenital heart disease, the need for reintervention on dysfunctional right ventricular outflow tracts is pervasive. Many such patients are poor candidates for both transcatheter pulmonary valve replacement and cardiopulmonary bypass, and hybrid surgical and transcatheter procedures have evolved to meet this need. We present two cases of hybrid pulmonary valve replacement involving pulmonary artery band placement via left anterior thoracotomy followed by transvenous placement of a SAPIEN S3 valve without prestenting. This approach avoids cardiopulmonary bypass as well as redo sternotomy and will likely see an increase in utilization in the future.

Approaching End-of-Life Decisions in Adults with Congenital Heart Disease.

PURPOSE: Despite tremendous advances in medical and surgical care, some adults with congenital heart disease (ACHD) develop terminal conditions where therapy is limited. This paper reviews the important role of palliative care, advance care planning (ACP), and end-of-life (EOL) care in ACHD. RECENT FINDINGS: Recent studies suggest that ACP is infrequently utilized in ACHD. Patients generally express interest in learning more about EOL care, though few ACHD providers have received adequate training to confidently conduct these discussions. Most barriers to communication are largely addressable, and an organized approach to ACP that encourages active patient participation followed by clear documentation is more likely to be successful. Palliative care appears complementary to standard medical care and can be introduced at any stage of illness, with proven benefit in similar patient populations. ACP is an important part of the routine care for all ACHD. Patient preferences should be identified early and palliative methods incorporated whenever necessary.

Heart Transplantation and Mechanical Circulatory Support in Adults with Congenital Heart Disease.

PURPOSE OF REVIEW: To assess current management strategies for advanced heart failure in adults with congenital heart disease, including heart transplantation and mechanical circulatory support. RECENT FINDINGS: Current data demonstrate that adults with CHD generally experience higher short-term mortality after heart transplantation and MCS implantation, but enjoy superior long-term survival. Such patients are nonetheless less likely to receive a transplant than non-ACHD peers due to a variety of factors, including lack of applicability of current listing criteria to HF in ACHD. MCS is underutilized in ACHD, but provides similar quality of life benefits for ACHD and non-ACHD patients alike. Heart failure in ACHD is complex and difficult to treat, and both heart transplantation and mechanical circulatory support are often challenging to implement in this patient population. However, long-term results are encouraging, and existing data supports increasing use of MCS and transplant earlier in their disease course. Multidisciplinary care is critical to success in these complex patients.

Utility of Cardiac Magnetic Resonance Imaging in Predicting Atrial Arrhythmias in Repaired Tetralogy of Fallot.

Arrhythmias are the leading cause of morbidity and mortality in repaired tetralogy of Fallot (TOF), and over 20% of these patients will develop a sustained atrial arrhythmia during their lifetimes. Cardiac magnetic resonance imaging (cMRI) is frequently performed in TOF, although its ability to identify patients at risk of atrial arrhythmias is uncertain. Adult TOF patients (n = 175) with no history of atrial arrhythmia who underwent cMRI between 2003 and 2020 at a single tertiary care center were identified. Clinical characteristics and imaging findings were evaluated to identify a predilection for atrial arrhythmias using Kaplan-Meier survival analysis and log-rank testing. Multivariable Cox regression was used to determine independent predictors of atrial arrhythmias. Over a median follow-up of 3.6 years, 29 patients (17%) developed atrial arrhythmias. Independent predictors of atrial arrhythmia included age (hazard ratio [HR] 1.06 per 1-year increase, 95% confidence interval [CI] 1.02 to 1.09, p = 0.002), diabetes mellitus (HR 4.26, 95% CI 1.26 to 14.41, p = 0.020), indexed right ventricular end-diastolic volume (RVEDVi), (HR 1.20 per 10-ml/m2 increase, 95% CI 1.05 to 1.39, p = 0.010), and moderate or greater tricuspid regurgitation (TR) (HR 6.32, 95% CI 2.15 to 18.60, p = 0.001). Utilizing Kaplan-Meier analysis, patients with at least mild right ventricular dilation (RVEDVi >100 ml/m2, p = 0.047) and greater than or equal to moderate TR (p <0.001) were found to be significantly more likely to develop atrial arrhythmias. In conclusion, cMRI can help to identify TOF patients at increased risk for atrial arrhythmia beyond standard clinical and imaging data by better quantifying RVEDVi and degree of TR.

Where Adults with Congenital Heart Disease Die: Insights from the CDC-WONDER Database.

The population of adults with congenital heart disease (ACHD) is rapidly increasing. There is limited understanding of location of death and associated disparities in these patients. From 2005-2018, a trend-level analysis was performed using death certificate data from the Centers for Disease Control and Prevention Wide-ranging Online Data for Epidemiologic Research Database, with individual-level mortality data obtained from National Center for Health Statistics. Places of death were classified as hospital, home, hospice facility, nursing home/long-term care and other. A total of 15,507 total deaths were identified in ACHD from 2005-2018 (54% Male, 84% White). ACHD patients were more likely to die in the hospital (64%) compared to general population (41%). Younger decedents (20-34) with ACHD were more likely to die in the hospital, while older decedents (≥65) were more likely to die at Hospice/Nursing facilities. Black and Hispanic patients with ACHD were more likely to die in the hospital compared to White and non-Hispanic patients. A significantly large proportion of ACHD deaths are observed in younger patients and occur in inpatient facilities. End-of-life planning among socially vulnerable populations should be prioritized.

The Evolving Surgical Burden of Fontan Failure: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database.

BACKGROUND: Fontan failure often requires surgical therapy in the form of Fontan revision or heart transplantation. We sought to characterize national trends in the surgical burden of Fontan failure and identify risk factors for adverse outcomes. METHODS: Fontan patients undergoing Fontan revision or transplantation from January 2010 to June 2018 were included. We evaluated baseline characteristics and outcomes and used multivariable logistic regression to identify risk factors for operative mortality and composite mortality and major morbidity. RESULTS: A total of 1135 patients underwent Fontan revision (n = 598) or transplantation (n = 537) at 100 centers. Transplantations increased from 34 in 2010 to 76 in 2017, largely owing to an increase in patients with hypoplastic left heart syndrome (HLHS) (18 in 2010 to 49 in 2017), while Fontan revision decreased (75 in 2010 to 49 in 2017). Transplantation patients were younger (median 14 years of age vs 18 years of age; P < .001), more often had preoperative risk factors (66% vs 40%; P < .001), and more often had HLHS (51% vs 15%; P < .001). Operative mortality and composite major morbidity and mortality were 7.6% and 35% for transplantation and 7.1% and 22% for Fontan revision, respectively. Multivariable risk factors for mortality included older age (odds ratio [OR], 1.08/y; P = .007), presence of preoperative risk factors (OR, 3.33; P = .002), and concomitant pulmonary artery reconstruction (OR, 2.7; P = .029) for Fontan revision but only older age (OR, 1.06/y; P = .020) for transplantation. CONCLUSIONS: Both transplantation and Fontan revision are associated with high morbidity and mortality. There has been evolution of practices in surgical therapy for Fontan failure, perhaps related to rising prevalence of HLHS staged palliation.

Impaired right ventricular reserve predicts adverse cardiac outcomes in adults with congenital right heart disease.

OBJECTIVE: The prevalence of heart failure (HF) among adult patients with congenital heart disease (ACHD) is rising. Right ventricle (RV) exercise reserve and its relationship to outcomes have not been characterised. We aim to evaluate the prognostic impact of impaired RV reserve in an ACHD population referred for cardiopulmonary exercise testing (CPET). METHODS: This retrospective study evaluates patients with ACHD who underwent CPET (n=147) with first-pass radionuclide ventriculography at a single tertiary care centre. RV reserve was categorised as normal, mild to moderately or severely impaired. The primary composite clinical outcome included clinical right HF, arrhythmia, transplantation or death. RESULTS: Patients were median age 41±13 years, 50% were female and median follow-up was 1.1 (IQR: 0.7-2.0) years. Exercise RV reserve was impaired in 103 patients (70%), of whom 32% were asymptomatic. Resting RV systolic function poorly predicted RV reserve, with 52% of patients with severe impairment having a qualitatively normal echocardiographic assessment. The severely impaired reserve group had lower peak oxygen consumption (VO2)(17.2 vs 22.5 mL/kg/min, p<0.0001) compared with the normal reserve group, and was more likely to develop the composite outcome (48% vs 9%, log-rank p<0.001). Severely impaired RV reserve predicted event-free survival after adjusting for peak VO2, age, sex, RV pathology, QRS duration, New York Heart Association class, resting RV ejection fraction and RV dilation by echocardiography or MRI (HR 3.7, 95% CI 1.1 to 13.0, p=0.039). CONCLUSION: Impaired RV reserve, occurred in asymptomatic patients, was not well predicted by resting systolic function assessment, and strongly predicted adverse cardiovascular outcomes.