Adult Congenital Heart Disease Database Research Articles

Outcomes of permanent pacemakers and implantable cardioverter-defibrillators in an adult congenital heart disease population

BackgroundBrady- and tachyarrhythmias commonly complicate adult congenital heart disease (ACHD). Permanent pacemakers (PPMs) or implantable cardioverter–defibrillators (ICDs) are often utilised to prevent morbidity or mortality related to arrhythmia, but can also be associated with significant morbidity themselves. MethodsWe analysed outcomes from patients in our comprehensive ACHD database who were seen at least twice since 2000 and once since 2018. Of 1953 ACHD patients, 134 had a PPM and 78 had an ICD (47 for primary and 31 for secondary prevention). ResultsFor PPM patients, 41% had a pacing percentage below 33%, 13% had 33–66%, and 46% had above 66%. One fifth required PPM upgrade, most to cardiac resynchronisation therapy, the rest to ICD. There were 33 appropriate ICD shocks in 15 patients (19%) and 34 inappropriate shocks in 13 patients (17%) over a median follow up of 4.6 years (IQR 0.9–8.3 years). Anti-tachycardia pacing was delivered appropriately for 28% of patients and inappropriately for 9%.Apart from inappropriate therapy, one third of PPM and ICD patients had other device-related complications. Acute PPM complications included lead dysfunction requiring revision (2%), pneumothorax (2%), pleural effusion (2%) and pocket infection (2%). ICDs were also acutely complicated by lead dysfunction (4%) as well as pocket hematoma (3%). The most common long-term complication overall was lead dysfunction, affecting one sixth of both PPM and ICD patients. Finally, the rate of device insertion increased significantly with disease severity. ConclusionsAnti-arrhythmic devices can be lifesaving in ACHD patients, but inappropriate therapy and device-related complications are very common.

Operational and Ethical Considerations for a National Adult Congenital Heart Disease Database.

As more adults survive with congenital heart disease, the need to better understand the long‐term complications, and comorbid disease will become increasingly important. Improved care and survival into the early and late adult years for all patients equitably requires accurate, timely, and comprehensive data to support research and quality‐based initiatives. National data collection in adult congenital heart disease will require a sound foundation emphasizing core ethical principles that acknowledge patient and clinician perspectives and promote national collaboration. In this document we examine these foundational principles and offer suggestions for developing an ethically responsible and inclusive framework for national ACHD data collection.

Hospital discharge codes and substantial underreporting of congenital heart disease

BackgroundHospital discharge codes are relied upon for research, accounting/invoicing and health systems planning. Congenital heart disease (CHD), however, is uniquely difficult for non-cardiologists to code due to the rarity, variety and complexity of lesions. It is therefore important that the accuracy of hospital discharge codes is regularly checked to ensure that the prevalence and burden of CHD is being correctly estimated and recorded. Methods and resultsWe identified all inpatient admissions of adults with CHD to Royal Prince Alfred Hospital in Sydney, Australia from January 2018 to March 2021 (257 admissions, 106 unique patients). The associated discharge coding summaries were extracted and compared to the codes in the separately collected and audited Adult CHD database. Only a quarter of discharge coding summaries contained any diagnosis of CHD, and just one-tenth accurately recorded all appropriate CHD diagnoses. Patients with simple lesions were most likely to have a coded diagnosis of CHD, while those with moderate and complex lesions were much less likely. Moreover, patients admitted under a cardiovascular specialty were twice as likely to have a coded diagnosis of CHD, compared with those admitted under non-cardiovascular specialties (p ​= ​0.006). Overall, less than half of patients had any hospital-coded diagnosis of CHD in any admission over the three-year study period. ConclusionsHospital discharge coding dramatically underreports CHD, especially for patients with moderate and severe CHD lesions and for admissions under non-cardiovascular specialties. This suggests that discharge coding-based estimates of the burden of CHD on hospitals and health systems may be substantially underestimated.

Late complication rates after aortic coarctation repair in patients with or without a bicuspid aortic valve

ObjectivePatients with previously repaired aortic coarctation (CoA) are at risk of developing late surgical complications. Many patients with CoA also have a bicuspid aortic valve (BAV). We sought to determine...

Defibrillators in adult congenital heart disease: Long-term risk of appropriate shocks, inappropriate shocks, and complications.

Sudden cardiac death (SCD) accounts for up to 25% of deaths in the adult congenital heart disease (ACHD) population. Current guidelines for defibrillator implantation are either extrapolated from acquired cardiac disease or are based upon single lesion studies, predominantly Tetralogy of Fallot (TOF). Defibrillator-related morbidity appears to be substantially higher in ACHD patients. We retrospectively evaluated all patients in our ACHD database who received an implantable cardioverter-defibrillator (ICD) between 2000 and 2019, and who were ≥16 years old at time of implant. Patients were followed for appropriate shocks, inappropriate shocks, and complications. Of 4748 patients in our database, 59 patients (1.2%) underwent ICD implantation. ICDs were for primary prevention in 63% and secondary prevention in 37%. Over a median follow-up of 6.6 years, 24% received an appropriate shock, 27% an inappropriate shock, and 42% suffered a device-related complication (annualized risks of 3.2%, 3.6%, and 5.7%, respectively). There were no significant predictors of appropriate shocks or inappropriate shocks. All appropriate shocks in primary prevention patients occurred in TOF or transposition of the great arteries (TGA) with atrial switch, typically in the presence of multiple SCD risk factors. The majority of inappropriate shocks were due to supraventricular arrhythmias. Device-related mortality was 1.7% (0.3% per annum). Appropriate shocks were relatively common in an ACHD ICD population followed in the long term. Device-related morbidity was significant. Although risk factors have been established for TOF, and to a lesser extent TGA, risk stratification for ICD implantation in ACHD remains challenging.

Left arm structure and function late after subclavian flap repair of aortic coarctation in childhood.

Concerns exist over the long-term consequences of subclavian artery ligation in subclavian flap repair for coarctation of the aorta. We sought to analyse upper limb structural and functional performance in adults who have had surgery in childhood for coarctation of the aorta, using either subclavian flap repair or end to end aortic anastomosis. Two-group observational design using anatomical and upper limb functional performance measures. Purposive sampling from our specialist adult congenital heart disease database of patients who received subclavian flap repair or end to end anastomosis for coarctation of the aorta as children. Upper limb measurements were completed using MRI and blood flow velocity with ultrasound imaging. Bilateral standardised upper limb functional testing of assessment of strength, dexterity and a standardised self-report of upper limb disability was completed. Eighteen right-handed patients, 9 with subclavian repair, (38 ± 12 years, 78% males) were studied. Age at repair was 4.7 ± 5.9 years; mean time from initial repair 32 ± 9 years. The subclavian group had a larger difference between right and left when compared the end to end anastomosis group in: lower arm muscle mass (94.5 ± 42.3 mls versus 37.8 ± 94.5 mls, p = 0.008), lower arm maximal cross-sectional area, (5.9 ± 2.8 cm2 versus 2.9 ± 2.6 cm2, p = 0.038) and grip strength (14.7 ± 8.3 lbs versus 5.9 ± 5.3 lbs, p = 0.016) There were no significant functional differences between groups. In adults with repaired coarctation of the aorta, those with subclavian flap repair had a greater right to left arm muscle mass and grip strength differential when compared to those with end to end anastomosis repair.

Mechanism and Risk Factors for Death in Adults With Tetralogy of Fallot

One of the goals of lifelong care in adults with tetralogy of Fallot (TOF) is early identification and treatment of patients at high risk for adverse events. Clinical risk stratification tools are critical for achieving this goal. We reviewed the Mayo Adult Congenital Heart Disease database and identified 465 TOF patients (age 37 ± 14 years, men 223 [48%]) seen at Mayo Clinic Rochester between 1990 and 2017. The aim was to determine the risk factors for death and/or heart transplant through a comprehensive analysis of 8 groups of variables (demographics, co-morbidities, medications, heart rhythm, echocardiography, cardiac magnetic resonance imaging, cardiac catheterization, and cardiopulmonary exercise test data) using univariable and multivariable Cox proportional hazard models. The end point of death and/or transplant occurred in 57 (12%) patients during a follow-up of 13.6 ± 8.2 years, yielding an event rate of 0.9% per year. Independent risk factors were age >42 years, atrial fibrillation, ≥moderate QRS fragmentation, left ventricular ejection fraction <50%, right ventricular end-diastolic pressure >16 mm Hg, and left ventricle end-diastolic pressure >16 mm Hg. There is nearly a twofold increase in the risk of death and/or transplant per unit increase in number of risk factors (hazard ratio 1.92, 95% confidence interval 1.62 to 2.27, p <0.001). In conclusion, the current study provides risk stratification indices based on a comprehensive risk model of all clinical variables in an unselected TOF population. Further studies are required to determine whether interventions targeted at modifying these risk factors will alter the annual event rate.

Role of QRS Fragmentation for Risk Stratification in Adults With Tetralogy of Fallot.

BackgroundPatients with tetralogy of Fallot (TOF) remain at risk for cardiovascular events despite successful repair. Some of the current risk stratification tools require advanced imaging and invasive studies, and hence are difficult to apply to routine patient care. A recent study showed that QRS fragmentation (QRS‐f) is predictive of mortality in patients with TOF. The current study aims to validate this result by assessing whether severity of QRS‐f could predict all‐cause mortality in a different TOF population.Methods and ResultsThe authors reviewed the Mayo Adult Congenital Heart Disease database for patients with TOF who had ECG from 1990–2017. QRS‐f was defined as notches in QRS complex in ≥2 contiguous leads on ECG, not related to bundle branch block, and classified as none, mild (≤3 leads), moderate (4 leads), or severe (≥5 leads). Of 465 patients (age 37±14 years) in the study, QRS‐f was present in 161 (35%): mild (n=43, 9%), moderate (n=77, 17%), and severe (n=41, 9%). There were 55 deaths (12%) during 13.6±8.2 years of follow‐up. Severity of QRS‐f remained an independent predictor of all‐cause mortality after adjustment for other ECG parameters, patient demographics, and atrial and ventricular arrhythmia (hazard ratio, 1.74 per class; 95% confidence interval, 1.08–2.93 [P=0.041]).ConclusionsThe presence of severe QRS‐f may be used as complementary data to the usual clinical indices to determine whether interventions such as invasive electrophysiology study should be performed in patients with nonsustained ventricular tachycardia or to proceed with pulmonary valve replacement in patients with severe pulmonary regurgitation with ventricular volumes below the guideline‐directed threshold for intervention.

Efficacy and adverse effects of sotalol in adults with congenital heart disease

IntroductionAdults with congenital heart disease (CHD) are predisposed to arrhythmias, which can often be refractory to medical therapy. Sotalol is an attractive alternative antiarrhythmic to amiodarone in this younger patient population, given the latter's toxicity profile, but it may have proarrhythmic effects. We therefore aimed to assess the efficacy and safety of sotalol in adults with CHD. MethodsWe retrospectively assessed our adult CHD database for all patients ≥16 years old, with moderate to highly complex defects, who were prescribed sotalol between 2000 and 2017. Efficacy in treating the clinical arrhythmia was assessed as complete, partial or failure. Adverse effects, including proarrhythmia, were documented. ResultsSotalol was prescribed in 82 of 902 adult CHD patients reviewed (9%). The mean age at sotalol commencement was 31.8 ± 13.1 years, with a median time on sotalol of 2.8 years. The average prescribed dose was 122 ± 51 mg/daily. Sotalol was completely effective in 48% (n = 39), partially effective in 46% (n = 38) and failed to control the clinical arrhythmia in 6% (n = 5). Fifteen patients (18%) discontinued sotalol due to a side effect, most commonly fatigue or dyspnoea. No episodes of torsades de pointes or sudden cardiac death were observed. Significant bradycardia related to sotalol occurred in 13% (n = 11, with permanent pacing implemented in 4), and was associated with Fontan anatomy. ConclusionsIn moderate to highly complex adult CHD, sotalol was reasonably effective and safe in low doses. Side effects limiting treatment were typically non-life-threatening, with significant bradycardia related to sotalol more likely in Fontan patients.

Incidence and clinical characteristics of sudden cardiac death in adult congenital heart disease

BackgroundThe life expectancy of adults with congenital heart disease (CHD) has significantly improved in recent decades, with non-cardiovascular causes of death now competing with traditional cardiovascular causes. The risk of sudden cardiac death (SCD), a devastating event, still remains elevated above that of the general population. MethodsWe reviewed 2935 patients in our adult CHD database (age≥16years, seen at least once in our centre) and documented all cases of SCD between 2000–2015. Incidence and characteristics of SCD cases by congenital defect and complexity of disease were determined. ResultsWe documented 35 cases of SCD, with an incidence of 0.4 deaths/1000patientyears (py). Incidence in simple, moderate and complex congenital categories was 0.04/1000py, 0.57/1000py and 2.0/1000py respectively. The highest risk category was Eisenmenger syndrome, with an incidence of 4.8 deaths/1000py. Moderate risk lesions included transposition of the great arteries (atrial switch surgery or congenitally corrected) and Fontan circulations. Repaired tetralogy, atrial septal defect and left ventricular outflow tract lesions were all relatively low risk. We observed a high prevalence of atrial arrhythmias (43%) and QRS prolongation (mean 132ms) in our SCD cases. ConclusionsThe adult CHD population remains at an elevated risk for SCD, particularly in the setting of complex underlying defects. Moderate to high risk lesions include Eisenmenger syndrome, transposition of the great arteries (atrial switch or congenitally corrected) and Fontan circulations.

Incidence, predictors and outcomes of infective endocarditis in a contemporary adult congenital heart disease population

BackgroundThe prevalence of congenital heart disease (CHD) in the adult population is steadily increasing. A substrate of prosthetic material and residual lesions, constantly evolving as surgical techniques change over time, predispose these patients to the potentially devastating complication of infective endocarditis (IE). MethodsWe retrospectively reviewed 2935 patients in our adult CHD database for all cases of endocarditis between 1991 and 2016. Incidence, clinical course and predictors of outcomes were analysed. ResultsWe document 74 episodes in 62 patients, with an incidence of 0.9 cases/1000 patient years (py). IE was more common in complex CHD (1.4 cases/1000py) and ventricular septal defects (VSDs) (1.9 cases/1000py). Prosthetic material was involved in 47% and left-sided infection predominated (66%). The incidence in bicuspid aortic valves post aortic valve replacement (AVR) was significantly higher than in unoperated valves, being 1.8 and 1.1 cases/1000 patient years respectively. Streptococcus was the most frequently implicated causative organism (37%). Emboli occurred in 34% of cases with a cerebral predilection. 46% of patients required surgery during the admission for IE, most frequently to replace a severely regurgitant bicuspid aortic valve. Early endocarditis-related mortality was 15%, associated with cerebral emboli and acute renal failure. ConclusionsIn a contemporary adult CHD cohort, those with complex underlying lesions, VSDs or an AVR were at higher risk for IE. Mortality remains substantial and is more likely in patients with cerebral emboli and/or acute renal failure.

O27-1 - Hepatitis C Virus Positivity Negatively Affects Cardiac Functions and Long-term Prognosis in Patients with Adult Congenital Heart Disease

Background: It was previously suggested that hepatitis C virus (HCV) positivity affects cardiac function. As the screening for HCV began in 1992 in Japan, we hypothesized that HCV positive rate would be higher in patients with adult congenital heart disease (ACHD) who underwent heart surgery before 1992, which would adversely affect cardiac functions and long-term prognosis in those patients. Methods and Results: We retrospectively examined 253 patients (median age, 20 years; M/F, 119/134) who were enrolled in our ACHD database between 1995 and 2010. Changes in cardiac functions were evaluated by both δleft ventricular ejection fraction (δLVEF, change from baseline LVEF) and δLVEF/year (δLVEF divided by follow-up year). Major adverse cardiac event (MACE) was defined as the composite of cardiac death, heart failure hospitalization, lethal ventricular arrhythmias, heart reoperation, and heart or pulmonary transplantation. HCV positivity was noted in 23 patients (9.1%) and all of them had undergone heart surgery before 1992. HCV-positive patients in the MACE-free group had greater reduction in LVEF; δLVEF (−22.5 ± 8.7 vs. −9.3 ± 11.5%, P = .0002) and δLVEF/year (−1.5 ± 0.7 vs. −0.8 ± 1.1%/year, P = .0066). Multivariate COX analysis showed that HCV positivity was a significant positive predictor of MACE (HR 2.21, 95% CI 1.02 to 4.39, P = .044). Conclusions: These findings suggest that more attention should be paid to HCV positivity in the management of ACHD patients.

Prevalence of Cancer in Adults With Congenital Heart Disease Compared With the General Population

The prevalence rate of cancer among adult patients with congenital heart disease (CHD) in North America has not been previously described. The Quebec adult CHD database was used to determine the prevalence rate of cancer among adult patients with CHD measured as the number of adults with CHD and cancer alive in 2005 per 1,000 adults with CHD. This prevalence rate was compared with the prevalence rate of cancer in the general population of adults in Canada. Types of cancer among the CHD group were described by gender and age. Adult patients with CHD had a 1.6 to 2 times higher prevalence of cancer at 2, 5, and 10years for both men and women. Overall, men had a greater prevalence of total cancers in all-time durations than did women. Breast, colon, and prostate cancer were the most common cancers reported in adults with CHD. In conclusion, we observed an increased prevalence of cancer among the adult CHD population of Quebec compared with the general Canadian population.

P1 Diagnosis and stroke risk of atrial fibrillation in patients with atrial septal defects: A single centre experience

Background Atrial fibrillation (AF) is one of the most common sustained tachyarrhythmias. It increases risk of developing stroke by up to five times. There is an increased incidence in patients with adult congenital heart disease (ACHD). The CHA 2 DS 2 VASC scoring system is validated for assessing the risk of ischaemic stroke in AF in the general population. Its role in ACHD is unclear. This study assesses the burden of AF in patients with uncomplicated atrial septal defects (ASD). Methods A retrospective analysis of the ACHD database of all patients at a large quaternary specialist ACHD centre. Results 135 (3%) patients with a diagnosis of ASD and co-existing AF were identified out of 4500 patients under regular follow up at Queen Elizabeth Hospital Birmingham. 101 (75%) secundum ASD, 12 (9%) sinus venosus ASD, 1 (1%) coronary sinus ASD and 21 (15%) unclear. 47 (35%) surgical closures, 28 (21%) percutaneous closure, 57 (42%) unrepaired, 3 (2%) both surgical and percutaneous. There were 7 residual defects post closure. -The mean age was 67.8(+/-12.8) years, range 36–90 years, 65 male (48.1%). -The mean CHA 2 DS 2 VASC score was 2.21 (Range 0–6). -The mean age of onset of AF was 56(+/-12.3) years. In 40% the AF was asymptomatic. -90% of patients with a CHA 2 DS 2 VASC score of 2 or more were anticoagulated with either warfarin or a novel oral anticoagulant (n = 75). 11 patients had suffered a stroke (8.15%). -7 presented initially with a stroke and asymptomatic AF; of these 6 were also found to have ASDs after the stroke. The mean age stroke occurred was 58.7(+/-20) years. The mean CHA 2 DS 2 VASC score for patients who had a stroke was 4.18 (Range 2–6). Discussion The incidence of AF in ASDs is high and is frequently asymptomatic. AF and strokes occur at a young age in this population. A significant proportion of our patients presented for the first time with a stroke and asymptomatic AF. Expert consensus currently advocates screening for arrhythmias in certain ACHD groups but not ASD. This data suggest that patients with ASD should be actively screened for AF. The role of the CHA 2 DS 2 VASC score if unclear in these patients but a high CHA 2 DS 2 VASC does appear to correlate with a higher incidence of stroke.

Pulmonary outflow obstruction protects against heart failure in adults with congenitally corrected transposition of the great arteries

BackgroundPulmonary outflow tract obstruction (POTO) reduces systemic atrioventricular valve (SAVV) regurgitation severity in congenitally corrected transposition of the great arteries (ccTGA). Therefore, pulmonary artery banding is proposed as a palliative intervention. We aimed to investigate the effect of native or surgically induced POTO on event-free survival, defined as the composite of all-cause mortality, heart transplantation, or congestive heart failure (CHF). Methods and resultsPatients with ccTGA (n=62; median age 27.5 (IQR 18.4–39.4) years; 39% with POTO) were selected from the Adult Congenital Heart Disease database of a tertiary hospital. At first visit, SAVV regurgitation ≥3/4, systemic RV dysfunction ≥moderate, and CHF were present in 26%, 26%, and 15% of patients, respectively. Over a mean follow-up time of 10.1±6.1years, all-cause mortality, rate of heart transplantation, and CHF were 18%, 8% and 40%, respectively. SAVV regurgitation (HR: 1.99; 95% CI: 1.01–3.92; P=0.048) and systemic RV dysfunction severity (HR: 1.89; 95% CI: 1.05–3.37; P=0.033) were associated with the composite endpoint, independently of age at baseline, POTO, Ebstein-like malformation, and systemic RV dilatation. Patients with POTO had lower risk for developing SAVV regurgitation ≥3/4 (HR: 0.18; 95% CI: 0.05–0.58; P=0.004) and moderate systemic RV dysfunction (HR: 0.34; 95% CI: 0.15–0.78; P=0.011). When POTO was present, the mean progression-free interval for the composite endpoint increased from 11.2 to 18.1years (P=0.035). ConclusionsPOTO is associated with an improved event-free survival in adults with ccTGA.

Optimizing CT angiography in patients with Fontan physiology: single-center experience of dual-site power injection

To identify adult patients with single-ventricle congenital heart disease and Fontan procedure palliation who have been misdiagnosed with or incompletely evaluated for pulmonary embolism. Additionally, this study was designed to demonstrate that simultaneous, dual-injection of contrast medium into an upper and lower extremity vein is superior to single-injection protocols for CT angiography (CTA) of the chest in this population. Patients included in the study were retrospectively selected from the Adult Congenital Heart Disease (ACHD) database. Search criteria included history of Fontan palliation and available chest CT examination. Patients were evaluated for (1) type of congenital heart disease and prior operations;(2) indication for initial CT evaluation;(3) route of contrast medium administration for the initial CT examination and resulting diagnosis;(4) whether or not anticoagulation therapy was initiated; and (5) final diagnosis and treatment plan.Results: The query of the ACHD database resulted in 28 individuals or patients with Fontan palliation (superior and inferior venae cavae anastomosed to the pulmonary arteries). Of these, 19 patients with Fontan physiology underwent CTA of the pulmonary circulation, and 17 had suboptimal imaging studies. Unfortunately, seven of these 17 patients (41%) were started on anticoagulation therapy due to a diagnosis of pulmonary embolism that was later excluded. Patients with single-ventricle/Fontan physiology are at risk of thromboembolic disease. Therefore, studies evaluating their complex anatomy must be performed with the optimal imaging protocol to ensure diagnostic accuracy, which is best achieved with dual-injection of an upper and lower extremity central vein.

Adult Congenital Heart Disease Database—Bay of Plenty District Health Board (BOPDHB)

Adult Congenital Heart Disease Database—Bay of Plenty District Health Board (BOPDHB)

Risk of Aortic Root or Ascending Aorta Complications in Patients With Bicuspid Aortic Valve With and Without Coarctation of the Aorta

The actual incidence of ascending aorta complications (AACs) in adults with bicuspid aortic valve (BAV) and the role of associated coarctation of the aorta (COA) as an independent risk factor for AACs remain unknown. From the Adult Congenital Heart Disease database at La Paz Hospital, 631 patients in whom a BAV was diagnosed by echocardiography or surgical inspection since December 1989 were identified. These patients were then further subdivided into 2 groups according to the presence of an associated COA. AACs included aortic aneurysms (ascending aorta > or =55 mm) and aortic dissection, rupture, or perforation. Patients with a BAV and COA had a greater prevalence of AACs (8.0%) than those with an isolated BAV (3.7%; p = 0.037). The coexistence of COA was the only significant predictor of AACs (odds ratio 4.7, 95% confidence interval 1.5 to 15; p = 0.01). From the total patient group with a BAV, the clinical and echocardiographic data were reviewed for 341 patients without an AAC at baseline (97 with and 244 without COA) who had undergone serial examinations >1 year apart. The median follow-up was 7 years (interquartile range 3.5 to 10.2; total 2,436 patient-years). A new AAC occurred in 13 patients (0.5/100 patient-years). The incidence of AACs was 1.3/100 patient-years in the COA group versus 0.2/100 patient-years in the non-COA group (hazard ratio 7.5, 95% confidence interval 2.0 to 28, p = 0.002). All acute aortic events (dissection or rupture) at follow-up occurred in patients with a BAV and COA. In conclusion, the long-term incidence of AACs in patients with isolated BAV is low, but patients with BAV and associated COA are at increased risk.