Adult Congenital Heart Disease Population Research Articles

Arrhythmia Burden Among Adult PatientsWith Congenital Heart Disease: APopulation-Based Study.

A significant percentage of patients with congenital heart disease surviving into adulthood will develop arrhythmias. These arrhythmias are associated with an increased risk of adverse events and death. We aimed to assess arrhythmia prevalence, risk factors, and associated health care usage in a large national cohort of patients with adult congenital heart disease. Adults with a documented diagnosis of congenital heart disease, insured by Clalit and Maccabi health services between January 2007 and December 2011, were included. We assessed the associations between arrhythmia and subsequent hospitalization rates and death with mixed negative binomial and Cox proportional hazard models, respectively. Among 11 653 patients with adult congenital heart disease (median age, 47 years [interquartile range, 31-62]), 8.7% had a tachyarrhythmia at baseline, 1.5% had a conduction disturbance, and 0.5% had both. Among those without a baseline arrhythmia, 9.2% developed tachyarrhythmias, 0.9% developed a conduction disturbance, and 0.3% developed both during the study period. Compared with no arrhythmia (reference group), arrhythmia in the previous 6 months was associated with a higher multivariable adjusted hospitalization rate, 1.33-fold higher than the rate of the reference group (95% CI, 1.00-1.76) for ventricular arrhythmia, 1.27-fold higher (95% CI, 1.17-1.38) for atrial arrhythmias, and 1.33-fold higher (95% CI, 1.04-1.71) for atrioventricular block. Atrial tachyarrhythmias were associated with an adjusted mortality hazard ratio (HR) of 1.65 (95% CI, 1.44-2.94), and ventricular tachyarrhythmias with a >2-fold increase in mortality risk (HR, 2.06 [95% CI, 1.44-2.94]). Arrhythmias are significant comorbidities in the adult congenital heart disease population and have a significant impact on health care usage and survival.

Characteristics and Outcomes of a Single-Centre Cohort of Adult Congenital Heart Disease Patients Referred for Heart Transplant

Adult congenital heart disease (ACHD) services increasingly encounter heart failure (HF) in the ageing ACHD population. Optimal timing of referral for heart transplant (HTx) evaluation in this heterogeneous population is complex and ill-defined. We aim to outline the characteristics and outcomes of ACHD patients referred for HTx from a large Australian ACHD centre. Retrospective review of ACHD patients referred for HTx from a primary ACHD centre (1992-2021). Database analysis of patient demographics, characteristics, wait-listing, and transplantation outcomes was performed. A total of 45 patients (mean age 37±9.9 years old; 69% male) were referred for HTx with a mean follow-up of 5.9±6.3 years. Of these, 22 of 45 (49%) were listed and transplanted, including one heart-lung transplant. The commonest diagnosis was dextro-transposition of the great arteries (13/45, 29%). Most patients, 33 of 45 (73.3%) had undergone at least one cardiac surgery in childhood. Indications for HTx referral included HF in 34 of 45 (75%), followed by pulmonary hypertension in 7 of 45 (11%). Median transplant wait-list time was 145 days (interquartile range, 112-256). Of the 23 patients not wait-listed, the reasons included clinical stability in 13 of 45 (29%), psychosocial factors in 2 of 45 (4.4%) and prohibitive surgical risk, including multiorgan dysfunction, in 8 of 45 (17.7%). Transplant was of a single organ in most, 21 of 22 (95.5%). Overall mortality was 5 of 22 (22.7%) in those after HTx, and 14 of 23 (60.9%) in those not listed (p=0.0156). Increasingly, ACHD patients demonstrate the need for advanced HF treatments. HTx decision-making is complex, and increased mortality is seen in those not wait-listed. Ultimately, the referral of ACHD patients for HTx is underpinned by local decision-making and experience, wait-list times and outcomes.

Safety of SGLT-2 inhibitors in the management of heart failure in the adult congenital heart disease patient population

BackgroundSodium glucose transporter 2 inhibitors (SGLT-2i) have shown safety and efficacy in patients with heart failure (HF). However, evidence for the use of SGLT-2i in adult congenital heart disease (ACHD) patients with HF is limited. MethodsWe performed a retrospective, single center analysis of 18 patients (>18 years of age) with ACHD and a diagnosis of HF who were initiated on an SGLT-2i. Patient characteristics, including vital signs, laboratory values, concomitant medications, clinical outcomes, and echocardiograms, were obtained as part of standardized clinical care at our ACHD program before and 2–6 months after initiation of SGLT-2i. The primary outcome was to demonstrate safety of SGLT-2i initiation via potential changes in systolic blood pressure, serum sodium, and serum creatinine. ResultsOf the 18 patients, 11 (61%) had moderate complexity congenital heart disease while 7 (39%) had great complexity congenital heart disease. Post initiation, there were no significant differences in systolic blood pressure (121.8 ± 20.8 mmHg to 114.4 ± 14.9 mmHg, p = 0.06), sodium level (138.7 ± 2.9 mMol/L to 138.0 ± 2.2 mMol/L, p = 0.75), and creatinine level (0.85 ± 0.18 mg/dL to 0.89 ± 0.18 mg/dL, p = 0.07). There was a statistically significant decline in weight (78.9 ± 22.9 kg to 76.0 ± 23.0 kg, p = 0.0039) but without a statistically significant change in NT-pro NBP (1358.2 ± 2735.0 pg/mL to 601.6 ± 786.1 pg/mL, p = 0.36). ConclusionsWe demonstrated the use of SGLT-2i in a small cohort of ACHD population, including patients with complex congenital heart disease, appears safe and well tolerated. The safety and potential efficacy of SGLT-2i in patients with ACHD will require further evaluation in prospective multicenter studies.

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.

Translation of Tools and Techniques from the Adult Electrophysiology World to Pediatric Cardiac Implantable Electronic Devices.

This article reviews various opportunities to translate established and novel tools and techniques used in adult electrophysiology to pediatrics and the adult congenital heart disease population. There is a specific focus on preoperative management of special population, implantation techniques, and postoperative programming of devices.

Adult Congenital Heart Disease Care in Canada: Has Quality of Care Improved in the Last Decade?

BackgroundPatients with adult congenital heart disease (ACHD) are at increased risk of comorbidity and death compared with the age-matched population. Specialized care is shown to improve survival. The purpose of this study was to analyze current measures of quality of care in Canada compared with those published by our group in 2012. MethodsA survey focusing on structure and process measures of care quality in 2020 was sent to 15 ACHD centres registered with the Canadian Adult Congenital Heart Network. For each domain of quality, comparisons were made with those published in 2012. ResultsIn Canada, 36,708 patients with ACHD received specialized care between 2019 and 2020. Ninety-five cardiologists were affiliated with ACHD centres. The median number of patients per ACHD clinic was 2000 (interquartile range [IQR]: 1050, 2875). Compared with the 2012 results, this represents a 68% increase in patients with ACHD but only a 19% increase in ACHD cardiologists. Compared with 2012, all procedural volumes increased with cardiac surgeries, increasing by 12% and percutaneous intervention by 22%. Wait time for nonurgent consults and interventions all exceeded national recommendations by an average of 7 months and had increased compared with 2012 by an additional 2 months. Variability in resources were noted across provincial regions. ConclusionsOver the past 10 years, ACHD care gaps have persisted, and personnel and infrastructure have not kept pace with estimates of ACHD population growth. Strategies are needed to improve and reduce disparity in ACHD care relative to training, staffing, and access to improved care for Canadians with ACHD.

Preprocedural Anxiety in Adults With Congenital Heart Disease: The PANIC Study

BackgroundPreprocedural anxiety may have detrimental effects both cognitively and physiologically. ObjectivesThe objective of this study was to determine the association between state (situational) and trait (persistent in everyday life) anxiety and differences between the adult congenital heart disease (ACHD) and acquired heart disease populations. MethodsThe State-Trait Anxiety Inventory and financial stress scale were administered to adults with acquired and CHD at 4 tertiary referral centers in the United States prior to cardiac catheterization. Student’s t-test and least absolute shrinkage and selection operator regression analyses were used to assess differences in anxiety between groups and identify the optimal model of predictors of anxiety. ResultsOf the 291 patients enrolled, those with CHD (n = 91) were younger (age 41.3 ± 16.3 years vs 64.7 ± 11.3 years, P < 0.001), underwent more cardiac surgeries (P < 0.001), and had higher levels of trait anxiety (t[171] = 2.62, P = 0.001, d = 0.33). There was no difference in state anxiety between groups (t[158.65] = 1.37, P = 0.17, d = 0.18). State anxiety was singularly associated with trait anxiety. Trait anxiety was negatively associated with age and positively associated with state anxiety and financial stress. Patients with CHD of great complexity were more trait (F[2,88] = 4.21, P = 0.02) and state anxious (F[2,87] = 4.59, P = 0.01), though with relatively small effect size. ConclusionsTrait anxiety levels are higher in the ACHD population and directly associated with state anxiety. Specialists caring for ACHD patients should not only recognize the frequency of trait anxiety but also high-risk subgroups that may benefit from psychological or social interventions to reduce preprocedural anxiety.

Novel Therapeutic Strategies in Heart Failure in Adult Congenital Heart Disease: of Medicines and Devices.

This paper reviews the latest literature on the growing field of heart failure in the adult congenital heart disease population. After highlighting the increasing prevalence and a few of the unique potential causes, including the concept of early senescence, this review begins with novel medical management strategies such as the angiotensin II receptor blocker and neprilysin inhibitors and sodium glucose cotransporter-2 inhibitors. Then, it addresses the latest applications of percutaneous techniques like implantable hemodynamic monitoring, transcatheter pulmonary and aortic valve replacement, and mitral clips. Cardiac resynchronization therapy and novel lymphatic system imaging and intervention are then described. Finally, the use of mechanical support devices, temporary and durable, is discussed as well as heart and combined heart and liver transplantation. There have been recent exciting advances in the strategies used to manage adult congenital heart disease patients with heart failure. As this population continues to grow, it is likely we will see further rapid evolution in this field.

The Effects of Cardiac Implantable Electronic Device Personal Protective Equipment Use in Patients with Pediatric and Adult Congenital Heart Disease.

Pediatric and adult congenital heart disease (ACHD) patients encounter physical and emotional barriers. Cardiac implantable electronic devices (CIEDs), including pacemakers and implantable cardiac defibrillators (ICD) often compound these issues. Patient fear associated with damaging the CIED system can lead to avoidance of physical activity and reduced quality of life (QOL). CIED personal protective equipment (PPE) is a potential treatment for decreasing this fear. We sought to determine the effects of CIED PPE use in the pediatric and ACHD population. Patients 5 years or older with a CIED at a single pediatric and ACHD heart rhythm center received a CIED protector and customized athletic shirt. QOL was assessed using the PedsQL TM 4.0 SF15 prior to and after 6-12months. Of the 77 patients enrolled, 26 completed repeat assessment. The mean age at enrollment was 15.6years (range 5-36) with a mean device age of 2.2years (< 1-10years). Pacemakers were present in 77% and 23% had ICDs. The PPE was used in 92% with no PPE malfunction. Fear associated with physical activity was reduced, z = -4, p < 0.001, with a large effect size (r = 0.55). There was a trend toward increased physical activity. Total QOL scores improved, z = -2.771, P < 0.05, with a medium effect size (r = 0.4). This first study of CIED PPE in children shows that providing CIED PPE to pediatric and ACHD patients may decrease their fear of damaging their device system, increasing physical activity levels, and improving quality of life.

Transvenous lead extraction of lumenless 3830 pacing lead in conduction system pacing: a single-center experience

BackgroundThe Medtronic SelectSecure Model 3830 lumenless lead (Medtronic, Inc., Minneapolis, MN) is commonly used for conduction system pacing (CSP). However, with this increased use, the potential need for transvenous lead extraction (TLE) also will increase. While extraction of endocardial 3830 leads is rather well described especially in pediatric and adult congenital heart disease population, there is very limited data on extraction of CSP leads. In the present study, we reported our preliminary experience on TLE of CSP leads and provided technical considerations.MethodsThe study population comprised 6 consecutive patients (67% male; mean age 70 ± 22 years) with CSP leads (3830 leads), including left bundle branch pacing (LBBP) lead (n = 3) and His pacing lead (n = 3) undergoing TLE. Overall target leads were 17. The mean implant duration time of CSP leads was 97 ± 90 months [range 8–193).ResultsManual traction was successful in 2 cases and mechanical extraction tools were required in the remaining cases. Sixteen leads (94%) were completely extracted, whereas incomplete removal was observed in one lead (6%) among 1 patient. Of note, in the only lead incompletely removed, we observed retention of < 1-cm remnant of lead material consisting of the screw of 3830 LBBP lead into the interventricular septum. No failure of lead extraction was reported and no major complications occurred.ConclusionsOur findings demonstrated that at an experienced center the success of TLE of chronically implanted CSP leads is high in the absence of major complications also when mechanical extraction tools are needed.

C43 RELATIONSHIP BETWEEN ECHOCARDIOGRAPHIC DATA AND CARDIOPULMONARY EXERCISE TEST PARAMETERS IN ADULT CONGENITAL HEART DISEASE PATIENTS: A RETROSPECTIVE CLINICAL STUDY

Abstract Introduction The interpretation of cardiopulmonary exercise test (CPET) in adult congenital heart disease (ACHD) is challenging because of the high variability in cardiac lesions and because the poor exercise tolerance is often associated with abnormal heart function. The aim of this work is to study the correlation between echocardiographic evaluation and CPET parameters in ACHD population. Methods This is a retrospective clinical study enrolling all ACHD patients afferent to our institution from March 2016 to February 2020 collecting clinical, CPET and echocardiographic data. Population was divided into three groups and compared: patients with prevalent left heart lesions (L group), patients with prevalent right heart lesions (R group) and patients with single ventricle physiology (SV group). Finally, correlation analysis were performed to assess any relationship between echocardiographic and CPET data. Results 60 patients were included in the study, 20 were male, median age was 36 [30–46.75] years old, 23 had a prevalent left side pathology, 31 a prevalent right side pathology and 6 had a single ventricle physiology. L group had a significantly lower predicted peak VO2 in comparison to SV group (p=0.03). Statistical analysis showed a correlation between the right ventricular function and the ventilatory reserve in the R group (R2 0.517). In SV group we found correlation between ventricular–arterial coupling and Cardiac mechanical efficiency with the ventilatory reserve (R2 0.6 and 0.65, respectively) and the oxygen pulse (R2 0.97 and 0.98, respectively), and a correlation between the ejection fraction and the Oxygen pulse (R2 0.52). Finally, a correlation between the TAPSE/PAPs index and the ventilation (R2=0.79), the ratio between the oxygen uptake and the workload (R2=0.61) and the oxygen consumption (R2=0.72) was found. Coclusion:In our preliminary experience, echocardiographic parameters of cardiocirculatory efficiency correlate with the oxygen consumption per heart beat evaluated at the CPET in single ventricle patients, and the breath rate is influenced not only by ventricular function but also by the coupling between the ventricle and the arterial system. In patients affected by right–side disease, the breath rate correlate with the right ventricular function. Finally, in the left heart lesion population, a correlation between the TAPSE/PAPs index with the ventilation, the oxygen consumption per beat and the oxygen consumption was found.

PE-452787-3 INITIAL EXPERIENCE OF PULSED FIELD ABLATION OF ATRIAL FIBRILLATION AND ATRIAL TACHYCARDIA IN ADULT PATIENTS WITH CONGENITAL HEART DISEASE

Prevalence of atrial tachycardias (AT) and atrial fibrillation (AF) is increasing in adult patients with congenital heart disease (CHD) significantly contributing to morbidity. Catheter ablation of AT and AF in the adult CHD population is challenging and recurrence is significant. Pulsed field ablation (PFA) is a new, non-thermal energy source for catheter ablation of AF. Due to its inherent properties of creating effective ablation lesions without permanent affection of non-myocardial tissue, it is attractive for the use in the adult CHD population. To date, its use in the adult CHD population has not been reported. This study describes initial experience of PFA of AT and AF in adult CHD patients. Prospective enrollment of adult CHD patients with a history of recurrent AT/AF, who had prior radiofrequency (RFA)/cryoenergy (Cryo) ablation of AT/AF. A high-density map using a 3D mapping system (EnSite™ Precision; Abbott) in conjunction with a high-density catheter (Advisor™ HD Grid; Abbott) was performed prior and after PFA. PFA was performed using the FARAWAVE™ ablation catheter (Boston Scientific) and applying repeated 2 kV pulses (2.5 s each) to the respective substrate. Procedural success was defined as complete substrate ablation (residual atrial electrograms < 0.05 mV on voltage map). Four adult CHD patients with recurrent AT/AF despite repeated previous RFA/cryoablation were enrolled. 2/4 had interrupted inferior V. cava and a transhepatic access was used to get access to the left atrium. PFA of all pulmonary vein ostia and the left atrial posterior wall was performed in 3 subjects. Additional PFA of a septal low voltage area was achieved in one of these patients. In the fourth patient with Ebstein’s anomaly, PFA of the cavotricuspid isthmus (CTI) and of a conduction gap within the crista terminalis was performed. Complete procedural success was achieved in all patients. Transient, fully reversible spasm of the right coronary artery without ECG changes occurred after PFA within the CTI. No recurrence of AT/AF was observed during follow-up of 5 months. Herein, we report for the first time the use of PFA in adult CHD patients with recurrent AT/AF. PFA turned out to be effective and generally safe. When using it in proximity to the coronary arteries, one must be aware of reversible coronary artery spasm. PFA might be a valuable tool to improve mid- and long-term outcome of AT/AF ablation in the adult CHD population.

Cardiac Drugs in ACHD Cardiovascular Medicine.

Adult congenital heart disease (ACHD) is a growing population that requires life-long care due to advances in pediatric care and surgical or catheter procedures. Despite this, drug therapy in ACHD remains largely empiric due to the lack of clinical data, and formalized guidelines on drug therapy are currently lacking. The aging ACHD population has led to an increase in late cardiovascular complications such as heart failure, arrhythmias, and pulmonary hypertension. Pharmacotherapy, with few exceptions, in ACHD is largely supportive, whereas significant structural abnormalities usually require interventional, surgical, or percutaneous treatment. Recent advances in ACHD have prolonged survival for these patients, but further research is needed to determine the most effective treatment options for these patients. A better understanding of the use of cardiac drugs in ACHD patients could lead to improved treatment outcomes and a better quality of life for these patients. This review aims to provide an overview of the current status of cardiac drugs in ACHD cardiovascular medicine, including the rationale, limited current evidence, and knowledge gaps in this growing area.

Heart failure in systemic right ventricle: Mechanisms and therapeutic options.

d-loop transposition of the great arteries (d-TGA) and congenitally corrected transposition of the great arteries (cc-TGA) feature a right ventricle attempting to sustain the systemic circulation. A systemic right ventricle (sRV) cannot support cardiac output in the long run, eventually decompensating and causing heart failure. The burden of d-TGA patients with previous atrial switch repair and cc-TGA patients with heart failure will only increase in the coming years due to the aging adult congenital heart disease population and improvements in the management of advanced heart failure. Clinical data still lags behind in developing evidence-based guidelines for risk stratification and management of sRV patients, and clinical trials for heart failure in these patients are underrepresented. Recent studies have provided foundational data for the commencement of robust clinical trials in d-TGA and cc-TGA patients. Further insights into the multifactorial nature of sRV failure can only be provided by the results of such studies. This review discusses the mechanisms of heart failure in sRV patients with biventricular circulation and how these mediators may be targeted clinically to alleviate sRV failure.

From Other Journals: A Review of Recent Articles by Our Editorial Team.

This review is meant to highlight recent publications from other journals that are relevant to pediatric cardiologists. The articles chosen for this edition look at the outcomes of catheter-based interventions for aortic stenosis, the effect of atherosclerotic cardiovascular disease risk factors on the adult congenital heart disease population, the difference in mortality from congenital heart disease between rural and urban America, preoperative NT-proBNP as a predictor of Fontan outcomes, and an overview of the utilization and outcomes of the Rastelli, Nikaidoh, and REV procedures.

Heart-Focused Anxiety Is Prevalent in Adults With Congenital Heart Disease and Associated With Reduced Exercise Capacity.

Psychological distress and anxiety are increased among adults with congenital heart disease (CHD). However, heart-focused anxiety (HFA) and its impact on peak oxygen uptake (V˙ o2peak ) have not yet been studied in this patient cohort. This study investigates HFA in adults with CHD and whether HFA is associated with V˙ o2peak in these patients. In this cross-sectional study, HFA was assessed in 984 adults with various CHD (34.2 ± 11.2 yr, 44% female) by the cardiac anxiety questionnaire. In 708 of those (33.5 ± 10.6 yr, 42% female), additional cardiopulmonary exercise testing was performed to assess exercise capacity by measurement of V˙ o2peak . Compared with the general population, this adult CHD population showed higher HFA in the total cardiac anxiety questionnaire score ( z score; 1.32 ± 1.17; P < .001), as well as in the three subdomains anxiety, avoidance, and attention (all P < .001). The higher the HFA, the lower was the V˙ o2peak % predicted ( r = -0.280, P < .001) independent of CHD diagnosis, CHD severity, and surgical status. The V˙ o2peak % predicted significantly differed between patients with no (85.9 ± 18.4% predicted), slight (80.4 ± 18.4% predicted), and severe HFA (74.2 ± 18.4% predicted) independent of CHD diagnosis, CHD severity, and surgical status ( P < .001). This study shows that HFA is a common sequela in adults with CHD, and high HFA is associated with reduced V˙ o2peak . Psychological screening and counseling for HFA therefore seem indicated in the aftercare of adults with CHD.

Heart Failure in Complex Congenital Heart Disease of the Adult.

Adult congenital heart disease (ACHD) patients have demonstrated improved survival, especially those with severely complex disease, mainly single-ventricle/Fontan physiology and those with a systemic right ventricle. We describe the heart failure phenotypes of complex CHD, reversible causes for heart failure, and considerations for advanced therapy. While initially marketed for application to patients with acquired causes for heart failure, newer devices and technologies have started to be used in the ACHD population. After reversible causes for heart failure in CHD are addressed, it is reasonable to consider use of new device-based technologies and orthotopic heart transplant (OHT) for end-stage disease. New heart failure technology and organ transplant should carefully be considered and applied in complex ACHD, where there may be significant improvement in morbidity and mortality.

Kidney disease and congenital heart disease: Partnership for life

The literature on the relationship between kidney and cardiovascular diseases is continuously expanding. Scientists have elucidated many of the neurohormonal and hemodynamic pathways involved in cardiorenal disease. However, little is known about kidney disease in patients with congenital heart disease. Given advances in the medical and surgical care of this highly complex patient population, survival rates have dramatically improved leading to a higher percentage of adults living with congenital heart disease. Accordingly, a noticeable increase in the prevalence of kidney disease is appreciated in these patients. Some of the main risk factors for developing chronic kidney disease in the adult congenital heart disease population include chronic hypoxia, neurohormonal derangements, intraglomerular hemodynamic changes, prior cardiac surgeries from minimally invasive to open heart surgeries with ischemia, and nephrotoxins. Unfortunately, data regarding the prevalence, pathophysiology, and prognosis of chronic kidney disease in the adult congenital heart disease population remain scarce. This has led to a lack of clear recommendations for evaluating and managing kidney disease in these patients. In this review, we discuss contemporary data on kidney disease in adults with congenital heart disease in addition to some of the gaps in knowledge we face. The article highlights the delicate interaction between disease of the heart and kidneys in these patients, and offers the practitioner tools to more effectively manage this vulnerable population.

Conduction system pacing in the adult congenital population; feasibility and outcomes

Abstract Funding Acknowledgements Type of funding sources: None. Background Left bundle pacing (LBP) is an evolving pacing method designed to capture the intrinsic conduction and minimise ventricular dys- synchrony. Anatomical complexity, scars of previous corrective surgeries and haemodynamic properties of adult congenital heart disease (ACHD) population could increase challenges of LBP. We describe our experience in LBP in ACHD patients and different patient groups. Purpose To compare the feasibility of LBP in ACHD patient population and non-ACHD patients. Methods We included all patients who had LBP at our institution in 2020 and 2021. Demographic data, imaging data, procedural parameters and follow up data were collected and analysed. Results A total of 36 patients were included and divided into two groups: ACHD (n=8, mean age, 54 years, 38% females) and non-ACHD (n=28, mean age, 74 years, 43% females). ACHD anatomy included simple lesions (4) as atrial and ventricular septal defects, partial anomalous pulmonary venous drainage, bicuspid aortic valve and Shone’s syndrome, moderate (3), as Tetralogy of Fallot with surgical repair, and complex (1), as transposition of great arteries with Mustard repair. Non-ACHD included patients with structurally normal heart, dilated cardiomyopathy, and those who underwent mitral and aortic valve interventions. Mean left ventricular ejection fraction in ACHD group was 59.5%, and 57.5% in non-ACHD. Late gadolinium enhancement in basal septal area was present in 5 patients in ACHD group. Acute success rate, defined as capture of left bundle branch, was 100% in ACHD group, and 88% in non-ACHD. No acute complications were recorded in both groups. Mean pre-procedural QRS duration was longer in ACHD group (170 ms, vs, 120, p=&amp;lt;0.001). Mean reduction in QRS duration in ACHD group was 27 ms, vs, 15, p= 0.856. Mean procedural and fluoroscopy times were similar in both groups (ACHD, 75.5 minutes, vs, 70, p= 0.26, and ACHD, 9 minutes, vs 7.13, respectively, p= 0.46). Pacing parameters at implantation and after 2 months were satisfactory in both groups (Fig 1). Conclusion Left bundle pacing is feasible in ACHD population as compared to non-ACHD patients, with low incidence of complications. Procedural and fluoroscopic times were similar in both groups. Pacing parameters were satisfactory and stable over 2 month-follow up.

PO-704-04 LEFT BUNDLE PACING IN ADULT CONGENITAL HEART DISEASE PATIENTS

Left bundle pacing (LBP) is an evolving pacing method designed to capture the intrinsic conduction and minimize ventricular dys-synchrony. Anatomical complexity, scars of previous corrective surgeries and hemodynamic properties of adult congenital heart disease (ACHD) population could increase challenges of LBP. We describe our experience in LBP in ACHD patients and different patient groups.