Systemic Ventricular Ejection Fraction Research Articles

Noninvasive Advanced Cardiovascular Magnetic Resonance-Derived Fontan Hemodynamics Are Associated With Reduced Kidney Function But Not Albuminuria.

Kidney disease is the most important predictor of death in patients with a Fontan circulation, yet its clinical and hemodynamic correlates have not been well established. A total of 53 ambulatory patients with a Fontan circulation (median age, 16.2 years, 52.8% male patients) underwent advanced cardiovascular magnetic resonance assessment, including 4-dimensional flow imaging and computational fluid dynamics. Estimated glomerular filtration rate (eGFR) <90 mL/min per 1.73 m2 was observed in 20.8% and albumin-to-creatinine ratio >3 mg/mmol in 39.6%. The average eGFR decline rate was -1.83 mL/min per 1.73 m2 per year (95% CI, -2.67 to -0.99; P<0.001). Lower eGFR was associated with older age, larger body surface area at examination, longer time since Fontan procedure, and lower systemic ventricular ejection fraction. Higher albumin-to-creatinine ratio was associated with absence of fenestration at the Fontan operation, and older age and lower systemic ventricular ejection fraction at the assessment. Lower cross-sectional area of the Fontan conduit indexed to flow (r=0.32, P=0.038), higher inferior vena cava-conduit velocity mismatch factor (r=-0.35, P=0.022), higher kinetic energy indexed to flow in the total cavopulmonary connection (r=-0.59, P=0.005), and higher total cavopulmonary connection resistance (r=-0.42, P=0.005 at rest; r=-0.43, P=0.004 during exercise) were all associated with lower eGFR but not with albuminuria. Kidney dysfunction and albuminuria are common among clinically well adolescents and young adults with a Fontan circulation. Advanced cardiovascular magnetic resonance-derived metrics indicative of declining Fontan hemodynamics are associated with eGFR and might serve as targets to improve kidney health. Albuminuria might be driven by other factors that need further investigation.

Multicenter Study of Survival Benefit of Cardiac Resynchronization Therapy in Pediatric and Congenital Heart Disease

BackgroundEvidence for the efficacy of cardiac resynchronization therapy (CRT) in pediatric and congenital heart disease (CHD) has been limited to surrogate outcomes. ObjectivesThis study aimed to assess the impact of CRT upon the risk of transplantation or death in a retrospective, high-risk, controlled cohort at 5 quaternary referral centers. MethodsBoth CRT patients and control patients were <21 years of age or had CHD; had systemic ventricular ejection fraction <45%; symptomatic heart failure; and significant electrical dyssynchrony (QRS duration z score >3 or single-site ventricular pacing >40%) at enrollment. Patients with CRT were matched with control patients via 1:1 propensity score matching. CRT patients were enrolled at CRT implantation; control patients were enrolled at the outpatient clinical encounter where inclusion criteria were first met. The primary endpoint was transplantation or death. ResultsIn total, 324 control patients and 167 CRT recipients were identified. Mean follow-up was 4.2 ± 3.7 years. Upon propensity score matching, 139 closely matched pairs were identified (20 baseline indices). Of the 139 matched pairs, 52 (37.0%) control patients and 31 (22.0%) CRT recipients reached the primary endpoint. On both unadjusted and multivariable Cox regression analysis, the risk reduction associated with CRT for the primary endpoint was significant (HR: 0.40; 95% CI: 0.25-0.64; P < 0.001; and HR: 0.44; 95% CI: 0.28-0.71; P = 0.001, respectively). On longitudinal assessment, the CRT group had significantly improved systemic ventricular ejection fraction (P < 0.001) and shorter QRS duration (P = 0.015), sustained to 5 years. ConclusionsIn pediatric and CHD patients with symptomatic systolic heart failure and electrical dyssynchrony, CRT was associated with improved heart transplantation-free survival.

Systemic Atrioventricular Valve Surgery in Patients With Congenitally Corrected Transposition of the Great Vessels.

Limited data exist regarding the long-term outcomes of systemic atrioventricular valve (SAVV) intervention (morphologic tricuspid valve) in congenitally corrected transposition (ccTGA). The goal of this study was to evaluate the mid- and long-term outcomes of SAVV surgery in ccTGA. We performed a retrospective review of 108 ccTGA patients undergoing SAVV surgery from 1979 to 2022. The primary outcome was a composite endpoint of mortality, cardiac transplantation, or ventricular assist device implantation. The secondary outcome was long-term systemic right ventricular ejection fraction (SVEF). Cox proportional hazard and linear regression models were used to analyze survival and late SVEF data. The median age at surgery was 39.5 years (Q1-Q3: 28.8-51.0 years), and the median preoperative SVEF was 39% (Q1-Q3: 33.2%-45.0%). Intrinsic valve abnormality was the most common mechanism of SAVV regurgitation (76.9%). There was 1 early postoperative mortality (0.9%). Postoperative complete heart block occurred in 20 patients (18.5%). The actuarial 5-, 10-, and 20-year freedom from death or transplantation was 92.4%, 79.1%, and 62.9%. The 10- and 20-year freedom from valve reoperation was 100% and 93% for mechanical prosthesis compared with 56.6% and 15.7% for bioprosthesis (P< 0.0001). Predictors of postoperative mortality were age at operation (P=0.01) and preoperative SVEF (P=0.04). Preoperative SVEF (P< 0.001), complex ccTGA (P=0.02), severe SAVV regurgitation (P=0.04), and preoperative creatinine (P=0.003) were predictors of late postoperative SVEF. SAVV surgery remains a valuable option for the treatment of patients with ccTGA, with low early mortality and satisfactory long-term outcomes, particularly in those with SVEF≥40%. Timely referral and accurate patient selection are the keys to better long-term outcomes.

Impact of heart failure on reoperation in adult congenital heart disease: An innovative machine learning model

ObjectivesThe study objectives were to evaluate the association between preoperative heart failure and reoperative cardiac surgical outcomes in adult congenital heart disease and to develop a risk model for postoperative morbidity/mortality. MethodsSingle-institution retrospective cohort study of adult patients with congenital heart disease undergoing reoperative cardiac surgery between January 1, 2010, and March 30, 2022. Heart failure defined clinically as preoperative diuretic use and either New York Heart Association Class II to IV or systemic ventricular ejection fraction less than 40%. Composite outcome included operative mortality, mechanical circulatory support, dialysis, unplanned noncardiac reoperation, persistent neurologic deficit, and cardiac arrest. Multivariable logistic regression and machine learning analysis using gradient boosting technology were performed. Shapley statistics determined feature influence, or impact, on model output. ResultsPreoperative heart failure was present in 376 of 1011 patients (37%); those patients had longer postoperative length of stay (6 [5-8] vs 5 [4-7] days, P < .001), increased postoperative mechanical circulatory support (21/376 [6%] vs 16/635 [3%], P = .015), and decreased long-term survival (84% [80%-89%] vs 90% [86%-93%]) at 10 years (P = .002). A 7-feature machine learning risk model for the composite outcome achieved higher area under the curve (0.76) than logistic regression, and ejection fraction was most influential (highest mean |Shapley value|). Additional risk factors for the composite outcome included age, number of prior cardiopulmonary bypass operations, urgent/emergency procedure, and functionally univentricular physiology. ConclusionsHeart failure is common among adult patients with congenital heart disease undergoing cardiac reoperation and associated with longer length of stay, increased postoperative mechanical circulatory support, and decreased long-term survival. Machine learning yields a novel 7-feature risk model for postoperative morbidity/mortality, in which ejection fraction was the most influential.

Improved Surgical Outcomes in Adults with Congenital Heart Disease and Decompensated Heart Failure: The Role of Perioperative Medical Optimization.

BACKGROUND Decompensated heart failure (HF) is recognized as a significant prognostic factor for unfavorable outcomes in both the general population and adults with congenital heart diseases (ACHD). Among ACHD patients, those with advanced heart failure may be candidates for heart transplantation. However, in ACHD patients requiring heart surgery, even with reduced ejection fraction, the administration of appropriate medications can result in improved circulatory parameters, functional class, and surgical outcomes. CASE REPORT We present 5 patients who exhibited indications for open-heart surgery, advanced heart failure (HF) accompanied by congestion, and significant physical activity limitations or symptoms at rest (NYHA class III-IV). Among the patients, 40% were male, with a mean age of 47.4 years (ranging from 32 to 62 years). Three patients displayed reduced systemic ventricular ejection fraction, while 4 patients experienced arrhythmia. Congenital heart diseases (CHD) observed in the patients included repaired double-outlet right ventricle, congenitally corrected transposition of the great arteries, repaired tetralogy of Fallot, partial anomalous right pulmonary venous return, and atrial septal defect. Comprehensive heart failure medications were administered, including an angiotensin receptor neprilysin inhibitor, levosimendan, beta-blockers, phosphocreatine, and diuretics. The preoperative period ranged from 8 days to 2 months. Notably, significant clinical and hemodynamic improvements were observed in all cases, and all open-heart surgeries were successfully completed. CONCLUSIONS Advanced and decompensated HF has a high impact on surgical outcomes. Preoperative care with prescribed medical management for ACHD patients is possible and provides good early and mid-term postoperative results.

Pacemaker lead insertion sites contribute to abnormalities of myocardial function and histopathology

Ventricular pacing can cause myocardial dysfunction, but how lead anchoring to the myocardium affects function has not been studied. The purpose of this study was to evaluate patterns of regional and global ventricular function in patients with a ventricular lead using cine cardiac computed tomography (CCT) and histology. This was a single-center retrospective study with 2 groups of patients with a ventricular lead: (1) those who underwent cine CCT from September 2020 to June 2021 and (2) those whose cardiac specimen was analyzed histologically. Regional wall motion abnormalities on CCT were assessed in relation to lead characteristics. For the CCT group, 122 ventricular lead insertion sites were analyzed in 43 patients (47% female; median age 19 years; range 3-57 years). Regional wall motion abnormalities were present at 51 of 122 lead insertion sites (42%) in 23 of 43 patients (53%). The prevalence of a lead insertion-associated regional wall motion abnormality was higher with active pacing (55% vs 18%; P < .001). Patients with lead insertion-associated regional wall motion abnormalities had a lower systemic ventricular ejection fraction (median 38% vs 53%; P < .001) than did those without regional wall motion abnormalities. For the histology group, 3 patients with 10 epicardial lead insertion sites were studied. Myocardial compression, fibrosis, and calcifications were commonly present directly under active leads. Lead insertion site-associated regional wall motion abnormalities are common and associated with systemic ventricular dysfunction. Histopathological alterations including myocardial compression, fibrosis, and calcifications beneath active leads may explain this finding.

Right ventricular cardiac resynchronization therapy in patients with right ventricular conduction delay and heart failure

Right ventricular cardiac resynchronization therapy in patients with right ventricular conduction delay and heart failure

Abstract 11015: Pacemaker Lead Insertion Sites Contribute to Regional and Global Ventricular Dysfunction

Introduction: Ventricular pacing can cause dysfunction but how the lead is anchored to the myocardium has not been studied. We evaluated patterns of regional and global ventricular function in patients with a ventricular pacemaker or defibrillator using cine cardiac CT (CCT). Methods: This was a single center retrospective study of patients with a ventricular pacemaker or defibrillator who underwent a cine CCT from September 2020 to June 2021. Regional wall motion abnormalities (RWMAs) were assessed in relation to lead insertion site (sew-on vs screw-in) and pacing activity (active vs. inactive). Results: A total of 122 ventricular lead insertion sites were analyzed in 43 patients (53% male, median age 20 years, range 3-57 years). Thirteen patients (30%) had palliated single ventricle physiology, twenty-nine patients had congenital heart disease with biventricular circulation (67%), and one patient had a structurally normal heart (2%). RWMAs were present in 51/122 (42%) lead insertion sites among 23/43 (53%) patients. The prevalence of a lead insertion site associated RWMA was higher with active pacing (54% vs 19%; p &lt; 0.001) and highest for active epicardial sew-on sites ( Figure 1 ). RWMAs were not associated with position of the insertion site relative to the chest wall (p = 0.85) or the distance between the two heads of a bipolar lead (p = 0.23). Patients with lead insertion site associated RWMAs had a lower systemic ventricular ejection fraction (mean of 37.5% vs 52.5%, p &lt; 0.001) compared to those without a RWMA. Conclusions: Pacemaker lead insertion site RWMAs are common and associated with systemic ventricular dysfunction. The mechanism and clinical significance of this regional and global dysfunction warrant further study.

Catheter ablation for atrial fibrillation in adult congenital heart disease: an international registry study

Abstract Background Life expectancies for patients with congenital heart disease (CHD) have dramatically increased in recent years, accompanied by a rise in atrial fibrillation (AF) prevalence. Data on AF ablation strategy and outcomes are limited in CHD. Purpose We aimed to investigate the characteristics of CHD patients presenting for AF ablation and their outcomes. Methods A multicenter, retrospective analysis was performed of CHD patients undergoing AF ablation between 2004 and 2020 at 13 participating centers. The severity of CHD was classified using the 2014 PACES/HRS guidelines. Clinical data were collected including ablation strategy and follow up. One-year procedural success was defined as freedom from AF in the absence of antiarrhythmic drugs (AADs, complete) or including previously failed AADs (partial). Results Of 240 patients, 127 (53.4%) had persistent AF, 62.5% were male, and mean age was 55.2±0.9 years. CHD complexity categories included 147 (61.3%) simple, 69 (28.8%) intermediate and 25 (10.4%) severe. The most common CHD type was atrial septal defect (n=78). More complex CHD conditions included transposition of the great arteries (n=14), anomalous pulmonary veins (n=13), tetralogy of Fallot (n=8), cor triatriatum (n=7), single ventricle physiology (n=2), among others. The majority (71.3%) of patients had AF despite at least one AAD. 46 patients (22.1%) had a reduced systemic ventricular ejection fraction &amp;lt;50%, and the mean left atrial diameter was 44.1±0.7 mm. PV isolation (PVI) was performed in 227 patients (94.6%); additional ablation strategies included left atrial linear ablations (25.4%), CFAE (19.2%), and cavotricuspid isthmus ablation (40.8). One-year complete and partial success rates were 45.0% and 20.5%, respectively, with no significant difference in the rate of complete success between complexity groups. Overall, 38 patients (15.8%) required more than one ablation procedure. There were 3 (1.3%) major and 13 (5.4%) minor procedural complications. Conclusion AF ablation in this complex population was safe and resulted in AF control in the majority of patients. Future work should address the most appropriate ablation targets in the challenging population. Funding Acknowledgement Type of funding sources: None.

Catheter Ablation for Atrial Fibrillation in Adult Congenital Heart Disease: An International Multicenter Registry Study.

Data on atrial fibrillation (AF) ablation and outcomes are limited in patients with congenital heart disease (CHD). We aimed to investigate the characteristics of patients with CHD presenting for AF ablation and their outcomes. A multicenter, retrospective analysis was performed of patients with CHD undergoing AF ablation between 2004 and 2020 at 13 participating centers. The severity of CHD was classified using 2014 Pediatric and Congenital Electrophysiology Society/Heart Rhythm Society guidelines. Clinical data were collected. One-year complete procedural success was defined as freedom from atrial tachycardia or AF in the absence of antiarrhythmic drugs or including previously failed antiarrhythmic drugs (partial success). Of 240 patients, 127 (53.4%) had persistent AF, 62.5% were male, and mean age was 55.2±13.3 years. CHD complexity categories included 147 (61.3%) simple, 68 (28.3%) intermediate, and 25 (10.4%) severe. The most common CHD type was atrial septal defect (n=78). More complex CHD conditions included transposition of the great arteries (n=14), anomalous pulmonary veins (n=13), tetralogy of Fallot (n=8), cor triatriatum (n=7), single ventricle physiology (n=2), among others. The majority (71.3%) of patients had trialed at least one antiarrhythmic drug. Forty-six patients (22.1%) had reduced systemic ventricular ejection fraction <50%, and mean left atrial diameter was 44.1±8.2 mm. Pulmonary vein isolation was performed in 227 patients (94.6%); additional ablation included left atrial linear ablations (40%), complex fractionated atrial electrogram (19.2%), and cavotricuspid isthmus ablation (40.8%). One-year complete and partial success rates were 45.0% and 20.5%, respectively, with no significant difference in the rate of complete success between complexity groups. Overall, 38 patients (15.8%) required more than one ablation procedure. There were 3 (1.3%) major and 13 (5.4%) minor procedural complications. AF ablation in CHD was safe and resulted in AF control in a majority of patients, regardless of complexity. Future work should address the most appropriate ablation targets in this challenging population.

Anesthetic Management in Parturients With Fontan Physiology

Anesthetic Management in Parturients With Fontan Physiology

Spanish registry of cardiac resynchronization therapy in adults with congenital heart disease (RETRACCA): clinical outcomes at one year follow-up

Abstract Funding Acknowledgements Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Spanish Society of Cardiology Background There are limited data about the impact of cardiac resynchronization therapy (CRT) in adults with congenital heart disease (CHD) with conflicting results in patients (P) with systemic right ventricle (SRV). Objectives We analyzed the one-year outcomes of adults with CHD implanted with CRT and the impact of CRT in P with SRV as compared with systemic left ventricle (SLV). Methods Data were analyzed from the Spanish Registry of CRT in adults with CHD (RETRACCA). This is an observational, ambispective (66 % retrospective), multicenter Registry, including 74 adults with CHD implanted with a CRT device from 6 Spanish centers from 2007 to 2021. Ventricular function was assessed by echocardiography and graded on a four-point ordinal scale. Response to CRT was defined as an improvement in NYHA functional class and/or systemic ventricular ejection fraction by at least one category. In addition to changes in functional class and ventricular function, other outcomes analyzed included mortality, heart transplantation and CRT-related complications. Results 68 P completed one year of follow-up. Table 1 shows the baseline characteristics of the P prior to CRT. The two main indications for CRT were depressed systolic function of the systemic ventricle with more than 40 % ventricular pacing (69 %) and severely depressed systolic function of systemic ventricle, NYHA FC II-IV and QRS &amp;gt; 150 (18 %). Sixty percent of P received a CRT-ICD, whereas 40 % received a CRT-pacemaker. The approach for CRT system implantation was transvenous in 70 % of the patients, mixed in 21 % and fully epicardial in 9 %. Overall, 43 out of 68 patients (75 %) responded to CRT either by improvement of NYHA FC (56 %) and/or systemic ventricular function (41 %). Compared with baseline, CRT was associated with significant improvement in NYHA FC (p&amp;lt;0.001; figure 1), QRS duration (169±31 vs 150±29; p=0.005), and systemic ventricle systolic function (severe or moderate 80 % vs 58 %; p=0.04). Percentage of responders was similar among patients with a SLV (73 %) and with SRV (81 %; p=0.51). Only pre-implant NYHA FC III-IV was predictive of a positive response in the univariate analysis (OR 3.82; 95 % CI 1.03-14.18). Seven complications occurred at implant (10 %). During the follow-up, there were 8 complications in 7 P: 2 P had heart transplantation, 3 P required hospital admission for decompensation of heart failure, 2 P presented pocket infections and 1 lead malfunction. No differences in complication rates were observed between the systemic LV and systemic RV subgroups. Conclusions In this series, at one-year follow-up, CRT resulted in improvement in functional class and/or systemic ventricular function in 75 % of P with adult CHD. The percentages of responders and complications were similar among patients with SLV or SRV.

Cardiac resynchronization therapy in patients with congenital heart disease and systemic right ventricle.

Although patients with systemic right ventricle (SRV) represent a significant proportion of patients with congenital heart disease (CHD) implanted with cardiac resynchronization therapy (CRT), there are limited and conflicting data in this specific patient group. We aimed to analyze outcomes of patients with SRV implanted with a CRT device. Data were analyzed from an observational, retrospective, multicenter cohort study including all patients with CHD implanted with a CRT device from 6 French centers from 2004 to 2020. Response to CRT was defined as an increase in systemic ventricular ejection fraction of ≥10% and/or an improvement in New York Heart Association functional class by at least 1 grade. A total of 85 patients with CHD were enrolled (mean age 39.8 ± 20.0 years; 55 [64.7%] males; 25 defibrillators [29.4%]), including 31 patients with SRV (36.5%) (mean age 43.9 ± 19.8 years; 16 [51.6%] males). The mean change in QRS duration after implantation was similar as compared with patients with systemic left ventricle (-46 ± 26 ms vs -35 ± 32 ms; P = .16). During a mean follow-up of 5.1 ± 3.5 years, late complications included 2 lead dysfunctions (6.5%), 3 CRT-related infections (9.7%), and 1 inappropriate implantable cardioverter-defibrillator shock (3.2%). The proportion of CRT responders at 6, 12, and 24 months were 82.6%, 80.0%, and 77.8% in patients with SRV vs 66.7%, 64.3%, and 69.6% in patients with systemic left ventricle (P = NS). In this multicenter cohort, one-third of patients with CHD implanted with a CRT device had SRV. CRT in patients with SRV was associated with a high rate of responders, comparable to that of patients with systemic left ventricle.

Angiography-guided mid/high septal implantation of ventricular leads in patients with congenital heart disease.

BackgroundConduction system pacing prevents pacing‐induced cardiomyopathy, but it can be challenging to perform in patients with congenital heart disease (CHD), and mid/high septal lead implantation is an alternative. This study aimed to assess intraprocedural angiography's utility as a guide for mid/high‐septal lead implantation in CHD patients.MethodsThe study subjects were CHD patients with Class I/IIa indications for permanent pacemaker implantation. To guide septal lead implantation, we performed an intraprocedural right ventricular angiogram in anteroposterior, 40° left anterior oblique, and 30° right anterior oblique. The primary endpoint was the lead tip in the mid/high septum on computed tomography (CT). The secondary endpoints were complications and systemic ventricular function on follow‐up.ResultsFrom January 2008 to December 2018, we enrolled 27 patients (mean age: 30 ± 20 years; M:F 17:10) with CHD (unoperated: 20, operated: 7). The mean paced QRS duration was 131.7 ± 5.8 ms, and CT done in 22/27 patients confirmed the lead tip in the mid‐septum in 16, high septum in 5, and apical septum in 1 patient. There were no procedural complications, and during a mean follow‐up of 58 ± 35.2 months, there was no significant change in the systemic ventricular ejection fraction (56.4 ± 8.3% vs 53.9 + 5.9%, P = .08). Two patients with Eisenmenger syndrome died because of refractory heart failure.ConclusionsIntraprocedural angiography is safe and useful to guide mid/high‐septal lead implantation in CHD patients. Mid/high septal lead position preserves systemic ventricular function in patients with CHD during medium‐term follow‐up.

Feasibility and outcome of supraventricular tachycardia ablation via a retrograde access using remote magnetic navigation in patients with physiologically univentricular hearts after Damus-Kaye-Stansel surgery

AimsSupraventricular tachycardia (SVT) is one of the most frequent complications in adult patients with congenital heart disease (CHD). Limited experience exists for successful ablation in complex CHD patients with prior Damus-Kaye-Stansel (DKS) surgery. MethodsAll patient files of a single-center were screened retrospectively to identify CHD patients with prior DKS surgery throughout a time-interval from 1998 to 2020. These were analysed with focus on SVT, treatment and outcome of catheter ablation (CA). ResultsOverall, 13 patients with prior DKS surgery were identified (median age 22.0 years (IQR 20.5–25.5 years), 76.9% male, 92.3% NYHA Class I, median systemic ventricular ejection fraction 58.5% (IQR 53.0–65.5), 69.2% extra cardiac tunnel). Seven patients (53.8%) presented with symptomatic SVT (median cycle length 440 ms (IQR 360–517 ms)). Of these, five patients underwent CA using remote magnetic navigation. The majority of procedures were performed through a retrograde access using three-dimensional image integration. Median procedure time was 180.5 min (IQR 160.8–274.5 min), median fluoroscopy time 1.5 min (IQR 0.5–4.7 min) and radiofrequency time 851 s (265–2005 s). No peri- or postinterventional complications were observed. After a mean of 1.5 procedures, all patients were complaint-free without documentation of tachycardia recurrence throughout a median follow-up of 13 months (IQR 5.8–17.5 months). ConclusionIn complex univentricular situations after Damus-Kaye-Stansel surgery, ablation for supraventricular tachycardia can be achieved safely and successfully. Techniques such as remote magnetic navigation and three-dimensional image integration may aid for successful procedure results.

Treatment of symptomatic atrial tachycardia in complex anatomic conditions: use and outcome of ablation in patients after damus-kaye-stansel surgery

Abstract Funding Acknowledgements Type of funding sources: None. Background Atrial tachycardias (AT) are amongst the most frequent complications in adult patients with congenital heart disease (ACHD). Their incidence mainly depends on age and complexity of underlying defect. AT has a marked impact on morbidity and mortality and is associated with sudden cardiac death particularly in complex ACHD patients. The arrhythmic substrate can be variable from re-entry to multifocal resulting in demanding requirements for electrophysiologic ablation procedures (EP) and thus, relatively high rates of recurrence. Methods and results Retrospective analysis of ACHD patients with prior Damus-Kaye-Stansel (DKS) surgery treated at one of the largest specialized tertiary centers for ACHD patients worldwide. Analysis focussed on occurrence of AT, respective treatment and outcome of EP. Over a median observation time of 15 years, 13 patients with prior DKS surgery and complex congenital heart defects of different types but uniformly Fontan circulation, were identified (median age 22.0 years (IQR 20.5 – 25.5), 76.9% male, 92.3% NYHA Class I, median systemic ventricular ejection fraction 58.5%, 69.2% extracardiac tunnel). Seven patients (53.8%) presented with symptomatic AT (median cyclic length 440 ms (IQR 360.0 – 517.5)). Of these, six patients underwent EP. Median procedure time was 180.5 min (IQR 160.8 – 274.5), median fluoroscopy time 1.56 min (IQR 0.51 – 4.73) and radiofrequency time 851 sec (265.0 -2005.0). Remote magnetic navigation was used to access ablation site through a retrograde access via the aortic as well as the neo-aortic valve. Three-dimensional image integration aided in mapping and ablation. Throughout the procedure as well as in follow-up visits, no reportable complications were observed. After a mean of 1.5 procedures, all analysed patients stayed complaint-free throughout a median time-interval of 13 months. Conclusion AT are frequent in complex univentricular situations after Damus-Kaye-Stansel surgery (53.8%). Although more than one procedure (mean 1.5) was necessary to achieve satisfactory results even in experienced hands, EP appears to be an appropriate treatment strategy in this young and otherwise healthy patient cohort. According to our analysis, EP is feasible and safe in centers with knowledge of specific anatomic characteristics of congenital heart disease and when the appropriate technical infrastructure is present. Access to ablation site was performed by remote magnetic navigation in the majority of procedures. The floppy catheter was navigated through the neo-aortic as well as through the aortic valve without observable peri- or postinterventional complications. Mapping with three-dimensional image integration facilitated EP and led to a relatively low radiation exposure with the known benefits for the patient as well as for the healthcare professionals involved in the procedure. Abstract Figure.

Long-Term Outcome of Patients With Congenital Heart Disease Undergoing Cardiac Resynchronization Therapy.

BackgroundCardiac resynchronization therapy (CRT) is rarely used in patients with congenital heart disease, and reported follow‐up is short. We sought to evaluate long‐term impact of CRT in a single‐center cohort of patients with congenital heart disease.Methods and ResultsThirty‐two consecutive patients with structural congenital heart disease (N=30) or congenital atrioventricular block (N=2), aged median of 12.9 years at CRT with pacing capability device implantation, were followed up for a median of 8.7 years. CRT response was defined as an increase in systemic ventricular ejection fraction or fractional area of change by >10 units and improved or unchanged New York Heart Association class. Freedom from cardiovascular death, heart failure hospitalization, or new transplant listing was 92.6% and 83.2% at 5 and 10 years, respectively. Freedom from CRT complications, leading to surgical system revision (elective generator replacement excluded) or therapy termination, was 82.7% and 72.2% at 5 and 10 years, respectively. The overall probability of an uneventful therapy continuation was 76.3% and 58.8% at 5 and 10 years, respectively. There was a significant increase in ejection fraction/fractional area of change (P<0.001) mainly attributable to patients with systemic left ventricle (P=0.002) and decrease in systemic ventricular end‐diastolic dimensions (P<0.05) after CRT. New York Heart Association functional class improved from a median 2.0 to 1.25 (P<0.001). Long‐term CRT response was present in 54.8% of patients at last follow‐up and was more frequent in systemic left ventricle (P<0.001).ConclusionsCRT in patients with congenital heart disease was associated with acceptable survival and long‐term response in ≈50% of patients. Probability of an uneventful CRT continuation was modest.

Abstract 14549: Outcome of Radiofrequency Catheter Ablation of Cavo-tricuspid Isthmus Dependent Flutter in Patients With Adult Congenital Heart Disease

Introduction: Both typical cavo-tricuspid isthmus (CTI) dependent (or its equivalent) and atypical atrial flutter (AFL) are common in adults with congenital heart disease (CHD) either due to its inherent cardiac anatomy or as a result of prior surgical procedures. Radiofrequency catheter ablation is routinely used as a therapeutic option. Hypothesis: Patients with CHD will have an improvement in systemic ventricular ejection fraction and a decreased incidence of atrial arrhythmias after ablation. Methods: A retrospective study was conducted on 99 patients with CHD who underwent ablation for clinical AFL at a single institution between 2010 and 2019. Of these patients, 62 had CTI-AFL. The 2 patients with unspecified CHD were excluded. CHD was divided into 3 categories according to the American College of Cardiology Task Force 1 of the 32nd Bethesda Conference - simple, moderate severity, and great complexity lesions. Outcomes of ablation at 1 year follow up were assessed. Results: Typical counterclockwise CTI-AFL was seen in 50 patients. The mean age was 49.1±13.6 years old with 29 (48.3%) females. Of the 60 patients, 35% (n=21) had simple lesions, 51.7% (n=31) had moderate lesions, and 13.3% (n=8) had great complexity lesions. The mean left or systemic ventricular ejection fraction (EF) significantly improved on follow-up echo among patients with simple CHD (49.1±13.1 to 56.5±4.7%, p=0.018) and moderate CHD (49.5±14.9 to 54±8.5%, p=0.02), but did not change among patients with great complexity CHD (52.5±6 to 52.5±9.25%, p=0.9). There was a significantly increased incidence of atrial fibrillation (9.5% vs. 16.1% vs. 50%, p=0.04) post ablation among simple, moderate, and great complexity lesions, respectively, but no significant difference in the recurrence rate of atrial flutter (p=0.3). Conclusions: Patients who underwent CTI-AFL ablation showed an improvement in EF in patients with simple and moderate CHD. There was also a significantly increased incidence in the development of atrial fibrillation post ablation in CHD with great complexity compared to simple or moderate CHD.

The Fate of Congenitally Corrected Transposition of the Great Arteries Unoperated Before Adulthood

The outcomes, therapeutic strategies, and risk factors of congenital corrected transposition of great arteries (ccTGA) unoperated before adulthood are unclear. From October 2009 to January 2018, 117 adult ccTGA patients, classified into ccTGA with intact ventricular septum, ventricular septum defect, and pulmonary valve or subpulmonary outflow tract stenosis (PS) groups, were reviewed. Statistical analysis was performed with SPSS 19.0 (IBM, Armonk, NY). At the first visit, no patients suffered operation. The PS group had the least systemic atrioventricular valve regurgitation and the greatest systemic ventricular ejection fraction. All 49 patients underwent surgery. From the first visit to last follow-up, systemic ventricular ejection fraction of unoperated ccTGA decreased significantly. In the intact ventricular septum group, patients receiving systemic atrioventricular valve replacement/valvuloplasty had a significantly increased systemic ventricular ejection fraction and statistically more freedom from death and transplant than unoperated. In the ventricular septum defect group the late systemic ventricular ejection fraction of operated patients was not statistically different from their basic data at first visit. In the PS group patients receiving physiologic repair had significantly decreased systemic ventricular ejection fractions. Severe systemic atrioventricular valve regurgitation, physiologic repair, and systemic ventricular dysfunction (ejection fraction <40%) were risk factors for mortality, transplant, and congestive heart failure. PS protects against systemic atrioventricular valve regurgitation and ventricular dysfunction. Systemic atrioventricular valve replacement/valvuloplasty improved systemic ventricular function for ccTGA with an intact ventricular septum. Physiologic repair was not ideal for ccTGA with PS. Severe systemic atrioventricular valve regurgitation and systemic ventricular dysfunction were associated with suboptimal outcomes.

Highlights From the Circulation Family of Journals

Highlights From the <i>Circulation</i> Family of Journals