Association of Health Status Metrics with Clinical Outcomes in Patients with Adult Congenital Heart Disease and Atrial Arrhythmias.

The prognostic value of health status metrics in patients with adult congenital heart disease (ACHD) and atrial arrhythmias is unclear. In this retrospective cohort study of an ongoing national, multicenter registry (PROTECT-AR, NCT03854149), ACHD patients with atrial arrhythmias on apixaban are included. At baseline, health metrics were assessed using the physical component summary (PCS), the mental component summary (MCS) of the Short-Form-36 (SF-36) Health Survey, and the modified European Heart Rhythm Association (mEHRA) score. Patients were divided into groups according to their SF-36 PCS and MCS scores, using the normalized population mean of 50 on the PCS and MCS as a threshold. The primary outcome was the composite of mortality from any cause, major thromboembolic events, major/clinically relevant non-major bleedings, or hospitalizations. Multivariable Cox-regression analyses using clinically relevant parameters (age greater than 60 years, anatomic complexity, ejection fraction of the systemic ventricle, and CHA2DS2-VASc and HAS-BLED scores) were performed to examine the association of health metrics with the composite outcome. Over a median follow-up period of 20 months, the composite outcome occurred in 50 of 158 (32%) patients. The risk of the outcome was significantly higher in patients with SF-36 PCS ≤ 50 compared with those with PCS > 50 (adjusted hazard ratio (aHR), 1.98; 95% confidence interval [CI], 1.02−3.84; p = 0.04) after adjusting for possible confounders. The SF-36 MCS ≤ 50 was not associated with the outcome. The mEHRA score was incrementally associated with a higher risk of the composite outcome (aHR = 1.44 per 1 unit increase in score; 95% CI, 1.03−2.00; p = 0.03) in multivariable analysis. In ACHD patients with atrial arrhythmias, the SF-36 PCS ≤ 50 and mEHRA scores predicted an increased risk of adverse events.

The Adult Congenital Heart Disease Anatomic and Physiological Classification: Associations with Clinical Outcomes in Patients with Atrial Arrhythmias.

The implications of the adult congenital heart disease anatomic and physiological classification (AP-ACHD) for risk assessment have not been adequately studied. A retrospective cohort study was conducted using data from an ongoing national, multicentre registry of patients with ACHD and atrial arrhythmias (AA) receiving apixaban (PROTECT-AR study, NCT03854149). At enrollment, patients were stratified according to Anatomic class (AnatC, range I to III) and physiological stage (PhyS, range B to D). A follow-up was conducted between May 2019 and September 2021. The primary outcome was a composite of death from any cause, any major thromboembolic event, major or clinically relevant non-major bleeding, or hospitalization. Cox proportional-hazards regression modeling was used to evaluate the risks for the outcome among AP-ACHD classes. Over a median 20-month follow-up period, 47 of 157 (29.9%) ACHD patients with AA experienced the composite outcome. Adjusted hazard ratios (aHR) with 95% confidence intervals (CI) for the outcome in PhyS C and PhyS D were 1.79 (95% CI 0.69 to 4.67) and 8.15 (95% CI 1.52 to 43.59), respectively, as compared with PhyS B. The corresponding aHRs in AnatC II and AnatC III were 1.12 (95% CI 0.37 to 3.41) and 1.06 (95% CI 0.24 to 4.63), respectively, as compared with AnatC I. In conclusion, the PhyS component of the AP-ACHD classification was an independent predictor of net adverse clinical events among ACHD patients with AA.

Trends in ablation procedures in Greece over the 2008-2018 period: Results from the Hellenic Cardiology Society Ablation Registry.

OBJECTIVE: In 2008, the radiofrequency ablation (RFA) procedures registry of the Hellenic Society of Cardiology was created. This online database allowed electrophysiologists around the country to input data for all performed ablation procedures. The aim of this study is to provide a thorough report and interpretation of the data submitted to the registry between 2008 and 2018. METHODS: In 2008, a total of 27 centers/medical teams in 24 hospitals were licensed to perform RFA in Greece. By 2018, the number had risen to 31. Each center was tasked with inserting their own data into the registry, which included patient demographics (anonymized), type of procedure and technique, complications, and outcomes. RESULTS: A total of 18587 procedures in 17900 patients were recorded in the period of 2008-2018. By 2018, slightly more than 70% of procedures were performed in 7 high-volume centers (>100 cases/year). The most common procedure since 2014 was atrial fibrillation ablation, followed by atrioventricular nodal reentry tachycardia ablation. Complication rates were low, and success rates remained high, whereas the 6-month relapse rates declined steadily. CONCLUSION: This online RFA registry has proved that ablation procedures in Greece have reached a very high standard, with results and complication rates comparable to European and American standards. Ablation procedures for atrial fibrillation are increasing constantly, with it being the most common intervention over the last 6-year period, although the absolute number of procedures still remains low, compared to other European countries.

Rapid right ventricular pacing for balloon aortic valvuloplasty: expanding its routine use in neonates and infants.

OBJECTIVE: Rapid right ventricular pacing during balloon aortic valvuloplasty is commonly used to achieve balloon stability in children and adults. There is no consensus for the use of the technique in neonates and infants. We sought to review our institutional experience with rapid right ventricular pacing-assisted balloon aortic valvuloplasty across all age groups and evaluate the safety and effectiveness of the technique in the sub-group of neonates and infants <12months. METHODS: Retrospective study between February, 2011 and February, 2020. RESULTS: A total of 37 patients (Group I: 21 neonates/infants <12months and Group II: 16 children 12 months-16 years) were analysed. Catheter-measured left ventricular to aortic gradient reduced from median of 66 mmHg (with a range from 30 to 125 mmHg) to 14 mmHg (with a range from 5 to 44 mmHg) in Group I and 44 mmHg (with a range from 28 to 93 mmHg) to 18 mmHg (with a range from 2 to 65 mmHg) in Group II (p < 0.001). Procedure and fluoroscopy times were identical in the two groups. Balloon:annulus ratio was 0.94 and 0.88 in Groups I and II, respectively. Freedom from reintervention was 100% for Group I at a median time of 3.2 years and 81% at 2.7 years for Group II. Reinterventions in Group II (3/16 pts) were performed predominantly for complex left ventricular outflow tract stenosis. At follow-up echocardiogram, 45% of patients in Group I had no aortic regurgitation, 30% trace-mild, 20% mild-moderate, and 5% moderate aortic regurgitation, whereas in Group II, 50% of patients had no aortic regurgitation, 32% had mild aortic regurgitation, and 18% mild-moderate aortic regurgitation. Unicuspid valves were only encountered in Group 1 (2/21 pts, 10%) and they were predictive of mild-aortic regurgitation during follow-up (p = 0.003). Ventricular fibrillation occurred in three neonates with suspicion of myocardial ischemia on the pre-procedure echocardiogram. All were successfully defibrillated. CONCLUSIONS: Rapid right ventricular pacing can be expanded in neonates and infants to potentially decrease the incidence of aortic regurgitation and reintervention rates, hence avoiding high-risk surgical bail-out procedures for severe aortic regurgitation in the first year of life. Myocardial ischemia may predispose to ventricular dysrhythmias during rapid right ventricular pacing.

Rationale and design of a prospective, observational, multicentre study on the safety and efficacy of apixaban for the prevention of thromboembolism in adults with congenital heart disease and atrial arrhythmias: the PROTECT-AR study.

INTRODUCTION: The risk for stroke in adults with congenital heart disease (ACHD) is increased, especially in the setting of commonly ensuing atrial arrhythmias (AA), namely atrial fibrillation, atrial flutter or intra-atrial re-entrant tachycardia. Data are limited regarding treatment with non-vitamin K oral anticoagulants in long-term studies involving patients with ACHD and AA. METHODS AND ANALYSIS: PReventiOn of ThromboEmbolism in Adults with Congenital HearΤ disease and Atrial aRrhythmias is a prospective, multicenter, single-arm, non-interventional cohort study designed to investigate the safety and efficacy of apixaban for the prevention of thromboembolism in ACHD with AA in a 'real-world' setting. Eligible patients will be evaluated by the means of available registries and clinical counter. The study aims to accumulate approximately 500 patient-years of exposure to apixaban as part of routine care. Enrolment will take place at four ACHD centres in Greece. The first patient was enrolled in July 2019. The primary efficacy endpoint is a composite of stroke, systemic or pulmonary embolism and intracardiac thrombosis. The primary safety endpoint is major bleeding, according to the International Society on Thrombosis and Haemostasis bleeding criteria. ETHICS AND DISSEMINATION: The study protocol has been approved by the institutional review board/independent ethics committee at each site prior to study commencement. All patients will provide written informed consent. Results will be disseminated at scientific meetings and published in peer-reviewed journals. TRIAL REGISTRATION NUMBER: NCT03854149; Pre-results.

Levoatriocardinal Vein: A Rarely Recognized Cause of Recurrent Cardiac and Cerebral Thromboembolic Events.

Levoatriocardinal vein (LACV) is a rare congenital anomaly of the systemic veins. We describe the case of a 43-year-old patient who presented with a transient ischemic attack episode, desaturation, and ventricular arrhythmias. Transesophageal echocardiography diagnosed a patent foramen ovale, and magnetic resonance imaging revealed 2 small myocardial infarcts, despite normal coronaries on subsequent angiography. Echocardiography and cardiac computed tomography revealed an LACV with bidirectional flow. The patient underwent percutaneous LACV and patent foramen ovale closure, with immediate saturation increase after occlusion of the LACV. To the best of our knowledge, this is the first time an LACV is identified as the cause of recurrent thromboembolic events.

Performing modified fontan before six years of age leads to superior long-term exercise capacity.

We investigated the influence of age at Fontan completion on the exercise capacity of patients who underwent a Fontan operation. Our study demonstrated that age at the time of the Fontan operation significantly affects the peak oxygen consumption at mid-term follow up and that exercise capacity is superior in patients who have undergone Fontan completion at an earlier age. These findings provide support for recommendations to perform Fontan completion procedures relatively early.

Coronary Artery Thrombosis in a Neonate With Critical Aortic Stenosis.

We describe a case of right coronary artery occlusion in a neonate with critical aortic stenosis. We hypothesize that the thrombus was either formed in situ resulting from low cardiac output or secondary to an atrial arrhythmia in the presence of severe left ventricular diastolic dysfunction that may have occurred during air-transfer to our facility. We conclude that attention should be given to the coronary arteries in low-flow critical aortic stenosis, particularly in neonates and infants whose stroke volume is extremely low.

Atrioventricular Nodal Reentrant Tachycardia in Patients With Congenital Heart Disease: Outcome After Catheter Ablation.

BACKGROUND: The relationship of atrioventricular nodal reentrant tachycardia to congenital heart disease (CHD) and the outcome of catheter ablation in this population have not been studied adequately. METHODS AND RESULTS: A multicenter retrospective study was performed on patients with CHD who had atrioventricular nodal reentrant tachycardia and were treated with catheter ablation. There were 109 patients (61 women), aged 22.1±13.4 years. The majority, 86 of 109 (79%), had CHD resulting in right heart pressure or volume overload. Patients were divided into 2 groups: group A (n=51) with complex CHD and group B (n=58) with simple CHD. There were no significant differences between groups in patients' growth parameters, use of 3-dimensional imaging, and type of ablation (radiofrequency versus cryoablation). Procedure times (251±117 versus 174±94 minutes; P=0.0006) and fluoroscopy times (median 20.8 versus 16.6 minutes; P=0.037) were longer in group A versus group B. There were significant differences between groups in the acute success of ablation (82% versus 97%; P=0.04), risk of atrioventricular block (14 versus 0%; P=0.004), and need for chronic pacing (10% versus 0%; P=0.008). There was no permanent atrioventricular block in patients who underwent cryoablation. After 3.2±2.7 years of follow-up, long-term success was 86% in group A and 100% in group B (P=0.004). CONCLUSIONS: Atrioventricular nodal reentrant tachycardia can complicate the course of patients with CHD. This study demonstrates that the outcome of catheter ablation is favorable in patients with simple CHD. Patients with complex CHD have increased risk of procedural failure and atrioventricular block.

Cryoablation of atrioventricular nodal reentrant tachycardia in children and adolescents: improved long-term outcomes with increasing experience.

BACKGROUND: Cryoablation is increasingly used for the treatment of atrioventricular nodal reentrant tachycardia (AVNRT). There are limited data regarding its efficacy and late outcomes in the pediatric population. METHODS: We reviewed the clinical records of 70 consecutive pediatric and adolescent patients (mean age: 12.2 ± 3, range: 5-19 years) with AVNRT, with or without additional structural heart disease, who underwent catheter ablation with the intention to use cryoablation as the primary modality between September 2004 and July 2011. RESULTS: The acute success rate was 97.14%. No long-term complications occurred. Patients were followed up for an average time of 46 ± 22.5 months (range 5-90). The recurrence rate was 16%, decreasing from 28% in the first 25 cases to 8.9% in the last 45 cases (p<0.05). Wenckebach point prolongation during cryoapplications was associated with a lower recurrence rate (p<0.05). No other risk factors for recurrence were identified, including persistence of slow pathway conduction. Patients with congenital heart disease had a similar success rate to patients with a structurally normal heart. CONCLUSIONS: When applied to pediatric patients, cryoablation has an excellent success and safety profile, which can improve with increasing experience. Transient Wenckebach point prolongation during cryo-applications may be associated with improved late outcomes.

Preservation of right ventricular structure and function following transatrial-transpulmonary repair of tetralogy of Fallot.

OBJECTIVES: Management strategy of patients with tetralogy of Fallot (TOF)-including timing, as well as surgical technique-remains a controversial topic. We sought to analyse both early and late results of our consistent policy of non-neonatal transatrial/transpulmonary (TA/TP) repair of TOF over almost 14 years, in order to assess preservation or possible deterioration of right ventricular (RV) function. METHODS: All 245 consecutive patients with TOF, referred to our group for repair between September 1997 and December 2010, have been prospectively followed up. Their clinical and echocardiographic data were retrospectively analysed. All underwent complete TA/TP repair at a median age of 1.6 years (range 0.2 to 55.6 years). RESULTS: Follow-up (median 8.5 years, range 0.5 to 14.6 years) was 100% completed. There was no operative death. There were three early re-operations: one for residual right ventricular outflow tract obstruction (RVOTO), one for intractable arrhythmias and one for remote second ventricular septal defect (VSD). There were three non-cardiac-related late deaths. Actuarial survival at 14 years was 98.8% (242/245). Twenty five patients required late re-operation, including 23 patients (9.4%, 23/245) who had pulmonary valve replacement (PVR). All other patients remained asymptomatic at follow-up. Mean residual right ventricular outflow tract (RVOT) pressure gradients have remained stable (6.8 ± 6.6 mmHg, 95% CI 5.9-7.6, early postoperatively, versus 7.6 ± 7.5 mmHg, 95% CI 6.6-8.6 at follow-up; P = 0.015). The mean qualitative grade of pulmonary valve insufficiency (PVI) increased (from 1.4 ± 0.9, 95% CI 1.3-1.5 at discharge to 2.1 ± 1.2, 95% CI 2-2.3 at follow-up; P < 0.001). Similarly, mean qualitative grade of tricuspid valve insufficiency (TVI) also increased (from 1 ± 0.7, 95% CI 0.9-1 at discharge to 1.5 ± 0.8, 95% CI 1.4-1.6 at follow-up; P < 0.001). However, RV function has remained normal in most patients. No significant arrhythmias have been noted. CONCLUSIONS: Our management strategy of non-neonatal TA/TP repair for all patients with TOF is associated with minimal early and late mortality and morbidity, relatively low re-operation rate, preserved RV function and excellent clinical outcomes at follow-up over almost 14 years. Since many patients demonstrated progressive increase in PVI and TVI, a much longer follow-up is necessary to determine the ultimate rates of late re-operation for pulmonary valve replacement (PVR).

The contegra® valved heterograft conduit for right ventricular outflow tract reconstruction: a reliable solution.

INTRODUCTION: The Contegra® bioprosthetic valved conduit, a glutaraldehyde-preserved valve-containing bovine jugular vein graft (Contegra, Medtronic Inc., Minneapolis MN, USA) introduced for clinical trials in 1998, is used for reconstruction of the right ventricular outflow tract (RVOT), mainly in children. This study evaluates our surgical experience with the Contegra® graft, emphasizing the assessment of conduit durability at mid-term follow up. METHODS: The intermediate results of RVOT reconstruction utilizing the Contegra conduit were retrospectively analyzed in a series of 34 consecutive patients (25 male, 9 female), with a mean age of 10.9 ± 11.2 years (range 0.2-46 years). Included were 14 patients with tetralogy of Fallot (TOF) with pulmonary atresia, 11 with reoperation of previously corrected TOF, 5 with truncus arteriosus, 2 with TOF with absent pulmonary valve, 1 reoperation of previously repaired double outlet right ventricle with pulmonary atresia, and 1 undergoing a Ross procedure. Contegra conduit sizes varied in diameter between 12 and 22 mm (mean 18.3 ± 3.2 mm). RESULTS: There were no hospital deaths. There was one early conduit replacement as a result of recurrent thrombosis. Four patients developed early thrombus formation in a valve cusp with complete resolution following anticoagulation therapy. At mean follow up of 85 months (range 6-136 months) and median follow up of 95 months, one patient required Contegra graft explantation in another institution (indications unknown). Freedom from reoperation for Contegra grafts was 94% at 11.4 years. Mean transpulmonary pressure gradients remained low (9.6 ± 5.3 mmHg postoperative, 19.6 ± 10.6 mmHg at follow up). Although there was a clear trend towards worsening of conduit valve insufficiency, this was neither statistically significant nor considered clinically so. CONCLUSIONS: In our experience of 34 consecutive operations, the Contegra® valved conduit for RVOT reconstruction seems to be a reliable alternative to homograft conduits, with promising mid-term freedom from structural deterioration and reoperation.

Radiofrequency ablation of accessory pathways in children and congenital heart disease patients: impact of a nonfluoroscopic navigation system.

BACKGROUND: We sought to assess the impact of routine use of a nonfluoroscopic navigation system in the procedural aspects of radiofrequency ablation of accessory pathways (APs) in pediatric and congenital heart disease (CHD) patients and the reduction of fluoroscopy in different pathway locations. METHODS: This was a retrospective review of 192 patients, divided in two groups: group A (76 patients, fluoroscopic only ablation) and group B (116 patients, combined use of fluoroscopy and a nonfluoroscopic system (NavX™). Comparison of procedural aspects (procedure time, fluoroscopy time, success, complications, and recurrences) was performed. RESULTS: The two groups were comparable in terms of age, AP location, and presence of CHD. The mean age was 11.34 ± 4.65 years in group A versus 10.91 ± 3.68 years in group B. The procedure duration was significantly shorter in group B than in group A (177.06 ± 62.18 vs 242.45 ± 99.07) (P < 0.001). There was a significant reduction in the fluoroscopy time in group B compared to group A (8.27 ± 8.23 vs 39.77 ± 32.65 minutes) (P < 0.001). The difference between the two groups was statistically significant in all categories of APs. The success rate was 97.4% in group A and 96.6% in group B. There were no complications directly related to the use of the nonfluoroscopic system. There was no difference in the recurrence rate. CONCLUSIONS: The use of a nonfluoroscopic system for catheter navigation resulted in significant reduction of total procedure and fluoroscopy time during catheter ablation of APs in pediatric and CHD patients, regardless of the location of the pathway, without a compromise in safety and efficacy.

The fontan procedure in Greece: early surgical results and excellent mid-term outcome.

INTRODUCTION: The Fontan operation (modified from its original version) affords excellent palliation for many patients with various forms of anatomic or functional single ventricle. The purpose of this study was to evaluate the outcome of our experience with the Fontan procedure in Greece. METHODS: Fifty-eight consecutive patients with single ventricle physiology had a modified Fontan operation between 1997 and 2009. Their records were reviewed retrospectively. Follow up, which included clinical evaluation and echocardiographic functional assessment, was complete. RESULTS: Median age at operation was 5.7 years (range 3 years to 29.4 years); 46.5% had multistage palliation (stage I and II); 79% had prior bidirectional cavopulmonary shunt (stage II) and 8.6% single stage Fontan. Fifty-four patients had an extracardiac conduit total cavopulmonary connection (EC-TCPC) and 4 an intra-atrial lateral tunnel (LT-TCPC). Fenestration was performed in 26 (44.8%) patients. Operative mortality was 0%. One patient required a short period of extracorporeal membrane oxygenator support. The most frequent complication was prolonged pleural effusion. Median duration of pleural effusion was 17 days (range 6-47 days). Median duration of follow up was 5.44 years (range 0.36 to 11.5 years). There were 2 late deaths (overall mortality 3.4%). One patient died from progressive deterioration of ventricular function within 2 years of operation and the other from fulminant endocarditis. Ten subjects have undergone device closure of a persistent fenestration. All 56 surviving patients are in excellent clinical condition (NYHA class I or II). CONCLUSIONS: We have performed the Fontan procedure over a period of 13 years in Greece with excellent mid- and long-term results. Longer follow up will be necessary to assess the possible incidence of late severe complications, some of which may necessitate heart transplantation.

Atresia of the superior vena cava causing cyanosis and increasing head circumference in an infant.

We report a case of congenital atresia of the superior vena cava (SVC) with stenotic anastomoses between systemic and pulmonary veins, resulting in cyanosis and symptoms consistent with SVC syndrome in an infant.