Complex Congenital Heart Disease Patients Research Articles

Combined subcutaneous implantable cardioverter defibrillator and pacemaker devices in complex congenital heart disease: a single-center experienced based study.

Subcutaneous implantable cardioverter defibrillators (S-ICD) are widely accepted therapy in congenital heart disease (CHD) patients at risk of life-threatening ventricular arrhythmias or sudden cardiac death (SCD) when pacing is not required. Occasionally, pacemaker (PM)-dependent CHD patients will subsequently develop an indication for a cardioverter defibrillator. The use of S-ICD in complex CHD patients who have had already PM devices implanted implies some specific considerations, as the safety for these patients in unknown and recommendations among physicians may vary widely. We review the data and studied the indications for S-ICD in complex CHD with previous PM and discuss its usefulness in clinical practice. From a large cohort of 345 patients enrolled in the S-ICD Monaldi care registry, which encompass all the patients implanted in the Monaldi Hospital of Naples, we considered 11 consecutive complex CHD patients (10M/1F aged 40.4 ±18.4 years) who underwent S-ICD implant after a previous PM implant, from February 2015 to October 2022. Mean follow-up was 25.5 ± 22 months. All the patients showed a good compliance to the device system with no complications (infections or skin erosions). In complex CHD with already implanted PM devices, S-ICD implant appears to be a safe alternative to PM upgrading to transvenous ICD system, avoiding abandoned leads or life-threatening lead extraction. However, there are important issues with regard to testing and programming that need to be addressed at the time of implantation.

Accuracy of pulmonary venous flow quantification by 2D phase contrast cardiac magnetic resonance

Abstract Funding Acknowledgements Type of funding sources: None. Background Determination of pulmonary venous flow by cardiac magnetic resonance (CMR) is frequently required in patients with complex congenital heart disease (CHD) lesions. Although new advanced imaging techniques like 4D flow are increasingly available, standard 2D phase contrast (2DPC) sequences remain the most frequently used method for quantification of flow. However, little is known on the accuracy of 2DPC flow measurements in the pulmonary veins. Purpose This study therefore aims to investigate the accuracy of pulmonary venous flow quantification by 2DPC. Methods Patients with CHD underwent a CMR on a 1.5T scanner. 2DPC flow sequences (typical temporal resolution 35–50ms, inplane spatial resolution 1.3–1.8 mm2, VENC 80–100) were acquired in the ascending aorta (AA), main, right and left pulmonary arteries (MPA, RPA, LPA), as well as left (LPV) and right (RPV) pulmonary veins. Pulmonary venous flow was compared to pulmonary arterial flow in all patients and to aortic flow in patients without left to right (L-R) shunt using Bland-Altman plots and Pearson’s correlations. Results Twenty CHD patients (age 27±3, range 7; 53 years, 7 women, tetralogy of Fallot N=7, Ross operation N=1; Fontan N=4, transposition of great arteries N=2, atrial septal defect N=2, Turner syndrome 1, Ebstein N=1, valvulopathies N=2) were included. Table 1 shows absolute flow values of the different vessels. Bland-Altman analyses and correlations (Table 2) showed a good agreement of total pulmonary venous (PV) to flow in the ascending aorta (group without shunt) and the sum of flow of both branch pulmonary arteries. However, agreement of total PV with MPA flow as well as of LPV and RPV with the respective branch pulmonary artery flow was reduced. Conclusions Total PV flow determined by 2DPC shows a good agreement with flow in the ascending aorta and the sum of the branch pulmonary arteries. Flow turbulences due to the pathologies of the right ventricular outflow tract probably contribute to the reduced agreement of total PV and MPA flow. However, the reduced agreement of the flow in left/right pulmonary veins and their respective branch pulmonary arteries underlines the need for newer advanced flow quantification techniques to improve accuracy of pulmonary venous flow measurements.

Combined Heart-Liver vs Isolated Heart Transplantation in Adults With Congenital Heart Disease

BackgroundDespite an increasing number of adults with congenital heart disease undergoing combined heart-liver transplantation (CHLT), there is a paucity of literature analyzing posttransplant outcomes. We analyzed the incidence and outcomes of congenital heart disease patients undergoing CHLT compared with those undergoing isolated heart transplantation (HT). MethodsThis was a retrospective analysis of all adult (≥18 years) congenital heart disease patients undergoing CHLT or HT between 2000 and 2020 in the Organ Procurement and Transplantation Network database. The primary outcome was death at 30 days and 1 year after transplantation. ResultsOf 1214 recipients included for analysis, 92 (8%) underwent CHLT and 1122 (92%) underwent HT. Patients undergoing CHLT and HT were similar in the distribution of age, sex, and serum bilirubin. Upon adjusted analysis with HT as the reference, undergoing CHLT was associated with a similar hazard of 30-day mortality between 2000 and 2017 (hazard ratio [HR], 0.51; 95% CI, 0.12-2.08; P = .35) and 2018 and 2020 (HR, 2.32; 95% CI, 0.88-6.13; P = .09). Similarly, there was no difference in the hazard of 1-year mortality for patients undergoing CHLT between 2000 and 2017 (HR, 0.60; 95% CI, 0.22-1.63; P = .32) and 2018 and 2020 (HR, 1.52; 95% CI, 0.66-3.53; P = .33) compared with HT. ConclusionsThe number of adults undergoing CHLT continues to rise. Given comparable survival outcomes between CHLT and HT, our findings demonstrate the former as a viable option for complex congenital heart disease patients with failing cavopulmonary circulation and associated liver disease. Future studies should delineate factors associated with early hepatic dysfunction to help identify congenital heart disease patients that would benefit from CHLT.

Effects of Sports, Exercise Training, and Physical Activity in Children with Congenital Heart Disease-A Review of the Published Evidence.

Children and adolescents with congenital heart disease (CHD) should be encouraged to adopt a physically active lifestyle, ideally by participating in sports activities at school and sports clubs. Children with complex CHD or other risk factors (for example, pacemakers, cardioverter-defibrillators, channelopathies) may, however, need specific individualized training programs. This review article summarizes the current knowledge regarding the clinical effects of sports and exercise training on CHD and its pathophysiologic mechanisms. An evidence-based approach based on a literature search, using PubMed, Medline, CINHAL, Embase, and the Cochrane Library was conducted, last completed on 30 December 2021. In studies with 3256 CHD patients in total, including 10 randomized controlled trials, 14 prospective interventional trials, 9 observational trials, and 2 surveys, exercise training has been shown to improve exercise capacity and physical activity, motoric skills, muscular function, and quality of life. Sports and exercise training appears to be effective and safe in CHD patients. Despite being cost-efficient, training programs are currently scarcely reimbursed; therefore, support from healthcare institutions, commissioners of healthcare, and research-funding institutions is desirable. There is a strong need to establish specialized rehabilitation programs for complex CHD patients to enhance these patients' access to this treatment intervention. Further studies may be desirable to confirm these data to investigate the impact on risk profiles and to identify the most advantageous training methodology and underlying pathophysiological mechanisms.

Nurses' perceptions and use of near infrared spectroscopy in paediatric cardiac intensive care.

Near-infrared spectroscopy (NIRS) is a non-invasive technology that estimates regional oxygen saturation. Literature demonstrates that NIRS can provide valuable data for clinical staff. However, little research has addressed the nursing care and management of NIRS in the critical care environment. To assess nurses' perception around the use of NIRS and current NIRS practice within paediatric cardiac intensive care unit (PCICUs). A 53-item cross-sectional electronic survey was developed to assess indications for NIRS, critical value thresholds and interventions, barriers to use, policies and procedures, and nursing perceptions. Descriptive statistics were used to summarize and aggregate data. Among the 28 responding sites (63.6% response rate), usage of NIRS was variable and patient-dependent. Most nurses reported using NIRS in patients with unstable physiology such as post-operative single ventricle (n=25, 89.3%) and concern for shock (n=21, 75.0%). Critically low cerebral values varied among respondents from less than 40 (n=3, 10.7%) to less than 60 (n=4, 14.3%), with lower critical values permitted for single ventricle physiology: less than 40 (n=8, 28.6%) to less than 50 (n=6, 21.4%). Reported barriers to using NIRS included skin breakdown (n=9, 32.1%), lack of consistency in decision-making among physicians (n=13, 46.4%), and not using NIRS data when developing a plan of care (n=11, 39.3%). Most (n=24, 85.7%) nurses reported that NIRS provided valuable information and was perceived to be beneficial for patients. NIRS monitoring is a common technology in the care of complex congenital heart disease patients. Most nurses valued this technology, but inconsistencies and practicalities around its use in guiding patient management were found to be problematic. NIRS is commonly used in the PCICU and although nurses perceived NIRS to be useful for their practice, the variability in the interpretation of values and inconsistent protocols and decision-making by physicians was challenging.

Aortic valve repair using geometric ring annuloplasty in pediatric and congenital heart disease patients

ObjectiveExisting replacement options for the aortic valve have significant drawbacks, especially in children. A geometric annuloplasty ring can help to achieve consistently successful aortic valve repair, but only limited experience with use of this device has been reported in pediatric and congenital heart disease patients. MethodsAll pediatric and adult congenital patients at our institution who underwent aortic valve repair with placement of a geometric annuloplasty ring were reviewed. The study period spanned from July 2018 to April 2022. Hemodynamic outcomes were evaluated using transthoracic echocardiography. ResultsThe study included 36 subjects. The median age was 17.4 years (range, 8-30 years). Twenty-one subjects were younger than age 18 years. The most common primary diagnoses were neoaortic valve insufficiency or neoaortic root dilation, and congenital aortic stenosis with bicuspid or functionally unicuspid aortic valve. Of the 34 subjects with procedural success, 31 (91%) had use of additional valve repair techniques and 26 (76%) had an additional concomitant procedure performed. Operative mortality was 0% (0/33), and major complication rate was 6% (2/33). The median follow-up time was 1.9 years (maximum, 3.8 years). The mean grade of aortic insufficiency was signific antly reduced after repair, with no change in mean gradients. Freedom from reoperation over the follow-up period was 97% (33/34), and freedom from ≥3+ recurrent aortic insufficiency was 94% (32/34). ConclusionsA geometric annuloplasty ring can be used to help achieve consistently successful aortic valve repair with excellent perioperative and follow-up outcomes, even in pediatric and complex congenital heart disease patients.

The high-density grid catheter: A safe adjunctive tool in pediatric and complex congenital heart disease patients.

The safety and utility of the Advisor™ High-Density Grid mapping catheter (HDGC) in children and congenital heart disease (CHD) patients are not well described. A single-center retrospective cohort study of pediatric and CHD patients undergoing electrophysiology study and ablation to determine the effect of HDGC use on outcomes including complications, fluoroscopy use, procedure duration, acute ablation success, and arrhythmia recurrence. HDGC was used in 74/261 (28.3%) cases. HDGC subjects differed by median age (17 vs. 13 years; p < 0.001),weight (68 vs. 50 kg; p < 0.001), and prevalence of significant CHD (42% vs. 3%; p < 0.001). Arrhythmia substrates were dissimilar: HGDC cases had higher frequencies of intra-atrial re-entrant tachycardia (25.7% vs. 0.5%), atrial flutter (8.1% vs. 1.1%), ectopic atrial tachycardia (13.5% vs. 3.7%), and premature ventricular contractions (9.5% vs. 0.5%), and lower incidences of atrioventricular re-entrant tachycardia (16.2% vs. 46.1%). Complications were rare (n = 5, 1.9%) with no significant difference between groups (p = 1.00). Procedure duration-but not fluoroscopy exposure-was significantly longer in HDGC cases (median 256 vs. 216 min, p < 0.001). Acute success was lower (93.2% vs. 99.4%; p = 0.01) and recurrences higher (13.2% vs. 3.8%; p = 0.016) in HDGC compared to non-HDGC cases. HDGC use in children and CHD patients is safe and not associated with higher complication rates. The lower acute success and higher recurrence rates in HDGC cases likely reflect a bias toward HDGC use in more complex arrhythmia substrates rather than less favorable ablation outcomes.

Abstract No. 22 Water-soluble pediatric ultra-high resolution computed tomography lymphangiography

To describe a novel lymphatic imaging technique: pediatric ultra-high resolution CT lymphangiography (UHR CTL) with water-soluble iodinated contrast via direct inguinal intranodal injection. UHR CTL is particularly beneficial in complex congenital heart disease patients with postoperative high-mortality continuous chylothorax and right-to-left shunt.

Neuromonitoring, neuroimaging, and neurodevelopmental follow-up practices in neonatal congenital heart disease: a European survey

BackgroundBrain injury and neurodevelopmental impairment remain a concern in children with complex congenital heart disease (CHD). A practice guideline on neuromonitoring, neuroimaging, and neurodevelopmental follow-up in CHD patients undergoing cardiopulmonary bypass surgery is lacking. The aim of this survey was to systematically evaluate the current practice in centers across Europe.MethodsAn online-based structured survey was sent to pediatric cardiac surgical centers across Europe between April 2019 and June 2020. Results were summarized by descriptive statistics.ResultsValid responses were received by 25 European centers, of which 23 completed the questionnaire to the last page. Near-infrared spectroscopy was the most commonly used neuromonitoring modality used in 64, 80, and 72% preoperatively, intraoperatively, and postoperatively, respectively. Neuroimaging was most commonly performed by means of cranial ultrasound in 96 and 84% preoperatively and postoperatively, respectively. Magnetic resonance imaging was obtained in 72 and 44% preoperatively and postoperatively, respectively, but was predominantly reserved for clinically symptomatic patients (preoperatively 67%, postoperatively 64%). Neurodevelopmental follow-up was implemented in 40% of centers and planned in 24%.ConclusionsHeterogeneity in perioperative neuromonitoring and neuroimaging practice in CHD in centers across Europe is large. The need for neurodevelopmental follow-up has been recognized. A clear practice guideline is urgently needed.ImpactThere is large heterogeneity in neuromonitoring, neuroimaging, and neurodevelopmental follow-up practices among European centers caring for neonates with complex congenital heart disease.This study provides a systematic evaluation of the current neuromonitoring, neuroimaging, and neurodevelopmental follow-up practice in Europe.The results of this survey may serve as the basis for developing a clear practice guideline that could help to early detect and prevent neurological and neurodevelopmental sequelae in neonates with complex congenital heart disease.

Impact of ferumoxytol vs gadolinium on 4D flow cardiovascular magnetic resonance measurements in small children with congenital heart disease

BackgroundCardiovascular magnetic resonance (CMR) allows for time-resolved three-dimensional phase-contrast (4D Flow) analysis of congenital heart disease (CHD). Higher spatial resolution in small infants requires thinner slices, which can degrade the signal. Particularly in infants, the choice of contrast agent (ferumoxytol vs. gadolinium) may influence 4D Flow CMR accuracy. Thus, we investigated the accuracy of 4D Flow CMR measurements compared to gold standard 2D flow phase contrast (PC) measurements in ferumoxytol vs. gadolinium-enhanced CMR of small CHD patients with shunt lesions.MethodsThis was a retrospective study consisting of CMR studies from complex CHD patients less than 20 kg who had ferumoxytol or gadolinium-enhanced 4D Flow and standard two-dimensional phase contrast (2D-PC) flow collected. 4D Flow clinical software (Arterys) was used to measure flow in great vessels, systemic veins, and pulmonary veins. 4D Flow accuracy was defined as percent difference or correlation against conventional measurements (2D-PC) from the same vessels. Subgroup analysis was performed on two-ventricular vs single-ventricular CHD, arterial vs venous flow, as well as low flows (defined as < 1.5 L/min) in 1V CHD.ResultsTwenty-one ferumoxytol-enhanced and 23 gadolinium-enhanced CMR studies were included, with no difference in age (2.1 ± 1.6 vs. 2.3 ± 1.9 years, p = 0.70), patient body surface area (0.50 ± 0.2 vs. 0.52 ± 0.2 m2, p = 0.67), or vessel diameter (11.4 ± 5.2 vs. 12.4 ± 5.6 mm, p = 0.22). Ten CMR studies with single ventricular CHD were included. Overall, ferumoxytol-enhanced 4D flow CMR measurements demonstrated less percent difference to 2D-PC when compared to gadolinium-enhanced 4D Flow CMR studies. In subgroup analyses of arterial vs. venous flows (high velocity vs. low velocity) and low flow in single ventricle CHD, ferumoxytol-enhanced 4D Flow CMR measurements had stronger correlation to 2D-PC CMR. The contrast-to-noise ratio (CNR) in ferumoxytol-enhanced studies was higher than the CNR in gadolinium-enhanced studies.ConclusionsFerumoxytol-enhanced 4D Flow CMR has improved accuracy when compared to gadolinium 4D Flow CMR, particularly for infants with small vessels in CHD.

Management of congenital heart disease in the peripartum period: An illustrative case series

More women with complex congenital heart disease (CHD) reach adulthood resulting in a cohort of patients who are at high risk for adverse events during pregnancy. The haemodynamic changes usual to pregnancy may be poorly tolerated in patients with poor systemic ventricular function, cyanosis, left-sided obstructive lesions and pulmonary hypertension. Complex CHD patients are best managed by a multi-disciplinary team at a high-risk centre. Pre-conception counselling aims at risk stratifying by means of a clinical evaluation, electrocardiogram and echocardiography. Echocardiography plays a vital role in delineating the initial lesions and residual lesions with its haemodynamic complications. The modified WHO (mWHO) classification provides a helpful tool to stratify anatomical and physiological lesions by maternal and foetal event rates and is recommended by the European Society of Cardiology (ESC). Patients with cyanosis, severe aortopathy and severe pulmonary hypertension fall into Class IV and termination of pregnancy is advised. Patients may however choose to continue their pregnancy. We present 3 such cases of complex CHD (Fontan circulation, severe aortopathy and severe pulmonary hypertension) and illustrate some pertinent management principles in the peripartum period.

Systemic Venous Reconstructions During Pediatric Heart Transplantation.

Congenital heart disease continues to be an important indication for pediatric heart transplantation (HT) and is often complicated by systemic venous anomalies. The need for reconstruction, surgical technique used, as well as the outcomes of these have limited documentation. Descriptive, retrospective study of patients transplanted at Emory University between 2006 and 2017. We reviewed surgical data, follow-up, and interventions for patients necessitating venous reconstruction during transplantation. A total of 179 transplants were performed during the time period of which 74 (41%) required systemic venous reconstruction. Mean age at transplant was 6.3 (±6.16) years, and 74.3% of these patients carried a diagnosis of single ventricle; 51 (68.9%) of 74 patients required pulmonary artery reconstruction at the time of HT. Forty patients required superior vena caval reconstruction, while 22 patients required inferior vena caval reconstruction due to prior palliation or anomaly. Venous anomalies along with other anatomic features necessitated biatrial transplantation in four patients. Posttransplant evaluation revealed systemic venous stenosis in 14 (18.9%) of 74 patients. Eight (10.8%) patients required 12 interventions for the systemic veins. Patients with bilateral Glenn anastomosis prior to transplant were at high risk for the development of stenosis and needing interventions. Systemic venous complications were uncommon in those with native systemic veins without Glenn or Fontan procedure. Systemic venous reconstruction needs are high in pediatric HT. Posttransplant stenosis and the need for interventions are relatively common. Current techniques for systemic venous reconstruction for complex congenital heart disease patients may deserve further review to optimize these outcomes.

An automatic framework for perioperative risks classification from retinal images of complex congenital heart disease patients

An automatic framework for perioperative risks classification from retinal images of complex congenital heart disease patients

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.

Optimal AV delay in ventricularly paced adults with congenital heart disease

BackgroundThe atrioventricular (AV) delay may be altered in ventricularly paced patients with either dual-chamber pacemakers or cardiac resynchronization therapy (CRT), to optimize diastolic filling and cardiac output, but such modifications are virtually unstudied in the setting of complex congenital heart disease (CHD). We sought to assess the optimal AV delay in a pilot study of ventricularly paced adults with CHD. MethodsAV delay optimization was performed by the iterative method utilising pulsed wave Doppler of systemic AV valve inflow, in the patient's presenting rhythm (atrial-sensed or paced). Diastolic filling time (DFT), inflow and outflow velocity time integrals (VTI) were compared at optimal and nominal AV delay settings. ResultsNine patients were studied with the following CHD diagnoses: congenitally corrected transposition of the great arteries (TGA) in 4, D-TGA post Senning in 2, tetralogy of Fallot in 2 and Fontan circulation in 1. The median optimal AV delay was 100 ± 20 ms, shorter than the median nominal AV delay of 150 ± 20 ms. The median difference between optimal and nominal AV delay setting was 50 ± 40 ms, with the optimal AV delay shorter than the nominal in all but one patient. At optimal AV delay, the DFT was longer, and inflow and outflow VTIs were larger, compared to nominal. ConclusionsAV delay optimization in ventricularly paced adult CHD patients resulted in an optimal AV delay that differed to the nominal setting in all patients, and typically was shorter.

Initial Experience Assessing the Feasibility of Conscious Sedation in Patients Undergoing Transcatheter Pulmonic Valve Implantation

ABSTRACT Background: Transcatheter pulmonic valve implantation (TPVI) is a minimally invasive procedure that treats patients with dysfunction of the pulmonic valve or right-ventricular-to-pulmonary artery conduit. TPVI procedures have traditionally been performed under general anesthesia (GA), yet conscious sedation (CS) has emerged as an alternative to GA. This study assessed the feasibility of CS-TPVI compared to GA-TPVI. Methods: We performed a retrospective review of 19 consecutive patients undergoing TPVI with CS or GA. The primary endpoint was procedure success, defined as a residual gradient <20 mmHg and freedom from reoperation within 30 days. Secondary endpoints included the procedure step durations, 30-day readmission rate, composite of 90-day rehospitalization and mortality rates, procedural characteristics, and periprocedural complications. Results: Nineteen patients underwent TPVI (CS-TPVI = 8). The procedural success rate was not statistically different between cohorts; only one case in the GA-TPVI group had an elevated transvalvular gradient. The CS-TPVI 90-day composite of rehospitalization and mortality rates was not different from that of GA-TPVI. Median in-room procedure duration was less with CS (131.5 minutes, [90–173, IQR] versus 196 minutes [165–260, IQR], p = 0.007), fewer patients required vasopressors, and no patients converted from CS to GA. Contrast use, radiation exposure, length of stay, and periprocedural complications were not different between groups. Conclusions: This small, nonrandomized study reports no differences in clinical outcomes between CS-TPVI and GA-TPVI with shorter in-room duration and less vasopressor use in the CS-TPVI group. This study highlights the importance of a multidisciplinary approach in complex congenital heart disease patients and underscores the need for larger studies investigating CS-TPVI. Abbreviations: CHD: congenital heart disease; CS: conscious sedation; GA: general anesthesia; IQR: interquartile range; PA: pulmonary artery; RV: right ventricle; RVOT: right ventricular outflow tract; TAVI: transcatheter aortic valve implantation; TPVI: transcatheter pulmonic valve implantation

Transcriptomic and Functional Analyses of Mitochondrial Dysfunction in Pressure Overload-Induced Right Ventricular Failure.

BackgroundIn complex congenital heart disease patients such as those with tetralogy of Fallot, the right ventricle (RV) is subject to pressure overload, leading to RV hypertrophy and eventually RV failure. The mechanisms that promote the transition from stable RV hypertrophy to RV failure are unknown. We evaluated the role of mitochondrial bioenergetics in the development of RV failure.Methods and ResultsWe created a murine model of RV pressure overload by pulmonary artery banding and compared with sham‐operated controls. Gene expression by RNA‐sequencing, oxidative stress, mitochondrial respiration, dynamics, and structure were assessed in pressure overload‐induced RV failure. RV failure was characterized by decreased expression of electron transport chain genes and mitochondrial antioxidant genes (aldehyde dehydrogenase 2 and superoxide dismutase 2) and increased expression of oxidant stress markers (heme oxygenase, 4‐hydroxynonenal). The activities of all electron transport chain complexes decreased with RV hypertrophy and further with RV failure (oxidative phosphorylation: sham 552.3±43.07 versus RV hypertrophy 334.3±30.65 versus RV failure 165.4±36.72 pmol/(s×mL), P<0.0001). Mitochondrial fission protein DRP1 (dynamin 1‐like) trended toward an increase, while MFF (mitochondrial fission factor) decreased and fusion protein OPA1 (mitochondrial dynamin like GTPase) decreased. In contrast, transcription of electron transport chain genes increased in the left ventricle of RV failure.ConclusionsPressure overload‐induced RV failure is characterized by decreased transcription and activity of electron transport chain complexes and increased oxidative stress which are associated with decreased energy generation. An improved understanding of the complex processes of energy generation could aid in developing novel therapies to mitigate mitochondrial dysfunction and delay the onset of RV failure.

Positive Association of Depression, Anxiety, and Attention Deficit Hyperactivity Disorder in Youth with Congenital Heart Disease: A Cross-Sectional Comparative Study

Purpose: Depression and anxiety in older adolescents and adults with complex congenital heart disease (CHD) has been well described. Additionally, studies report higher rates of ADHD in children with complex CHD. These data are lacking for younger and simple CHD patients. The goal of our study was to characterize anxiety/depression and ADHD in children with all types of CHD compared to those without CHD. Methods: Data was obtained using electronic medical records between 2011-2016 from a large tertiary care pediatric hospital. Inclusion criteria were youth < 18 years …

Transbaffle/transconduit puncture using a simple CARTO-guided approach without echocardiography in patients with congenital heart disease.

Catheter ablation (CA) of atrial tachyarrhythmias (ATs) in patients with complex congenital heart disease (CHD) often requires technically challenging transbaffle or transconduit puncture. The aim was to assess the feasibility and safety of transbaffle/transconduit puncture based on computed tomography (CT) three-dimensional (3D) reconstruction merged with electro-anatomical mapping (EAM) without per-procedure echocardiographic guidance. We included 18 consecutive CHD patients in two centers who had atrial-switch or Fontan surgery and underwent CA of AT by an antegrade approach requiring intracardiac puncture. Twelve patients with atrial-switch surgery and six patients with extracardiac Fontan surgery were referred for CA of AT. Cardiac CT with 3D reconstruction was performed before the procedure. The 3D volume of the systemic venous atrium or extracardiac conduit acquired by EAM was merged with the corresponding CT 3D reconstruction. The ablation catheter was positioned at the optimal puncture site. Under fluoroscopic guidance, the needle was positioned next to the ablation and the puncture was performed. Balloon expansion of the puncture site was performed in every case of transconduit puncture and in two (17%) cases of transbaffle puncture. Overall, 17 intra-atrial reentrant tachycarrythmias and 9 focal ATs were successfully ablated, with no acute complications. The median time to access the pulmonary atrium was 78.5 minutes (range, 55-185) and total median fluoroscopy time was 23 minutes (range, 7-53). Transbaffle and transconduit punctures can be performed safely in CHD patients by using a simple technique relying on CT 3D reconstruction and EAM.

Atrial fibrillation ablation in adults with congenital heart disease on uninterrupted oral anticoagulation is safe and efficient.

The prevalence of atrial fibrillation (AF) is significantly higher in adults with congenital heart disease (ACHD) compared to patients without congenital heart disease (CHD). As AF in ACHD patients might have significant hemodynamic consequences, rhythm control is particularly desirable but rarely achieved by antiarrhythmic drugs. The aim of this study was to investigate safety and long-term outcome of AF ablation in ACHD patients. All ACHD patients (n = 46) that underwent AF ablation at our centre from 2013 to 2017 were included in the study. CHD was classified as simple (46%), moderate (41%) or complex (13%). The majority of patients (61%) suffered from persistent AF (paroxysmal AF 39%). Persistent AF was present in 57% of patients with simple, in 58% of patients with moderate and 83% of patients with complex CHD. All patients underwent radiofrequency (RF) ablation on uninterrupted oral anticoagulation. Pulmonary vein isolation (PVI) was performed in patients with paroxysmal AF, whereas patients with persistent AF underwent PVI and ablation of complex fractionated atrial electrograms (CFAE). No major complications occurred. Single-procedure success after 18months off antiarrhythmic drugs was 61% for paroxysmal AF and 29% for persistent AF (p = 0.003). Multiple procedures (mean 2.1 ± 1.4) increased long-term success to 82% for paroxysmal AF and 48% for persistent AF (p = 0.05). Long-term ablation success was 64% for simple, 62% for moderate and 50% for complex CHD patients. AF ablation in ACHD patients is feasible and safe regardless of CHD complexity. Success rates in patients with paroxysmal AF are high and comparable to patients without CHD. In ACHD patients with persistent AF, success rates of ablation are markedly reduced which might be due to a different and/or more extensive (bi-)atrial substrate. In the cohort of complex ACHD patients with persistent AF as the dominant AF type, long-term success of AF ablation is limited.