Balloon atrial septostomy for transposition of the great arteries: Safety and experience with the Z-5 balloon catheter.

BACKGROUND: Balloon atrial septostomy (BAS) is an emergent and essential cardiac intervention to enhance intercirculatory mixing at atrial level in deoxygenated patients diagnosed with transposition of the great arteries (TGA) and restrictive foramen ovale. The recent recall of several BAS catheters and the changes in the European legal framework for medical devices (MDR 2017/745), has led to an overall scarcity of BAS catheters and raised questions about the use, safety, and experience of the remaining NuMED Z-5 BAS catheter. AIMS: To evaluate and describe the practice and safety of the Z-5 BAS catheter, and to compare it to the performance of other BAS catheters. METHODS: A retrospective single-center cohort encompassing all BAS procedures performed with the Z-5 BAS catheter in TGA patients between 1999 and 2022. RESULTS: A total of 182 BAS procedures were performed in 179 TGA-newborns at Day 1 (IQR 0-5) days after birth, with median weight of 3.4 (IQR 1.2-5.7) kg. The need for BAS was urgent in 90% of patients. The percentage of BAS procedures performed at bedside increased over time from 9.8% (before 2010) to 67% (2017-2022). Major complication rate was 2.2%, consisting of cerebral infarction (1.6%) and hypovolemic shock (0.5%). The rate of minor complications was 9.3%, including temporary periprocedural AV-block (3.8%), femoral vein thrombosis (2.7%), transient intracardiac thrombus (0.5%), and atrial flutter (2.2%). BAS procedures performed at bedside and in the cardiac catheterization laboratory had similar complication rates. CONCLUSIONS: BAS using the Z-5 BAS catheter is both feasible and safe at bedside and at the cardiac catheterization laboratory with minimal major complications.

The impact of unilateral pulmonary artery stenosis on right ventricular to pulmonary arterial coupling in patients with transposition of the great arteries.

BACKGROUND: Unilateral pulmonary artery (PA) stenosis is common in the transposition of the great arteries (TGA) after arterial switch operation (ASO) but the effects on the right ventricle (RV) remain unclear. AIMS: To assess the effects of unilateral PA stenosis on RV afterload and function in pediatric patients with TGA-ASO. METHODS: In this retrospective study, eight TGA patients with unilateral PA stenosis underwent heart catheterization and cardiac magnetic resonance (CMR) imaging. RV pressures, RV afterload (arterial elastance [Ea]), PA compliance, RV contractility (end-systolic elastance [Ees]), RV-to-PA (RV-PA) coupling (Ees/Ea), and RV diastolic stiffness (end-diastolic elastance [Eed]) were analyzed and compared to normal values from the literature. RESULTS: In all TGA patients (mean age 12 ± 3 years), RV afterload (Ea) and RV pressures were increased whereas PA compliance was reduced. RV contractility (Ees) was decreased resulting in RV-PA uncoupling. RV diastolic stiffness (Eed) was increased. CMR-derived RV volumes, mass, and ejection fraction were preserved. CONCLUSION: Unilateral PA stenosis results in an increased RV afterload in TGA patients after ASO. RV remodeling and function remain within normal limits when analyzed by CMR but RV pressure-volume loop analysis shows impaired RV diastolic stiffness and RV contractility leading to RV-PA uncoupling.

Endocardial substrate of ventricular tachycardia in an adult patient with congenitally corrected transposition of great arteries without surgical repair.

Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart disease which may present sudden cardiac death presumably due to malignant ventricular tachycardia (VT). In patients with congenital heart disease, knowledge of arrhythmogenic substrate is crucial for planning an ablation procedure. We present the first description of the arrhythmogenic endocardial substrate of a non-iatrogenic scar-related VT in a patient with CCTGA.

A short RP tachycardia in congenitally corrected transposition. What is the mechanism?

A 51-year-old female with congenitally corrected transposition of great arteries (CCTGA), situs solitus, dextrocardia, atrial septal defect and persistent left superior vena cava underwent electrophysiology study for recurrent palpitations with documented narrow complex, short RP tachycardia. With a catheter in the region of the anterior mitral annulus, a His signal was recorded and HV interval was 35 msec. Tachycardia was induced with a ventricular extrastimulus. During the tachycardia there was 1:1 ventriculo-atrial conduction and central atrial activation with a VA interval of 20 msec. The recorded His signal could be seen after the QRS. What is the mechanism of the tachycardia?

En Bloc Rotation of the Outflow Tracts - Double Root Translocation.

En bloc rotation of the outflow tracts or double root translocation offers an anatomic repair of transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction and closely related forms of double outlet right ventricle. The technical principle is to excise aortic and pulmonary root en bloc, rotate them as a whole by 180°, and reimplant them. The left ventricular outflow tract is enlarged with the patch closing the ventricular septal defect. In our experience, two thirds of the pulmonary valves could be preserved. Growth of the aortic and pulmonary root could be demonstrated in several studies performed by our group. It is still a complex and technically demanding procedure with long cardiopulmonary bypass periods and cross-clamp times. However, perioperative mortality and complications do not differ significantly from other forms of reconstruction. The reoperation rate is significantly lower. Presently, the best time to perform this operation seems to be after the newborn period within the first year of life.

Complete Transposition of the Great Arteries: 5-Year Experience From a Single Center.

Complete transposition of the great arteries (TGA) is the most common cause of cyanosis in the first week of life. Prenatal diagnosis helps with counseling, planning delivery, and postnatal management for resource-rich health services. In a resource-limited setting, postnatal diagnosis is the norm. This work examines cases of complete TGA in one center in Cambodia without prenatal testing. Twenty-four cases were studied over 5 years. Complex TGA was frequently seen. The majority had normal coronary anatomy and arterial switch operation (ASO) was performed in most cases with a favorable outcome.

End-stage heart failure in congenitally corrected transposition of the great arteries: a multicentre study.

BACKGROUND AND AIMS: For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear. METHODS: This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained. The composite primary outcome was mechanical circulatory support, heart transplantation, or death. RESULTS: From 558 patients (48% female, age at first visit 36 ± 14.2 years, median follow-up 8.7 years), the event rate of the primary outcome was 15.4 per 1000 person-years (11 mechanical circulatory support implantations, 12 transplantations, and 52 deaths). Patients experiencing the primary outcome were older and more likely to have a history of atrial arrhythmia. The primary outcome was highest in those with both moderate/severe right ventricular (RV) dysfunction and tricuspid regurgitation (n = 110, 31 events) and uncommon in those with mild/less RV dysfunction and tricuspid regurgitation (n = 181, 13 events, P < .001). Outcomes were not different based on anatomic complexity and history of tricuspid valve surgery or of subpulmonic obstruction. New CHF admission or ventricular arrhythmia was associated with the primary outcome. Individuals who underwent childhood surgery had more adverse outcomes than age- and sex-matched controls. Multivariable Cox regression analysis identified older age, prior CHF admission, and severe RV dysfunction as independent predictors for the primary outcome. CONCLUSIONS: Patients with ccTGA have variable deterioration to end-stage heart failure or death over time, commonly between their fifth and sixth decades. Predictors include arrhythmic and CHF events and severe RV dysfunction but not anatomy or need for tricuspid valve surgery.

Modified Senning procedure in a patient with dextrocardia with left atrial isomerism and anomalous systemic venous drainage: a rare case report.

The atrial switch procedure by Senning or Mustard technique primarily aims in correcting parallel systemic and pulmonary circulations at atrial level. This procedure may be used in late presenting D-transposition of great arteries with a deconditioned left ventricle, congenitally corrected transposition of great arteries and isolated ventricular inversion. We describe the case of a child with dextrocardia, left atrial isomerism with complex pulmonary and systemic venous drainage resulting in mixing at atrial level. She was successfully operated by modified Senning procedure performed through the left-sided atrium.

Long-term incidence of infective endocarditis among patients with congenital heart disease.

BACKGROUND: Patients with congenital heart disease (CHD) are at lifelong high risk of infective endocarditis (IE). The risk of IE presumably differs among different CHD, but little knowledge exists on the area. METHODS: In this observational cohort study, all CHD-patients born in 1977 to 2018 were identified using Danish nationwide registries and followed from the date of birth until first-time IE, emigration, death, or end of study (December 31, 2018). The comparative risk of IE among CHD-patients vs age- and sex-matched controls from the background population was assessed. The risk of IE was stratified according to the type of CHD and factors associated with IE including sex and relevant time-varying coefficients (ie, cyanosis, cardiac prostheses, diabetes mellitus, chronic kidney disease, and cardiac implantable electronic devices) were examined using Cox-regression analysis. RESULTS: A total of 23,464 CHD-patients (50.0% men) were identified and matched with 93,856 controls. During a median follow-up of 17.7 years, 217(0.9%) CHD-patients and 4(0.0%) controls developed IE, corresponding to incidence rates of 5.2(95%CI 4.6-6.0) and 0.02(95%CI 0.01-0.1) per 10,000 person-years, respectively. The incidence of IE was greatest among patients with tetralogy of fallot, malformations of the heart chambers (including transposition of the great arteries, univentricular heart, and truncus arteriosus), atrioventricular septal defects, and heart valve defects. Factors associated with IE among CHD-patients included male sex, cyanosis, cardiac prostheses, chronic kidney disease, and cardiac implantable electronic devices. CONCLUSIONS: CHD-patients have a substantially higher associated incidence of IE than the background population. With the increasing longevity of these patients, relevant guidelines concerning preventive measures are important.

Design of a multi-institutional neurocognitive discovery study in adult congenital heart disease (MINDS-ACHD).

BACKGROUND: Neurocognitive dysfunction (NCD) is a common comorbidity among children with congenital heart disease (CHD). However, it is unclear how underlying CHD and its sequelae combine with genetics and acquired cardiovascular and neurological disease to impact NCD and outcomes across the lifespan in adults with CHD. METHODS: The Multi-Institutional Neurocognitive Discovery Study in Adults with Congenital Heart Disease (MINDS-ACHD) is a partnership between the Pediatric Heart Network (PHN) and the Adult Alliance for Research in Congenital Cardiology (AARCC) that examines objective and subjective neurocognitive function and genetics in young ACHD. This multicenter cross-sectional pilot study is enrolling 500 young adults between 18 and 30 years with moderate or severe complexity CHD at 14 centers in North America. Enrollment includes 4 groups (125 participants each): (1) d-looped Transposition of the Great Arteries (d-TGA); (2) Tetralogy of Fallot (TOF); (3) single ventricle (SV) physiology; and (4) "other moderately or severely complex CHD." Participants complete the standardized tests from the NIH Toolbox Cognitive Battery, the NeuroQoL, the Hospital Anxiety and Depression Scale, and the PROMIS Global QoL measure. Clinical and demographic variables are collected by interview and medical record review, and an optional biospecimen is collected for genetic analysis. Due to the COVID-19 pandemic, participation may be done remotely. Tests are reviewed by a Neurocognitive Core Laboratory. CONCLUSIONS: MINDS-ACHD is the largest study to date characterizing NCD in young adults with moderate or severely complex CHD in North America. Its results will provide valuable data to inform screening and management strategies for NCD in ACHD and improve lifelong care.

Congenital Heart Disease with Congenital Diaphragmatic Hernia: Surgical Decision Making and Outcomes.

OBJECTIVE: To describe the types of congenital heart disease (CHD) in a congenital diaphragmatic hernia (CDH) cohort in a large volume center and evaluate surgical decision making and outcomes based on complexity of CHD and associated conditions. STUDY DESIGN: A retrospective review of patients with CHD and CDH diagnosed by echocardiogram between 01/01/2005 and 07/31/2021. The cohort was divided into 2 groups based on survival at discharge. RESULTS: Clinically important CHD was diagnosed in 19% (62/326) of CDH patients. There was 90% (18/20) survival in children undergoing surgery for both CHD and CDH as neonates, and 87.5 (22/24) in those undergoing repair initially for CDH alone. A genetic anomaly identified on clinical testing was noted in 16% with no significant association with survival. A higher frequency of other organ system anomalies was noted in nonsurvivors compared with survivors. Nonsurvivors were more likely to have unrepaired CDH (69% vs 0%, P < .001) and unrepaired CHD (88% vs 54%, P < .05), reflecting a decision not to offer surgery. CONCLUSIONS: Survival was excellent in patients who underwent repair of both CHD and CDH. Patients with univentricular physiology have poor survival and this finding should be incorporated into pre and postnatal counseling about eligibility for surgery. In contrast, patients with other complex lesions including transposition of the great arteries have excellent outcomes and survival at 5 years follow-up at a large pediatric and cardiothoracic surgical center.

An unmet need: arrhythmia detection by implantable loop recorder in the systemic right ventricle.

AIMS: Patients with systemic right ventricles are at high risk of sudden cardiac death. Arrhythmia is a significant risk factor. Routine Holter monitoring is opportunistic with poor adherence. The aim of this study was to determine if continuous rhythm monitoring with an implantable loop recorder (ILR) could allow early detection of clinically important arrhythmias. METHODS AND RESULTS: Implantable loop recorder implantation was offered to patients with atrial switch repair for transposition of the great arteries. Recordings were made with symptoms or, automatically for pauses, significant bradycardia or tachycardia and reviewed by the multi-disciplinary team. Twenty-four out of 36 eligible patients underwent ILR implantation with no complication. Forty-two per cent had preserved ventricular function, 75% were NYHA functional class I, 88% had low sudden cardiac death risk, 33% had previous intra-atrial re-entrant tachycardia (IART), and none had known conduction disease. Eighteen out of 24 (75%) patients made 52 recordings (52% automated) over 39.5 months (1.6-72.5). Thirty-two out of 52 (62%) recordings in 15/24 (63%) of the cohort were clinically significant and included sinus node disease (two patients), atrioventricular block (two patients), IART (seven patients), and IART with sinus node disease or atrioventricular block (four patients). Implantable loop recorder recordings prompted medication change in 11 patients [beta-blockers (n = 9), anti-coagulation (n = 5), and stopping anti-coagulation (n = 1)] and device therapy recommendation in seven patients [five pacemakers (three: atrioventricular block) and two defibrillators]. Two patients declined intervention; one suffered an arrhythmic death. Intra-atrial re-entrant tachycardia and clinically relevant conduction disease were detected in patients irrespective of sudden cardiac death risk. CONCLUSION: Continuous monitoring with an ILR in patients with systemic right ventricle following atrial switch detects clinically relevant arrhythmias that impact decision-making. In this cohort, clinically relevant arrhythmias did not correlate with sudden cardiac death risk.

Three-dimensional-mapping-guided permanent conduction system pacing in paediatric patients with congenitally corrected transposition of the great arteries.

AIMS: In congenitally corrected transposition of the great arteries (CCTGA) the right ventricle (RV) is systemic. Atrioventricular block (AVB) and systolic dysfunction are frequently observed. Permanent pacing of the subpulmonary left ventricle (LV) may worsen RV dysfunction. The aim of this study was to seek out if LV conduction system pacing (LVCSP) guided by three-dimensional-electroanatomic mapping systems (3D-EAMs) can preserve RV systolic function in paediatric CCTGA patients with AVB. METHODS AND RESULTS: Retrospective analysis of CCTGA patients who underwent 3D-EAM-guided LVCSP. Three-dimensional-pacing map guided lead implantation towards septal sites with narrower paced QRS. Electrocardiograms (ECGs), echocardiograms, and lead parameters (threshold, sensing, and impedance) were compared at baseline (pre-implantation) and at 1-year follow-up. Right ventricle function was evaluated by 3D ejection fraction (EF), fractional area change (FAC), RV global longitudinal strain (GLS). Data are reported as median (25th-75th centiles). Seven CCTGA patients aged 15 (9-17) years, with complete/advanced AVB (4 with prior epicardial pacing), underwent 3D-guided LVCSP (5 DDD, 2 VVIR). Baseline echocardiographic parameters were impaired in most patients. No acute/chronic complications occurred. Ventricular pacing was >90%. At 1-year follow-up QRS duration showed no significant changes compared with baseline; however, QRS duration shortened in comparison with prior epicardial pacing. Lead parameters remained acceptable despite ventricular threshold increased. Systemic RV function was preserved: FAC and GLS improved significantly, and all patients showed normal RV EF (>45%). CONCLUSION: Three-dimensional-EAM-guided LVCSP preserved RV systolic function in paediatric patients with CCTGA and AVB after short-term follow-up.

Hypertrophic cardiomyopathy in the systemic right ventricle in a patient with congenitally corrected transposition of the great arteries: A case report.

Congenitally corrected transposition of the great arteries is a rare clinical entity, which usually presents during adulthood with associated defects; atrioventricular block, heart failure, systemic valve failure, and arrhythmias usually complicate the clinical course. Even rarer is associated hypertrophic cardiomyopathy, which complicates the disease course and clinical decision-making. Herein, we present a patient with this condition who underwent heart transplantation, with adequate clinical resolution.

A new score for life-threatening ventricular arrhythmias and sudden cardiac death in adults with transposition of the great arteries and a systemic right ventricle.

AIMS: To investigate the incidence of major adverse ventricular arrhythmias and related events (MAREs) and to develop a stratification tool predicting MAREs in adults with a systemic right ventricle (sRV). METHODS AND RESULTS: In a multicentre approach, all adults (≥16 years old) with a sRV undergoing follow-up between 2000 and 2018 were identified. The incidence of MAREs, defined as sudden cardiac death, sustained ventricular tachycardia, and appropriate implantable cardioverter-defibrillator (ICD) therapy, was analysed. The association of MAREs with clinical, electrical, and echocardiographic parameters was evaluated. A total of 1184 patients (median age 27.1 years; interquartile range 19.9-34.9 years; 59% male; 70% with atrial switch repair for D-transposition of the great arteries) were included. The incidence of MAREs was 6.3 per 1000 patient-years. On multivariate analysis, age, history of heart failure, syncope, QRS duration, severe sRV dysfunction and at least moderate left ventricular outflow tract obstruction were retained in the final model with a C-index of 0.78 [95% confidence interval (CI) 0.72-0.83] and a calibration slope of 0.93 (95% CI 0.64-1.21). For every five ICDs implanted in patients with a 5-year MARE risk >10%, one patient may potentially be spared from a MARE. CONCLUSION: Sudden cardiac death remains a devastating cause of death in a contemporary adult cohort with a sRV. A prediction model based on clinical, electrocardiographic, and echocardiographic parameters was devised to estimate MARE risk and to identify high-risk patients who may benefit from primary prevention ICD implantation.

Corrected Transposition of Great Arteries with Cor Triatriatum and Atrial Septal Defect-Case Report.

A 37-year-old male patient with corrected transposition of great arteries (ccTGA) with cor triatriatum sinister (CTS), left superior vena cava, and atrial septal defects is reported in our case. None of these impacted the patient's growth or development, nor daily work until age 33. Later, the patient developed symptoms of obvious impaired heart function, which improved after medical treatment. However, the symptoms reappeared and gradually worsened two years later, and we decided to treat it with surgery. In this case, we selected tricuspid mechanical valve replacement, cor triatriatum correction, and atrial septal defect repair. During the follow-up of five years, the patient had no obvious symptoms, ECG did not change significantly from five years ago, and the cardiac color Doppler ultrasound showed RVEF 0.51.

Transcatheter systemic atrioventricular valve-in-valve implantation in a congenitally corrected transposition of the great arteries patient.

We present an asymptomatic pregnant patient with congenitally corrected transposition of the great arteries and severe atrioventricular bioprosthesis regurgitation - with increased maternal and fetal risk due to volume overload. She was considered high risk for reintervention and was submitted to an off-label post-partum transcatheter valve-in-valve implantation with a Sapiens 3 valve. The procedure was successful, and she remains asymptomatic 30 months after - and even went through another successful pregnancy.

TGA + IVS + LVOTO: patterns of practice and outcomes.

PURPOSE: Transposition of great arteries with intact ventricular septum and left ventricular outflow tract obstruction (TGA + IVS + LVOTO) is uncommon. We reviewed operations performed in patients with TGA + IVS + LVOTO in the European Congenital Heart Surgeons Association Congenital Database (ECHSA-CD). METHODS: All 109 patients with a diagnosis of TGA + IVS + LVOTO in ECHSA-CD who underwent cardiac surgery during a 21-year period (01/2000-02/2021, inclusive) were included. Preoperative variables, operative data, and postoperative outcomes were collected. RESULTS: These 109 patients underwent 176 operations, including 37 (21.0%) arterial switch operations (ASO), 26 (14.2%) modified Blalock-Taussig-Thomas shunts (MBTTS), 11 (6.2%) Rastelli operations, and 13 (7.3%) other palliative operations (8 superior cavopulmonary anastomosis[es], 4 Fontan, and 1 other palliative procedure). Of 37 patients undergoing ASO, 22 had a concomitant procedure.There were 68 (38.6%) reoperations, including 11 pacemaker procedures and 8 conduit operations. After a systemic-to-pulmonary artery shunt, reoperations included shunt reoperation (n = 4), Rastelli (n = 4), and superior cavopulmonary anastomosis (n = 3).Overall Operative Mortality was 8.2% (9 deaths), including three following ASO, two following "Nikaidoh, Kawashima, or LV-PA conduit" procedures, and two following Rastelli. Postoperative complications occurred after 36 operations (20.4%). The most common complications were delayed sternal closure (n = 11), postoperative respiratory insufficiency requiring mechanical ventilation >7 days (n = 9), and renal failure requiring temporary dialysis (n = 8). CONCLUSION: TGA + IVS + LVOTO is rare (109 patients in ECHSA-CD over 21 years). ASO, MBTTS, and Rastelli are the most common operations performed for TGA + IVS + LVOTO. Larger international studies with long-term follow-up are needed to better define the anatomy of the LVOTO and to determine the optimal surgical strategy.

Right ventricular dissection after arterial switch operation.

Herein we present the right ventricular dissection and describe its successful management after arterial switch operation in a full-term male neonate. There are no evidence-based recommendations for the management of this rare complication. Our management included veno-arterial extracorporeal membrane oxygenation placement and delayed surgical evacuation of the dissecting haematoma with beneficial outcomes.

Situs inversus with dextrocardia and transposition of great arteries.

Transposition of the great arteries with left ventricle outflow tract obstruction in combination with situs inversus totalis and dextrocardia is a very rare anomaly and carries high morbidity and mortality. Only few cases have been reported with this anomaly. We describe a 21-day of infant girl with transposition of the great arteries and mirror image dextrocardia and pulmonary stenosis who underwent successful neonatal arterial switch operation and left ventricle outflow tract obstruction resection following PDA stent implantation.