Atrial Switch Operation Research Articles

A meta-analysis of the effects of sacubitril-valsartan on surrogate markers of systemic right ventricular dysfunction in congenital heart disease

Abstract Introduction Dysfunction of systemic right ventricle (sRV) in adult congenital heart disease (ACHD) patients is expectable and often associated with heart failure and other dismal cardiovascular events. Despite limited evidence, several neurohormonal modulators approved for heart failure with reduced ejection fraction (HFrEF) have been used off-label in patients with RV dysfunction1. Sacubitril-valsartan (ARNI) effects on sRV remains poorly studied, with current evidence being limited to small cohorts. Purpose Review and summarize current evidence about the effects of ARNI in ACHD patients with sRV. Methods We performed a systematic review on MEDLINE, EMBASE, COCHRANE, and SCOPUS for articles that included "systemic right ventricle" and "sacubitril valsartan" until January 2024. Two investigators screened the literature and extracted data. Treatment effects were measured by Cohen's d effect-size (EZ) pre and post-ARNI, with a 95% confidence interval (CI), using a restricted maximum likelihood model. Results From 197 publications screened, five studies fulfilled our criteria, comprising a total of 135 patients. Follow-up after ARNI therapy ranged from 12 to 27 months. No randomized clinical trials were found, and only two studies had a prospective design. Dextro-transposition of the great arteries (D-TGA) after atrial switch operation was the most common type of congenital heart disease with sRV (n=95; 70%). Meta-analysis demonstrates that ARNI reduced NT-proBNP levels during follow-up (EZ= -0.34, CI -0.59 to 0.08; p=0.01; I2=4.0%). Regarding RV function, ARNI improved RV fractional area change (RVFAC) (EZ= 0.75, CI 0.01 to 1.50; p= 0.048; I2=85.0%). A trend for RV global longitudinal strain improvement was also observed with ARNI (EZ= 1.74, CI -0.05 to 3.54; p=0.06; I2=95.9%) but not for tricuspid annular plane systolic excursion (EZ= -0.07, CI -0.33 to 0.20; p=0.62; I2=0.0%). RV diastolic diameter did not change significantly with ARNI (EZ= 0.07, CI -0.43 to 0.56; p=0.79; I2=68.2%). A trend for improvement on the 6-minute walk test was observed (EZ= 1.89, CI -0.13 to 3.91; p=0.07; I2=96.3%), but no effect on exercise oxygen consumption (VO2) (EZ= -0.09, CI -1.32 to 1.15; p=0.89; I2=91.4%). During follow-up ARNI did not change significantly systolic blood pressure (EZ= -0.32, CI -0.74 to 0.11; p=0.15; I2=58.4%) or serum creatinine (EZ= 0.23, CI -0.04 to 0.49; p=0.09; I2=0.0%). Conclusions Despite heterogeneity among these studies, this meta-analysis demonstrates that ARNI might improve NT-proBNP levels and RV function. ARNI seems safe and well tolerated, with no significant effect on blood pressure and renal function. More well-powered studies are needed to confirm the beneficial effects of ARNI in ACHD with sRV.

Right ventricular global longitudinal strain by cardiac magnetic resonance feature tracking is associated with outcome in patients with a systemic right ventricle

Abstract Introduction It is well known, that patients with a systemic (sub-aortic) right ventricle (sRV) for congenitally-corrected transposition of the great arteries (TGA) or with complete TGA after atrial switch operation are more threatened to develop RV dysfunction, relevant supra- and ventricular tachycardia and have a reduced life expectancy. While the randomized, placebo-controlled, double-blinded, multi-centre SERVE trial to investigate the effect of phosphodiesterase-5 inhibitor tadalafil in sRV patients showed negative results on the primary end-point RV endsystolic volume (RVESV), aim of this sub-analysis was to evaluate RV global longitudinal strain (GLS), a more sensitive measure of RV function, by cardiac magnetic resonance (CMR) feature tracking (FT) over time, for treatment effect and to investigate whether RV GLS predicts clinical outcome in sRV patients. Methods CMR volumetric RV and FT parameters were analysed (blinded for patient and assessment time and treatment) at baseline and compared to follow-up (FU) after 3 years, or after 1 year in case of withdrawal of the study drug. FT was performed using appropriate software. Treatment effect was analysed using ANCOVA with baseline values and FU-time as covariates. Values of > 4th quartile of RV GLS were used as cut-off for Kaplan-Meier analysis on the primary outcome, defined as a composite of all-cause death, clinically relevant arrhythmias and hospitalisation for heart failure. Results CMR exams were available for 78 patients at baseline (40±11 years, 33% females) and for 71 patients at FU. No treatment effect of tadalafil compared to the placebo was discovered for RV GLS (p=0.26), RVESVi (p=0.63) and RV ejection fraction (RVEF, p=0.6). Over a 3 years FU-time, RV GLS mildly increased (p=0.0023, figure 1). During FU, 14 patients experienced a clinical outcome: 1 death, 4 hospitalisations for heart failure, and 12 clinically relevant arrhythmic events. An RV GLS > -13.4% was associated with adverse clinical outcome (p=0.003, figure 2). Conclusion In patients with a systemic RV, treatment with Tadalafil had no effect on CMR-derived RV global longitudinal strain compared to placebo. RV GLS changed significantly over the 3 years FU-time. A RV GLS value of > -13.4% is associated with adverse clinical outcome in these patients.Figure 1Figure 2

The Sub-Pulmonary Left Ventricle in Patients with Systemic Right Ventricle, the Paradoxical Neglected Chamber: A Cardiac Magnetic Resonance Feature Tracking Study.

Background/Objective: The impact of subpulmonary left ventricle (LV) dysfunction in patients with a systemic right ventricle (SRV) is insufficiently characterized, with only a few studies suggesting its prognostic significance. Additionally, its evaluation through imaging techniques is a challenge. To assess the correlation between quantitative cardiac magnetic resonance-feature tracking (CMR-FT) data and the risk of clinical events related to the natural history of SRV failure. Methods: In this cross-sectional study, 21 patients with a diagnosis of transposition of the great arteries (TGA) and atrial switch operation (AtSO) or congenitally corrected transposition (ccTGA) were recruited. All participants underwent CMR-FT analysis. Considered clinical events included NYHA class deterioration (from I-II to III-IV), increased diuretic therapy, arrhythmias, sudden cardiac death, and hospitalizations. Results: The cohort consisted of 52.4% males (mean age: 25.4 ± 11.9 years). Eleven patients were diagnosed with ccTGA. Of the 10 patients with TGA post-AtSO, 50% had undergone Mustard repair. Clinical events occurred in 11 patients, with 47.6% experiencing hospitalizations and 28.6% developing arrhythmias. Left ventricular global longitudinal strain (LV GLS) was significantly associated with event-risk in both univariate and multivariate analyses (p = 0.011; p = 0.025). A cut-off value of LV GLS > -19.24 was proposed to stratify high-risk patients (p = 0.001). Conclusions: Our study confirms the role of subpulmonary LV function in determining outcomes of SRV patients. The assessment of LV GLS by using CMR-FT could significantly enhance clinical management during follow-up.

Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II.

Dextro-transposition of the great arteries (D-TGA) is the second most common cyanotic heart disease, accounting for 5-7% of all congenital heart defects (CHDs). It is characterized by ventriculoarterial (VA) connection discordance, atrioventricular (AV) concordance, and a parallel relationship with D-TGA. As a result, the pulmonary and systemic circulations are separated [the morphological right ventricle (RV) is connected to the aorta and the morphological left ventricle (LV) is connected to the pulmonary artery]. This anomaly is included in the group of developmental disorders of embryonic heart conotruncal irregularities, and their pathogenesis is multifactorial. The anomaly's development is influenced by genetic, epigenetic, and environmental factors. It can occur either as an isolated anomaly, or in association with other cardiac defects. The typical concomitant cardiac anomalies that may occur in patients with D-TGA include ventriculoseptal defects, patent ductus arteriosus, left ventricular outflow tract obstruction (LVOTO), mitral and tricuspid valve abnormalities, and coronary artery variations. Correction of the defect during infancy is the preferred treatment for D-TGA. Balloon atrial septostomy (BAS) is necessary prior to the operation. The recommended surgical correction methods include arterial switch operation (ASO) and atrial switch operation (AtrSR), as well as the Rastelli and Nikaidoh procedures. The most common postoperative complications include coronary artery stenosis, neoaortic root dilation, neoaortic insufficiency and neopulmonic stenosis, right ventricular (RV) outflow tract obstruction (RVOTO), left ventricular (LV) dysfunction, arrhythmias, and heart failure. Early diagnosis and treatment of D-TGA is paramount to the prognosis of the patient. Improved surgical techniques have made it possible for patients with D-TGA to survive into adulthood.

Long-term myocardial performance of the systemic right ventricle during exercise in patients with transposition of the great arteries and atrial switch operation

ObjectiveThe echocardiographic assessment of the systemic right ventricle (sRV) performance during stress testing is limited and evaluation is not routinely performed. The aim of the study is to investigate sRV myocardial performance at rest and with exercise in patients with complete transposition of the great arteries (dTGA) who have undergone atrial switch operation. MethodsIn a single-center cross-sectional study, 41 patients with dTGA following the atrial switch operation and gender-matched 20 healthy volunteers underwent exercise echocardiography on a bicycle ergometer in the semi-supine position to assess sRV systolic function indices: tricuspid annular plane systolic excursion (TAPSE), right ventricular area change (FAC), global longitudinal strain (GLS) and systemic velocity time integral (VTI). ResultsPatients with sRV were characterized by lower systolic function assessed by TAPSE, s’, FAC, GLS both at baseline and at peak exercise, compared with the control group. sRV GLS decreased during exercise in patients with sRV (−6 + 2.84) compared to increased in patients with systemic left ventricle (0.47 + 2.74), p < 0.001. There was no increase in VTI during exercise in patients with sRV, compared to controls (Δ VTI −0.01 ± 2.96 cm vs. Δ VTI 4.50 ± 3.13 cm, p < 0.001). There was a trend towards higher chronotropic incompetence in patients with sRV vs. control (61% vs. 45%, p = 0.28). ConclusionOur results confirmed that patients with dTGA have reduced ability to increase myocardial contractility and stroke volume during exercise. Chronotropic incompetence was prevalent in dTGA patients.

Long-term Outcomes of Surgical Repair for Corrected Transposition of the Great Arteries

BackgroundThis study investigated the long-term outcomes of physiological and anatomical repair for corrected transposition of the great arteries and double-outlet right ventricle with discordant atrioventricular connection. MethodsThis single-center retrospective study included 146 patients who underwent biventricular repair of corrected transposition of the great arteries or double-outlet right ventricle with discordant atrioventricular connections from 1972 to 2023. Survival rate, freedom from reoperation, New York Heart Association (NYHA) Functional Classification, and incidence of systemic ventricular dysfunction in the long-term were compared between physiological repair (PR) and anatomical repair (AR) groups. ResultsThe PR group consisted of 55 patients, with median age at repair of 10.3 years. Thirty-one patients underwent conventional Rastelli procedure, and 24 patients underwent atrial and/or ventricular septal defect closure. The AR group consisted of 91 patients, with median age at repair of 5.8 years. Seventy-two patients underwent atrial switch plus Rastelli procedure and 19 patients underwent atrial plus arterial switch operation. The 30-year survival was 63.5% in the PR group and 72.3% in the AR group (P = .448). The 30-year freedom from reoperation was 71.9% in the PR group and 62.2% in the AR group (P = .220). There was a significant difference in the incidence of systemic ventricular dysfunction between the groups (87.5% in the PR group and 35.3% in the AR group, P < .001) and in the NYHA classification of long-term survivors (mean NYHA class of 1.9 in the PR group and 1.5 in the AR group, P = .009). ConclusionsThe systemic ventricular function and general status in the long-term were significantly better in AR patients, suggesting the potential advantage of AR.

Transbaffle or retrograde aortic approach for atrial arrhythmias ablation in patients with D-TGA treated with atrial switch surgery

Abstract Introduction Patients with D- transposition of the great arteries (TGA) treated with Senning or Mustard surgeries have high incidence of supraventricular tachycardias (SVTs). Accessing to the pulmonary venous atrium (PVAt) is frequently required to perform the ablation, however it can be challenging. Aim To investigate the differences between a retrograde aortic approach (Re-Aa) and transbaffle approach (TBa) for PVAt ablation in this set of patients. Methods Prospective observational study in a third level hospital since April 2018 until October 2022, with a medium-term follow-up. All consecutive patients (pt) with history of SVT and atrial switch surgery that underwent electrophysiologic study (EP) and electroanatomic mapping using a high-density Grid mapping catheter and a contact-force ablation catheter were included. Results A total of 25 EPs were performed in 20 pt (13 (52%) Female, median age 37 y.o [IQR 34-41], median follow-up time 13 month [IQR 10-37]). In 14 pt the PVAt was mapped and ablated via Re-Aa, and in 11 with TBa. Table 1 shows basal patients characteristics, note that no significant differences between groups were found. In 19 pt (76%) a CTI-dependent flutter was documented (10 (71%) in Re-Aa group and 9 (82%) in TBa, p=0.6). Furthermore, in 7 pt other tachycardia circuits were found. Accurate mapping of all the CTI walls were achieved with both strategies, however, the posterior PVAt wall and the pulmonary veins were suboptimal mapped with Re-Aa due to a lack of catheter contact. Acute ablation success was 83% in Re-Aa and 100% in TBa (p=0.5). Despite a higher recurrence rate in the Re-Aa in the first months (20% vs 0%), no significant differences in recurrences were found between groups within 1 year of follow-up (HR 1.3 [95%CI: 0,2-7], p=0.8) (FIGURE). Complication rate was 20% in Re-Aa and 0% in TBa. All complications were directly related to the arterial vascular access (1 femoral pseudoaneurysm, 1 iliac dissection, 1 mediastinal hematoma), and were successfully treated. Conclusion In D-TGA treated with atrial switch surgery, PVAt mapping and ablation is feasible with both strategies with a high acute success rate and without significant differences in recurrences at mid-term follow up. However, TBa is associated with a lower complications rate and also with a better catheter contact and accurate mapping in the PVAt posterior wall.

High-Throughput Genomics Identify Novel FBN1/2 Variants in Severe Neonatal Marfan Syndrome and Congenital Heart Defects.

Fibrillin-1 and fibrillin-2, encoded by FBN1 and FBN2, respectively, play significant roles in elastic fiber assembly, with pathogenic variants causing a diverse group of connective tissue disorders such as Marfan syndrome (MFS) and congenital contractural arachnodactyly (CCD). Different genomic variations may lead to heterogeneous phenotypic features and functional consequences. Recent high-throughput sequencing modalities have allowed detection of novel variants that may guide the care for patients and inform the genetic counseling for their families. We performed clinical phenotyping for two newborn infants with complex congenital heart defects. For genetic investigations, we employed next-generation sequencing strategies including whole-genome Single-Nucleotide Polymorphism (SNP) microarray for infant A with valvular insufficiency, aortic sinus dilatation, hydronephrosis, and dysmorphic features, and Trio whole-exome sequencing (WES) for infant B with dextro-transposition of the great arteries (D-TGA) and both parents. Infant A is a term male with neonatal marfanoid features, left-sided hydronephrosis, and complex congenital heart defects including tricuspid regurgitation, aortic sinus dilatation, patent foramen ovale, patent ductus arteriosus, mitral regurgitation, tricuspid regurgitation, aortic regurgitation, and pulmonary sinus dilatation. He developed severe persistent pulmonary hypertension and worsening acute hypercapnic hypoxemic respiratory failure, and subsequently expired on day of life (DOL) 10 after compassionate extubation. Cytogenomic whole-genome SNP microarray analysis revealed a deletion within the FBN1 gene spanning exons 7-30, which overlapped with the exon deletion hotspot region associated with neonatal Marfan syndrome. Infant B is a term male prenatally diagnosed with isolated D-TGA. He required balloon atrial septostomy on DOL 0 and subsequent atrial switch operation, atrial septal defect repair, and patent ductus arteriosus ligation on DOL 5. Trio-WES revealed compound heterozygous c.518C>T and c.8230T>G variants in the FBN2 gene. Zygosity analysis confirmed each of the variants was inherited from one of the parents who were healthy heterozygous carriers. Since his cardiac repair at birth, he has been growing and developing well without any further hospitalization. Our study highlights novel FBN1/FBN2 variants and signifies the phenotype-genotype association in two infants affected with complex congenital heart defects with and without dysmorphic features. These findings speak to the importance of next-generation high-throughput genomics for novel variant detection and the phenotypic variability associated with FBN1/FBN2 variants, particularly in the neonatal period, which may significantly impact clinical care and family counseling.

External Validation of a Risk Score Model for Predicting Major Clinical Events in Adults After Atrial Switch.

A risk model has been proposed to provide a patient individualized estimation of risk for major clinical events (heart failure events, ventricular arrhythmia, all-cause mortality) in patients with transposition of the great arteries and atrial switch surgery. We aimed to externally validate the model. A retrospective, multicentric, longitudinal cohort of 417 patients with transposition of the great arteries (median age, 24 years at baseline [interquartile range, 18-30]; 63% men) independent of the model development and internal validation cohort was studied. The performance of the prediction model in predicting risk at 5 years was assessed, and additional predictors of major clinical events were evaluated separately in our cohort. Twenty-five patients (5.9%) met the major clinical events end point within 5 years. Model validation showed good discrimination between high and low 5-year risk patients (Harrell C index of 0.73 [95% CI, 0.65-0.81]) but tended to overestimate this risk (calibration slope of 0.20 [95% CI, 0.03-0.36]). In our population, the strongest independent predictors of major clinical events were a history of heart failure and at least mild impairment of the subpulmonary left ventricle function. We reported the first external validation of a major clinical events risk model in a large cohort of adults with transposition of the great arteries. The model allows for distinguishing patients at low risk from those at intermediate to high risk. Previous episode of heart failure and subpulmonary left ventricle dysfunction appear to be key markers in the prognosis of patients. Further optimizing risk models are needed to individualize risk predictions in patients with transposition of the great arteries.

Severe hypoalbuminemia complicating a successful atrial switch operation (Senning) with cardiopulmonary bypass - report on a rare case

Post-operative hypoalbuminemia has been reported in 12% of neonates and 20% of infants; 97% occurs in the first 48 hours in neonates and infants undergoing cardiac surgery with cardiopulmonary bypass (CPB). Perioperative hypoalbuminemia is not independently associated with any post-operative morbidity. However, when analysed as a continuous variable, lower serum albumin levels have been reported to be associated with increased post-operative morbidity. Pre-operative low serum albumin level is independently associated with increased odds of post-operative hypoalbuminemia, and prolonged length of hospital stay. Immediate post-CPB (0-24 hour), lower serum albumin level is independently associated with an increased duration of mechanical ventilation. There is a relative paucity of literature on the impact of hypoalbuminemia in the paediatric population. Hypoalbuminemia is a marker of poor outcome in critically ill children and those undergoing a wide range of medical interventions. Albumin infusions may be an effective therapy for fluid resuscitation and for patients with severe hypoalbuminemia. We present a successful perioperative management of a 3 months old-male child, weighing 3.5 kg with dextro-transposition of great arteries (D-TGA) with regressed LV undergoing an atrial switch (Senning) procedure, and uneventful intraoperative course i.e. easy weaning from CPB. However, in the ICU, the baby suffered an unusual complication as severe hypoproteinaemia resulting in acute kidney injury (AKI) with complete anuria, generalized anasarca, hypotension requiring inotropes and vasopressors, and difficult weaning from ventilator.

Continuous Long-Term Assessment of Heart Rate Variability in Adults with Cyanotic Congenital Heart Disease after Surgical Repair.

Background: Heart rate variability (HRV) is an established, non-invasive parameter for the assessment of cardiac autonomic nervous activity and the health status in general cardiology. However, there are few studies on HRV in adults with congenital heart defects (CHDs). The aim of the present study was to evaluate the use of long-term continuous HRV measurement for the assessment of global health status in adults with cyanotic CHD. Methods: This prospective study included 45 adults (40% female, mean age = 35.2 ± 9.2 [range: 19-58] years) after cardiac surgical repair. HRV parameters were calculated from continuous 24 h measurements using a Bittium Faros 180 sensor (Bittium Corp., Oulu, Finland). Results: Postoperative patients with transposition of the great arteries (TGA) (n = 18) achieved significantly higher values of standard deviation of NN intervals (SDNN) (175.4 ± 59.9 ms vs. 133.5 ± 40.6 ms; p = 0.013) compared with patients with other conotruncal anomalies (n = 22). Comparing patients with TGA after a Senning-Brom or Mustard operation (n = 13) with all other heart surgery patients (n = 32), significantly higher HRV parameters were found after atrial switch (root mean square of successive RR interval differences: 53.6 ± 20.7 ms vs. 38.4 ± 18.3 ms; p = 0.019; SDNN: 183.5 ± 58.4 ms vs. 136.3 ± 45.3 ms; p = 0.006). A higher SDNN was also measured after Senning-Brom or Mustard operations than after a Rastelli operations (n = 2) (SDNN: 183.5 ± 58.4 ms vs. 84.5 ± 5.2 ms; p = 0.037). When comparing atrial switch operations (n = 3) with Rastelli operations, the SDNN value was significantly shorter in the Rastelli group (p = 0.004). Conclusions: Our results suggest that continuous HRV monitoring may serve as a marker of cardiac autonomic dysfunction in adults with cyanotic CHD after surgical repair. Impaired cardiac autonomic nervous activity may be associated with an increased risk of adverse reactions in patients with repaired CHD. Therefore, a longitudinal assessment of HRV patterns and trends may provide a deeper insight into dynamic changes in their autonomic regulation and disease progression, lifestyle changes, or treatments. As each person has individual variability in heart rate, HRV may be useful in assessing intra-individual disease progression and may help to improve personalized medicine. Further studies are needed to better understand the underlying mechanisms and to explore the full potential of HRV analysis to optimize medical care for ACHDs.

Prognostic significance of echocardiographic deformation imaging in adult congenital heart disease.

Due to medical and surgical advancements, the population of adult patients with congenital heart disease (ACHD) is growing. Despite successful therapy, ACHD patients face structural sequalae, placing them at increased risk for heart failure and arrhythmias. Left and right ventricular function are important predictors for adverse clinical outcomes. In acquired heart disease it has been shown that echocardiographic deformation imaging is of superior prognostic value as compared to conventional parameters as ejection fraction. However, in adult congenital heart disease, the clinical significance of deformation imaging has not been systematically assessed and remains unclear. According to the Preferred Reporting Items for Systematic Reviews checklist, this systematic review included studies that reported on the prognostic value of echocardiographic left and/or right ventricular strain by 2-dimensional speckle tracking for hard clinical end-points (death, heart failure hospitalization, arrhythmias) in the most frequent forms of adult congenital heart disease. In total, 19 contemporary studies were included. Current data shows that left ventricular and right ventricular global longitudinal strain (GLS) predict heart failure, transplantation, ventricular arrhythmias and mortality in patients with Ebstein's disease and tetralogy of Fallot, and that GLS of the systemic right ventricle predicts heart failure and mortality in patients post atrial switch operation or with a congenitally corrected transposition of the great arteries. Deformation imaging can potentially impact the clinical decision making in ACHD patients. Further studies are needed to establish disease-specific reference strain values and ranges of impaired strain that would indicate the need for medical or structural intervention.

CASE-TA-05 - A Baffle Leak with Right-to-left Shunting in an Adult with D-TGA After Atrial Switch Operation

CASE-TA-05 - A Baffle Leak with Right-to-left Shunting in an Adult with D-TGA After Atrial Switch Operation

Long-Term outcomes in adult patients with congenital heart disease considered for transplantation: A single center study.

Adult congenital heart disease (ACHD) patients pose unique challenges in identifying the time for transplantation and factors influencing outcomes. To identify hemodynamic, functional, and laboratory parameters that correlate with 1- and 10-year outcomes in ACHD patients considered for transplantation. A retrospective chart review of long-term outcomes in adult patients with congenital heart disease (CHD) evaluated for heart or heart + additional organ transplant between 2004 and 2014 at our center was performed. A machine learning decision tree model was used to evaluate multiple clinical parameters correlating with 1- and 10-year survival. We identified 58 patients meeting criteria. D-transposition of the great arteries (D-TGA) with atrial switch operation (20.7%), tetralogy of Fallot/pulmonary atresia (15.5%), and tricuspid atresia (13.8%) were the most common diagnosis for transplant. Single ventricle patients were most likely to be listed for transplantation (39.8% of evaluated patients). Among a comprehensive list of clinical factors, invasive hemodynamic parameters (pulmonary capillary wedge pressure (PCWP), systemic vascular pressure (SVP), and end diastolic pressures (EDP) most correlated with 1- and 10-year outcomes. Transplanted patients with SVP<14 and non- transplanted patients with PCWP<15 had 100% survival 1-year post-transplantation. For the first time, our study identifies that hemodynamic parameters most strongly correlate with 1- and 10-year outcomes in ACHD patients considered for transplantation, using a data-driven machine learning model.

Overview of Long-Term Outcome in Adults with Systemic Right Ventricle and Transposition of the Great Arteries: A Review.

The population of patients with a systemic right ventricle (sRV) in biventricular circulation includes those who have undergone an atrial switch operation for destro-transposition of the great arteries (d-TGA) and those with congenitally corrected transposition of the great arteries (ccTGA). Despite the life expectancy of these patients is significantly increased, the long-term prognosis remains suboptimal due to late complications such as heart failure, arrhythmias, and premature death. These patients, therefore, need a close follow-up to early identify predictive factors of adverse outcomes and to implement all preventive therapeutic strategies. This review analyzes the late complications of adult patients with an sRV and TGA and clarifies which are risk factors for adverse prognosis and which are the therapeutic strategies that improve the long-term outcomes. For prognostic purposes, it is necessary to monitor sRV size and function, the tricuspid valve regurgitation, the functional class, the occurrence of syncope, the QRS duration, N-terminal pro B-type natriuretic peptide levels, and the development of arrhythmias. Furthermore, pregnancy should be discouraged in women with risk factors. Tricuspid valve replacement/repair, biventricular pacing, and implantable cardioverter defibrillator are the most important therapeutic strategies that have been shown, when used correctly, to improve long-term outcomes.

Myocardial work of the systemic right ventricle and its association with outcomes

Abstract Funding Acknowledgements Type of funding sources: None. Background Impaired systolic function of the systemic right ventricle (SRV) is well-known factor associated with adverse outcomes in patients with transposition of great arteries after atrial switch operation or with congenitally corrected transposition of great arteries. A recently proposed echocardiographic method of evaluating the RV myocardial work enables quantitative assessment of myocardial deformation also accounting for afterload and myocardial dyssynchrony. Purpose To evaluate clinical and prognostic significance of myocardial work parameters of the SRV. Methods Adult patients with the SRV were enrolled in a single-centre prospective observational study.SRV systolic function was assessed by 3D-echocardiography (3DE) derived ejection fraction (EF), 2D longitudinal strain and myocardial work analysis. The study endpoint was the combination of all-cause mortality and heart transplantation. Results 38 patients were included (mean age 45 ± 12 years, 63% male). Global constructive work (GCW) and global work index (GWI) demonstrated moderate correlation with the 3DE SRV EF (Rho 0.64, p&amp;lt;0.0001 and Rho 0.63, p&amp;lt;0.0001, respectively). GCW showed strong correlation with the BNP level (Rho −0.77, p&amp;lt;0.0001), closely followed by GWI, 4-chamber longitudinal strain and 3DE EF (all Rho −0.73, p&amp;lt;0.0001). GCW and GWI were significantly lower in patients with moderate or severe tricuspid regurgitation compared with less than moderate regurgitation (1226±439 vs 1509±264mmHg%, p = 0.02, and 984±348 vs 1259±278 mmHg%, p = 0.01, respectively). During a follow-up period of 3.5 (2.8–3.9) years, 7 patients (18%) died and one received transplantation (3%). They had significantly lower GCW and GWI compared with patients who did not reach the study endpoint (Figure 1). Cox regression including GCW, GWI and 3DE SRV EF were the best-fit models based on the Akaike Information Criterion, outperforming longitudinal strain parameters (Figure 2). Conclusion GWI and GCW provided reliable quantification of the SRV systolic function. GWI, GCW and 3DE-derived SRV parameters were the best predictors of all-cause mortality and heart transplantation in patients with the SRV.

Intracavitary blood flow dynamics in the systemic right ventricle: a 4D-Flow MRI study

Abstract Funding Acknowledgements Type of funding sources: None. Background Failure of the systemic right ventricle (SRV) is based on morphological differences between right and left ventricles (RVs and LVs). RV adaptation to systemic afterload includes increased circumferential (GCS) over longitudinal global myocardial strain (GLS) with an unknown impact on intracavitary blood flow distribution. This study aimed to explore the SRV pattern of hemodynamic forces (HFs). Methods 4D-Flow cardiovascular magnetic resonance data were acquired using a prototype sequence on a 1.5-T MAGNETOM Aera. The ratio between transverse (inferior-anterior, HFIA and septal-lateral, HFSL) and longitudinal (basal-apical, HFBA) HFs (RRMS) was calculated as in Figure 1, for systole and diastole. Results We enrolled 12 adults with SRV (6 D-transposition of great arteries after atrial switch operation and 6 L-transpositions) and 12 age-matched healthy subjects (41±12 vs 42±13,p = 0.89). SRVs reported comparable end-diastolic volumes (83±18 ml/m2), ejection fraction (60±8%) and GLS (−20.2±3.8%) to control RVs (75±13 ml/m2,p = 0.18; 64±5%,p = 0.25; −23±5.6%,p = 0.21). Differently, SRV mass (55±24 g/m2) and GCS (−18.9±7.8%) were greater than RVs (20±3 g/m2,p&amp;lt;0.001 and −12.4±4,p = 0.016) and comparable to control LVs (57±11 g/m2,p = 0.7 and −20.2±3.8%,p = 0.3). The 4D-Flow analysis showed that SRV systolic RRMS (0.98±0.31) was similar to LVs (0.94±0.27,p = 0.78) but lower than RVs (1.32±0.45,p = 0.04). This reflected a significantly increased HFBA with respect to RVs (0.338±0.150 vs. 0.162±0.097, p = 0.0025) and similar to LVs (0.462 ± 0.186, p = 0.087). Concomitantly, a moderate correlation was demonstrated between SRV systolic HFBA magnitude and GCS (r2=0.47,p = 0.013). During diastole, SRVs showed lower HFBA (0.173±0.086) than LVs (0.304±0.104,p = 0.0028), revealing a diastolic RRMS (0.74 ± 0.14) comparable to RVs (0.73 ± 0.17,p = 0.95) and significantly different from LVs (0.50 ± 0.19,p = 0.003). Conclusions In SRVs, RRMS is similar to LVs during systole, possibly as a result of increased GCS. Inversely, the SRV filling appears to be closely related to ventricular morphology as suggested by RRMS comparable to RVs during diastole.

Myocardial Perfusion Scintigraphy in a Patient With Transposition of Great Arteries Post-Atrial Switch Surgery.

We report a case of a 33-year-old woman who underwent stress and rest myocardial perfusion scintigraphy (MPS) to exclude coronary artery disease. MPS images showed an apparent dextrocardia with a right-sided septal wall uptake. The electrocardiograph showed a right axis deviation with dominant R waves at leads aVR and V1. Upon retrieval of the patient's medical records, she had an underlying transposition of great arteries and underwent a Senning atrial switch surgery. Hence, the MPS images demonstrated a prominent right ventricular wall due to its function as the "systemic" ventricle with minimal uptake in the "pulmonary" left ventricle.

Long Term Follow-Up of Patients with Systemic Right Ventricle and Biventricular Physiology: A Single Centre Experience.

A progressively increasing prevalence of congenital heart disease (CHD) in adulthood has been noticed in recent decades; CHD cases with a systemic right ventricle have a poorer outcome. Seventy-three patients with SRV evaluated in an outpatient clinic between 2014 and 2020 were enrolled in this study. Thirty-four patients had a transposition of the great arteries treated with an atrial switch operation; 39 patients had a congenitally corrected transposition of the great arteries (ccTGA). Mean age at the first evaluation was 29.6 ± 14.2 years; 48% of the patients were female. The NYHA class at the visit was III or IV in 14% of the cases. Thirteen patients had at least one previous pregnancy. In 25% of the cases, complications occurred during pregnancy. Survival free from adverse events was 98.6% at one year and 90% at 6-year follow-up without any difference between the two groups. Two patients died and one received heart transplantation during follow-up. The most common adverse event during follow-up was the presence of arrhythmia requiring hospitalization (27.1%), followed by heart failure (12.3%). The presence of LGE together with lower exercise capacity, higher NYHA class and more dilated and/or hypokinetic RV predicted a poorer outcome. Quality of life was similar to the QoL of the Italian population. Long-term follow-up of patients with a systemic right ventricle is characterized by a high incidence of clinical events, prevalently arrhythmias and heart failure, which cause most of the unscheduled hospitalizations.

EVAHEART 2 with double cuff tipless inflow cannula is suitable for long-term support atrial switch operation in transposition of great arteries.

Implantation of continuous-flow left ventricular assist device in a narrow lumen is technically challenging to secure an optimal support. We experienced a patient with the transposition of the great arteries after the Senning procedure who was initially implanted with Jarvik 2000®. She presented with worsening heart failure symptoms 2 years after implanting Jarvik 2000®. We assumed that the inflow cannula was stuck in the highly developed trabeculae on the interventricular septum, which disturbed the VAD to maintain an expected support. After converting to the EVAHEART® 2, we successfully obtained an adequate inflow. We consider that the tipless cannula of EVAHEART® 2 is the most suitable when there is no sufficient room to place a conventional inflow cannula in the systemic ventricle.