Echocardiography is a standard tool for assessing right ventricular (RV) function in the perioperative setting, but in high-risk cases, additional monitoring may be required. Patients with pulmonary hypertension, pre-existing RV failure, or undergoing complex surgeries (eg, pulmonary endarterectomy, LVAD implantation, or transplantation) are particularly vulnerable. Catheter-based techniques, such as pulmonary artery catheterization (PAC), provide continuous, functional data and may be valuable in intensive care or when echocardiography is limited. Despite concerns over complications, PACs can help assess hemodynamics, cardiac output, and RV performance, aiding early detection of RV failure in select high-risk patients.

Surgical management of congenital heart disease encompasses a spectrum of procedures, ranging from biventricular repair to univentricular palliation, each tailored to the specific anatomical and hemodynamic features of individual cases. Among these, biventricular repair, which preserves a functional ventricle to sustain pulmonary circulation, is prioritized whenever feasible. Advances in approaches have significantly improved outcomes, enabling many patients with congenital heart disease to reach adulthood, including the majority who have undergone biventricular repair. Despite these advancements, long-term complications-such as valvular disease, arrhythmias, heart failure, outflow tract obstruction, and dysfunction of extracardiac conduits-pose persistent challenges in the lifelong care of these patients. This review examines the distinct challenges and management strategies associated with adult patients who have undergone biventricular repair for complex congenital heart disease. The discussion focuses on key conditions, including repaired tetralogy of Fallot, transposition of the great arteries following atrial or arterial switch procedures, surgically managed or untreated congenitally corrected transposition of the great arteries with significant tricuspid regurgitation necessitating intervention, pulmonary atresia with intact ventricular septum, and Ebstein's anomaly. By addressing the long-term complications and therapeutic considerations unique to this patient population, this review aims to provide a comprehensive framework for optimizing care as these individuals transition into adulthood.

This study investigates outcomes of the reverse double switch operation (R-DSO) and ventricular switch, novel approaches for patients with D-looped borderline left hearts that utilize the morphologic right ventricle as the systemic ventricle and the hypoplastic left ventricle as the subpulmonary ventricle.

This retrospective review analyzed early outcomes of children who underwent R-DSO/ventricular switch at a single institution between 2015 and 2023. Our primary end points were right ventricular (RV) function and tricuspid regurgitation. Secondary outcomes included mortality, reoperation, and perioperative complications.

Twenty-eight patients underwent either R-DSO (n = 19) or ventricular switch (n = 9). In 19 patients, a decompressing bidirectional cavopulmonary shunt was utilized, creating a reverse 1.5 switch. Median age at R-DSO/ventricular switch was 3.1 years (range, 9 months-12 years). At a median follow-up of 1.0 year (range, 38 days-7.2 years), no mortalities or heart transplants had occurred. Mild-moderate or greater RV dysfunction was detected in 4 patients, and mild-moderate or worse tricuspid regurgitation was also detected in 4 patients. Three patients required reoperations. Preoperative RV ejection fraction <55% was associated with higher incidence of postoperative RV dysfunction.

The R-DSO/VS strategy is an alternative to single-ventricle palliation or anatomic biventricular repair in the borderline left heart. Concerns for RV dysfunction and tricuspid regurgitation mandate close monitoring. Patients with preoperative RV dysfunction may be at risk for postoperative RV dysfunction. Further studies with longer follow-up are needed to delineate outcomes in comparison to the Fontan pathway and identify optimal candidates for this novel strategy.

Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart anomaly that often leads to systemic heart failure, necessitating mechanical circulatory support or transplantation. We report a case of a 54 year old male diagnosed with dextrocardia and ccTGA (I, D, D), who had been suffering from congestive heart failure for over 7 years. Despite receiving intensive treatment, his condition deteriorated. Preoperative evaluation revealed significant systemic right ventricular dysfunction with severe valvular regurgitation and pulmonary hypertension. The surgical approach included tricuspid and aortic bioprosthetic valve replacement, mitral valve annuloplasty, and right thoracic ventricular assist device (VAD) implantation. The patient recovered well postoperatively and was discharged on postoperative day 39 with New York Heart Association (NYHA) class I. This case demonstrates the feasibility of using VAD in right heart ccTGA as a bridge to transplantation or destination therapy, emphasizing the importance of meticulous preoperative planning and intraoperative management for successful outcomes.

Broadly speaking, right heart failure (RHF) can be defined as a clinical syndrome with signs and symptoms of heart failure resulting from right ventricular dysfunction, as evidenced by abnormal structure or function. The heterogeneity of conditions associated with RHF combined with the challenges of characterizing the structural and functional relationships of the right ventricle make a singular definition of RHF elusive. We performed a concise scoping review of the literature that provides knowledge synthesis of RHF in different clinical populations, and extracted the recommended criteria to define the syndrome.

Searches were conducted to identify reviews, guidelines, consensus statements, systematic reviews, meta-analyses, and consensus statements concerning right heart or right ventricular failure in well-described adult human disease populations from the previous 10 years. After removal of case reports and duplicates, publications describing syndromes in the context of left-sided heart disease were also removed. Data extracted from selected manuscripts included the patient population and quantitative criteria to define RHF, categorized based on diagnostic modalities.

Of 9487 articles initially identified, 71 were selected for full text review. We found that the majority of the reviewed literature offered multifaceted diagnostic approaches, including clinical, echocardiographic, hemodynamic, and therapeutic characteristics, with unique additions or omissions depending on the clinical contexts. Nomenclature for RHF was variable, and only 40% of articles articulated a narrative definition.

At this time, development of consistent criteria and a universal definition for RHF remains a work in process.

Patients with systemic right ventricle (SRV), either d-transposition of the great arteries following an atrial switch procedure or congenitally corrected transposition of the great arteries, develop severe right ventricular dysfunction, prompting appropriate medical therapy. However, the efficacy of beta-blockers and angiotensin receptor blockers or angiotensin-converting enzyme inhibitors (ACEI) in SRV patients is unproven.

The objective of this study was to determine the effects of ACEI/ARB and beta-blockers on outcomes in SRV patients after accounting for likely cofounders affecting their use.

From a retrospective, multicenter study on heart failure-related outcome in individuals with SRV, those who were taking an ACEI/ARB, beta-blocker, or both of these medication were identified. We performed a propensity analysis to match them to those not using these medications at their initial visit. Matching was based on a propensity score, which captured co-morbidities, demographics, and baseline echocardiographic parameters. Primary outcome of death, transplant, or mechanical circulatory support, and secondary outcomes of heart failure hospitalizations/atrial arrhythmias were analyzed respectively.

We identified 393 patients taking ACEI/ARB or beta-blocker, or taking both a beta-blocker and ACEI/ARB (62.1% male, median age 31.3 years) and 484 patients (56.4% male, median age of 26.0 years) who were neither on a beta-blocker nor on ACEI/ARB at the time of initial clinic visit. Median follow-up was ∼8 years. After propensity matching, medication use was not associated with decreased mortality, heart failure hospitalizations, or arrhythmias. Hazard ratios remained positive for beta blockers, implying potential harm rather than benefit.

In this large multicenter propensity-matched observational study, patients with SRV taking beta-blockers or ACEI/ARB did not have a benefit in survival or reduced hospitalization. The likelihood of demonstrating favorable effects in larger studies appears remote.

A 35-year-old man with congenitally corrected transposition of the great arteries (ccTGA) and prior ventricular septal defect repair developed pacing-induced cardiomyopathy after 30 years of single-chamber VVI pacemaker use. Right bundle branch area pacing (RBBAP) was chosen for device upgrade due to its potential benefits over conventional cardiac resynchronization therapy. Advanced three-dimensional electroanatomic mapping was employed to navigate a synthetic patch from previous surgery and guide mid-septal electrode placement. Successful RBBAP was achieved with optimal parameters, complemented by atrial and defibrillator lead implantation. At 12 months, the patient showed improved ejection fraction (from 30% to 40%) and stable electrocardiographic results. This case illustrates the role of RBBAP and advanced mapping in addressing complex structural and conduction abnormalities in ccTGA.

Congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac anomaly. The management strategy historically consisted of physiologic repair, leaving the morphologic right ventricle to support the systemic circulation. More recently, anatomic repair has been implemented to bring the left ventricle into the systemic circulation. Uncertainty persists about which repair strategy has the best outcome.

This meta-analysis aimed to summarize the long-term mortality risks following anatomic and physiologic repair of ccTGA.

PubMed, Embase, and the Cochrane Database were searched. Data were extracted using prespecified data forms. The primary outcome was the composite risk of all-cause mortality or heart transplantation during hospitalization and at 1, 5, and 10 years of follow-up. Secondary outcomes included reintervention risk.

Forty-seven studies totaling 2,844 patients were included. The incidence risk of mortality at 10 years was 11.7% (95% CI: 8.5%-15.3%) and 17.4% (95% CI: 12.4%-23.0%) in the anatomic and physiologic repair groups, respectively. The incidence risk of reintervention at 10 years was 24.5% (95% CI: 19.2%-30.1%) and 30.3% (95% CI: 23.5%-37.6%), respectively. The primary outcome was significantly lower at 10 years in anatomically repaired patients who had surgery at <5 years of age and who had preoperative pulmonary artery banding (Pheterogeneity < 0.01).

Anatomic repair of ccTGA patients results in higher overall and reintervention-free survival compared to physiologic repair. Specifically, patients who had anatomic repair at <5 years of age or who had preoperative pulmonary artery banding have better survival.

Congenitally corrected transposition of the great arteries (ccTGA) is an infrequent and complex congenital malformation, which accounts for approximately 0.5% of all congenital heart defects. This defect is characterized by both atrioventricular and ventriculoarterial discordance, with the right atrium connected to the morphological left ventricle (LV), ejecting blood into the pulmonary artery, while the left atrium is connected to the morphological right ventricle (RV), ejecting blood into the aorta. Due to this double discordance, the blood flow is physiologically normal. Most patients have coexisting cardiac abnormalities that require further treatment. Untreated natural course is often associated with progressive failure of the systemic right ventricle (RV), tricuspid valve (TV) regurgitation, arrhythmia, and sudden cardiac death, which occurs in approximately 50% of patients below the age of 40. Some patients do not require surgical intervention, but most undergo physiological repair leaving the right ventricle in the systemic position, anatomical surgery which restores the left ventricle as the systemic ventricle, or univentricular palliation. Various types of anatomic repair have been proposed for the correction of double discordance. They combine an atrial switch (Senning or Mustard procedure) with either an arterial switch operation (ASO) as a double-switch operation or, in the cases of relevant left ventricular outflow tract obstruction (LVOTO) and ventricular septal defect (VSD), intra-ventricular rerouting by a Rastelli procedure. More recently implemented procedures, variations of aortic root translocations such as the Nikaidoh or the half-turned truncal switch/en bloc rotation, improve left ventricular outflow tract (LVOT) geometry and supposedly prevent the recurrence of LVOTO. Anatomic repair for congenitally corrected ccTGA has been shown to enable patients to survive into adulthood.

Ventricular remodeling leads to fibrotic changes in systemic right ventricles (RV). Native T1 mapping provides a quantitative measure in myocardial tissue characterization. The aim of our study was to correlate native T1 values of the systemic RV to function and volumetric data. Native T1 maps were generated with a single breath hold Modified Look-Locker Inversion-recovery pulse (MOLLI) sequence was acquired in the mid-ventricular short axis. Regions of interest (ROI) were drawn in both ventricular free walls, the interventricular septum (IVS), superior insertion point (SIP) and inferior insertion point (IIP) to obtain native T1 values. T1 values were compared to CMR ventricular volumes and function using Spearman correlation. The median age was 36 years (IQR 27-48 years). There were elevated mean native left ventricular (LV) T1 and IIP T1 values at 1122 ± 171 ms and 1117 ± 96 ms, respectively. RV dysfunction was associated with elevated IIP T1 (p = 0.007). Significant moderate negative correlations were seen between RV T1 and LV ejection fraction (LVEF) (r= -0.63, p = 0.01), between RV: IVS T1 ratio and LVEF (r= -0.68, p = 0.006), between LVEF and SIP: IVS T1 ratios (r= -0.54, p = 0.04), and RVEF and IIP T1 (r= -0.59, p = 0.02). Fibrosis measured by native T1 mapping in the systemic RV is most prominent in the LV wall and septal insertion point and correlates with decreased function. T1 values can be used in non-invasive imaging assessment of the RV, but further studies with larger cohorts are needed to assess ability to risk stratify and guide therapy.

There is a paucity of data regarding sex-related differences on cardiac outcomes in the context of transposition of the great arteries (TGA) with a systemic right ventricle and biventricular physiology (sRV-biV). Moreover, the long-term impact of pregnancy on cardiac outcomes remains unknown.

The purpose of this study was to identify sex-related differences and the influence of pregnancy on cardiac outcomes in TGA sRV-biV population.

A retrospective cohort study was conducted on 213 adults with TGA sRV-biV, 82 (38.4%) women, age 42.6 ± 12.8 years, with a median follow-up of 16 years. Cardiac events, interventions, last follow-up sRV-biV dysfunction, and heart failure (HF) medications were compared between men vs women, and women with vs without pregnancies resulting in live births.

Women had a lower incidence of nonsustained ventricular tachycardia (HR: 1.80; 95% CI: 1.04-3.09, P = 0.035) and nonsignificantly fewer HF-related hospitalizations than men (HR: 2.10; 95% CI: 0.95-4.67, P = 0.069) in univariable analysis. At the last follow-up, women had a lower prevalence of moderate to severe sRV-biV dysfunction than men (P < 0.001) and were less frequently prescribed HF therapy. Women had fewer implantable cardioverter-defibrillators for primary prevention than men (P = 0.016), with no difference for secondary prevention. Women who had pregnancies resulting in live births (N = 47), had a high prevalence of cardiac events in the 15 (IQR: 9-28) years following pregnancy with no significant differences with those without (N = 32) pregnancies.

Women with a sRV-biV have fewer adverse cardiovascular events than men. Due to sRV-biV, pregnancy remains with high maternal risk but is not associated with worse long-term cardiac outcomes under rigorous multidisciplinary cardio-obstetrical care.

Background: Congenitally corrected transposition of the great arteries (cc-TGA) is a defect characterized by arterio-ventricular and atrioventricular disconcordance. Most patients have co-existing cardiac abnormalities that warrant further treatment. Some patients do not require surgical intervention, but most undergo physiological repair or anatomical surgery, which enables them to reach adulthood. Aims: We aimed to evaluate mortality risk factors in patients with cc-TGA. Results: We searched the PubMed database and included 10 retrospective cohort studies with at least a 5-year follow-up time with an end-point of cardiovascular death a minimum of 30 days after surgery. We enrolled 532 patients, and 83 met the end-point of cardiovascular death or equivalent event. As a risk factor for long-term mortality, we identified New York Heart Association (NYHA) class ≥III/heart failure hospitalization (OR = 10.53; 95% CI, 3.17-34.98) and systemic ventricle dysfunction (SVD; OR = 4.95; 95% CI, 2.55-9.64). We did not show history of supraventricular arrhythmia (OR = 2.78; 95% CI, 0.94-8.24), systemic valve regurgitation ≥moderate (SVR; OR = 4.02; 95% Cl, 0.84-19.18), and pacemaker implantation (OR = 1.48; 95% Cl, 0.12-18.82) to affect the long-term survival. In operated patients only, SVD (OR = 4.69; 95% CI, 2.06-10.71) and SVR (OR = 3.85; 95% CI, 1.5-9.85) showed a statistically significant impact on survival. Conclusions: The risk factors for long-term mortality for the entire cc-TGA population are NYHA class ≥III/heart failure hospitalization and systemic ventricle dysfunction. In operated patients, systemic ventricle dysfunction and at least moderate systemic valve regurgitation were found to affect survival.

Systemic atrioventricular valve (morphologic tricuspid valve [TV]) regurgitation has been implicated in the development of systemic ventricular failure in congenitally corrected transposition of the great arteries (ccTGA), leading to timely referral for valve replacement. However, the surgical management of subpulmonary atrioventricular valve (morphologic mitral valve [MV]) regurgitation and outcomes has not been well studied.

Of 108 ccTGA patients undergoing atrioventricular valve surgery from 1979 to 2022, 22 patients (20%) underwent MV surgery. Demographics, etiology of valve regurgitation, operative details, and outcomes of these 22 patients were retrospectively reviewed. Follow-up at 1, 5, and 10 years was available for 18 (82%), 13 (59%), and 11 patients (50%), respectively.

Median age was 37 years (interquartile range, 29-57 years). Intrinsic MV pathology was present in 18 individuals (82%). Operations included repair in 16 patients (73%), replacement in 6 (27%), and concomitant replacement of TV in 16 (73%). There was 1 perioperative death (5%) in a patient undergoing an emergent operation for severe acute-on-chronic heart failure due to worsening TV regurgitation. During a median follow-up of 12 years (interquartile range, 2-19 years), 7 patients (32%) died. Among the 16 patients who underwent repair, recurrent moderate or greater regurgitation was seen in 15%, 29%, and 43% of repairs in patients with annular, leaflet, and lead-induced regurgitation, respectively.

Concomitant TV and MV disease occur much less frequently than isolated TV disease in ccTGA. Intrinsic MV disease is most commonly observed but appears less amenable to successful repair compared with mitral repair in the systemic position and suggests MV replacement may be preferred in ccTGA patients.

We report a case of heart transplantation in a 68-year-old Jehovah's Witness patient with congenitally corrected transposition of the great arteries (ccTGA) who developed heart failure due to right ventricular dysfunction. The patient underwent successful heart transplantation without the use of blood products, employing meticulous hemostasis and careful surgical planning. This case highlights the anatomical considerations and challenges in transplanting a heart in a patient with ccTGA, including the reversed positions of the pulmonary artery and the ascending aorta. It also emphasizes the importance of tailored surgical strategies to achieve hemostasis and avoid blood transfusion in Jehovah's Witness patients. This case adds to the limited literature on heart transplantation in patients with ccTGA and demonstrates that heart transplantation can be successfully performed without blood transfusion in Jehovah's Witness patients.

The right ventricle (RV) is one of the four pumping chambers of the heart, pumping blood to the lungs. In severe forms of congenital heart disease and pulmonary hypertension, the RV is made to pump into the systemic circulation. Such systemic RVs typically display early failure due to pressure overload. In rare cases a systemic RV persists into later decades of life - colloquially called a 'Super RV'. Here we present the single-nucleus transcriptome of a systemic RV from a 60-year-old with congenitally corrected transposition of great arteries (ccTGA). Our data shows two specific signaling pathways enriched in the ccTGA RV myocardium. First, we show increased insulin like growth factor (IGF1) signaling within the systemic RV myocardium: there is increased expression of the main receptor IGFR1 within the cardiomyocytes, and IGF1 ligands within the cardiofibroblasts and macrophages. Second, we find increased VEGF and Wnt9 ligand expression in cardiomyocytes and increased VEGF1R and Wnt9 receptors in endothelial cells, which are implicated in angiogenesis. We show that increased insulin and angiogenesis signaling are potentially beneficial RV adaptations to increased pressure overload. This study of an adult systemic RV provides an important framework for understanding RV remodeling to systemic pressures in congenital heart disease and pulmonary hypertension.

Limited data exist regarding the long-term outcomes of systemic atrioventricular valve (SAVV) intervention (morphologic tricuspid valve) in congenitally corrected transposition (ccTGA).

The goal of this study was to evaluate the mid- and long-term outcomes of SAVV surgery in ccTGA.

We performed a retrospective review of 108 ccTGA patients undergoing SAVV surgery from 1979 to 2022. The primary outcome was a composite endpoint of mortality, cardiac transplantation, or ventricular assist device implantation. The secondary outcome was long-term systemic right ventricular ejection fraction (SVEF). Cox proportional hazard and linear regression models were used to analyze survival and late SVEF data.

The median age at surgery was 39.5 years (Q1-Q3: 28.8-51.0 years), and the median preoperative SVEF was 39% (Q1-Q3: 33.2%-45.0%). Intrinsic valve abnormality was the most common mechanism of SAVV regurgitation (76.9%). There was 1 early postoperative mortality (0.9%). Postoperative complete heart block occurred in 20 patients (18.5%). The actuarial 5-, 10-, and 20-year freedom from death or transplantation was 92.4%, 79.1%, and 62.9%. The 10- and 20-year freedom from valve reoperation was 100% and 93% for mechanical prosthesis compared with 56.6% and 15.7% for bioprosthesis (P < 0.0001). Predictors of postoperative mortality were age at operation (P = 0.01) and preoperative SVEF (P = 0.04). Preoperative SVEF (P < 0.001), complex ccTGA (P = 0.02), severe SAVV regurgitation (P = 0.04), and preoperative creatinine (P = 0.003) were predictors of late postoperative SVEF.

SAVV surgery remains a valuable option for the treatment of patients with ccTGA, with low early mortality and satisfactory long-term outcomes, particularly in those with SVEF ≥40%. Timely referral and accurate patient selection are the keys to better long-term outcomes.

Congenitally corrected levo-transposition of the great arteries (L-TGA) is a congenital heart disease in which the ventricles and great arteries are transposed from their typical anatomy. In L-TGA, the double discordance, atrioventricular and ventriculoarterial, create an acyanotic milieu which allows patients to survive their early decades, however, progressive systemic right ventricle (sRV) dysfunction creates complications later in life. sRV dysfunction and remodeling predisposes patients to intracardiac thrombus (ICT) formation.

A 40-year-old male with L-TGA presented with symptoms of acute decompensated heart failure. In childhood, he had surgical repair of a ventricular septal defect. In adulthood, he developed sRV dysfunction, systemic tricuspid valve (sTV) regurgitation, and left-bundle branch block for which he underwent cardiac resynchronization therapy. Transthoracic echocardiogram showed a sRV ejection fraction of 40%, severe sTV regurgitation, and a newly identified sRV ICT. ICT was confirmed by ultrasound-enhancing agents and transesophageal echocardiography. Our patient was optimized with guideline-directed medical therapy and diuresis. Anticoagulation was achieved with a vitamin K antagonist (VKA) and he was later referred for evaluation by advanced heart failure and heart transplant services.

Anticoagulation with VKA is the mainstay of treatment in the absence of conclusive data supporting direct oral anticoagulant use in ICT in patients with congenital heart disease. This case illustrates the natural history of L-TGA and highlights the importance of surveillance and monitoring with dedicated cardiac imaging to identify complications.

The right ventricle (RV) has complex geometry and function, with motion along three separate axes-longitudinal, radial, and anteroposterior. Quantitative assessment of RV function by two-dimension echocardiography (2DE) has been limited as a consequence of this complexity, whereas newer three dimensional (3D) analysis offers the potential for more comprehensive assessment of the contributors to RV function. The aims of this study were to quantify the longitudinal, radial and anteroposterior components of global RV function using 3D echocardiography in a cohort of healthy children and to examine maturational changes in these parameters.

Three-dimensional contours of the RV were generated from a cohort of healthy pediatric patients with structurally normal hearts at two centers. Traditional 2D and 3D echo characteristics were recorded. Using offline analysis of 3D datasets, RV motion was decomposed into three components, and ejection fractions (EF) were calculated (longitudinal-LEF; radial-REF; and anteroposterior-AEF). The individual decomposed EF values were indexed against the global RVEF. Strain values were calculated as well.

Data from 166 subjects were included in the analysis; median age was 13.5 years (range 0 to 17.4 years). Overall, AEF was greater than REF and LEF (29.2 ± 6.2% vs. 25.1 ± 7.2% and 25.7 ± 6.0%, respectively; p < 0.001). This remained true when indexed to overall EF (49.8 ± 8.7% vs. 43.3 ± 11.6% and 44.4 ± 10%, respectively; p < 0.001). Age-related differences were present for global RVEF, REF, and all components of RV strain.

In healthy children, anteroposterior shortening is the dominant component of RV contraction. Evaluation of 3D parameters of the RV in children is feasible and enhances the overall understanding of RV function, which may allow improvements in recognition of dysfunction and assessment of treatment effects in the future.

Congenitally corrected transposition of the great arteries (ccTGAs) represents a complex form of congenital heart disease that is associated with several cardiac complications. Herein is a case series of three children with ccTGA and ventricular assist device (VAD) inserted for systemic right ventricle failure at a single institution. All patients remained hemodynamically stable postimplant and were successfully discharged from the intensive care unit to undergo postoperative rehabilitation. All three patients received an orthotopic heart transplant with uneventful posttransplant courses. This case series provides insight into the medical management and technical feasibility of VAD support in children with ccTGA with end-stage heart failure.

The presence of a systemic right ventricle (sRV) with biventricular physiology (biV) is associated with increased patient morbidity and mortality. To date, no pharmacologic therapy for heart failure has been proven effective for patients with systolic dysfunction of the sRV-biV. We designed a randomized, double-blind, placebo-controlled crossover trial to compare sacubitril/valsartan treatment to placebo in adults (aged ≥ 18 years) with moderate-to-severe sRV-biV dysfunction and New York Heart Association functional class II to III symptoms. Two primary efficacy endpoints are assessed in the trial: exercise capacity (submaximal exercise duration) and neurohormonal activation (N-terminal prohormone brain natriuretic peptide). Secondary objectives include assessing a change in the Kansas City Cardiomyopathy Questionnaire score and evaluating the safety and tolerance of sacubitril/valsartan. A 6-week open run-in phase identifies the maximum tolerated dose of sacubitril/valsartan, up to 97 mg/103 mg twice daily. After a 2-week washout period, patients are randomized 1:1 to sacubitril/valsartan treatment vs placebo for a 24-week phase, followed by another 2-week washout period and subsequent crossover to the alternative treatment arm for an additional 24-week phase. Data to assess primary and secondary endpoints are collected at baseline and at the end of each phase. A total of 48 patients is required to provide > 80% power to detect a 30% difference in distance walked and in N-terminal prohormone brain natriuretic peptide levels with sacubitril/valsartan treatment vs placebo, each with a 2-sided P-value of 0.025. In summary, the Prospective Comparison of Angiotensin Receptor-Neprilysin Inhibitor vs Placebo in Patients With Congenital Systemic Right Ventricular Heart Failure Trial (PARACYS-RV) should determine the role of sacubitril/valsartan in treating heart failure in patients with sRV-biV and carries the potential to alter management of this patient population.

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.

The objectives of this study was to compare the long-term outcomes of anatomic repair using atrial switch with the Rastelli procedure versus physiological repair with left ventricle-to-pulmonary artery conduit for patients with levo-transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction.

Of patients with levo-transposition of the great arteries who underwent biventricular repair between 1978 and 2001, 31 hospital survivors after anatomic repair of atrial switch and the Rastelli (anatomic group) and 14 hospital survivors after physiological repair with left ventricle-to-pulmonary artery conduit (physiological group) were enrolled. Survival rates, reoperation rates, and most recent conditions were compared.

The overall survival rate at 20 years was 79.7% (95% CI, 66.4%-95.6%) in the anatomic group and 85.1% (95% CI, 68.0%-100%) in the physiological group (P = .87). The reoperation rate at 10 years was 19.8% (95% CI, 5.6%-34.0%) in the anatomic group and 52.0% (95% CI, 25.0%-79.1%) in the physiological group (P = .067). Only patients in the physiological group underwent systemic tricuspid valve replacement. The anatomic group showed a better cardiac index at catheterization (2.79 ± 0.75 L/min/m2 vs 2.30 ± 0.54 L/min/m2; P = .035), lower serum brain natriuretic peptide (73 ± 86 pg/mL vs 163 ± 171 pg/mL; P = .024), and better maximal oxygen uptake in the treadmill test (64.1 ± 16.5% vs 52.7 ± 17.8% of predicted normal; P = .036), although the period until most recent catheterization, blood inspection, and treadmill testing were earlier in the anatomic group.

Preservation of the left ventricle as the systemic ventricle using anatomic repair contributes to better cardiopulmonary condition compared with physiological repair.

d-loop transposition of the great arteries (d-TGA) and congenitally corrected transposition of the great arteries (cc-TGA) feature a right ventricle attempting to sustain the systemic circulation. A systemic right ventricle (sRV) cannot support cardiac output in the long run, eventually decompensating and causing heart failure. The burden of d-TGA patients with previous atrial switch repair and cc-TGA patients with heart failure will only increase in the coming years due to the aging adult congenital heart disease population and improvements in the management of advanced heart failure. Clinical data still lags behind in developing evidence-based guidelines for risk stratification and management of sRV patients, and clinical trials for heart failure in these patients are underrepresented. Recent studies have provided foundational data for the commencement of robust clinical trials in d-TGA and cc-TGA patients. Further insights into the multifactorial nature of sRV failure can only be provided by the results of such studies. This review discusses the mechanisms of heart failure in sRV patients with biventricular circulation and how these mediators may be targeted clinically to alleviate sRV failure.

Management strategies for congenitally corrected transposition of the great arteries (ccTGA) historically consisted of a physiologic repair, resulting in the morphologic right ventricle (mRV) supporting systemic circulation. This strategy persisted despite the development of heart failure by middle age because of the reasonable short-term outcomes, and the natural history of some patients with favorable anatomy (felt to demonstrate the mRV's ability to function in the long-term), and due to the less-than-optimal outcomes associated with anatomical repair. As outcomes with anatomical repair improved, and the long-term risk of systemic mRV dysfunction became apparent, more have begun to realize its advantages. In addition to the decision on whether or not to pursue anatomical repair, and the optimal timing, studies demonstrating the nuance to morphologic left ventricle retraining have demonstrated its feasibility. Further considerations in ccTGA have begun to be better understood, including: the management of a poorly functioning mRV, systemic tricuspid valve regurgitation, the utility of morphologic left ventricle outflow tract obstruction (native or surgically created) and pacing strategies. While some considerations are apparent: biventricular pacing is superior to univentricular, tricuspid regurgitation must be managed early with either progression towards anatomical repair (pulmonary artery banding if needed for retraining) or tricuspid replacement (not repair) based on the patient's age; others remain to be completely elucidated. Overall, the heterogeneity of ccTGA, as well as the unique presentation with each patient regarding ventricular and valvular function and center-to-center variability in management strategies has made the interpretation of published data difficult. That said, more recent long-term outcomes favor anatomical repair in most situations.

Interventions in adults with congenital heart disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To bring rigour to our process and amplify the cumulative nature of evidence ACHD care we used the ADAPTE process; we systematically adjudicated, updated, and adapted existing guidelines by Canadian, American, and European cardiac societies from 2010 to 2020. We applied this to interventions related to right and left ventricular outflow obstruction, tetralogy of Fallot, coarctation, aortopathy associated with bicuspid aortic valve, atrioventricular canal defects, Ebstein anomaly, complete and congenitally corrected transposition, and patients with the Fontan operation. In addition to tables indexed to evidence, clinical flow diagrams are included for each lesion to facilitate a practical approach to clinical decision-making. Excluded are recommendations for pacemakers, defibrillators, and arrhythmia-directed interventions covered in separate designated documents. Similarly, where overlap occurs with other guidelines for valvular interventions, reference is made to parallel publications. There is a paucity of high-level quality of evidence in the form of randomized clinical trials to support guidelines in ACHD. We accounted for this in the wording of the strength of recommendations put forth by our national and international experts. As data grow on long-term follow-up, we expect that the evidence driving clinical practice will become increasingly granular. These recommendations are meant to be used to guide dialogue between clinicians, interventional cardiologists, surgeons, and patients making complex decisions relative to ACHD interventions.

Systemic tricuspid regurgitation and impaired aerobic capacity are common in adults with congenitally corrected transposition of great arteries (cc-TGA), but it is unknown whether systemic tricuspid valve replacement (sTVR) is associated with improvement in aerobic capacity.

The purpose of this study was to determine whether sTVR was associated with postoperative improvement in aerobic capacity as measured by peak oxygen consumption (VO2).

This is a retrospective cohort study of adults with cc-TGA and ≥moderate tricuspid regurgitation that underwent sTVR and exercise test pre- and post-sTVR (2003-2019). We selected a propensity-matched control group of patients with cc-TGA and ≥moderate tricuspid regurgitation that did not undergo sTVR between exercise tests. Improvement in aerobic capacity was calculated as the difference between baseline and follow-up exercise tests.

The study comprised 48 patients in each group, and both groups had similar peak VO2 at baseline. Compared to the baseline exercise test, the sTVR group had more improvement in peak VO2 (6 ± 4% vs -8 ± 3%-predicted, P = 0.001), and the factors associated with postoperative improvement in peak VO2 were baseline systolic blood pressure (<120 mm Hg), systemic right ventricular global longitudinal strain (<-16%), and preoperative peak VO2 (>60%-predicted).

There was an improvement in aerobic capacity after sTVR in patients with cc-TGA and severe tricuspid regurgitation. The determinants of improvement in aerobic capacity were baseline systolic blood pressure, systemic right ventricular global longitudinal strain, and preoperative peak VO2. Further studies are required to determine whether strict blood pressure control and optimal timing of sTVR based on these indices would result in improved clinical outcomes in this population.

Patients with transposition of the great arteries (TGA) after an atrial switch or congenitally corrected TGA (ccTGA) are prone to systemic right ventricular (sRV) failure. Tricuspid valve (TV) regurgitation aggravates sRV dysfunction. Timely TV surgery stabilizes sRV function, yet the development of atrioventricular (AV)-conduction disturbances in the course of sRV failure can contribute to sRV dysfunction through pacing-induced dyssynchrony. This study aims to explore the incidence, timing, and functional consequences of AV-block requiring ventricular pacing after TV surgery in patients with sRV.

Consecutive adolescent and adult patients with an sRV who underwent TV surgery between 1989 and 2020 and followed-up at our center were included in this observational cohort study.

The data of 28 patients (53% female, 57% ccTGA, and a mean age at surgery 38 ± 13 years) were analyzed. The mean follow-up was 9.7 ± 6.8 years. Of the remaining 22 patients at the risk of developing high degree AV-block after TV surgery, 9 (41%) developed an indication for chronic ventricular pacing during follow-up, of which 5 (56%) within 24 months postoperatively (3 prior to hospital discharge). The QRS duration, a surrogate marker for dyssynchrony, was significantly higher in patients with chronic left ventricular pacing than in patients with native AV-conduction (217 ± 24 vs. 116 ± 23 ms, p = 0.000), as was the heart failure biomarker NT-pro-BNP [2,746 (1,242-6,879) vs. 495 (355-690) ng/L, p = 0.004] and the percentage of patients with ≥1 echocardiographic class of deterioration of systolic sRV function (27 vs. 83%, p = 0.001). Of the patients receiving chronic subpulmonary ventricular pacing (n = 12), 9 (75%) reached the composite endpoint of progressive heart failure [death, ventricular assist device implantation, or upgrade to cardiac resynchronization therapy (CRT)]. Only 4 (31%) patients with native AV-conduction (n = 13) reached this composite endpoint (p = 0.027).

Patients with a failing sRV who undergo TV surgery are prone to develop AV-conduction abnormalities, with 41% developing an indication for chronic ventricular pacing during 10 years of follow-up. Patients with chronic subpulmonary ventricular pacing have a significantly longer QRS complex duration, have higher levels of the heart failure biomarker NT-pro-BNP, and are at a higher risk of deterioration of systolic sRV function and progressive heart failure.

To determine the contemporary outcomes of the double switch operation (DSO) (ie, Mustard or Senning + arterial switch).

A single-institution, retrospective review of all patients with congenitally corrected transposition of the great arteries undergoing a DSO.

Between 1999 and 2019, 103 patients underwent DSO with a Mustard (n = 93) or Senning (n = 10) procedure. Segmental anatomy was (S, L, L) in 93 patients and (I, D, D) in 6 patients. Eight patients had heterotaxy and 71 patients had a ventricular septal defect. Median age was 2.1 years (range, 1.8 months-40 years), including 34 patients younger than age 1 year (33%). Median weight was 10.9 kg (range, 3.4-64 kg). Sixty-one patients had prior pulmonary artery bands for a median of 1.1 years (range, 14 days-12.9 years; interquartile range, 0.7-3.1 years). Median intensive care unit and hospital lengths of stay were 5 and 10 days, respectively. Median follow-up was 3.4 years (interquartile range, 1-9.8 years) and 5.2 years (interquartile range, 2.3-10.7 years) in 79 patients with >1 year follow-up. At latest follow-up, aortic, mitral, tricuspid valve regurgitation, and left ventricle dysfunction was less than moderate in 96%, 98%, 96%, and 93%, respectively. Seventeen patients underwent reoperation: neoaortic valve intervention (n = 10), baffle revision (n = 5), and ventricular septal defect closure (n = 4). At latest follow-up, 17 patients (17%) had a pacemaker and 27 (26%) had cardiac resynchronization therapy devices. There were 2 deaths and 2 transplants. Transplant-free survival was 94.6% at 5 years. Risk factors for death or transplant included longer cardiopulmonary bypass time and older age at DSO.

The outcomes of the DSO are promising. Earlier age at operation might favor better outcomes. Progressive neoaortic regurgitation and reinterventions on the neo-aortic valve are anticipated problems.

We performed a systematic review of the literature on the assessment of subpulmonary and systemic right ventricular (RV) functional reserve during pharmacological and exercise stress in congenital heart patients and patients with pulmonary arterial hypertension (PAH). Literature search was conducted using PubMed, EMBASE, and MEDLINE from their inception up to August 2020. Of 913 records identified, 56 studies with a total of 1730 patients were included. Of the 56 studies, 23 assessed subpulmonary RV functional reserve in repaired tetralogy of Fallot patients, 19 assessed systemic RV reserve in patients with transposition of the great arteries (TGA) after atrial switch and those with congenitally corrected TGA, and 14 assessed subpulmonary RV research in patients with PAH. Pharmacological and exercise stress was used, respectively, in 22 and 34 studies. The main findings were (1) impairment of RV systolic and diastolic functional reserve, (2) associations between impaired functional reserve and worse baseline functional parameters, and (3) prognostic implications of RV systolic functional reserve on clinical outcomes in patients with volume and/or pressure-loaded subpulmonary and systemic right ventricles. Further studies are required to establish the incremental value of incorporating stress studies of RV systolic and diastolic function in the clinical management algorithm of congenital heart patients and patients with PAH.

The purpose of this study was to assess the role of echocardiography for a comprehensive assessment of cardiac remodeling, and the relationship between indices of cardiac remodeling and cardiovascular events (defined as the composite end point of heart failure hospitalization, heart transplant, or cardiovascular death) in adults with congenitally corrected transposition of great arteries (cc-TGA).

This is a retrospective study of adults with cc-TGA who underwent echocardiogram (2003-2020). Offline image analysis was performed in all patients. Chamber (atrial and ventricular) function and size were assessed by strain imaging and 2-dimensional echocardiography.

Of 233 patients with cc-TGA (40±15 years), 123 (55%) had at least one cardiac procedure before baseline echocardiogram. Of 233 patients, 76% and 61% had left atrial dysfunction and systemic right ventricular dysfunction, respectively; while 43% and 11% had right atrial dysfunction and left ventricular dysfunction, respectively. During a median follow-up of 8.9 years, 114 (49%) underwent additional cardiac procedures, and 66 (28%) had cardiovascular events. Left atrial reservoir strain, right ventricular global longitudinal strain, right atrial reservoir strain, left ventricular systolic pressure, and left ventricular global longitudinal strain were independently associated with cardiovascular events.

In addition to the clinical importance of right ventricular systolic dysfunction in cc-TGA that is already well described, the current study demonstrated, for the first time, that biatrial dysfunction was common and was associated with clinical outcomes. Since there are currently no effective therapies for atrial and ventricular dysfunction in patients with cc-TGA, there is a need for research to identify novel strategies to prevent atrial and ventricular dysfunction in this population.