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BackgroundCardiac resynchronisation therapy (CRT) is a key treatment for heart failure (HF) in acquired heart disease, but its benefits in adults with congenital heart disease and a systemic right ventricle (sRV) remain unclear. This study aimed to assess whether CRT improves outcomes in patients with sRV.MethodsThis is an international, retrospective study including patients >18 years from 33 centres with transposition of the great arteries (TGA) following atrial switch operation and congenitally corrected TGA. The primary endpoint included overall survival and survival free from HF. The secondary endpoint was a composite of death, hospitalisation for HF, heart transplant, mechanical support and ventricular tachycardia/implantable cardioverter-defibrillator therapies.ResultsWe identified 105 out of 1721 patients (3.5%) who underwent CRT. Median follow-up after CRT implant was 4.6 (1.6–8) years. QRS improvement was limited to those with previous pacing (167±35 vs 154±28 ms; p=0.002). Following CRT, there was no significant change in B-type natriuretic peptide values, peak VO2 and tricuspid regurgitation severity by echocardiography. CRT complications occurred in 10 (9.5%), though they were usually minor. Patients with CRT were propensity-matched to controls according to age, sex, anatomy, presence of complex disease, previous HF and sRV dysfunction at baseline. At univariable analysis, CRT (HR 4.39–95%, CI 1.6 to 11.9; p=0.003), older age and moderate-to-severe sRV dysfunction at baseline were predictive of death, while CRT (HR 3–95%, CI 1.3 to 7; p=0.01) and sRV dysfunction were associated with HF admission. By multivariable analysis, CRT (HR 8.8–95%, CI 2.9 to 26.6; p=0.0001) and age (HR 1.1%–95%, CI 1.01 to 1.15; p<0.0001) were independently associated with poorer outcome.ConclusionIn this retrospective study in the largest population thus far described with an sRV, CRT implant was not associated with improved survival, even after controlling for key confounders.

In selected patients with transposition of the great arteries (TGA), ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO), the arterial switch operation (ASO) may be the procedure of choice. This study reviews the clinical outcomes of TGA-VSD-LVOTO patients after ASO and compares mechanisms of LVOTO in this patient group to a historical series of cardiac specimens. This retrospective analysis included all cases with TGA-VSD-LVOTO who underwent ASO between January 1977 and December 2023. Additionally, a series of non-operated cardiac specimens with TGA-VSD-LVOTO was selected and examined for morphological comparison. Eleven patients with TGA-VSD-LVOTO underwent ASO. Eight of them had TGA-VSD, and three had Taussig-Bing anomaly. LVOTO mechanisms were multifactorial, including posteriorly deviated infundibular septum and fibrous tissue masses. Median age at ASO was 0.4 (0.07-1.8) years. Ten patients underwent primary LVOTO relief during ASO; no in-hospital mortality occurred. Two patients died >30 days post-ASO at 3.1 months and 6.0 years. Median follow-up was 19.0 (11.1-26.8) years, all survivors in NYHA class I. The patient without initial LVOTO relief did require reoperation during follow-up for progressive LVOTO at 3.5 months post-ASO. Two patients had moderate residual LVOTO at latest follow-up (gradient 30-50 mmHg). No significant neoaortic valve regurgitation was observed. From the anatomical specimen series, 10 of 33 TGA-VSD-LVOTO specimen were deemed eligible for ASO, revealing similar LVOTO mechanisms as the clinical cases. ASO is feasible in selected patients with TGA-VSD-LVOTO showing good long-term outcomes with preserved neoaortic valve function and no reoperations for LVOTO after initial relief.

BackgroundTo date, clinical trials have been underpowered to demonstrate a benefit from ACE inhibitors (ACEis) or angiotensin II receptor blockers (ARBs) in preventing systemic right ventricle (sRV) failure and disease progression in patients with transposition of the great arteries (TGA). This observational study aimed to estimate the effect of ACEi and ARB on heart failure (HF) incidence and mortality in a large population of patients with an sRV.MethodsData on all patients with an sRV under active follow-up at two tertiary centres between January 2007 and September 2018 were studied. The effect of ACEi and ARB on the incidence of HF and mortality was estimated using a propensity score weighting approach to control confounding.ResultsAmong the 359 patients with an sRV (32.2 (IQR 26.4–38.3) years, 59.3% male, 66% complete TGA with atrial switch repair and 34% congenitally corrected TGA), 79 (22%) had a moderate to severe sRV dysfunction and 138 (38%) were treated with ACEi or ARB. Fourteen (3.6%) patients died, 8 (2.1%) underwent heart transplantation and 46 (11.8%) had a new HF event over a median follow-up of 7.1 (IQR 4.0–9.4) years. On multivariate Cox analysis with adjustment using propensity score weighting approaches, ACEi or ARBs treatment was not significantly associated with a lower HF incidence or mortality in patients with an sRV.ConclusionsDespite significant neurohormonal activation described in patients with an sRV, there is still no evidence of a beneficial effect of ACEi or ARB on morbidity and mortality in this population.

Background To evaluate the relationship between IL-1α -889C/T (rs1800587), IL-1β -511C > T (rs16944), TNFα -308G > A (rs1800629), TNFα -238G > A (rs361525), IL-6 -174G > C (rs1800795), and IL-6 -572G > C (rs1800796) polymorphisms and the susceptibility to transposition of the great arteries (TGA). Methods A prospective analysis was performed on mothers whose newborns were diagnosed as having TGA. For each case of TGA, a mother who gave birth to a healthy neonate in the same period was randomly selected for the control group. The sample size was calculated before planning the study with 80% power and 5% alpha. Results Twenty-seven mothers whose newborn had TGA anomalies (group 1) and 27 mothers whose newborn had no TGA (group 2) were included in the study. There were no significant differences between the groups in terms of maternal age, pregestational body mass index, gestational age at birth and infant sex ( p  > 0.05). The genotype and allele distributions of IL-1α -889C/T (rs1800587), IL-1β -511C > T (rs16944), TNFα -308G > A (rs1800629), TNFα -238G > A (rs361525), IL-6 -174G > C (rs1800795) and IL-6 -572G > C (rs1800796) gene variants were not different between the two groups ( p  > 0.05). Conclusions There was no relation between IL-1α, IL-1β, IL-6, and TNFα promoter gene polymorphisms and TGA occurrence in our study group. Trial registration : This present prospective case–control study was conducted in Baskent University Hospital, Ankara, Turkey, between May 2020 and November 2021. Ethical approval was obtained from the university’s Clinical Research Ethics Commitee (No: KA20/211) in accordance with the Declaration of Helsinki.

ObjectiveTo test the hypothesis that ventricular and atrial function are different between patients with transposition of the great arteries (TGA) after arterial switch operation (ASO) and healthy controls.Methods103 consecutive patients with TGA (median age: 16.7 years, 4.3–39.6 years, 71.8% male) were compared with 77 controls (median age: 15.4 years, 6.3–43.2 years, 66.2% male). Biventricular and biatrial function were assessed using standard cardiovascular magnetic resonance (CMR) techniques and feature tracking. Group comparison was performed with conventional non-parametrical statistics and machine learning methods to find the variables most discriminative between patients and controls. These variables were used to build a multivariable logistic regression model to assess the case–control status.ResultsMarkers of left and right ventricular function (LV; RV) (ejection fraction, MAPSE, TAPSE, LV long-axis strain) as well as LV global longitudinal (−20.7 (−24.1; −17.9) vs −23.7 (−26.1; −21.6), p<0.001), circumferential (−29.4 (−32.2; −26.5) vs −30.5 (−33.6; 29), p=0.001) and atrial longitudinal strain (left atrium (LA): 23.3 (18.6; 28.8) vs 36.7 (30.7; 44), p<0001; right atrium: 21.7 (18.2; 27.8) vs 34.9 (26.9; 40.3), p<0.001) were reduced in patients compared with controls using non-parametrical testing. The logistic regression model including the most discriminative variables from univariate and machine learning analysis demonstrated significant differences between patients and controls only for TAPSE and LA global longitudinal strain.ConclusionsBiventricular and biatrial function are largely preserved after ASO for TGA. Using a comprehensive CMR protocol along with statistical machine learning methods and a regression approach, only RV longitudinal function and LA function are significantly impaired.

Congenitally corrected transposition is a rare cardiac malformation characterized by the combination of discordant atrioventricular and ventriculo-arterial connections, usually accompanied by other cardiovascular malformations. Incidence has been reported to be around 1/33,000 live births, accounting for approximately 0.05% of congenital heart malformations. Associated malformations may include interventricular communications, obstructions of the outlet from the morphologically left ventricle, and anomalies of the tricuspid valve. The clinical picture and age of onset depend on the associated malformations, with bradycardia, a single loud second heart sound and a heart murmur being the most common manifestations. In the rare cases where there are no associated malformations, congenitally corrected transposition can lead to progressive atrioventricular valvar regurgitation and failure of the systemic ventricle. The diagnosis can also be made late in life when the patient presents with complete heart block or cardiac failure. The etiology of congenitally corrected transposition is currently unknown, and with an increase in incidence among families with previous cases of congenitally corrected transposition reported. Diagnosis can be made by fetal echocardiography, but is more commonly made postnatally with a combination of clinical signs and echocardiography. The anatomical delineation can be further assessed by magnetic resonance imaging and catheterization. The differential diagnosis is centred on the assessing if the patient is presenting with isolated malformations, or as part of a spectrum. Surgical management consists of repair of the associated malformations, or redirection of the systemic and pulmonary venous return associated with an arterial switch procedure, the so-called double switch approach. Prognosis is defined by the associated malformations, and on the timing and approach to palliative surgical care.

Transposition of the great arteries (TGA) after atrial switch operation and congenitally corrected TGA (ccTGA) are commonly associated with impaired systemic right ventricular (RV) function and impaired prognosis. We aimed to investigate the value of indices of myocardial deformation on speckle-tracking echocardiography for quantifying ventricular function and their potential role in assessing ventricular-ventricular interaction and outcome in patients with a systemic RV. A total of 129 patients (87 with TGA and atrial switch and 42 with ccTGA, 71 men, age 35 ± 12 years) were investigated, and biventricular myocardial deformation was compared with findings in healthy subjects (n = 38, age 36 ± 10 years). Systemic ventricular longitudinal 2-dimensional (2D) peak systolic strain (RV 2D-LS) was significantly reduced compared with controls (−12.9 ± 3.6 and −15.4 ± 5.1 vs −21.0 ± 5.5 in TGAs, ccTGAs, and controls, P < .0001). Systemic and pulmonary 2D-LS were correlated in patients with TGA (r = 0.46, P < .0001) and ccTGA (r = 0.64, P < .0001), suggesting interventricular interaction, and this was confirmed when ejection fraction on magnetic resonance imaging was assessed (r = 0.53, P < .0001). More importantly, systemic 2D-LS (hazard ratio 1.31, P = .01) was related to adverse clinical outcome (symptomatic progression to New York Heart Association class ≥3, clinically relevant cardiac arrhythmia, or death) in patients with TGA and atrial switch independently of ejection fraction on cardiac magnetic resonance imaging, history of clinically relevant arrhythmia, or functional class. Global longitudinal systolic strain is significantly reduced in patients with a systemic RV, is related to subpulmonary ventricular function, and predicts adverse clinical outcome in adults with atrial switch TGA.

Background Transposition of the great arteries is a critical congenital heart defect characterized by the reversal of the aorta and pulmonary arteries, resulting in two parallel circulatory pathways. This condition is typically incompatible with life unless a shunt, such as an atrial or ventricular septal defect, facilitates the adequate mixing of oxygenated and deoxygenated blood in the heart. Survival into adulthood without surgical correction is exceptionally rare. While a limited number of cases have been reported globally, none, to the best of our extensive search, have been associated with a patent ductus arteriosus and pulmonary artery aneurysm. Case presentation We present a unique case of a 25-year-old Ethiopian male with complex dextrotransposition of the great arteries, who survived into adulthood and presented with acute decompensated heart failure. Remarkably, this patient had a constellation of associated anomalies, including an atrial septal defect, ventricular septal defect, patent ductus arteriosus, and severe pulmonary hypertension. Owing to resource limitations, insufficient medical knowledge, and financial constraints, the patient was managed conservatively. Despite these challenges, the patient showed symptomatic improvement with the conservative management provided. Conclusion This case offers novel insights into the natural history of untreated dextrotransposition of the great arteries with multiple associated anomalies in adults. It also underscores the challenges of managing complex congenital heart defects in resource-limited settings and highlights the urgent need for accessible advanced diagnostic and therapeutic interventions to improve outcomes for similar patients.

Background For major congenital heart disease, the benefits of antenatal diagnosis on some post-natal measures have been suggested. However, findings have been inconclusive and focus on short term outcome measures alone with little data from a UK population. Our aim is to describe differences in reported outcomes for patients born with isolated Hypoplastic Left Heart Syndrome or Transposition of the Great Arteries in a UK population, following either antenatal or postnatal diagnosis. Methods Retrospective population-based study with case note review covering a 15 year period (1st January 1998 to 31st December 2012) in the British county of Leicestershire. Cases were identified from two local registers: the East Midlands and South Yorkshire Congenital Anomaly Register and a list of surgical patient held by the East Midlands Congenital Heart Centre. Results In total 52 cases of Hypoplastic Left Heart Syndrome or Transposition of the Great Arteries were identified with 24 (46.2%) diagnosed antenatally. Maximum and minimum follow up was 181 and 16 months respectively. Median follow up was 83 months (IQR: 44–111). The risk of intubation in the postnatal period (OR: 4.64, 95% CI: 1.40 - 15.32) was greater in cases of Hypoplastic Left Heart Syndrome or Transposition of the Great Arteries diagnosed after birth when compared to those diagnosed antenatally. There was a non-significant increase in the risk of metabolic acidosis in the postnatal period (OR: 12.5, 95% CI: 0.64 - 245.46). No differences in mortality or long-term outcomes were demonstrated between antenatally and postnatally diagnosed cohorts. Conclusions These results confirm data from American and European populations that, for a British population, an antenatal diagnosis of a major congenital heart disease can have a favourable impact on some postnatal outcome measures. There appears to be no evidence that time of diagnosis impacts on long-term outcome measures.