Search Results (16)

Objectives: This study sought to echocardiographic manifestations and the related risk factors affecting the prognosis of isolated congenitally corrected transposition of the great arteries (CCTGA). Methods: A total of 143 patients (≥18 years of age) were diagnosed with isolated CCTGA at Anzhen Hospital. The patients were classified as the operation group and the non-operation group depending on whether they had undergone tricuspid valve surgery. The echocardiographic data and follow-up were compared, and the primary outcomes examined were defined as death or heart transplantation. Results: The average age of 143 patients with isolated CCTGA was 39.93 ± 13.50 years. Tricuspid valve surgery was performed in 31 patients with isolated CCTGA, and 112 patients did not undergo tricuspid valve surgery. The incidence of tricuspid valve structural changes in the operation group was 39.1%, and this group had higher numbers of patients with right ventricular diastolic diameter, right ventricular systolic diameter, left atrial dimensions, and regurgitation before surgery compared with the non-operation group (p < 0.05). Follow-up results showed no significant difference in the number of death/heart transplantations, and the incidence of systemic ventricular ejection fraction (SVEF) < 40% between the two groups. The survival rate of the surgery group was higher than that of the non-surgery group, although not statistically significant (p = 0.123). Age, right ventricular end-diastolic diameter, and decreased SVEF at the first diagnosis are independent predictive risk factors for major adverse outcomes. Conclusions: Adult patients with isolated CCTGA may have structural abnormalities in their tricuspid valves. There were no significant differences in the incidence of adverse outcomes, morphological right ventricular systolic dysfunction, and survival between the surgery group and the non-surgery group. However, this study is a retrospective study, and the sample size of the surgical group is relatively small, which may limit the generalizability of the research conclusions. In the future, a prospective, large-scale study will be conducted to evaluate the therapeutic effect of tricuspid valve surgery on such patients. Full article

Background/Objectives: The arterial switch operation (ASO) has been performed in the neonatal period as an anatomical correction for d-transposition of the great arteries since the 1980s. As the population of adult congenital heart disease patients grows, it is essential for healthcare providers across various disciplines to comprehend the complexities of these patients. We report on outcomes up to the third decade after ASO. Methods: All patients who underwent ASO from May 1985 to December 2020 were included, and a retrospective chart review with follow-up until December 2021 was conducted. Additionally, vital status verification (90.3% complete) was performed through the national health insurance. Survival analysis and competing risk analysis were performed to determine outcomes in the third decade after ASO. Results: One-hundred-ninety-five patients (72.3% male; median age at surgery 6 days; median weight at surgery 3.4 kg) underwent ASO from May 1985 to December 2020. Patients with a prenatal diagnosis showed a lower rate of preoperative cardiac decompensation (p = 0.017). Early in-hospital mortality was 8.7%, and no early in-hospital deaths occurred in the study era from 2006 to 2020. Four late deaths occurred, and the Kaplan–Meier estimated survival of the 178 hospital survivors was 98.3% ± 1.2% at 10 years, 96.8% ± 1.9% at 20 years and 92.4% ± 4.7 at 30 years. The cumulative incidence of left ventricular outflow tract (LVOT) reoperation after ASO was 1.3% at 10 years, 3.4% at 20 years and 11.3% at 30 years. The cumulative incidence of right ventricular outflow tract (RVOT) reoperation after ASO was 7.2% at 10 years and 17.5% at 20 and 30 years. Conclusions: Overall long-term survival of the hospital survivors is good. The reoperation rate on the LVOT is favorable. Percutaneous interventions, if feasible after the Lecompte maneuver, might pose an option to delay the more common reoperations on the RVOT, though further studies are needed to determine their long-term impact. Full article

Background: Pregnancy in women with biventricular circulation and a systemic right ventricle (sRV) is considered high risk, with limited data available on pregnancy outcomes. This study aimed to investigate pregnancy outcomes in this population. Materials and Methods: A systematic review was conducted using four major electronic databases. Pregnant women with a complete transposition of great arteries (d-TGA) after an atrial switch operation or a congenitally corrected transposition of the great arteries (ccTGA) were included. Results: In total, 15 studies including 632 pregnancies in 415 women with an sRV and biventricular circulation were identified, of whom 299 (72%) had d-TGA and 116 (28%) ccTGA. Maternal mortality or cardiac transplantation occurred in 0.8% of pregnancies. The most frequent maternal complications were the worsening of systemic atrioventricular valve regurgitation [pooled estimate (PE): 16%, 95% CI: 5;26], the deterioration of sRV function (PE: 15%, 95% CI: 2;27), the worsening of the NYHA class (PE: 13%, 95% CI: 6;20), all-cause hospitalization (PE): 10%, 95% CI: 7;12), arrhythmias (PE: 8%, 95% CI: 5;11), and symptomatic heart failure (PE: 6%, 95% CI: 3;10). Stillbirth occurred in 0.7% of pregnancies and neonatal death in 0.4%. Small-for-gestational-age neonates were encountered in 36% (95% CI: 21;52) of pregnancies and preterm delivery in 22% (95% CI: 14;30). A subgroup analysis showed no significant difference in outcomes between women with d-TGA and those with ccTGA, except for the worsening of the NYHA class, which occurred more often in d-TGA (18%, 95% CI: 12;27 vs. 6%, 95% CI: 3;15, respectively, p = 0.03). Conclusions: Maternal and fetal/neonatal mortality are low among pregnant women with biventricular circulation and an sRV. However, significant maternal morbidity and poor neonatal outcomes are frequently encountered, rendering management in specialized centers imperative. Full article

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. Full article

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. Full article

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. Full article

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. Full article

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. Full article

Background: 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. Methods: 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). Results: 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. Conclusions: 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. Full article

(1) Background: Transposition of the great arteries (TGA) is the most common congenital heart disease, accounting for 5–7% of all cardiac anomalies, with a prevalence of 0.2–0.3 per 1000 live births. (2) Aim: Our main objectives were to evaluate the clinical safety of balloon atrial septostomy in neonates and the possible complications. Furthermore, we tried to establish whether the procedure should be performed in all TGA patients with small atrial septal defects, regardless of oxygen saturation, within a center where corrective surgery cannot be performed on an emergency basis due to the lack of a permanent cardiac surgery team for arterial switch surgery. (2) Methods: We conducted an observational, retrospective, single tertiary-care center study between January 2008 and April 2022, which included 92 neonates with TGA transferred to our institution for specialized treatment. (3) Results: The median age at the time of the Rashkind procedure was four days. The rate of immediate complications after balloon atrial septostomy (BAS) was high (34.3%), but most were transient (metabolic acidosis and arterial hypotension—21.8%). Twenty patients with TGA managed in our hospital underwent definitive and corrective surgical intervention (arterial switch operation) at a median age of 13 days. Most patients (82.6%) were term neonates, but 16 were born preterm. (4) Conclusions: Urgent balloon atrial septostomy is often the only solution to restore adequate systemic perfusion. Bedside balloon atrial septostomy is a safe, effective, and initial palliative intervention in neonates with TGA, which can be performed in the neonatal unit. Full article

The transposition of great arteries (TGA) is one of the most frequent and severe congenital heart diseases. After newborn stabilization and while pending surgical correction, echocardiographic monitoring with a careful evaluation of left ventricle (LV) performance is warranted. In this study, our objectives were (i) to compare myocardial function, assessed via speckle-tracking echocardiography, between neonates with TGA and neonates without TGA and (ii) to identify a strain parameter with a good discriminatory ability for TGA. We conducted a retrospective, single-center study. A total of 90 neonates were examined, of whom 66 were included (16 comprised the TGA group and 50 comprised the control group). The results of a bivariate analysis showed that classic echocardiography parameters displayed no significant differences between the two studied groups (p = 0.785 for EF, p = 0.286 for MAPSE and p = 0.315 for TAPSE). We found a statistically significant difference between the two groups for the mean values of the LVpGLS parameter (adjusted p = 0.0047), with impaired LV myocardium function being observed in the TGA group after adjusting for other covariates. Regarding segmental strain, the mean medial and apical inter-ventricular septum strain values were found to be significantly lower in the neonates with TGA than in the controls (95% CI for difference in means: [−6.45, −0.65], [−8.56, −1.97]). The results of an ROC analysis showed that LVpGLS had a significant ability to differentiate between neonates with TGA and controls (AUC = 0.712, 95% CI: [0.52, 0.903], p = 0.011). In conclusion, LVpGLS is a parameter with a significant discriminatory ability for LV dysfunction, and it is useful in the evaluation of ventricular myocardial function in newborns with TGA. Full article

Introduction: Automated echocardiography image interpretation has the potential to transform clinical practice. However, neural networks developed in general cohorts may underperform in the setting of altered cardiac anatomy. Methods: Consecutive echocardiographic studies of patients with congenital or structural heart disease (C/SHD) were used to validate an existing convolutional neural network trained on 14,035 echocardiograms for automated view classification. In addition, a new convolutional neural network for view classification was trained and tested specifically in patients with C/SHD. Results: Overall, 9793 imaging files from 262 patients with C/SHD (mean age 49 years, 60% male) and 62 normal controls (mean age 45 years, 50.0% male) were included. Congenital diagnoses included among others, tetralogy of Fallot (30), Ebstein anomaly (18) and transposition of the great arteries (TGA, 48). Assessing correct view classification based on 284,250 individual frames revealed that the non-congenital model had an overall accuracy of 48.3% for correct view classification in patients with C/SHD compared to 66.7% in patients without cardiac disease. Our newly trained convolutional network for echocardiographic view detection based on over 139,910 frames and tested on 35,614 frames from C/SHD patients achieved an accuracy of 76.1% in detecting the correct echocardiographic view. Conclusions: The current study is the first to validate view classification by neural networks in C/SHD patients. While generic models have acceptable accuracy in general cardiology patients, the quality of image classification is only modest in patients with C/SHD. In contrast, our model trained in C/SHD achieved a considerably increased accuracy in this particular cohort. Full article

Background: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect (CHD). Contemporary data regarding its outcome in adults are scarce. Methods: Retrospective, single-center study of all ccTGA patients over the age of 16 years treated at our center during the time period 2006–2018. Only patients with a biventricular circulation were included. The primary endpoint was all-cause mortality. Results: Altogether, 96 patients (mean age 32.8 ± 16.0 years, female 50%) with ccTGA and a systemic right ventricle (SRV) were included in the study. An additional CHD was present in 81 patients (84.4%); most common were a ventricular septal defect (VSD) and a left ventricular outflow tract obstruction. Out of the whole cohort, 45 (46.9%) had already undergone cardiac surgery at baseline. During a median follow-up of 6.5 (IQR 2.8–12.7) years, the primary endpoint occurred in 10 patients (10.8%). Cause of death was cardiac in nine patients and suicide in one. Hospitalizations due to heart failure occurred in 48 patients (51.6%). Upon univariate Cox analysis, an NYHA class ≥III, severe tricuspid regurgitation, severe SRV systolic impairment, as well as a reduced left ventricular systolic function were predictors of the primary endpoint. Upon multivariable analysis, only NYHA class ≥ III (HR: 18.66, CI 95%: 3.01–115.80, p = 0.0017) and a reduced left ventricular systolic function (HR: 7.36, CI 95%: 1.18–45.99, p = 0.038) remained as independent predictors. Conclusions: Adults with ccTGA and an SRV are burdened with significant morbidity and mortality. Predictors for mortality are NYHA class and subpulmonary left ventricular function. Full article

To appreciate congenital heart disease fully, a detailed understanding of the anatomical presentation, as well as the physiology, is required. This is often introduced at an advanced stage of training. Professor Anderson has been influential in the Clinical Anatomy Intercalated BSc programme at the University of Birmingham, in particular in his teaching on Sequential Segmental Analysis. This article describes the experiences of the latest cohort of students on this programme, who undertook varying research projects using the Birmingham Cardiac Archive, with the guidance of Professor Anderson. The projects outlined include various aspects of isomerism, encompassing both the cardiac and abdominal manifestations, as well as details of congenitally corrected transposition of the great arteries and prenatally diagnosed right aortic arch and double arch. These studies all aimed to increase the knowledge base of their respective cardiac malformations and provide a basis for further research. Full article

Background: The right ventricle serves as the subaortic systemic ventricle (sysRV) in patients with congenitally corrected transposition of the great arteries (ccTGA) and in patients with transposition of the great arteries (TGA) surgically repaired by an atrial switch. SysRV can lead to late complications, primarily heart failure, significant regurgitation of the systemic atrioventricular (AV) valve, and ventricular arrhythmias with sudden cardiac death. We sought to investigate the value of 2D- and 3D-echocardiographic parameters of sysRV function. Methods: Consecutive adult patients with sysRV who presented at the adult congenital heart disease outpatient clinic were prospectively enrolled. All patients received comprehensive transthoracic echocardiography, including 3D-echocardiography, cardiac magnetic-resonance-imaging (CMR), cardiopulmonary-exercise-testing, and blood analysis for NT-proBNP. Results. A total of 27 patients were included, 18 with TGA and nine with ccTGA. Median age was 37 years (Q1 = 31, Q3 = 44), 44% were male, median NT-proBNP was 189 pg/mL (Q1 = 155, Q3 = 467); sufficient 3D-echocardiography datasets were acquired in 78% of patients. All echocardiographic 2D and 3D volumetric function parameters correlated with CMR data, whereas a correlation was not seen with any of the longitudinal function parameters. NT-proBNP correlated with tricuspid annular plane systolic excursion (r = −0.43, p = 0.02) and CMR ejection fraction (EF) (r = −0.62, p = 0.003). Conclusion: Systematic evaluation of sysRV is complex and should include not only volumetric parameters but also parameters of longitudinal function in addition to measurement of NT-proBNP. In patients with good image quality, 3D-echocardiography can be used to assess volumes and EF. Full article