Neoaortic Regurgitation Detected by Echocardiography After Arterial Switch Operation: A Systematic Review and Meta-Analysis.

Background: Neoaortic root dilatation (NeoARD) and neoaortic regurgitation (NeoAR) are common sequelae following the arterial switch operation (ASO) for transposition of the great arteries. Objectives: The authors aimed to estimate the cumulative incidence of NeoAR, assess whether larger neoaortic root dimensions were associated with NeoAR, and evaluate factors associated with the development of NeoAR during long-term follow-up. Methods: Electronic databases were systematically searched for articles that assessed NeoAR and NeoARD after ASO, published before November 2022. The primary outcome was NeoAR, classified based on severity categories (trace, mild, moderate, and severe). Cumulative incidence was estimated from Kaplan-Meier curves, neoaortic root dimensions using Z-scores, and risk factors were evaluated using random-effects meta-analysis. Results: Thirty publications, comprising a total of 6,169 patients, were included in this review. Pooled estimated cumulative incidence of ≥mild NeoAR and ≥moderate NeoAR at 30-year follow-up were 67.5% and 21.4%, respectively. At last follow-up, neoaortic Z-scores were larger at the annulus (mean difference [MD]: 1.17, 95% CI: 0.52-1.82, P < 0.001; MD: 1.38, 95% CI: 0.46-2.30, P = 0.003) and root (MD: 1.83, 95% CI: 1.16-2.49, P < 0.001; MD: 1.84, 95% CI: 1.07-2.60, P < 0.001) in patients with ≥mild and ≥moderate NeoAR, respectively, compared to those without NeoAR. Risk factors for the development of any NeoAR included prior pulmonary artery banding, presence of a ventricular septal defect, aorto-pulmonary mismatch, a bicuspid pulmonary valve, and NeoAR at discharge. Conclusions: The risks of NeoARD and NeoAR increase over time following ASO surgery. Identified risk factors for NeoAR may alert the clinician that closer follow-up is needed. (Risk factors for neoaortic valve regurgitation after arterial switch operation: a meta-analysis; CRD42022373214).

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

BACKGROUND: 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. METHODS AND RESULTS: 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. CONCLUSIONS: 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.

Association of European Paediatric and Congenital Cardiology practical recommendations for surveillance and prevention of cardiac disease in childhood cancer survivors: the importance of physical activity and lifestyle changes From the Association of European Paediatric and Congenital Cardiology Working Group Sports Cardiology, Physical Activity and Prevention, Working Group Adult Congenital Heart Disease, Working Group Imaging and Working Group Heart Failure.

BACKGROUND: Childhood cancer survivors are at increased risk of developing cardiovascular diseases, presenting as the main causes of morbidity and mortality within this group. Besides the usual primary and secondary prevention in combination with screening during follow-up, the modifiable lifestyle factors of physical activity, nutrition, and body weight have not yet gained enough attention regarding potential cardiovascular risk reduction. OBJECTIVE: These practical recommendations aim to provide summarised information and practical implications to paediatricians and health professionals treating childhood cancer survivors to reduce the risk of cardiovascular late effects. METHODS: The content derives from either published guidelines or expert opinions from Association of European Paediatric and Congenital Cardiology working groups and is in accordance with current state-of-the-art. RESULTS: All usual methods of prevention and screening regarding the risk, monitoring, and treatment of occurring cardiovascular diseases are summarised. Additionally, modifiable lifestyle factors are explained, and clear practical implications are named. CONCLUSION: Modifiable lifestyle factors should definitely be considered as a cost-effective and complementary approach to already implemented follow-up care programs in cardio-oncology, which can be actively addressed by the survivors themselves. However, treating physicians are strongly encouraged to support survivors to develop and maintain a healthy lifestyle, including physical activity as one of the major influencing factors. This article summarises relevant background information and provides specific practical recommendations on how to advise survivors to increase their level of physical activity.

Preterm Birth Is Associated With Adverse Cardiac Remodeling and Worse Outcomes in Patients With a Functional Single Right Ventricle.

OBJECTIVE: To assess the effects of preterm birth on cardiac structure and function and transplant-free survival in patients with hypoplastic left heart syndrome and associated anomalies throughout the staged palliation process. STUDY DESIGN: Data from the Single Ventricle Reconstruction trial were used to assess the impact of prematurity on echocardiographic measures at birth, Norwood, Stage II, and 14 months in 549 patients with a single functional right ventricle. Medical history was recorded once a year using medical records or telephone interviews. Cox regression models were applied to analyze transplant-free survival to age 6 years. Causal mediation analysis was performed to estimate the mediating effect of birth weight within this relationship. RESULTS: Of the 549 participants, 64 (11.7%) were born preterm. Preterm-born participants had lower indexed right ventricle end-diastolic volumes at birth but higher volumes than term-born participants by age 14 months. Preterm-born participants had an increased risk of death or heart transplantation from birth to age 6 years, with an almost linear increase in the observed risk as gestational age decreased below 37 weeks. Of the total effect of preterm birth on transplant-free survival, 27.3% (95% CI 2.5-59.0%) was mediated through birth weight. CONCLUSIONS: Preterm birth is associated with adverse right ventricle remodeling and worse transplant-free survival throughout the palliation process, in part independently of low birth weight. Further investigation into this vulnerable group may allow development of strategies that mitigate the impact of prematurity on outcomes in patients with hypoplastic left heart syndrome.

Long-Term Consequences of Acute Kidney Injury After Pediatric Cardiac Surgery: A Systematic Review.

OBJECTIVE: The objective of this study was to evaluate the available data on long-term kidney dysfunction, hypertension, and mortality after cardiac surgery-associated acute kidney injury (AKI) in the pediatric population. STUDY DESIGN: PubMed/MEDLINE, Embase, Scopus, and reference lists of relevant articles were searched for eligible studies published from inception through March 2022. Long-term outcomes after pediatric cardiac surgery complicated by AKI and those without were investigated. RESULTS: We identified 14 studies published between 2013 and 2022 that included a total of 6701 patients (AKI: 1376 patients; no AKI: 5325 patients). These studies used different well-established classifications to define AKI. All the studies suggested that AKI after heart surgery is common in the pediatric patient population and reported a potential link between cardiac surgery-associated AKI and important clinical outcomes. However, only 4 out of 11 studies found a strong association between (absence of recovery from) cardiac surgery-associated AKI and risk of developing chronic kidney disease, and 3 out of 5 studies found a significant increase in mortality rates for pediatric patients who developed AKI after cardiac surgery. Only 1 out of 4 studies found an association between AKI and hypertension at 12 months postoperatively, but found no association at later follow-up times. CONCLUSIONS: Although there is a trend, evidence on the long-term consequences of cardiac surgery-associated AKI in the pediatric population is mixed. Genetic syndromes, preexisting kidney disease, univentricular or cyanotic heart conditions, and/or high-complexity surgery may be more important for the development of kidney dysfunction by adolescence and early adulthood. Regardless, these children may benefit from a long-term kidney follow-up.

Right Heart Failure in a Patient with Critical Pulmonary Stenosis, Absent Right Pulmonary Artery, and Lung Cancer.

BACKGROUND Unilateral absence of a pulmonary artery (UAPA) is a rare congenital cardiovascular malformation. More than half of UAPA cases have other cardiac lesions such as tetralogy of Fallot or septal defects. Clinical manifestations are diverse and range from heart failure after birth to an incidental finding on chest imaging during adulthood. Whereas early surgical revascularization is recommended in infancy, this is usually not feasible in the adult population. Management in these patients is aimed at treating the complications of UAPA. CASE REPORT A 67-year-old woman was evaluated for subacute right heart failure. An echocardiogram revealed pulmonary stenosis, tricuspid regurgitation, and depressed right ventricular function. Chest computed tomography (CT) showed absence of the right pulmonary artery. Additionally, there was a lung tumor in the right upper lobe. Right-heart catheterization confirmed a critically obstructed pulmonary orifice shown by hemodynamic collapse when crossing the pulmonary valve with the catheter. The patient underwent pulmonary valve balloon dilatation with right ventricular outflow tract stenting followed by percutaneous implantation of a balloon-expandable stent-valve. The clinical course was complicated by a complete heart block. Oncologic management consisted of stereotactic radiotherapy. CONCLUSIONS The combination of UAPA, pulmonary stenosis, and lung cancer is rare. Pulmonary stenosis worsens prognosis in adult patients with UAPA, but also constitutes a therapeutic target. The decision to treat the pulmonary stenosis should be based on the severity of stenosis, the degree of pulmonary hypertension, and individual anatomy. We chose percutaneous pulmonary valve implantation because our patient had a critical pulmonary stenosis with normal pulmonary pressures.

Last year of life of adults with congenital heart diseases: causes of death and patterns of care.

AIMS: Although life expectancy in adults with congenital heart diseases (CHD) has increased dramatically over the past five decades, still a substantial number of patients dies prematurely. To gain understanding in the trajectories of dying in adults with CHD, the last year of life warrants further investigation. Therefore, our study aimed to (i) define the causes of death and (ii) describe the patterns of healthcare utilization in the last year of life of adults with CHD. METHODS AND RESULTS: This retrospective mortality follow-back study used healthcare claims and clinical data from BELCODAC, which includes patients with CHD from Belgium. Healthcare utilization comprises cardiovascular procedures, CHD physician contacts, general practitioner visits, hospitalizations, emergency department (ED) visits, intensive care unit (ICU) admissions, and specialist palliative care, and was identified using nomenclature codes. Of the 390 included patients, almost half of the study population (45%) died from a cardiovascular cause. In the last year of life, 87% of patients were hospitalized, 78% of patients had an ED visit, and 19% of patients had an ICU admission. Specialist palliative care was provided to 17% of patients, and to only 4% when looking at the patients with cardiovascular causes of death. CONCLUSIONS: There is a high use of intensive and potentially avoidable care at the end of life. This may imply that end-of-life care provision can be improved. Future studies should further examine end-of-life care provision in the light of patient's needs and preferences, and how the healthcare system can adequately respond.

MeVisLab-OpenVR prototyping platform for virtual reality medical applications.

PURPOSE: Virtual reality (VR) can provide an added value for diagnosis and/or intervention planning. Several VR software implementations have been proposed but they are often application dependent. Previous attempts for a more generic solution incorporating VR in medical prototyping software (MeVisLab) were still lacking functionality precluding easy and flexible development. METHODS: We propose an alternative solution that uses rendering to a graphical processing unit (GPU) texture to enable rendering arbitrary Open Inventor scenes in a VR context. It facilitates flexible development of user interaction and rendering of more complex scenes involving multiple objects. We tested the platform in planning a transcatheter cardiac stent placement procedure. RESULTS: This approach proved to enable development of a particular implementation that facilitates planning of percutaneous treatment of a sinus venosus atrial septal defect. The implementation showed it is intuitive to plan and verify the procedure using VR. CONCLUSION: An alternative implementation for linking OpenVR with MeVisLab is provided that offers more flexible development of VR prototypes which can facilitate further clinical validation of this technology in various medical disciplines.

Persistent Markers of Kidney Injury in Children Who Developed Acute Kidney Injury After Pediatric Cardiac Surgery: A Prospective Cohort Study.

Background Acute kidney injury (AKI) after pediatric cardiac surgery is common. Longer-term outcomes and the incidence of chronic kidney disease after AKI are not well-known. Methods and Results All eligible children (aged <16 years) who had developed AKI following cardiac surgery at our tertiary referral hospital were prospectively invited for a formal kidney assessment ≈5 years after AKI, including measurements of estimated glomerular filtration rate, proteinuria, α1-microglobulin, blood pressure, and kidney ultrasound. Longer-term follow-up data on kidney function were collected at the latest available visit. Among 571 patients who underwent surgery, AKI occurred in 113 (19.7%) over a 4-year period. Fifteen of these (13.3%) died at a median of 31 days (interquartile range [IQR], 9-57) after surgery. A total of 66 patients participated in the kidney assessment at a median of 4.8 years (IQR, 3.9-5.7) after the index AKI episode. Thirty-nine patients (59.1%) had at least 1 marker of kidney injury, including estimated glomerular filtration rate <90 mL/min per 1.73 m2 in 9 (13.6%), proteinuria in 27 (40.9%), α1-microglobinuria in 5 (7.6%), hypertension in 13 (19.7%), and abnormalities on kidney ultrasound in 9 (13.6%). Stages 1 to 5 chronic kidney disease were present in 18 (27.3%) patients. Patients with CKD were more likely to have an associated syndrome (55.6% versus 20.8%, P=0.015). At 13.1 years (IQR, 11.2-14.0) follow-up, estimated glomerular filtration rate <90 mL/min per 1.73 m² was present in 18 of 49 patients (36.7%), suggesting an average estimated glomerular filtration rate decline rate of -1.81 mL/min per 1.73 m² per year. Conclusions Children who developed AKI after pediatric cardiac surgery showed persistent markers of kidney injury. As chronic kidney disease is a risk factor for cardiovascular comorbidity, long-term kidney follow-up in this population is warranted.

Pulmonary Valve Replacement in Tetralogy of Fallot: An Updated Meta-Analysis.

BACKGROUND: The benefits of pulmonary valve replacement (PVR) for pulmonary insufficiency in patients with repaired tetralogy of Fallot are still incompletely understood, and optimal timing remains challenging. METHODS: We systematically reviewed databases (PubMed/MEDLINE, Embase, Cochrane Central Register of Controlled Trials /Cochrane Controlled Trials Register, ClinicalTrials.gov, Scientific Electronic Library Online, Literatura Latino Americana em Ciências da Saúde, and Google Scholar) and reference lists of relevant articles for studies about PVR in repaired tetralogy of Fallot patients that reported any of the following outcomes: mortality and redo PVR rates, right ventricular (RV) and left ventricular measures, QRS duration, cardiopulmonary exercise test results, or brain natriuretic peptide. In addition to calculating the pooled treatment effects using a random-effects meta-analysis, we evaluated the effect of preoperative measures on PVR outcomes using meta-regressions. RESULTS: Eighty-four studies involving 7544 patients met the eligibility criteria. Pooled mortality at 30 days, 5 years, and 10 years after PVR was 0.87% (63 of 7253 patients, 80 studies), 2.7% (132 of 4952 patients, 37 studies), and 6.2% (510 of 2765 patients, 15 studies), respectively. Pooled 5- and 10-year redo PVR rates were 3.7% (141 of 3755 patients, 23 studies) and 16.8% (172 of 3035 patients, 16 studies), respectively. The results of the previous meta-analysis could be confirmed. In addition, we demonstrated that after PVR (1) QRS duration, cardiopulmonary exercise test results, and RV and left ventricular measures longitudinal strain do not significantly change; (2) brain natriuretic peptide decreases; and (3) greater indexed RV end-diastolic and end-systolic volumes are associated with lower chances of RV volume normalization after PVR. CONCLUSIONS: This updated meta-analysis provides evidence about the benefits of PVR.

Tricuspid Valve Intervention at the Time of Pulmonary Valve Replacement in Adults With Congenital Heart Disease: A Systematic Review and Meta-Analysis.

Background Tricuspid regurgitation (TR) is a common finding in adults with congenital heart disease referred for pulmonary valve replacement (PVR). However, indications for combined valve surgery remain controversial. This study aimed to evaluate early results of concomitant tricuspid valve intervention (TVI) at the time of PVR. Methods and Results Observational studies comparing TVI+PVR and isolated PVR were identified by a systematic search of published research. Random-effects meta-analysis was performed, comparing outcomes between the 2 groups. Six studies involving 749 patients (TVI+PVR, 278 patients; PVR, 471 patients) met the eligibility criteria. In the pooled analysis, both TVI+PVR and PVR reduced TR grade, pulmonary regurgitation grade, right ventricular end-diastolic volume, and right ventricular end-systolic volumes. TVI+PVR, but not PVR, was associated with a decrease in tricuspid valve annulus size (mean difference, -6.43 mm, 95% CI, -10.59 to -2.27; P=0.010). Furthermore, TVI+PVR was associated with a larger reduction in TR grade compared with PVR (mean difference, -0.40; 95% CI, -0.75 to -0.05; P=0.031). No evidence could be established for an effect of either treatment on right ventricular ejection fraction or echocardiographic assessment of right ventricular dilatation and dysfunction. There was no evidence for a difference in hospital mortality or reoperation for TR. Conclusions While both strategies are effective in reducing TR and right ventricular volumes, routine TVI+PVR can reduce TR grade to a larger extent than isolated PVR. Further studies are needed to identify the subgroups of patients who might benefit most from combined valve surgery.

Strategies to Prevent Acute Kidney Injury after Pediatric Cardiac Surgery: A Network Meta-Analysis.

BACKGROUND AND OBJECTIVES: AKI is a common complication after pediatric cardiac surgery and has been associated with higher morbidity and mortality. We aimed to compare the efficacy of available pharmacologic and nonpharmacologic strategies to prevent AKI after pediatric cardiac surgery. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: PubMed/MEDLINE, Embase, Cochrane Controlled Trials Register, and reference lists of relevant articles were searched for randomized controlled trials from inception until August 2020. Random effects traditional pairwise, Bayesian network meta-analyses, and trial sequential analyses were performed. RESULTS: Twenty randomized controlled trials including 2339 patients and 11 preventive strategies met the eligibility criteria. No overall significant differences were observed compared with control for corticosteroids, fenoldopam, hydroxyethyl starch, or remote ischemic preconditioning in traditional pairwise meta-analysis. In contrast, trial sequential analysis suggested a 80% relative risk reduction with dexmedetomidine and evidence of <57% relative risk reduction with remote ischemic preconditioning. Nonetheless, the network meta-analysis was unable to demonstrate any significant differences among the examined treatments, including also acetaminophen, aminophylline, levosimendan, milrinone, and normothermic cardiopulmonary bypass. Surface under the cumulative ranking curve probabilities showed that milrinone (76%) was most likely to result in the lowest risk of AKI, followed by dexmedetomidine (70%), levosimendan (70%), aminophylline (59%), normothermic cardiopulmonary bypass (57%), and remote ischemic preconditioning (55%), although all showing important overlap. CONCLUSIONS: Current evidence from randomized controlled trials does not support the efficacy of most strategies to prevent AKI in the pediatric population, apart from limited evidence for dexmedetomidine and remote ischemic preconditioning.

Patient-Reported Outcomes in Adults With Congenital Heart Disease Following Hospitalization (from APPROACH-IS).

In this international study, we (1) compared patient-reported outcomes (PROs) in adults with congenital heart disease (CHD) who had versus had not been hospitalized during the previous 12 month, (2) contrasted PROs in patients who had been hospitalized for cardiac surgery versus nonsurgical reasons, (3) assessed the magnitude of differences between the groups (i.e., effect sizes), and (4) explored differential effect sizes between countries. APPROACH-IS was a cross-sectional, observational study that enrolled 4,028 patients from 15 countries (median age 32 years; 53% females). Self-report questionnaires were administered to measure PROs: health status; anxiety and depression; and quality of life. Overall, 668 patients (17%) had been hospitalized in the previous 12 months. These patients reported poorer outcomes on all PROs, with the exception of anxiety. Patients who underwent cardiac surgery demonstrated a better quality of life compared with those who were hospitalized for nonsurgical reasons. For significant differences, the effect sizes were small, whereas they were negligible in nonsignificant comparisons. Substantial intercountry differences were observed. For various PROs, moderate to large effect sizes were found comparing different countries. In conclusion, adults with CHD who had undergone hospitalization in the previous year had poorer PROs than those who were medically stable. Researchers ought to account for the timing of recruitment when conducting PRO research as hospitalization can impact results.

Assessment of long-term cardiac adaptation in adult patients with type II atrial septal defect : A cardiovascular magnetic resonance (CMR) study.

OBJECTIVES: In type II atrial septal defect (ASD) patients, the left-to-right (LR) shunt causes adaptation of the heart and circulation. The study objective was to evaluate with cardiovascular magnetic resonance imaging (CMR) the impact of LR shunt on left (LV) and right ventricular (RV) volumes, function, and myocardial strain. METHODS: Thirty-five patients (42 ± 17 years, 17 male) were compared to a control group (n = 40). Cine imaging was used to calculate ventricular volumes and ejection fraction (EF), global longitudinal (GLS) and circumferential strain (GCS), and longitudinal free wall (FWS) and interventricular septal (IVS) strain. Phase-contrast imaging was used to calculate pulmonary flow to systemic flow ratio (Qp/Qs). RESULTS: The LR shunt (Qp/Qs 2.2 ± 0.6) resulted in larger RV end-diastolic volume (EDVi) (152 ± 42 vs 82 ± 11 ml/m2), lower LV EDVi (72 ± 16 vs 83 ± 9 ml/m2), and higher RV/LV EDVi ratio (2.2 ± 0.5 vs 1.0 ± 0.1) than controls (all p < 0.001). Functionally, stroke volumes were larger in RV and lower in LV (both p < 0.001) with a strong trend toward lower RV EF in patients (p = 0.08). The LR shunt negatively impacted RV GLS (p = 0.03) but not RV GCS. Longitudinal IVS but not RV FWS were significantly lower in patients, i.e., p < 0.001, of longitudinal IVS. Shunt severity correlated with RV size and stroke volume, right atrial size, and pulmonary trunk diameter (all p < 0.001), but not with functional nor strain parameters. CONCLUSION: Long-term cardiac adaptation in ASD patients, with RV overfilling and LV underfilling, has a negative impact on systolic RV performance, a phenomenon which likely can be attributed to longitudinal dysfunction of the interventricular septum. KEY POINTS: • An LR shunt in type II ASD patients causes cardiac remodeling characterized by RV overfilling and conversely underfilling of the left ventricle. • At the long term, there is evidence of systolic dysfunction of the right ventricle in this group of patients. • Septal dysfunction underlies the observed impairment in RV function.

Perioperative Risk Factors for Mortality in Patients Undergoing Surgery for Ebstein's Anomaly at a Single Institution: A Retrospective Analysis.

BACKGROUND: Mortality after cardiac surgery for Ebstein's anomaly ranges from 2.5% to 31%. Independent predictors for mortality and morbidity remain poorly defined because of the low incidence of this congenital anomaly. To identify potentially modifiable factors, this retrospective study investigates the prognostic value of perioperative variables for mortality and morbidity. METHODS: We reviewed the charts of 171 patients with Ebstein's anomaly who were at one point in follow-up at our center. Only patients who underwent cardiac surgery for this anomaly were included. The primary endpoint was a composite of mortality or rehospitalization for cardiac reasons within 1 year of surgery. Logistic regression and Cox regression models were used to study the predictive value of various variables. RESULTS: We identified 32 patients (median age 12 years; range 7 days to 70 years) who underwent a total of 49 surgical procedures for Ebstein's anomaly at our institution between November 1987 and March 2015. The following variables were significantly associated with the primary outcome: increased severity of tricuspid valve stenosis (odds ratio 2.089; 95% confidence interval 1.175 to 3.713) and right ventricular dysfunction (1.826; 1.109 to 3.006), partial corrective surgery (versus corrective surgery) (6.709; 1.436 to 31.344), occurrence of major postoperative complications (5.460; 1.419 to 21.008), and increased length of stay in the intensive care unit (1.051; 1.010 to 1.093). A better outcome was observed with the use of intraoperative cardioplegic arrest (0.185; 0.063 to 0.550), atrial septal defect closure during surgery, and longer duration of surgery (0.991; 0.984 to 0.998). CONCLUSION: Several patient-specific characteristics and perioperative characteristics were associated with a poorer outcome after cardiac surgery for Ebstein's anomaly. The outcome seems to be primarily determined by the severity of the valve dysfunction and right ventricular performance, with only a minor role for perioperative surgical or anesthetic technical determinants.

Outcome of arterial switch operation for transposition of the great arteries. A 35-year follow-up study.

BACKGROUND: Arterial switch operation (ASO) is today the first-choice surgical treatment for patients with transposition of the great arteries. Long-term outcome data still remain scarce. Moreover, the course of these patients is not uneventful. Therefore, we wanted to evaluate long-term outcome and determine on which variables to focus during follow-up. METHODS: Clinical records of 318 patients who underwent ASO between October 1981 and July 2018 were reviewed. Perioperative, post-operative, and interventional data were collected to determine mortality and the need for re-intervention. Descriptive statistics and Kaplan-Meier survival analysis were performed. RESULTS: Mean follow-up time was 11.1 SD 8.5 years (range 0-35) with a mean age of 12.5 SD 9.0 years (range 0-37) at latest follow-up. In-hospital mortality was 7.5% and overall survival 90.9% for a maximum follow-up time of 35 years. Causes of early mortality were cardiogenic shock, severe pulmonary hypertension, septic shock and multiple organ failure. Causes of late mortality were cardiogenic shock, severe pulmonary hypertension, pacemaker lead fracture and fire death. Re-intervention free survival at 5-year was 91.6%, at 10-year 90.7%, at 20-year 79.2%. For all survivors, the most frequent sequelae after ASO were pulmonary artery stenosis (80.9%), of which 13.5% needed an intervention. The threshold for intervening on lesions at the level of the pulmonary artery bifurcation was higher and the percutaneous re-intervention rate was higher for non-bifurcation lesions. CONCLUSIONS: Despite a relatively high peri-operative mortality, TGA patients have an excellent overall long-term survival. However, a large proportion of patients requires re-interventions, mainly for pulmonary artery stenosis.

Cardiac interventions in pregnancy and peripartum - a narrative review of the literature.

In the UK, mortality rate during pregnancy/in the peripartal period is 14.1 per 100,000 maternities with heart disease being the leading cause of non-obstetric maternal mortality (10% to 15% of all maternal deaths). Owing to the advances in the treatment of congenital heart disease (CHD), an increasing percentage of women has reached childbearing age, making CHD nowadays the most frequent cardiovascular disease during pregnancy in the Western world. In the pregnant woman, several adaptive changes occur in the cardiovascular and pulmonary system that in the worst case can lead to cardiovascular collapse in women with pre-existing heart disease. If medical management is not sufficient, cardiac interventions have to be considered, including percutaneous endovascular interventions, cardiac surgery, the use of extracorporeal membrane oxygenation or the placement of assist devices. While all these interventions seem to be relatively safe for the mother, fetal mortality remains considerably high. A thorough understanding of maternal physiology during pregnancy and of the perfusion of the feto-maternal unit is mandatory for the successful management of pregnant patients in need of cardiac (surgical) interventions.

Health behaviours reported by adults with congenital heart disease across 15 countries.

BACKGROUND: Health behaviours are essential to maintain optimal health and reduce the risk of cardiovascular complications in adults with congenital heart disease. This study aimed to describe health behaviours in adults with congenital heart disease in 15 countries and to identify patient characteristics associated with optimal health behaviours in the international sample. DESIGN: This was a cross-sectional observational study. METHODS: Adults with congenital heart disease (n = 4028, median age = 32 years, interquartile range 25-42 years) completed self-report measures as part of the Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study (APPROACH-IS). Participants reported on seven health behaviours using the Health Behaviors Scale-Congenital Heart Disease. Demographic and medical characteristics were assessed via medical chart review and self-report. Multivariate path analyses with inverse sampling weights were used to investigate study aims. RESULTS: Health behaviour rates for the full sample were 10% binge drinking, 12% cigarette smoking, 6% recreational drug use, 72% annual dental visit, 69% twice daily tooth brushing, 27% daily dental flossing and 43% sport participation. Pairwise comparisons indicated that rates differed between countries. Rates of substance use behaviours were higher in younger, male participants. Optimal dental health behaviours were more common among older, female participants with higher educational attainment while sports participation was more frequent among participants who were younger, male, married, employed/students, with higher educational attainment, less complex anatomical defects and better functional status. CONCLUSIONS: Health behaviour rates vary by country. Predictors of health behaviours may reflect larger geographic trends. Our findings have implications for the development and implementation of programmes for the assessment and promotion of optimal health behaviours in adults with congenital heart disease.

Outcome of the Glenn procedure as definitive palliation in single ventricle patients.

OBJECTIVES: In selected single ventricle patients, a Glenn procedure (SV-Glenn) may be considered as definitive palliation. Either the patient is unsuited to progress to a Fontan circulation or a SV-Glenn circulation is preferred. This study aimed at describing the clinical course, and long-term mortality/morbidity of SV-Glenn patients. METHODS: All SV-Glenn patients followed at the University Hospitals Leuven before May 2018 were included. Patients who underwent, or were awaiting, TCPC completion and those who underwent a Glenn in the setting of a biventricular circulation one-and-a-half repair (OAHR), were excluded. RESULTS: Of 65 Glenn-only patients identified, 21 (32%) had OAHR, whereas 44 (68%) were SV-Glenn patients. Of SV-Glenn patients, 19 died within 6 months after the Glenn procedure. Of 25 SV-Glenn survivors, median age at Glenn was 6.3 (IQR 1.2-29.7) years. Eight were unsuited for TCPC completion; in 17 SV-Glenn was preferred over TCPC completion. Over a median follow-up time of 11 (IQR 3-18) years after the Glenn procedure, 5 (20%) patients died. At latest follow-up 10 (40%) had heart failure, 5 (20%) had atrial and 4 (16%) ventricular arrhythmias, 2 (8%) a thromboembolic event, 7 (28%) required pacemaker implantation, and 2 (8%) had infective endocarditis but none developed cirrhosis or protein-losing enteropathy. Mean saturation at latest follow-up was 87 ± 7%. CONCLUSION: SV-Glenn patients represent a unique and heterogeneous patient population. Outcome was reasonable, although comorbidities, such as heart failure and arrhythmias were not uncommon. In SV-Glenn patients, 'classic' complications related to Fontan physiology, such as cirrhosis and protein-losing enteropathy, were absent.