Biventricular responses to exercise and their relation to cardiorespiratory fitness in pediatric pulmonary hypertension.

Despite exercise intolerance being predictive of outcomes in pulmonary arterial hypertension (PAH), its underlying cardiac mechanisms are not well described. The aim of the study was to explore the biventricular response to exercise and its associations with cardiorespiratory fitness in children with PAH. Participants underwent incremental cardio-pulmonary exercise testing and simultaneous exercise echocardiography on a recumbent cycle ergometer. Linear mixed models were used to assess cardiac function variance and associations between cardiac and metabolic parameters during exercise. Eleven participants were included with a mean age 13.4 ±2.9 years. Right ventricle (RV) systolic pressure (RVsp) increased from a mean of 59 ±25 mmHg at rest to 130 ±40 mmHg at peak exercise (p<0.001), while RV fractional area change (RV-FAC) and RV free wall longitudinal strain (RVFW-Sl) worsened (35.2% vs 27%, p=0.09 and -16.6% vs -14.6%, p=0.1, respectively). At low and moderate intensity exercise, RVsp was positively associated with stroke volume and O2 pulse (p<0.1). At high intensity exercise RV-FAC, RVFW-Sl and left ventricular longitudinal strain were positively associated with oxygen uptake and O2 pulse (p<0.1), while stroke volume decreased towards peak (p=0.04). In children with PAH, the increase of pulmonary pressure alone does not limit peak exercise, but rather the concomitant reduced RV functional reserve, resulting in RV-PA uncoupling, worsening of inter-ventricular interaction and LV dysfunction. A better mechanistic understanding of PAH exercise physiopathology can inform stress testing and cardiac rehabilitation in this population.

Prospective evaluation of acute neurological events after paediatric cardiac surgery.

OBJECTIVES: Children with CHD are at heightened risk of neurodevelopmental problems; however, the contribution of acute neurological events specifically linked to the perioperative period is unclear. AIMS: This secondary analysis aimed to quantify the incidence of acute neurological events in a UK paediatric cardiac surgery population, identify risk factors, and assess how acute neurological events impacted the early post-operative pathway. METHODS: Post-operative data were collected prospectively on 3090 consecutive cardiac surgeries between October 2015 and June 2017 in 5 centres. The primary outcome of analysis was acute neurological event, with secondary outcomes of 6-month survival and post-operative length of stay. Patient and procedure-related variables were described, and risk factors were statistically explored with logistic regression. RESULTS: Incidence of acute neurological events after paediatric cardiac surgery in our population occurred in 66 of 3090 (2.1%) consecutive cardiac operations. 52 events occurred with other morbidities including renal failure (21), re-operation (20), cardiac arrest (20), and extracorporeal life support (18). Independent risk factors for occurrence of acute neurological events were CHD complexity 1.9 (1.1-3.2), p = 0.025, longer operation times 2.7 (1.6-4.8), p < 0.0001, and urgent surgery 3.4 (1.8-6.3), p < 0.0001. Unadjusted comparison found that acute neurological event was linked to prolonged post-operative hospital stay (median 35 versus 9 days) and poorer 6-month survival (OR 13.0, 95% CI 7.2-23.8). CONCLUSION: Ascertainment of acute neurological events relates to local measurement policies and was rare in our population. The occurrence of acute neurological events remains a suitable post-operative metric to follow for quality assurance purposes.

Protecting the stars of tomorrow: do international cardiovascular preparticipation screening policies account for the paediatric athlete? A systematic review and quality appraisal.

OBJECTIVE: (1) Identify and review current policies for the cardiovascular screening of athletes to assess their applicability to the paediatric population and (2) evaluate the quality of these policy documents using the Appraisal of Guidelines for Research & Evaluation II (AGREE II) tool. DESIGN: Systematic review and quality appraisal of policy documents. DATA SOURCES: A systematic search of PubMed, MEDLINE, Scopus, Web of Science, SportDiscus and CINAHL. ELIGIBILITY CRITERIA FOR SELECTING STUDIES: An article was included if it was a policy/position statement/guideline/consensus or recommendation paper relating to athletes and cardiovascular preparticipation screening. RESULTS AND SUMMARY: Of the 1630 articles screened, 13 met the inclusion criteria. Relevance to paediatric athletes was found to be high in 3 (23%), moderate in 6 (46%) and low in 4 (31%), and only 2 provide tailored guidance for the athlete aged 12-18 years. A median 5 related citations per policy investigated solely paediatric athletes, with study designs most commonly being retrospective (72%). AGREEII overall quality scores ranged from 25% to 92%, with a median of 75%. The lowest scoring domains were rigour of development; (median 32%) stakeholder involvement (median 47%) and Applicability (median 52%). CONCLUSION: Cardiac screening policies for athletes predominantly focus on adults, with few providing specific recommendations for paediatric athletes. The overall quality of the policies was moderate, with more recent documents scoring higher. Future research is needed in paediatric athletes to inform and develop cardiac screening guidelines, to improve the cardiac care of youth athletes.

Investigating the Accuracy of Quantitative Echocardiographic-Modified Task Force Criteria for Arrhythmogenic Ventricular Cardiomyopathy in Adolescent Male Elite Athletes.

Athlete preparticipation screening focuses on preventing sudden cardiac death (SCD) by detecting diseases such as arrhythmogenic ventricular cardiomyopathy (AVC), which affects primarily the right ventricular myocardium. Diagnosis may be obscured by physiological remodeling of the athlete heart. Healthy athletes may meet the 2010 Task Force Criteria right ventricular outflow tract (RVOT) dimension cut-offs, questioning the suitability of the modified Task Force Criteria (mTFC) in adolescent athletes. In this study, 67 male adolescent footballers undergoing preparticipation screening were reviewed. All athletes underwent a screening for resting ECG and echocardiogram according to the English FA protocol, as well as cardiopulmonary exercise testing, stress ECG, and exercise echocardiography. Athletes' right ventricular outflow tract (RVOT) that met the major AVC diagnostic criteria for dilatation were identified. Of 67 evaluated athletes, 7 had RVOT dilatation that met the major criteria, all in the long axis parasternal view measurement. All had normal right ventricular systolic function, including normal free-wall longitudinal strain (ranging from - 21.5 to - 32.7%). Left ventricular ejection fraction ranged from 52 to 67%, without evidence of structural changes. Resting ECGs and cardiopulmonary exercise tests were normal in all individuals. In a series of healthy athletes meeting the major AVC diagnostic criteria for RVOT dilatation, none had any other pathological changes on a detailed screening including ECG, exercise testing, and echocardiography. This report highlights that current AVC echocardiographic diagnosis criteria have limitations in this population.

The Role of Speckle-Tracking Echocardiography in Predicting Mortality and Morbidity in Patients With Congenital Heart Disease: A Systematic Review and Meta-analysis.

BACKGROUND: Speckle-tracking echocardiography (STE) is now routinely included in cardiac evaluations, but its role in predicting mortality and morbidity in congenital heart disease (CHD) is not well described. We conducted a systematic review to evaluate the prognostic value of STE in patients with CHD. METHODS: The EMBASE, Medline, Web of Science, Scopus, and Cochrane Central Register of Controlled Trials (CENTRAL) databases were searched from inception to January 2023 for terms related to all CHD, STE, and prognosis. Meta-analysis of association of right ventricle and left ventricle strain (RV Sl and LV Sl, respectively) with major adverse cardiovascular events (MACEs) was performed in atrial switch transposition of the great arteries (asTGA)/congenitally corrected TGA (ccTGA), tetralogy of Fallot (ToF), and congenital aortic stenosis (cAS)/bicuspid aortic valve (BAV). P-value combination analysis was additionally performed for all CHD groups. RESULTS: A total of 33 studies (30 cohorts, n = 8,619 patients, children, and adults) were included. Meta-analysis showed the following parameters as being associated with MACE: RV Sl in asTGA/ccTGA (hazard ratio [HR] = 1.1/%; CI, [1.03; 1.18]), RV Sl and LV Sl in ToF (HR = 1.14/%; CI, [1.03; 1.26] and HR = 1.14/%; CI, [1.08; 1.2], respectively), and LV Sl in cAS/BAV (HR = 1.19/%; CI, [1.15; 1.23]). The RV Sl and strain rate were associated with outcomes also in single ventricle/hypoplastic left heart syndrome (at all palliation stages except before Norwood stage 1) and LV Sl in Ebstein's anomaly. CONCLUSIONS: This systematic review and meta-analysis showed that biventricular strain and strain rate were associated with outcomes in a variety of CHD, highlighting the need for updated recommendations on the use of STE in the current guidelines, specific to disease types.

A new protocol for a single-stage combined cardiopulmonary and echocardiography exercise test: a pilot study.

Aims: The value of cardiopulmonary exercise testing (CPET) and exercise stress echocardiography (ESE) in managing cardiac disease is well known, but no standard CPET-ESE protocol is currently recommended. This pilot study aims to compare feasibility and cardiac function responses between a new high-intensity single-stage combined test (CPET-hiESE) and a standard maximal ESE (smESE). Methods and results: After screening and maximal CPET, all volunteers (n = 21) underwent three ESE modalities: (i) based on the gas exchange threshold (hiESE-GET, 40% of peak-GET, 6 min), (ii) based on heart rate (HR) (hiESE-HR, 80% of peak HR, 6 min), and (iii) smESE (85% of predicted peak HR for age, 3 min). Speckle tracking echocardiography (STE) and tissue Doppler imaging (TDI) were measured at each step. There was superior image quality and data completeness for the right ventricle strain for both hiESE modalities compared with smESE (71.4 and 76.2 vs. 42.9%, P = 0.07). Left ventricular STE data completeness was similar for all three conditions. Despite systematically higher HR, work rate and levels of exertion in the smESE compared with hiESE, STE and TDI parameters were not systematically different. Concordance correlation coefficients ranged from 0.56 to 0.88, lowest for strain rate parameters and mean difference from -0.34 to 1.53, highest for TDI measurements. Conclusion: The novel CPET-hiESE protocol allowed for better data completeness, at lower levels of exertion compared with smESE, without systematically different cardiac reserve measurements in healthy participants. This single-stage protocol can be individualized to clinical populations, which would provide practical advantages to standard testing.

Unprotected Left Main Bifurcation Stenting in Acute Coronary Syndromes: Two-Stent Technique versus One-Stent Technique.

AIMS: There is little evidence guiding the choice between a one-stent and a two-stent approach in unprotected distal left main coronary artery disease (UDLMCAD) presenting as acute coronary syndrome (ACS). We aim to compare these two techniques in an unselected ACS group. METHODS AND RESULTS: We conducted a single center retrospective observational study, that included all patients with UDLMCAD and ACS undergoing PCI between 2014 and 2018. Group A underwent PCI with a one-stent technique (n = 41, 58.6%), Group B with a two-stent technique (n = 29, 41.4%). A total of 70 patients were included, with a median age of 63 years, including n = 12 (17.1%) with cardiogenic shock. There were no differences between Group A and B in terms of patient characteristics, including SYNTAX score (median 23). The 30-day mortality was 15.7% overall, and was lower in Group B (3.5% vs. 24.4%, p = 0.02). Mortality rate at 4 years was significantly lower in Group B (21.4% vs. 44%), also when adjusted in a multivariable regression model (HR 0.26, p = 0.01). CONCLUSIONS: In our study, patients with UDLMCAD and ACS undergoing PCI using a two-stent technique had lower early and midterm mortality compared to one-stent approach, even after adjusting for patient-related or angiographic factors.

Arterial Switch for Transposition of the Great Arteries: Treatment Timing, Late Outcomes, and Risk Factors.

Background: Reports of long-term mortality and reintervention after transposition of the great arteries with intact ventricular septum treatment, although favorable, are mostly limited to single-center studies. Even less is known about hospital resource utilization (days at hospital) and the impact of treatment choices and timing on outcomes. Objectives: The purpose of this study was to describe survival, reintervention and hospital resource utilization after arterial switch operation (ASO) in a national dataset. Methods: Follow-up and life status data for all patients undergoing ASO between 2000 and 2017 in England and Wales were collected and explored using multivariable regressions and matching. Results: A total of 1,772 patients were identified, with median ASO age of 9.5 days (IQR: 6.5-14.5 days). Mortality and cardiac reintervention at 10 years after ASO were 3.2% (95% CI: 2.5%-4.2%) and 10.7% (95% CI: 9.1%-12.2%), respectively. The median time spent in hospital during the ASO spell was 19 days (IQR: 14, 24). Over the first year after the ASO patients spent 7 days (IQR: 4-10 days) in hospital in total, decreasing to 1 outpatient day/year beyond the fifth year. In a subgroup with complete risk factor data (n = 652), ASO age, and balloon atrial septostomy (BAS) use were not associated with late mortality and reintervention, but cardiac or congenital comorbidities, low weight, and circulatory/renal support at ASO were. After matching for patient characteristics, BAS followed by ASO and ASO as first procedure, performed within the first 3 weeks of life, had comparable early and late outcomes, including hospital resource utilization. Conclusions: Mortality and hospital resource utilization are low, while reintervention remains relatively frequent. Early ASO and individualized use of BAS allows for flexibility in treatment choices and a focus on at-risk patients.

Hospital resource utilization in a national cohort of functionally single ventricle patients undergoing surgical treatment.

Objective: The study objective was to provide a detailed overview of health resource use from birth to 18 years old for patients with functionally single ventricles and identify associated risk factors. Methods: All patients with functionally single ventricles treated between 2000 and 2017 in England and Wales were linked to hospital and outpatient records using data from the Linking AUdit and National datasets in Congenital HEart Services project. Hospital stay was described in yearly age intervals, and associated risk factors were explored using quantile regression. Results: A total of 3037 patients with functionally single ventricles were included, 1409 (46.3%) undergoing a Fontan procedure. During the first year of life, the median days spent in hospital was 60 (interquartile range, 37-102), mostly inpatient days, mirroring a mortality of 22.8%. This decreases to between 2 and 9 in-hospital days/year afterward. Between 2 and 18 years, most hospital days were outpatient, with a median of 1 to 5 days/year. Lower age at the first procedure, hypoplastic left heart syndrome/mitral atresia, unbalanced atrioventricular septal defect, preterm birth, congenital/acquired comorbidities, additional cardiac risk factors, and severity of illness markers were associated with fewer days at home and more intensive care unit days in the first year of life. Only markers of early severe illness were associated with fewer days at home in the first 6 months after the Fontan procedure. Conclusions: Hospital resource use in functionally single ventricle cases is not uniform, decreasing 10-fold during adolescence compared with the first year of life. There are subsets of patients with worse outcomes during their first year of life or with persistently high hospital use throughout their childhood, which could be the target of future research.

The use of 2-D speckle tracking echocardiography in assessing adolescent athletes with left ventricular hypertrabeculation meeting the criteria for left ventricular non-compaction cardiomyopathy.

BACKGROUND: Current echocardiographic criteria cannot accurately differentiate exercise induced left ventricular (LV) hypertrabeculation in athletes from LV non-compaction cardiomyopathy (LVNC). This study aims to evaluate the role of speckle tracking echocardiography (STE) in characterising LV myocardial mechanics in healthy adolescent athletes with and without LVNC echocardiographic criteria. METHODS: Adolescent athletes evaluated at three sports academies between 2014 and 2019 were considered for this observational study. Those meeting the Jenni criteria for LVNC (end-systolic non-compacted/compacted myocardium ratio > 2 in any short axis segment) were considered LVNC+ and the rest LVNC-. Peak systolic LV longitudinal strain (Sl), circumferential strain (Sc), rotation (Rot), corresponding strain rates (SRl/c) and segmental values were calculated and compared using a non-inferiority approach. RESULTS: A total of 417 participants were included, mean age 14.5 ± 1.7 years, of which 6.5% were LVNC+ (n = 27). None of the athletes showed any additional LVNC clinical criteria. All average Sl, SRl Sc, SRc and Rot values were no worse in the LVNC+ group compared to LVNC- (p values range 0.0003-0.06), apart from apical SRc (p = 0.2). All 54 segmental measurements (Sl/Sc SRl/SRc and Rot) had numerically comparable means in both LVNC+ and LVNC-, of which 69% were also statistically non-inferior. CONCLUSIONS: Among healthy adolescent athletes, 6.5% met the echocardiographic criteria for LVNC, but showed normal LV STE parameters, in contrast to available data on paediatric LVNC describing abnormal myocardial function. STE could better characterise the myocardial mechanics of athletes with LV hypertrabeculation, thus allowing the transition from structural to functional LVNC diagnosis, especially in suspected physiological remodelling.

The use of 2-D speckle tracking echocardiography in differentiating healthy adolescent athletes with right ventricular outflow tract dilation from patients with arrhythmogenic cardiomyopathy.

AIMS: Echocardiographic assessment of adolescent athletes for arrhythmogenic cardiomyopathy (ACM) can be challenging owing to right ventricular (RV) exercise-related remodelling, particularly RV outflow tract (RVOT) dilation. The aim of this study is to evaluate the role of RV 2-D speckle tracking echocardiography (STE) in comparing healthy adolescent athletes with and without RVOT dilation to patients with ACM. METHODS AND RESULTS: A total of 391 adolescent athletes, mean age 14.5 ± 1.7 years, evaluated at three sports academies between 2014 and 2019 were included, and compared to previously reported ACM patients (n = 38 definite and n = 39 borderline). Peak systolic RV free wall (RVFW-Sl), global and segmental strain (Sl), and corresponding strain rates (SRl) were calculated. The participants meeting the major modified Task Force Criteria (mTFC) for RVOT dilation were defined as mTFC+ (n = 58, 14.8%), and the rest as mTFC- (n = 333, 85.2%). Mean RVFW-Sl was -27.6 ± 3.4% overall, -28.2 ± 4.1% in the mTFC+ group and - 27.5 ± 3.3% in the mTFC- group. mTFC+ athletes had normal RV-FW-Sl when compared to definite (-29% vs -19%, p < 0.001) and borderline ACM (-29% vs -21%, p < 0.001) cohorts. In addition, all mean global and regional Sl and SRl values were no worse in the mTFC+ group compared to the mTFC- (p values range < 0.0001 to 0.1, inferiority margin of 2% and 0.1 s-1 respectively). CONCLUSIONS: In athletes with RVOT dilation meeting the major mTFC, STE evaluation of the RV can demostrate normal function and differentiate physiological remodelling from pathological changes found in ACM, improving screening in grey-area cases.

The role of cardiopulmonary exercise testing in predicting mortality and morbidity in people with congenital heart disease: a systematic review and meta-analysis.

AIMS: The role of cardiopulmonary exercise testing (CPET) in predicting major adverse cardiovascular events (MACE) in people with congenital heart disease (ConHD) is unknown. A systematic review with meta-analysis was conducted to report the associations between CPET parameters and MACE in people with ConHD. METHODS AND RESULTS: Electronic databases were systematically searched on 30 April 2020 for eligible publications. Two authors independently screened publications for inclusion, extracted study data, and performed risk of bias assessment. Primary meta-analysis pooled univariate hazard ratios across studies. A total of 34 studies (18 335 participants; 26.2 ± 10.1 years; 54% ± 16% male) were pooled into a meta-analysis. More than 20 different CPET prognostic factors were reported across 6 ConHD types. Of the 34 studies included in the meta-analysis, 10 (29%), 23 (68%), and 1 (3%) were judged as a low, medium, and high risk of bias, respectively. Primary univariate meta-analysis showed consistent evidence that improved peak and submaximal CPET measures are associated with a reduce risk of MACE. This association was supported by a secondary meta-analysis of multivariate estimates and individual studies that could not be numerically pooled. CONCLUSION: Various maximal and submaximal CPET measures are prognostic of MACE across a variety of ConHD diagnoses. Further well-conducted prospective multicentre cohort studies are needed to confirm these findings.

Exercise training in paediatric congenital heart disease: fit for purpose?

Exercise and physical activity (PA) have been shown to be effective, safe and feasible in both healthy children and children with congenital heart disease (CHD). However, implementing exercise training as an intervention is still not routine in children with CHD despite considerable evidence of health benefits and well-being. Understanding how children with CHD can safely participate in exercise can boost participation in PA and subsequently reduce inactivity-related diseases. Home-based exercise intervention, with the use of personal wearable activity trackers, and high-intensity interval training have been beneficial in adults' cardiac rehabilitation programmes. However, these remain underutilised in paediatric care. Therefore, the aims of this narrative review were to synthesise prescribed exercise interventions in children with CHD, identify possible limitation to exercise training prescription and provide an overview on how to best integrate exercise intervention effectively for this population into daily practice.

Left main coronary artery compression by dilated pulmonary artery in pulmonary arterial hypertension: a systematic review and meta-analysis.

OBJECTIVE: Pulmonary arterial hypertension (PAH) can lead to left main coronary artery compression (LMCo), but data on the impact, screening and treatment are limited. A meta-analysis of LMCo cases could fill the knowledge gaps in this topic. METHODS: Electronic databases were searched for all LMCo/PAH studies, abstracts and case reports including pulmonary artery (PA) size. Restricted maximum likelihood meta-analysis was used to evaluate LMCo-associated factors. Specificity, sensitivity and accuracy of PA size thresholds for diagnosis of LMCo were calculated. Treatment options and outcomes were summarized. RESULTS: A total of five case-control cohorts and 64 case reports/series (196 LMCo and 438 controls) were included. LMCo cases had higher PA diameter (Hedge's g 1.46 [1.09; 1.82]), PA/aorta ratio (Hedge's g 1.1 [0.64; 1.55]) and probability of CHD (log odds-ratio 1.22 [0.54; 1.9]) compared to non-LMCo, but not PA pressure or vascular resistance. A 40 mm cut-off for the PA diameter had balanced sensitivity (80.5%), specificity (79%) and accuracy (79.7%) for LMCo diagnosis, while a value of 44 mm had higher accuracy (81.7%), higher specificity (91.5%) but lower sensitivity (71.9%). Pooled mortality after non-conservative treatment (n = 150, predominantly stenting) was 2.7% at up to 22 months of mean follow-up, with 83% survivors having no angina at follow-up. CONCLUSION: PA diameter, PA/aorta ratio and CHD are associated with LMCo, while hemodynamic parameters are not. Data from this study support that a PA diameter cut-off between 40 and 44 mm can offer optimal accuracy for LMCo screening. Preferred treatment was coronary stenting, associated with low mid-term mortality and symptom relief. Diagnosis and management of left main coronary artery compression (LMCo) in patients with pulmonary arterial hypertension (PAH).

Aortic root translocation and en bloc rotation of the outflow tracts surgery for complex forms of transposition of the great arteries and double outlet right ventricle: A multicenter study.

OBJECTIVE: There are several choices for the correction of complex transposition of the great arteries and double outlet right ventricle not amenable to the Rastelli-type surgery, but outcome data are limited to small series. This study aims to report results after the aortic root translocation and en bloc rotation of the outflow tract procedures. METHODS: This is a retrospective, multicentric, observational study. Clinical, anatomy, procedural, and detailed follow-up data (median, 4.43 years) were collected. RESULTS: A total of 70 patients (62.9% male; median age, 1 year; range 4 days to 12.4 years) were included: n = 43 in the aortic root translocation group and n = 27 in the en bloc rotation group. Those in the aortic root translocation group were older (P = .01) and more likely to have had previous procedures (P < .0001), but cardiac anatomy was similar in both groups. Aortic root translocation and en bloc rotation early mortality (30 days) was similar (4.7% vs 3.7%, P = .8). Late survival and freedom from any cardiac reintervention were 92.7% and 16.9% at 15 years overall, respectively. Freedom from right ventricular outflow tract/conduit reintervention was better in the en bloc rotation group than in the aortic root translocation group (100% vs 24.5%, P = .0003), but more patients in the en bloc rotation group had moderate (or worse) aortic valve regurgitation during follow-up (16% vs 2.6%, P = .07). CONCLUSIONS: Both aortic root translocation and en bloc rotation are valuable surgical options for the treatment of complex transposition of the great arteries and double outlet right ventricle. In the en bloc rotation group, there was better freedom from right ventricular outflow tract reinterventions, but a higher probability of aortic valve regurgitation. Identifying the main driving forces for these observed differences requires further study of these procedures.

The Role of Speckle Tracking Echocardiography in the Evaluation of Common Inherited Cardiomyopathies in Children and Adolescents: A Systematic Review.

Speckle tracking echocardiography (STE) has gained importance in the evaluation of adult inherited cardiomyopathies, but its utility in children is not well characterized. We conducted a systematic review to evaluate the role of STE in pediatric inherited cardiomyopathies. PubMed, EMBASE, Web of Science, Scopus, CENTRAL and CINAHL databases were searched up to May 2020, for terms related to inherited cardiomyopathies and STE. Included were dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), left ventricular non-compaction (LVNC) and arrhythmogenic cardiomyopathy (ACM). A total of 14 cohorts were identified, of which six were in DCM, four in HCM, three in LVNC and one in ACM. The most commonly reported STE measurements were left ventricular longitudinal strain (Sl), circumferential strain (Sc), radial strain (Sr) and rotation/torsion/twist. Sl, Sc and were abnormal in all DCM and LVNC cohorts, but not in all HCM. Apical rotation and twist/torsion were increased in HCM, and decreased in LVNC. Abnormal STE parameters were reported even in cohorts with normal non-STE systolic/diastolic measurements. STE in childhood cardiomyopathies can detect early changes which may not be associated with changes in cardiac function detectable by non-STE methods. Longitudinal and circumferential strain should be introduced in the cardiomyopathy echocardiography protocol, reflecting current practice in adults.

Four-year outcomes after percutaneous coronary intervention of unprotected left main coronary artery disease in patients with stable angina and acute coronary syndrome.

Background. Percutaneous coronary intervention (PCI) of unprotected left main coronary artery disease (ULMCAD) have become a feasible and efficient alternative to coronary artery bypass surgery, especially in patients with acute coronary syndrome (ACS). There are limited data regarding early and late outcomes after ULMCAD PCI in patients with ACS and stable angina.The aim of this study was to compare early and four-year clinical outcomes in patients with ULMCAD PCI presenting as ACS or stable angina in a high-volume PCI center.Methods. We conducted a single center retrospective observational study, which included 146 patients with ULMCAD undergoing PCI between 2014 and 2018. Patients were divided in two groups: Group A included patients with stable angina (n = 70, 47.9%) and Group B patients with ACS (n = 76, 52.1%).Results. 30-day mortality was 8.22% overall, lower in Group A (1.43% vs 14.47%, p = 0.02). Mortality and major adverse cardiac events (MACE) rates at 4 years were significantly lower in Group A (9.64% vs 33.25%, p = 0.001, and 24.06% vs 40.11%, p = 0.012, respectively). Target lesion revascularization (TLR) at 4 year did not differ between groups (15% in Group A vs 12.76% in Group B, p = 0.5).Conclusions. In our study patients with ULMCAD and ACS undergoing PCI had higher early and long-term mortality and MACE rates compared to patients with stable angina, with similar TLR rate at 4-year follow-up.

Factors associated with unplanned reinterventions and their relation to early mortality after pediatric cardiac surgery.

OBJECTIVE: Unplanned reintervention (uRE) is used as an indicator of patient morbidity and quality of care in pediatric cardiac surgery. We investigated associated factors and early mortality after uREs. METHODS: Morbidity data were prospectively collected in 5 UK centers between 2015 and 2017; uRE included surgical cardiac, interventional transcatheter cardiac, permanent pacemaker, and diaphragm plication procedures. Mortality (30-day and 6-month) in uRE/no-uRE patients was reported before and after matching. Predicted 30-day mortality was calculated using the Partial Risk Adjustment in Surgery score. RESULTS: A total of 3090 procedures (2861 patients) were included (median age, 228 days). There were 146 uREs, resulting in an uRE rate of 4.7%. Partial Risk Adjustment in Surgery score, 30-day mortality and 6-month mortality in uRE and no-uRE groups were 2.4% versus 1.3%, 8.9% versus 1%, and 17.1% versus 2.4%, respectively. After matching, mortality at 6 months remained higher in uRE compared with no-uRE (12.2% vs 1.4%; P = .02; 74 pairs). In the uRE group, 21 out of 25 deaths at 6 months occurred when at least 1 additional postoperative complication was present. In multivariable analysis, neonatal age (P = .002), low weight (P = .009), univentricular heart (P < .001), and arterial shunt (P < .001) were associated with increased risk of uRE, but Partial Risk Adjustment in Surgery score was not (only in univariable analysis). CONCLUSIONS: uREs are a relatively frequent complication after pediatric cardiac surgery and are associated with some patient characteristics, but not the Partial Risk Adjustment in Surgery risk score. Early mortality was higher after uRE, independent of preoperative factors, but linked to other postoperative complications.

Does routine fenestration improve early and late postoperative outcomes in patients undergoing Fontan palliation?

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was: 'In [patients undergoing Fontan palliation] does [fenestration] affect [early and late postoperative outcomes]?' Altogether 509 papers were found using the reported search, of which 11 papers represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. Current data suggest that the use of fenestration has advantages in the immediate postoperative course, with fewer complications such as pleural effusions, shorter hospitalization and decreased early Fontan failure, but comparable long-term outcomes to a non-fenestrated approach. Fenestration should be used in high-risk patients or based on the haemodynamic parameters measured before weaning from cardiopulmonary bypass. Routine use may potentially lead to additional late fenestration closure procedures in some patients, without improving long-term outcomes.

Outcomes following aortic valve procedures in 201 complex congenital heart disease cases-results from the UK National Audit.

OBJECTIVES: Some patients with complex congenital heart disease (cCHD) also require aortic valve (AoV) procedures. These cases are considered high risk but their outcome has not been well characterized. We aim to describe these scenarios in the current practice, and provide outcome data for counselling and decision-making. METHODS: This was a retrospective study using the UK National Congenital Heart Disease Audit data on cCHD patients undergoing aortic valve replacement, balloon dilation (balloon aortic valvuloplasty) or surgical repair (surgical aortic valve repair) between 2000 and 2012. Coarsened exact matching was used to pair cCHD with patients undergoing AoV procedures for isolated valve disease. RESULTS: A total of 201 patients with a varied spectrum of cCHD undergoing 242 procedures were included, median age 9.4 years (1 day-65 years). Procedure types were: balloon aortic valvuloplasty (n = 31, 13%), surgical aortic valve repair (n = 57, 24%) and aortic valve replacement (n = 154, 63%). Mortality at 30 days was higher in neonates (21.8% vs 5.3%, P = 0.02). Survival at 10 years was 83.1%, freedom from aortic valve replacement 83.8% and freedom from balloon aortic valvuloplasty/surgical aortic valve repair 86.3%. Neonatal age (P < 0.001), single ventricle (P = 0.08), concomitant Fontan/Glenn (P = 0.002) or aortic arch procedures (0.02) were associated with higher mortality. cCHD patients had lower survival at 30 days (93% vs 100%, P = 0.003) and at 10 years (86.4% vs 96.1%, P = 0.005) compared to matched isolated AoV disease patients. CONCLUSIONS: AoV procedures in cCHD can be performed with good results outside infancy, but with higher mortality than in isolated AoV disease. Neonates and patients with single ventricle defects, especially those undergoing concomitant Fontan/Glenn, have worse outcomes.