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.

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.

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.

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.

Age over 35 years is associated with increased mortality after pulmonary valve replacement in repaired tetralogy of Fallot: results from the UK National Congenital Heart Disease Audit database.

OBJECTIVES: Many adults with repaired tetralogy of Fallot will require a pulmonary valve replacement (PVR), but there is no consensus on the best timing. In this study, we aim to evaluate the impact of age at PVR on outcomes. METHODS: This is a national multicentre retrospective study including all patients >15 years of age with repaired tetralogy of Fallot who underwent their first PVR between 2000 and 2013. The optimal age cut-off was identified using Cox regression and classification and regression tree analysis. RESULTS: A total of 707 patients were included, median age 26 (15-72) years. The mortality rate at 10 years after PVR was 4.2%, and the second PVR rate of 6.8%. Age at PVR of 35 years was identified as the optimal cut-off in relation to late mortality. Patients above 35 years of age had a 5.6 fold risk of death at 10 years compared with those with PVR under 35 years (10.4% vs 1.3%, P < 0.001), more concomitant tricuspid valve repair/replacement (15.1% vs 5.7%, P < 0.001) and surgical arrhythmia treatment (18.4% vs 5.9%, P < 0.001). In those under 50 years, there was an 8.7 fold risk of late death compared with the general population, higher for those with PVR after 35 than those with PVR below 35 years (hazard ratio 9.9 vs 7.4). CONCLUSIONS: Patients above 35 years of age with repaired tetralogy of Fallot have significantly worse mortality after PVR, compared with younger patients and a higher burden of mortality relative to the general population. This suggests that there are still cases where the timing of initial PVR is not optimal, warranting a re-evaluation of criteria for intervention.

MicroRNAs as potential biomarkers in congenital heart surgery.

OBJECTIVE: Pediatric congenital heart surgery (CHS) involves intracardiac, valvular, and vascular repairs. Accurate tools to aid short-term outcome prediction in pediatric CHS are lacking. Clinical scores, such as the vasoactive-inotrope score and ventilation index, are used to define outcome in clinical studies. MicroRNA-1-3p (miR-1) is expressed by both cardiomyocytes and vascular cells and is regulated by hypoxia. In adult patients, miR-1 increases in the circulation after open-heart cardiac surgery, suggesting its potential as a clinical biomarker. Thus, we investigated whether perioperative circulating miR-1 measurements can help predict post-CHS short-term outcomes in pediatric patients. METHODS: Plasma miR-1 was retrospectively measured in a cohort of 199 consecutive pediatric CHS patients (median age 1.2 years). Samples were taken before surgery and at the end of the operation. Plasma miR-1 concentration was measured by reverse transcription-quantitative polymerase chain reaction and expressed as miR-1 copies/µL and as relative expression to spiked-in exogenous cel-miR-39. RESULTS: Baseline plasma miR-1 did not vary across different diagnoses, increased during surgery (204-fold median relative increase, P < .001), and was associated with aortic crossclamp duration postoperatively (P < .001). Importantly, miR-1 levels at the end of the operation positively correlated with intensive care stay (P < .001), early severe cardiovascular events (P = .01), and with high vasoactive-inotrope score (P = .001) and ventilation index (P < .001), suggesting that miR-1 could accelerate the identification of patients with cardiopulmonary bypass-related ischemic complications, requiring more intensive support. CONCLUSIONS: Our study suggests miR-1 as a novel potential circulating biomarker to predict early postoperative outcome and inform clinical management in pediatric heart surgery.

Surgical Repair of Tetralogy of Fallot With Absent Pulmonary Valve: Favorable Long-Term Results.

Tetralogy of Fallot with absent pulmonary valve syndrome (ToF-APVS) is a rare variant of tetralogy, associated with severe pulmonary valve regurgitation and aneurysmal dilation of the pulmonary arteries (PAs). Reported outcomes after surgical corrections are limited to single center, older series and might not reflect the current outcome. We aim to use data from a national registry to evaluate short- and long-term outcomes after surgical repair of ToF-APVS, to serve for counselling and planning. All children undergoing ToF-APVS repair in the UK between 2002 and 2013 were included. Survival and freedom from reintervention were estimated using the Kaplan-Meier method, and univariable analysis was done using the Weibull regression model. A total of 98 children, 45% male, 10% with DiGeorge syndrome, median age of 213 days (1 day to 13 years) were included. Mortality at 30 days was 3.3%, higher for neonates (6.7% vs 2.7%, P = 0.4) and those on preoperative mechanical ventilatory support (16.7% vs 1.3%, P = 0.04). Survival was 92.1% and freedom from pulmonary valve or conduit replacement (PVR) 73.2% at 10 years. Neonates had worse survival (hazard ratio [HR] 6.2, P = 0.02), freedom from PVR (HR 4.5, P = 0.01), freedom from PAs arterioplasty (HR 6.6, P = 0.001), and overall freedom from any reintervention (HR 5.3, P < 0.001). Low weight at repair was associated with worse freedom from PVR (P = 0.02) and from PAs arterioplasty (P = 0.009), preoperative ventilatory support with increased mortality (P = 0.009), the presence of DiGeorge syndrome was associated with worse freedom from PVR (HR 4, P = 0.02). Surgical repair of ToF-APVS can be performed with low early and late mortality, with improving results even in those with preoperative mechanical ventilatory support. The need for right ventricular outflow tract reintervention, including on the PAs, is an expected issue in the long term for the majority of patients.

Primary repair versus surgical and transcatheter palliation in infants with tetralogy of Fallot.

OBJECTIVES: Treatment of infants with tetralogy of Fallot (ToF) has evolved in the last two decades with increasing use of primary surgical repair (PrR) and transcatheter right ventricular outflow tract palliation (RVOTd), and fewer systemic-to-pulmonary shunts (SPS). We aim to report contemporary results using these treatment options in a comparative study. METHODS: This a retrospective study using data from the UK National Congenital Heart Disease Audit. All infants (n=1662, median age 181 days) with ToF and no other complex defects undergoing repair or palliation between 2000 and 2013 were considered. Matching algorithms were used to minimise confounding due to lower age and weight in those palliated. RESULTS: Patients underwent PrR (n=1244), SPS (n=311) or RVOTd (n=107). Mortality at 12 years was higher when repair or palliation was performed before the age of 60 days rather than after, most significantly for primary repair (18.7% vs 2.2%, P<0.001), less so for RVOTd (10.8% vs 0%, P=0.06) or SPS (12.4% vs 8.3%, P=0.2). In the matched groups of patients, RVOTd was associated with more right ventricular outflow tract (RVOT) reinterventions (HR=2.3, P=0.05 vs PrR, HR=7.2, P=0.001 vs SPS) and fewer pulmonary valve replacements (PVR) (HR=0.3 vs PrR, P=0.05) at 12 years, with lower mortality after complete repair (HR=0.2 versus PrR, P=0.09). CONCLUSIONS: We found that RVOTd was associated with more RVOT reinterventions, fewer PVR and fewer deaths when compared with PrR in comparable, young infants, especially so in those under 60 days at the time of the first procedure.

Aortic Valve Replacement and the Ross Operation in Children and Young Adults.

BACKGROUND: There are several options available for aortic valve replacement (AVR), with few comparative reports in the literature. The optimal choice for AVR in each age group is not clear. OBJECTIVES: The study sought to report and compare outcomes after AVR in the young using data from a national database. METHODS: AVR procedures were compared after advanced matching, both in pairs and in a 3-way manner, using a Bayesian dynamic survival model. RESULTS: A total of 1,501 patients who underwent AVR in the United Kingdom between 2000 and 2012 were included. Of these, 47.8% had a Ross procedure, 37.8% a mechanical AVR, 10.9% a bioprosthesis AVR, and 3.5% a homograft AVR, with Ross patients being significantly younger when compared to the other groups. Overall survival at 12 years was 94.6%. In children, the Ross procedure had a 12.7% higher event-free probability (death or any reintervention) at 10 years when compared to mechanical AVR (p = 0.05). We also compared all procedures except the homograft in a matched population of young adults, where the bioprosthesis had the lowest event-free probability of 78.8%, followed by comparable results in mechanical AVR and Ross, with 86.3% and 89.6%, respectively. Younger age was associated with mortality and pulmonary reintervention in the Ross group and with aortic reintervention in the mechanical AVR. Of all 3 options, only the patients undergoing the Ross procedure approached the survival of the general population. CONCLUSIONS: AVR in the young achieves good results, with the Ross being overall better suited for this age group, especially in children. Although freedom from aortic valve reintervention is superior after the Ross procedure, the need for homograft reinterventions is an issue to take into account. All methods have advantages and limitations, with reinterventions being an issue in the long term for all, more crucially in smaller children.

Surgery for simple and complex subaortic stenosis in children and young adults: results from a prospective, procedure-based national database.

OBJECTIVE: To identify the outcomes of surgically treated subaortic stenosis in a national population. METHODS: From 2000 to 2013, 1047 patients aged < 40 years underwent 1142 subaortic stenosis procedures. Of the 1047 patients, 484 (46.2%) were considered to have complex stenosis (CS) because at or before the first operation they had mitral valve (MV) disease, aortic valve disease, aortic coarctation or an interrupted aortic arch. RESULTS: The 30-day mortality was 0.7% for simple stenosis (SS), 2.3% for CS (P = .06), and 1.6% overall. Age < 1 year (P < .01), MV procedure (P = .02) and an interrupted aortic arch at the index procedure (P < .01) were risk factors for early death. Konno-type procedure early mortality was 2.4%. The 12-year survival was 97.1%, with a significant difference between SS and CS (hazard ratio [HR], 4.53; P = .02). Having MV disease alone (HR, 4.11; P = .02), MV disease plus aortic coarctation (HR, 6.73; P = .008), and age < 1 year (HR, 6.72; P < .001) were risk factors for late mortality. Freedom from subaortic reintervention overall was 92.3% and 88.5% at 5 and 12 years, respectively, much greater with CS than with SS (HR, 4.91; P < .0001). The independent risk factors for reintervention were younger age at the index procedure (HR, 0.1/y; P = .002), concomitant MV procedure (HR, 2.68; P = .019), ventricular septal defect plus interrupted aortic arch (HR, 3.19; P = .014), and ventricular septal defect plus aortic coarctation (HR, 2.41; P = .023). Undergoing a concomitant aortic valve procedure at the index procedure was protective (HR, 0.29; P = .025). CONCLUSIONS: Patients with SS had excellent outcomes. However, those with CS had worse long-term survival and freedom from reintervention, with morbidity and mortality greatest in young patients with multiple lesions. Additional evaluation in large-scale prospective studies is warranted.