Guido Pieles

University of Bristol | UB · Bristol Heart Institute

Background Barth syndrome is a rare, life-threatening X-linked recessive mitochondrial disorder of lipid metabolism primarily affecting males. Previous research suggests that bezafibrate may ameliorate cellular lipid abnormalities and reduce cardiac dysfunction in an animal model. Objectives Estimate the effect of bezafibrate on clinical, biochemi...

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-p...

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) a...

INTRODUCTION: Patients with congenital heart disease (CHD) typically exhibit reduced exercise capacity and life quality, even after their structural heart defects have been clinically corrected. However, little is known about the interactions between physical activity (PA), sedentary time (ST), exercise capacity (peak VO2) and health-related qualit...

INTRODUCTION: Young-people with congenital heart disease (ConHD) have reduced cardiorespiratory fitness (CRF), which has been associated with mortality and morbidity. Previous studies have explored the determinants of CRF using inappropriate ratio-scaling techniques and have not accounted for physical activity (PA). The aims of this study were to i...

Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): UK Research and Innovation Medical Research Council Doctoral Training Programme Grant Work supported as part of a research partnership between the University of Bristol and Canon Medical Systems UK that determines the independence of th...

Background and Aim: Young people with congenital heart disease (CHD) are less active than their healthy peers negatively affecting quality of life and long-term outcomes. Despite no specific guidelines, a commonly used measure of physical activity (PA) is moderate- to-vigorous PA (MVPA), which has the disadvantage of neglecting light-intensity acti...

The number of adult patients with congenital heart disease (CHD) is steadily increasing and exceeds nowadays the number of children with CHD. This is due to significant advances in therapeutic possibilities that became available over the last four decades. As such, this aging population survives the CHD complications and is exposed to the tradition...

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 adoles...

The project is related to the use of exercise echo cardiography and young athletes, as part fo an on-going heart health of yougn athletes.

Aim: To determine if published Z-scores for left ventricular (LV), left atrial (LA) and aortic structure as well as indices of LV function (Doppler and TDI) in paediatric athletes and non-athletes are appropriate for application in male Arab and black paediatric athletes. If inappropriate, we aim to provide new nomograms and Z-scores for clinical a...

Background Increased physical activity has been shown to result in improved physical fitness, quality of life, self-confidence, and reduced anxiety in adults with congenital heart disease (CHD). In comparison to adults there are relatively few data on the benefit of physical activity for children and adolescents with CHD. It is clear that increasin...

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 do...

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 wit...

The athlete's heart is a well-known phenomenon in adults practicing competitive sports. Unfortunately, to date, most of the studies on training-induced cardiac remodelling have been conducted in adults and the current recommendations refer mainly to adult individuals. However, an appropriate interpretation of resting ECG and imaging in children pra...

Sports Cardiology practice commonly involves the evaluation of athletes for genetically determined cardiac conditions that may predispose to malignant arrhythmias, heart failure, and sudden cardiac death. High-level exercise can lead to electrical and structural cardiac remodelling which mimics inherited cardiac conditions (ICCs). Differentiation b...

Cardiac sequelae after COVID-19 have been described in athletes, prompting the need to establish a return-to-play (RTP) protocol to guarantee a safe return to sports practice. Sports participation is strongly associated with multiple short- and long-term health benefits in children and adolescents and plays a crucial role in counteracting the psych...

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...

Funding Acknowledgements Type of funding sources: Public Institution(s). Main funding source(s): UK Research and Innovation - Medical Research Council GW4 Studentship Background Right ventricle inflow (RV) dilation is a common adaptation to training in professional athletes, but how this impacts myocardial mechanics is yet unclear. Previous studie...

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 Criteri...

Background Barth syndrome (BS) is a life-threatening genetic disease caused by abnormal lipids in the mitochondria of cells and mostly affects young males. Those living with BS have severe exercise intolerance, lethargy and fatigue due to muscle disease which affect their daily life. Previous research suggests a need for qualitative exploration of...

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 chil...

The 12-lead electrocardiogram is a key component of cardiac screening in elite adolescent footballers. Current technology hampers mobile electrocardiogram monitoring that could reduce the time-to-diagnosis in symptomatic athletes. Recently, a 22-lead mobile electrocardiogram monitor, CardioSecur (Personal MedSystems GmbH), has been approved for use...

Background Barth syndrome is a rare, life-threatening, X-linked recessive genetic disease that predominantly affects young males and is caused by abnormal mitochondrial lipid metabolism. Currently, there is no definitive treatment for Barth syndrome other than interventions to ameliorate acute symptoms, such as heart failure, cardiac arrhythmias, n...

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 se...

Funding Acknowledgements Type of funding sources: Other. Main funding source(s): The study was support by a contractual research partnership between the University of Bristol and Canon Medical Systems UK. Background Arrhytmogenic cardiomyopathy (ACM) is a major cause of sudden cardiac death among young athletes. Screening these individuals can be...

Funding Acknowledgements Type of funding sources: Other. Main funding source(s): The study was support by a contractual research partnership between the University of Bristol and Canon Medical Systems UK Background Left ventricular non-compaction (LVNC) is a rare cardiomyopathy, with hypertrabeculation often observed in athletes. In confirmed LVNC...

Purpose Few data exist on the descriptions of LV myocardial mechanics and reserve during dynamic exercise of adolescent athletes. The aim of this study was to describe the LV myocardial and cardiopulmonary changes during exercise using 2-D strain deformation imaging. Methods Elite adolescent male football players (n = 42) completed simultaneous ca...

While invasive assessment of hemodynamics and testing of acute vasoreactivity in the catheterization laboratory is the gold standard for diagnosing pulmonary hypertension (PH) and pulmonary vascular disease (PVD) in children, transthoracic echocardiography (TTE) serves as the initial diagnostic tool. International guidelines suggest several key ech...

Background Right ventricular (RV) pressure loading from pulmonary hypertension (PH) and volume loading from pulmonary regurgitation (PR) lead to RV dysfunction, a critical determinant of clinical outcomes, but their impact on regional RV mechanics and fibrosis are poorly characterized. Here, we hypothesized that regional myocardial mechanics and ef...

Background Congenital heart disease (ConHD) affects approximately 1% of all live births. People with ConHD are living longer due to improved medical intervention and are at risk of developing non‐communicable diseases. Cardiorespiratory fitness (CRF) is reduced in people with ConHD, who deteriorate faster compared to healthy people. CRF is known to...

Background Numerous studies have measured the prognostic associations between cardiorespiratory fitness and patient outcomes in congenital heart disease, but no systematic review has assessed these associations for all types of congenital heart disease. It is therefore a timely opportunity to syntheses all available data using a systematic review m...

SARS-CoV-2 is the causative virus responsible for the COVID-19 pandemic. This pandemic has necessitated that all professional and elite sport is either suspended, postponed or cancelled altogether to minimise the risk of viral spread. As infection rates drop and quarantine restrictions are lifted, the question how athletes can safely resume competi...

Improved clinical care has led to an increase in the number of adults with congenital heart disease (CHD) engaging in leisure time and competitive sports activities. Although the benefits of exercise in patients with CHD are well established, there is a low but appreciable risk of exercise-related complications. Published exercise recommendations f...

Background: The 12-lead electrocardiogram (ECG) is the primary cardiac screening and diagnostic tool for athletes. However, limited portability hampers its use at the pitch-side where it would be invaluable in making fast and reliable cardiac diagnoses. Recently, smartphone apps have become available that can record 12-lead ECGs using only 4 electr...

BACKGROUND Barth syndrome is a rare, life-threatening, X-linked recessive genetic disease that predominantly affects young males and is caused by abnormal mitochondrial lipid metabolism. Currently, there is no definitive treatment for Barth syndrome other than interventions to ameliorate acute symptoms, such as heart failure, cardiac arrhythmias, n...

The systematic development of early age talent in sports academies has led to the professionalization of pediatric sport and the sports physician need to be aware of pediatric cardiological problems. Research into the medical cardiac care and assessment of the pediatric athlete are accumulating, but specific pediatric international guidelines are n...

The success of systematic early age talent development has led to the professionalisation of youth sports academies used by clubs and governing bodies alike, and sports physicians are nowadays commonly confronted with paediatric cardiological problems. Medical cardiac care of the paediatric athlete is however in its infancy, and the international g...

Information on genetic etiology of pediatric hypertrophic cardiomyopathy (HCM) rarely aids in risk stratification and prediction of disease onset. Little data exist on the association between genetic modifiers and phenotypic expression of myocardial performance, hampering an individual precision medicine approach. Single-nucleotide polymorphism gen...

Background Tetralogy of Fallot (TOF) repair results in long term chronic pulmonary regurgitation requiring pulmonary valve replacement (PVR). Homograft and stented bio-prosthesis are currently used for PVR but whether one should be considered superior to another remains unknown. Aim To compare echocardiographic and clinical outcomes after PVR with...

This is a protocol for a Cochrane Review (Intervention). The objectives are as follows: To assess the effectiveness and safety of physical activity promotion and exercise training interventions in individuals with congenital heart disease.

The European Pediatric Pulmonary Vascular Disease Network is a registered, non-profit organization that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of pediatric pulmonary hypertensive vascular disease, including pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, PH a...

Background: The usefulness of echocardiographic indices, including those already used by modified Task Force Criteria (mTFC), and others such as strain imaging, to identify arrhythmogenic right ventricular cardiomyopathy (ARVC) in adolescence is not well established. Methods: Echocardiograms from 120 adolescents investigated for ARVC (13±4 years...

An increasing number of patients with congenital heart disease (CHD) are participating in regular sport at levels ranging from gentle recreational activities to elite professional sport. In general, children and adults with CHD should be encouraged to engage in regular physical activity, but long-term complications, such as heart failure, arrhythmi...

Athlete’s heart occurs in childhood but is less well understood than in adults. In children, exercise-related cardiac remodelling occurs but with more heterogeneity than in adults. It can be difficult to distinguish age-related cardiac maturation, exercise-related adaptation, and the early manifestation of cardiac disease such as cardiomyopathy. Th...

Sport and exercise have been intensely advocated as protective lifestyle measures, preventing or reducing the risk of severe health issues including cardiovascular disease. More extreme forms of sport (for instance at high altitudes) have been identified as an important way of promoting cardiovascular adaptation, but have also been associated with...

Background: International electrocardiographic (ECG) recommendations regard anterior T-wave inversion (ATWI) in athletes under 16 years to be normal. Design: The aim of this study was to identify the prevalence, distribution and determinants of TWI by ethnicity, chronological and biological age within paediatric athletes. A second aim was to est...

Objective Historically, electrocardiographic (ECG) interpretation criteria for athletes were only applicable to adults. New international recommendations now account for athletes ≤16 years, but their clinical appropriateness is unknown. We sought to establish the diagnostic accuracy of new international ECG recommendations against the Seattle crite...

Background Reports on the incidence and causes of sudden cardiac death among young athletes have relied largely on estimated rates of participation and varied methods of reporting. We sought to investigate the incidence and causes of sudden cardiac death among adolescent soccer players in the United Kingdom. Methods From 1996 through 2016, we scre...

Aim: To determine the prevalence of paediatric athletes whom meet the structural and functional diagnostic Task Force Criteria of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC). Methods: 505 (Arab 361, Black 144) male paediatric athletes, aged 11-18 years, were evaluated by 12-lead ECG and 2D echocardiography. Structural variables were sca...

Further investigations were carried out in 42; 15 (2.1%) due to an ECG abnormality, 9 due to an abnormal examination and 18 due to history. Of these, 15 were confirmed to have a normal cardiovascular system. 1 player with AV node reentry underwent successful ablation. 1 of the 9 with an abnormal history was found to have a bicuspid aortic valve is...

Quantification of cardiac structure and function is central in cardiovascular research. Rabbits are valuable research models of cardiovascular human disease; however, there is little normal data available. The aim of this study was to investigate feasibility and provide normal values for comprehensive echocardiographic assessment of biventricular f...

Objectives: Right ventricular (RV) failure from increased pressure loading is a frequent consequence of acquired and congenital heart diseases. However, the mechanisms involved in their pathophysiology are still unclear, and few data exist on RV pressure-loading models and early versus late effects on RV and left ventricular responses. We characte...

Aim: To describe the electrocardiographic (ECG) and echocardiographic manifestations of the paediatric athlete's heart, and examine the impact of age, race and sex on cardiac remodelling responses to competitive sport. Design: Systematic review with meta-analysis. Data sources: Six electronic databases were searched to May 2016: MEDLINE, PubMe...

Theme park operators and medical professionals advise children with heart conditions against using rollercoaster rides, but these recommendations are not evidence-based. The underlying assumption is that the combination of adrenergic stimulation through stress and acceleration might trigger arrhythmias in susceptible individuals. We conducted a cro...

The sports cardiologist must be alert to the presence of underlying congenital heart disease (CHD), as intervention may be necessary before the onset of major symptoms. Cardiovascular mortality accounts for 75% of all deaths in CHD – predominantly due to sudden cardiac death (SCD) and heart failure. Important risk factors may include arrhythmias, h...

Background: The aim of this study was to quantify the degree of the effect of in-plane partial volume averaging on recorded peak velocity in phase contrast magnetic resonance angiography (PCMRA). Methods: Using cardiac optimized 1.5 Tesla MRI scanners (Siemens Symphony and Avanto), 145 flow measurements (14 anatomical locations; ventricular outlets...

Background: LV and RV myocardial reserve during exercise in adolescents has not been directly characterized. The aim of this study was to quantify myocardial performance response to exercise using two dimensional speckle tracking echocardiography and describe the relationship between myocardial reserve, respiratory and metabolic exercise parameter...

Tricuspid atresia and common truncus arteriosus are rare forms of congenital heart disease; the coexistence of both anomalies is therefore an extremely uncommon event. Without treatment, early mortality is the natural course so diagnostic and therapeutic management must be performed without delay. We report a case of a newborn with a postnatal diag...

Compared to one-dimensional M-mode technique for the assessment of ventricular myocardial function, two-dimensional speckle tracking echocardiographic (2DSTE) imaging is a novel and more sensitive technique, ideally suited for paediatric studies. PURPOSE: To measure left ventricular myocardial systolic function using 2DSTE at rest, exercise and in...

Assessment of LV function response to incremental dynamic exercise in healthy children and adolescents using 2-D speckle tracking echocardiography

Cardiovascular adaptations to aerobic exercise are mediated through interactions of cardiac, hormonal, metabolic, and muscular mechanisms with a dominant role for increase in cardiac output to enhance oxygen delivery. However, increased demands for oxygen uptake and use during exercise depend on adequate adaptations of systemic and pulmonary vascul...

Increased oxygen uptake and utilisation during exercise depend on adequate adaptations of systemic and pulmonary vasculature. Recent advances in magnetic resonance imaging techniques allow for direct quantification of aortic and pulmonary blood flow using phase-contrast magnetic resonance angiography (PCMRA). This pilot study tested quantification...

Exercise training is an underused intervention in paediatric healthcare. This is surprising, since initial evidence demonstrates its effectiveness and safety; furthermore it confers socioeconomic benefits for healthcare systems. Pilot studies have assessed and confirmed the feasibility of exercise training in many paediatric disease settings. Howev...

Williams-Beuren syndrome (WBS) is a multisystem genetic disorder comprising of craniofacial, developmental, and cardiac malformations. The most common cardiac defects found are supravalvar aortic stenosis and peripheral pulmonary stenosis. However, WBS should be regarded as a general arteriopathy consisting of stenoses of medium- and large-sized ar...

Vertebrate organs show consistent left-right (L-R) asymmetry in placement and patterning. To identify genes involved in this process we performed an ENU-based genetic screen. Of 135 lines analyzed 11 showed clear single gene defects affecting L-R patterning, including 3 new alleles of known L-R genes and mutants in novel L-R loci. We identified six...

We have identified an ethylnitrosourea (ENU)-induced recessive mouse mutation (Vcc) with a pleiotropic phenotype that includes cardiac, tracheoesophageal, anorectal, anteroposterior patterning defects, exomphalos, hindlimb hypoplasia, a presacral mass, renal and palatal agenesis, and pulmonary hypoplasia. It results from a C470R mutation in the pro...

To identify, map, clone, and functionally validate a novel mouse model for impaired glucose tolerance and insulin secretion. Haploinsufficiency of the insulin receptor and associated mild insulin resistance has been used to sensitize an N-ethyl-N-nitrosourea (ENU) screen to identify novel mutations resulting in impaired glucose tolerance and diabet...