The tricuspid valve in hypoplastic left heart syndrome: Echocardiography provides insight into anatomy and function

Tricuspid regurgitation (TR) is commonly seen in surgically palliated patients with hypoplastic left heart syndrome, and when significant, is associated with an increase in both morbidity and mortality. Tricuspid valve dysfunction appears to be the result of a combination of inherent structural malformations and the unique physiological circumstances resulting from right ventricular pressure and volume overload. Valve dysfunction evolves rapidly, and manifests early on in the surgical pathway. Whilst traditional echocardiographic imaging can identify anatomical defects and dysfunction resulting in varying degrees of regurgitation even at early stages, more sophisticated investigations such as 3D echocardiography, strain imaging and transesophageal 3DE might prove useful to better demonstrate the complex interactions between abnormal anatomy of the valve complex, ventricular function, mechanical synchrony, and TR. Recognition of specific mechanisms of TR can enhance patient-specific care by directing precise surgical interventions and by informing the best timing for intervention on the valve

“No official help is available” - experience of parents and children with congenital heart disease during COVID-19

Introduction: the purpose was to explore the experience, information, support needs and decision-making of parents with congenital heart disease (CHD) during the COVID-19 crisis. Materials and methods: a survey study of parents of children with CHD, children and young people, capturing experiences, decision-making, information and support needs during the COVID-19 crisis. The survey launched for 1 month (9thApril 2020) during the first infection wave in the UK and subsequent restriction of free movement under lockdown rules from March 23rd2020 until May 31st2020. Results: 184 parents and 36 children/young people completed the survey. Parents were more likely to worry about the virus (86.4%) than children/young people (69.4%), whilst (89%) parents were more vigilant for symptoms of the virus vs. children/young people (69.4%). A thematic analysis of the qualitative comments covered 34 subthemes, forming eight-overarching themes: Virus 1)risk of infection, 2)information, guidance and advice, 3)change in health care provision, and 4)fears and anxieties; Lockdown and isolation 5)psychological and social impact, 6)keeping safe under lockdown, 7)provisions and dependence on others, 8)employment and income.Conclusions: there was widespread concern over the virus especially amongst parents. Parents and children/young people however, were frustrated with the lack of specific and paediatric focused information and guidance, expressing disappointment with the adult centric information available. Parents also felt alone, especially with their concerns around the implications of cardiac service suspension and the implication for their child’s health. In order to better support children and their families, resources need to be developed to address families’ and children/young people’s concerns for their health during this pandemic. <br/

COVID-19 in congenital heart disease (COaCHeD) study

Background: COVID-19 has caused significant worldwide morbidity and mortality. Congenital heart disease (CHD) is likely to increase vulnerability and understanding the predictors of adverse outcomes is key to optimising care.// Objective: Ascertain the impact of COVID-19 on people with CHD and define risk factors for adverse outcomes.// Methods: Multicentre UK study undertaken 1 March 2020–30 June 2021 during the COVID-19 pandemic. Data were collected on CHD diagnoses, clinical presentation and outcomes. Multivariable logistic regression with multiple imputation was performed to explore predictors of death and hospitalisation.// Results: There were 405 reported cases (127 paediatric/278 adult). In children (age <16 years), there were 5 (3.9%) deaths. Adjusted ORs (AORs) for hospitalisation in children were significantly lower with each ascending year of age (OR 0.85, 95% CI 0.75 to 0.96 (p<0.01)). In adults, there were 24 (8.6%) deaths (19 with comorbidities) and 74 (26.6%) hospital admissions. AORs for death in adults were significantly increased with each year of age (OR 1.05, 95% CI 1.01 to 1.10 (p<0.01)) and with pulmonary arterial hypertension (PAH; OR 5.99, 95% CI 1.34 to 26.91 (p=0.02)). AORs for hospitalisation in adults were significantly higher with each additional year of age (OR 1.03, 95% CI 1.00 to 1.05 (p=0.04)), additional comorbidities (OR 3.23, 95% CI 1.31 to 7.97 (p=0.01)) and genetic disease (OR 2.87, 95% CI 1.04 to 7.94 (p=0.04)).// Conclusions: Children were at low risk of death and hospitalisation secondary to COVID-19 even with severe CHD, but hospital admission rates were higher in younger children, independent of comorbidity. In adults, higher likelihood of death was associated with increasing age and PAH, and of hospitalisation with age, comorbidities and genetic disease. An individualised approach, based on age and comorbidities, should be taken to COVID-19 management in patients with CHD

COVID-19 in congenital heart disease (COaCHeD) study

BACKGROUND: COVID-19 has caused significant worldwide morbidity and mortality. Congenital heart disease (CHD) is likely to increase vulnerability and understanding the predictors of adverse outcomes is key to optimising care.OBJECTIVE: Ascertain the impact of COVID-19 on people with CHD and define risk factors for adverse outcomes.METHODS: Multicentre UK study undertaken 1 March 2020-30 June 2021 during the COVID-19 pandemic. Data were collected on CHD diagnoses, clinical presentation and outcomes. Multivariable logistic regression with multiple imputation was performed to explore predictors of death and hospitalisation.RESULTS: There were 405 reported cases (127 paediatric/278 adult). In children (age &lt;16 years), there were 5 (3.9%) deaths. Adjusted ORs (AORs) for hospitalisation in children were significantly lower with each ascending year of age (OR 0.85, 95% CI 0.75 to 0.96 (p&lt;0.01)). In adults, there were 24 (8.6%) deaths (19 with comorbidities) and 74 (26.6%) hospital admissions. AORs for death in adults were significantly increased with each year of age (OR 1.05, 95% CI 1.01 to 1.10 (p&lt;0.01)) and with pulmonary arterial hypertension (PAH; OR 5.99, 95% CI 1.34 to 26.91 (p=0.02)). AORs for hospitalisation in adults were significantly higher with each additional year of age (OR 1.03, 95% CI 1.00 to 1.05 (p=0.04)), additional comorbidities (OR 3.23, 95% CI 1.31 to 7.97 (p=0.01)) and genetic disease (OR 2.87, 95% CI 1.04 to 7.94 (p=0.04)).CONCLUSIONS: Children were at low risk of death and hospitalisation secondary to COVID-19 even with severe CHD, but hospital admission rates were higher in younger children, independent of comorbidity. In adults, higher likelihood of death was associated with increasing age and PAH, and of hospitalisation with age, comorbidities and genetic disease. An individualised approach, based on age and comorbidities, should be taken to COVID-19 management in patients with CHD.</p

The relationship between maximal left ventricular wall thickness and sudden cardiac death in childhood onset hypertrophic cardiomyopathy

Background: Maximal left ventricular wall thickness (MLVWT) is a risk factor for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). In adults, the severity of left ventricular hypertrophy has a nonlinear relationship with SCD, but it is not known whether the same complex relationship is seen in childhood. The aim of this study was to describe the relationship between left ventricular hypertrophy and SCD risk in a large international pediatric HCM cohort. Methods: The study cohort comprised 1075 children (mean age, 10.2 years [±4.4]) diagnosed with HCM (1–16 years) from the International Paediatric Hypertrophic Cardiomyopathy Consortium. Anonymized, noninvasive clinical data were collected from baseline evaluation and follow-up, and 5-year estimated SCD risk was calculated (HCM Risk-Kids). Results: MLVWT Z score was <10 in 598 (58.1%), ≥10 to <20 in 334 (31.1%), and ≥20 in 143 (13.3%). Higher MLVWT Z scores were associated with heart failure symptoms, unexplained syncope, left ventricular outflow tract obstruction, left atrial dilatation, and nonsustained ventricular tachycardia. One hundred twenty-two patients (71.3%) with MLVWT Z score ≥20 had coexisting risk factors for SCD. Over a median follow-up of 4.9 years (interquartile range, 2.3–9.3), 115 (10.7%) had an SCD event. Freedom from SCD event at 5 years for those with MLVWT Z scores <10, ≥10 to <20, and ≥20 was 95.6%, 87.4%, and 86.0, respectively. The estimated SCD risk at 5 years had a nonlinear, inverted U-shaped relationship with MLVWT Z score, peaking at Z score +23. The presence of coexisting risk factors had a summative effect on risk. Conclusions: In children with HCM, an inverted U-shaped relationship exists between left ventricular hypertrophy and estimated SCD risk. The presence of additional risk factors has a summative effect on risk. While MLVWT is important for risk stratification, it should not be used either as a binary variable or in isolation to guide implantable cardioverter defibrillator implantation decisions in children with HCM

Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy

BACKGROUND Up to one-half of childhood sarcomeric hypertrophic cardiomyopathy (HCM) presents before the age of 12 years, but this patient group has not been systematically characterized. OBJECTIVES The aim of this study was to describe the clinical presentation and natural history of patients presenting with nonsyndromic HCM before the age of 12 years. METHODS Data from the International Paediatric Hypertrophic Cardiomyopathy Consortium on 639 children diagnosed with HCM younger than 12 years were collected and compared with those from 568 children diagnosed between 12 and 16 years. RESULTS At baseline, 339 patients (53.6%) had family histories of HCM, 132 (20.9%) had heart failure symptoms, and 250 (39.2%) were prescribed cardiac medications. The median maximal left ventricular wall thickness z-score was 8.7 (IQR: 5.3-14.4), and 145 patients (27.2%) had left ventricular outflow tract obstruction. Over a median follow-up period of 5.6 years (IQR: 2.3-10.0 years), 42 patients (6.6%) died, 21 (3.3%) underwent cardiac transplantation, and 69 (10.8%) had life-threatening arrhythmic events. Compared with those presenting after 12 years, a higher proportion of younger patients underwent myectomy (10.5% vs 7.2%; P = 0.045), but fewer received primary prevention implantable cardioverter-defibrillators (18.9% vs 30.1%; P = 0.041). The incidence of mortality or life-threatening arrhythmic events did not differ, but events occurred at a younger age. CONCLUSIONS Early-onset childhood HCM is associated with a comparable symptom burden and cardiac phenotype as in patients presenting later in childhood. Long-term outcomes including mortality did not differ by age of presentation, but patients presenting at younger than 12 years experienced adverse events at younger ages. (C) 2022 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation.Peer reviewe

Relationship Between Maximal Left Ventricular Wall Thickness and Sudden Cardiac Death in Childhood Onset Hypertrophic Cardiomyopathy

Background: Maximal left ventricular wall thickness (MLVWT) is a risk factor for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). In adults, the severity of left ventricular hypertrophy has a nonlinear relationship with SCD, but it is not known whether the same complex relationship is seen in childhood. The aim of this study was to describe the relationship between left ventricular hypertrophy and SCD risk in a large international pediatric HCM cohort. Methods: The study cohort comprised 1075 children (mean age, 10.2 years [+/- 4.4]) diagnosed with HCM (1-16 years) from the International Paediatric Hypertrophic Cardiomyopathy Consortium. Anonymized, noninvasive clinical data were collected from baseline evaluation and follow-up, and 5-year estimated SCD risk was calculated (HCM Risk-Kids). Results: MLVWT Z score was = 10 to = 20 in 143 (13.3%). Higher MLVWT Z scores were associated with heart failure symptoms, unexplained syncope, left ventricular outflow tract obstruction, left atrial dilatation, and nonsustained ventricular tachycardia. One hundred twenty-two patients (71.3%) with MLVWT Z score >= 20 had coexisting risk factors for SCD. Over a median follow-up of 4.9 years (interquartile range, 2.3-9.3), 115 (10.7%) had an SCD event. Freedom from SCD event at 5 years for those with MLVWT Z scores = 10 to = 20 was 95.6%, 87.4%, and 86.0, respectively. The estimated SCD risk at 5 years had a nonlinear, inverted U-shaped relationship with MLVWT Z score, peaking at Z score +23. The presence of coexisting risk factors had a summative effect on risk. Conclusions: In children with HCM, an inverted U-shaped relationship exists between left ventricular hypertrophy and estimated SCD risk. The presence of additional risk factors has a summative effect on risk. While MLVWT is important for risk stratification, it should not be used either as a binary variable or in isolation to guide implantable cardioverter defibrillator implantation decisions in children with HCM.Peer reviewe

Paediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2 (PIMS-TS): Prospective, national surveillance, United Kingdom and Ireland, 2020

Background: Paediatric Multisystem Inflammatory Syndrome temporally associated with SARS-CoV-2 (PIMS-TS), first identified in April 2020, shares features of both Kawasaki disease (KD) and toxic shock syndrome (TSS). The surveillance describes the epidemiology and clinical characteristics of PIMS-TS in the United Kingdom and Ireland. Methods: Public Health England initiated prospective national surveillance of PIMS-TS through the British Paediatric Surveillance Unit. Paediatricians were contacted monthly to report PIMS-TS, KD and TSS cases electronically and complete a detailed clinical questionnaire. Cases with symptom onset between 01 March and 15 June 2020 were included. Findings: there were 216 cases with features of PIMS-TS alone, 13 with features of both PIMS-TS and KD, 28 with features of PIMS-TS and TSS and 11 with features of PIMS-TS, KD and TSS, with differences in age, ethnicity, clinical presentation and disease severity between the phenotypic groups. There was a strong geographical and temporal association between SARS-CoV-2 infection rates and PIMS-TS cases. Of those tested, 14.8% (39/264) children had a positive SARS-CoV-2 RT-PCR, and 63.6% (75/118) were positive for SARS-CoV-2 serology. In total 44·0% (118/268) required intensive care, which was more common in cases with a TSS phenotype. Three of five children with cardiac arrest had TSS phenotype. Three children (1·1%) died. Interpretation: the strong association between SARS-CoV-2 infection and PIMS-TS emphasises the importance of maintaining low community infection rates to reduce the risk of this rare but severe complication in children and adolescents. Close follow-up will be important to monitor long-term complications in children with PIMS-T

Making every contact count: recognising obesity in paediatric and young adult cardiology.

Introduction: with increased survival, children with CHD are reaching adulthood, however, obesity amongst this cohort is an emerging problem. Making every contact count encourages clinicians to utilise contact to elicit behaviour change. The aim of this work was to identify whether the body habitus of children classified as obese was addressed during a clinical review. Methods: a retrospective observational cohort study was completed using a cardiology outpatient dataset from 2010 to 2019. Inclusion criteria are all children with a body mass index z score classified as obese (≥ 2 z scores). Individual electronic patient records were reviewed to identify long-term anthropometric measures including (i) recognition of body habitus, (ii) prescription of physical activity or dietary intervention, and (iii) referral to a weight management programme or dietitian. Results: from the cohort of 95 patients, 285 "obese clinical encounters"were identified, at the time of a cardiology clinic attendance. Of those, obesity was acknowledged in 25 clinic letters (8.65%), but only 8 used the correct terms "obese"or "obesity"(2.77%). Action to tackle obesity was recorded in 9.3% of cases with a direct referral to a dietitian being made on 3 occasions (1.04%). Conclusions: body habitus is not being routinely addressed by cardiologists caring for paediatric and young adult cardiac patients. This study has recognised an alarmingly high incidence of missed opportunities to make every contact count, to manage those with obesity and associated risk factors. </p

A multiplanar three dimensional echocardiographic study of mitral valvar annular function in children with normal and regurgitant valves

Introduction - The mitral valvar complex is difficult to visualise accurately in only two dimensions. Three-dimensional echocardiography gives new insight into the dynamic changes of intra-cardiac structures during the cardiac cycle. The aim of this study was to study the mitral annulus in systole and diastole in normal children using three-dimensional echocardiography, and to analyse the effect of regurgitation on annular function.Materials and methods - Three-dimensional echocardiographic datasets, acquired in 11 consecutive subjects with mitral regurgitation, and 20 normal subjects, were analysed offline using simultaneous multiplanar review.Results - The mitral valvar annular area decreased in diastole, and increased in systole, in both groups. The annulus in patients with mitral regurgitation is dilated compared to normal subjects, the systolic value for those with regurgitation having a mean of 6.79 plus or minus 2.55 centimetres2/metres2, and the diastolic value a mean of 5.01 plus or minus 1.78 centimetres2/metres2, as opposed to a systolic mean value of 5.28 centimetres2/metres2 plus or minus 1.68, p = 0.091, and diastolic mean value of 3.05 centimetres2/metres2 plus or minus 0.90, in normal subjects (p less than 0.0001). The proportional change in mitral valvar annular area from systole to diastole showed a trend towards being smaller in those with mitral regurgitation, although this did not reach significance (24.8% versus 41.13%, p equal to 0.249). Analysis of subgroups of patients with moderate or severe mitral regurgitation showed mitral excursion, expressed as percentage of left ventricular length, to be significantly less than in normal subjects, at 12.78 plus or minus 5.10% versus 15.84 plus or minus 4.23% (p equal to 0.012).Conclusions - Mitral valvar annular area in children decreases in diastole, and increases in systole. In those with mitral regurgitation, the annulus is dilated and the dynamic annular function is depressed.<br/