The aim of this study is to investigate the frequency of fragmented QRS and its associations with clinical findings and prognosis in children diagnosed with dilated cardiomyopathy with or without left ventricular non-compaction.

This retrospective study was conducted between 2010 and 2020. Patients with dilated cardiomyopathy were classified into two groups according to the presence of left ventricular non-compaction: Dilated cardiomyopathy with left ventricular non-compaction and dilated cardiomyopathy without left ventricular non-compaction. Patients were also divided into two groups according to the presence of fragmented QRS (fragmented QRS group and non-fragmented QRS group).

Twenty-three of 44 patients (52.3%) were male. Among left ventricular non-compaction patients, the fragmented QRS group had more complex ventricular arrhythmias (p = 0.003). Patients with fragmented QRS had a significantly higher rate of major adverse cardiac events and/or cardiac death in both cardiomyopathy groups (p = 0.003 and p = 0.005). However, the rate of major adverse cardiac events and/or cardiac death was similar between dilated cardiomyopathy patients with and without left ventricular non-compaction. Multivariate logistic regression analysis showed that the presence of fragmented QRS strongly predicts major adverse cardiac events and/or cardiac death (odds ratio, 31.186; 95% confidence interval, 2.347–414.307). Although the survival rates between cardiomyopathy groups were similar, patients with fragmented QRS had a markedly lower survival rate during the follow-up period, as mean of 15 months (p = 0.001).

Our study showed that the presence of fragmented QRS may be an important ECG sign predicting an major adverse cardiac event and/or cardiac death in patients with dilated cardiomyopathy. We believe that recognising fragmented QRS could be valuable in forecasting patient prognosis and identifying high-risk patients who require additional support.

There are limited data documenting sources of medical information that families use to learn about paediatric cardiac conditions. Our study aims to characterise these resources and to identify any disparities in resource utilisation. We hypothesise there are significant variations in the resources utilised by families from different educational and socio-economic backgrounds.

A survey evaluating what resources families use (websites, healthcare professionals, social media, etc.) to better understand paediatric cardiac conditions was administered to caretakers and paediatric patients at Morgan Stanley Children’s Hospital. Patients with a prior diagnosis of CHD, cardiac arrhythmia, and/or heart failure were included. Caretakers’ levels of education (fewer than 16 years vs. 16 years or more) and patients’ medical insurance types (public vs. private) were compared with regard to the utilisation of resources.

Surveys completed by 137 (91%) caretakers and 27 (90%) patients were analysed. Websites were utilised by 72% of caretakers and 56% of patients. Both private insurance and higher education were associated with greater reported utilisation of websites, healthcare professionals, and personal networks (by insurance p = 0.009, p = 0.001, p = 0.006; by education p = 0.022, p < 0.001, p = 0.018). They were also more likely to report use of electronic devices (such as a computer) compared to those with public medical insurance and fewer than 16 years of education (p < 0.001, p < 0.001, respectively).

Both levels of education and insurance status are associated with the utilisation of informative resources and digital devices by families seeking to learn more about cardiac conditions in children.

This study aimed to evaluate the cardiovascular manifestations and surveillance of multisystem inflammatory syndrome in children (MIS-C) and to determine the correlation of echocardiographic findings with cardiac magnetic resonance imaging findings.

Forty-four children diagnosed as MIS-C with cardiac involvement were enrolled in this observational descriptive study. The diagnosis of MIS-C was made according to the criteria of Centers for Disease Control and Prevention. Clinical findings, laboratory parameters, and electrocardiographic and echocardiographic findings at the time of diagnosis and during follow-up were evaluated. Cardiac magnetic resonance was performed on 28 (64%) cases. The 1-year follow-up imaging was performed in all cases with abnormal initial cardiac magnetic resonance findings.

Forty-four patients (56.8% male) with a mean age of 8.5 ± 4.8 years were enrolled in this study. There was a significant positive correlation between high-sensitivity cardiac troponin T (mean: 162 ± 444.4 pg/ml) and N-terminal pro b-type natriuretic peptide (mean: 10,054 ± 11,604 pg/ml) (p < 0.01). Number of cases with an electrocardiographic and echocardiographic abnormality was 34 (77%) and 31 (70%), respectively. Twelve cases (45%) had left ventricular systolic dysfunction and 14 (32%) cases had pericardial effusion on admission. Three cases (11%) had cardiac magnetic resonance findings that may be attributed to the presence of myocardial inflammation, and pericardial effusion was present in seven (25%) cases. Follow-up cardiac magnetic resonances of all cases were normal. Cardiac abnormalities were completely resolved in all except two cases.

Myocardial involvement can be seen during acute disease, but MIS-C generally does not lead to prominent damage during a year of surveillance. Cardiac magnetic resonance is a valuable tool to evaluate the degree of myocardial involvement in cases with MIS-C.

Aortic/arterial stiffness is a reliable, independent predictor and a risk factor for cardiovascular mortality. Arterial stiffness is assessed by pulse wave velocity and echocardiography. The purpose of this study is to analyse aortic/arterial stiffness in patients using echocardiographic and pulse wave velocity techniques.

The participants of this study consisted of 62 patients who presented to the Gazi University Pediatric Endocrinology and Pediatric Cardiology outpatient clinics, including 21 obese, 20 overweight, and 21 normal-weight patients. Echocardiography was performed on all patients, and echocardiographic measurements were compared to pulse wave velocity measurements.

The mean (min-max) arterial strain measurements were 0.146 ± 0.0 (0.06–0.3) in the obese group and 0.106 ± 0.0 (0.05–0.18) in the overweight group. In comparison to the overweight group, the obese group had greater arterial strain measurements. The pulse wave velocity measurements in the obese and overweight groups were greater than those in the normal weight group (p > 0.05). Elastic modulus and aortic stiffness ß index values were shown to be positively correlated with pulse wave velocity measurements in the obese group (r = 0.56, r = 0.53, respectively; p = 0.008, p = 0.01, respectively). Systolic and diastolic blood pressure measurements were correlated with pulse wave velocity measurements in the obese group (r = 0.98, p = 0.0001, respectively).

In our study, echocardiographic aortic measurements showing the vessel wall were correlated with pulse wave velocity measurements. Echocardiographic evaluation should be included in the routine follow-up of patients because pulse wave velocity measurement devices are not available in all centres, echocardiography is available in many centres, it is easily applicable, and it facilitates the follow-up of patients.

The use of peritoneal catheters for prophylactic dialysis or drainage to prevent fluid overload after neonatal cardiac surgery is common in some centres; however, the multi-centre variability and details of peritoneal catheter use are not well described.

Twenty-two-centre NEonatal and Pediatric Heart Renal Outcomes Network (NEPHRON) study to describe multi-centre peritoneal catheter use after STAT category 3–5 neonatal cardiac surgery using cardiopulmonary bypass. Patient characteristics and acute kidney injury/fluid outcomes for six post-operative days are described among three cohorts: peritoneal catheter with dialysis, peritoneal catheter with passive drainage, and no peritoneal catheter.

Of 1490 neonates, 471 (32%) had an intraoperative peritoneal catheter placed; 177 (12%) received prophylactic dialysis and 294 (20%) received passive drainage. Sixteen (73%) centres used peritoneal catheter at some frequency, including six centres in >50% of neonates. Four centres utilised prophylactic peritoneal dialysis. Time to post-operative dialysis initiation was 3 hours [1, 5] with the duration of 56 hours [37, 90]; passive drainage cohort drained for 92 hours [64, 163]. Peritoneal catheter were more common among patients receiving pre-operative mechanical ventilation, single ventricle physiology, and higher complexity surgery. There was no association with adverse events. Serum creatinine and daily fluid balance were not clinically different on any post-operative day. Mortality was similar.

In neonates undergoing complex cardiac surgery, peritoneal catheter use is not rare, with substantial variability among centres. Peritoneal catheters are used more commonly with higher surgical complexity. Adverse event rates, including mortality, are not different with peritoneal catheter use. Fluid overload and creatinine-based acute kidney injury rates are not different in peritoneal catheter cohorts.

Lifetime radiation exposure for paediatric orthotopic heart transplant (OHT) patients is significant with cardiac catheterisation as the dominant source. Interventional cardiac magnetic resonance is utilised to obtain simultaneous, radiation-free haemodynamics and flow/function measurements. We sought to compare invasive haemodynamic measurements and radiation exposure in traditional cardiac catheterisation, to comprehensive interventional cardiac magnetic resonance.

Twenty-eight OHT patients who underwent 67 interventional cardiac magnetic resonance procedures at Children’s National Hospital were identified. Both invasive oximetry with peripheral oxygen saturation (Fick) and cardiac magnetic resonance phase contrast measurements of pulmonary and systemic blood flow were performed. Systemic and pulmonary blood flow from the two modalities was compared using Bland–Altman, concordance analysis, and inter-reader correlation. A mixed model was implemented to account for confounding variables and repeat encounters. Radiation dosage data were collected for a contemporaneous cohort of orthotopic heart transplant patients undergoing standard, X-ray-guided catheterisation.

Simultaneous cardiac magnetic resonance and Fick have poor agreement in our study based on Lin’s correlation coefficient of 0.68 and 0.73 for pulmonary and systemic blood flow, respectively. Bland–Altman analysis demonstrated a consistent over estimation of cardiac magnetic resonance cardiac output by Fick. The average indexed dose area product for patients undergoing haemodynamics with endomyocardial biopsy was 0.73 (SD ±0.6) Gy*m2/kg. With coronary angiography added, the indexed dose area product was 14.6 (SD ± 7.8) Gy*m2/kg.

Cardiac magnetic resonancemeasurements of cardiac output/index in paediatric orthotopic heart transplant patients have poor concordance with Fick estimates; however, cardiac magnetic resonance has good internal validity and inter-reader reliability. Radiation doses are small for haemodynamics with biopsy and increase exponentially with angiography, identifying a new target for cardiac magnetic resonance imaging.

Transcatheter pulmonary valve replacement serves as a successful alternative to surgical replacement of a right ventricle to pulmonary artery conduit. Guidelines for recommending transcatheter pulmonary valve replacement depend on MRI right ventricular volumes, which have been correlated to the echocardiographic measure of right ventricular annular tilt. We aim to assess whether right ventricular annular tilt can be a clinically useful alternative tool in the acute and long-term periods after transcatheter pulmonary valve replacement to assess right ventricular health.

We reviewed 70 patients who underwent transcatheter pulmonary valve replacement at a single institution. Echocardiographic measurements were obtained prior to transcatheter pulmonary valve replacement, immediately after transcatheter pulmonary valve replacement, and within 6 months to 1 year after transcatheter pulmonary valve replacement. Right ventricular annular tilt measures the angle of the tricuspid valve plane relative to the mitral valve plane at end-diastole in the apical four-chamber view. Right ventricular fractional area change, right ventricular systolic strain, tissue Doppler velocity, and tricuspid annular plane systolic excursion Z-scores were obtained using published methods.

Right ventricular annular tilt decreased significantly immediately after transcatheter pulmonary valve replacement (p = 0.0004), and this reduction in right ventricular volume persisted at the mid-term follow-up (p < 0.0001). Fractional area change did not change significantly after transcatheter pulmonary valve replacement while right ventricular global strain improved at mid-term follow-up despite no significant difference immediately after transcatheter pulmonary valve replacement.

Right ventricular annular tilt decreases both immediately after transcatheter pulmonary valve replacement and at mid-term follow-up. Right ventricular strain also improved after transcatheter pulmonary valve replacement, corresponding to the improved volume load. Right ventricular annular tilt can be considered as an additional echocardiographic factor to assess right ventricular volume and remodeling after transcatheter pulmonary valve replacement.

The diagnosis of Duchenne-linked cardiomyopathy may be challenging. Conventional echocardiographic measurements typically show deterioration beyond the second decade. Global longitudinal strain has been proposed as an earlier marker than left ventricular ejection fraction.

A prospective, observational, cross-sectional, case-control study was carried out. Both Duchenne patients and control subjects underwent transthoracic echocardiogram in order to assess left ventricle function. Bayesian linear regression was the main tool for inference. Age effects were parameterised by means of a spline function because of its simplicity to characterise continuous variables and smooth contributions. The posterior distribution of the marginal age effects was used to assess the earliest age of deterioration of each marker.

Sixteen Duchenne patients and twenty-two healthy male subjects were enrolled. On overage, cardiac function measures were found for ejection fraction and longitudinal strain among different groups. Age effects on global longitudinal strain are more reliably found at ages of 6 and above, while ejection fraction starts to deteriorate at an older age. Progressive left ventricular dysfunction in Duchenne patients is one of the key issues and starts at an early age with subtle symptoms.

This cross-sectional study provides supporting evidence that global longitudinal strain is an earlier marker of disease progression than ejection fraction in Duchenne patients.

It is often assumed, that adult patients with CHD (ACHD) have impairments regarding their cognitive function (CF) and health-related quality of life. In particular, it seems reasonable to assume that cyanosis may have a potential impact on CF as well as surgical or drug treatment into adulthood. This study assesses neuromental health aspects such as CF and health-related quality of life in ACHD patients.

Seventy-eight ACHD patients (female n = 39 (50%); 34.1 ± 12.9 years; cyanotic CHD n = 49 (62.8%) with a cyanosis duration of 159.8 ± 196.2 month) who underwent open heart surgery as first intervention were asked to participate during routinely follow-up in 2018. Wechsler Intelligence Scale IV was used for CF and the Short Form 36 Health Survey to assess health-related quality of life.

Intelligence quotient measures showed significant differences comparing never cyanotic and with a cyanotic phase in verbal comprehension (p = 0.013). There was no association of CF with cyanosis duration, number of surgery or catheter, CHD severity, and time of first surgery. The group of early surgery showed significantly better results in physical function (p = 0.040) of health-related quality of life, and in comparison with their assigned reference, both groups showed significantly reduced results in all domains except in bodily pain and mental health. Full-Scale intelligence quotient correlates with physical function of health-related quality of life.

The results show normal CF in ACHD. Health-related quality of life was weak in comparison with the reference. There is a need to improve the well-being of our ACHD with structured programmes, including physical activity programmes. This growing ACHD population should be focused in order of their needs, medical ones on one hand and on the other hand psychosocial matters.

Trivial regurgitation from a normal aortic valve is rarely seen in healthy children and adolescents. The aim of this study was to evaluate timing of presentation, associated conditions, and medium-term prognosis of this incidental finding.

Paediatric patients observed to have trivial aortic valve regurgitation with otherwise normal echocardiograms were retrospectively analysed. Clinical and echocardiographic parameters were measured and categorised on presentation and follow-up.

Sixty patients (39 males) were identified over a 13-year period. Age at presentation was 14.8 years (IQR 12.9–16.0), height z-score was + 0.71 (95% CI + 0.48– + 0.94), and body mass index z-score was + 0.66 (95% CI + 0.40– + 0.92). Median aortic regurgitation vena contracta diameter was 1.0 mm (IQR 0.8–1.3). Aortic valve strands were visualised in 28% and physiologic mitral regurgitation in 32%. Aortic annulus, sinotubular junction, and mid-ascending aorta diameters were normal, and mean aortic sinus diameter was only slightly increased (z-score + 0.23, 95% CI + 0.02– + 0.44). Follow-up data were obtained in 36 patients from 1 to 6.7 years later (median 2.1). Aortic regurgitation was no longer detectable in 28%, and none exhibited worsening. Mitral regurgitation prevalence was lower in those with aortic regurgitation resolution versus persistence (10% versus 50%, p = 0.03).

Trivial aortic regurgitation in paediatric patients with normal hearts is more common in adolescents and is associated with an increased prevalence of aortic valve strands and physiologic mitral regurgitation. These findings do not worsen during growth and may resolve consistent with being physiologic rather than pathologic.

Stenting of stenotic right ventricular outflow tract is a palliative measure for severely impaired small babies with Tetralogy of Fallot or similar pathologies. Little is known about the histopathological fate of the stents in the right ventricular outflow tract.

Eight samples of surgically removed right ventricular outflow tract stents were histologically analysed according to a predefined protocol.

The most frequent diagnosis was Tetralogy of Fallot in four patients, pulmonary atresia with ventricular septal defect in two patients, double outlet right ventricle with pulmonary obstruction in one patient, and muscular obstruction of the right ventricular outflow tract in one patient with a syndromic disease with hypertrophic cardiomyopathy. Stents mean implantation duration was 444 days ranging from 105 to 1117 days (median 305.5 days). Histology revealed a variable degree of pseudointima formation consisting of fibromuscular cells surrounded by extracellular matrix. Four of the specimen contained adjacent myocardial tissue fragments, which showed regressive changes. Neither myocardium nor pseudointima tissue or tissue parts locally related to stent struts were infiltrated by inflammatory cells.

Histological analysis after explantation of early-in-life implanted right ventricular outflow tract stents revealed predominantly pronounced neo-intimal proliferation with a visible endothelial layer, no signs of inflammation, and no prolapse of muscular tissue through the stent struts. Thus, implantation of stents in early life seems to interfere little with the hosts’ immune system and might help to open up the right ventricular outflow tract by mechanical forces and regressive changes in adjacent muscular tissue.

Parents of children with CHD face several barriers when trying to access the services needed to support their child’s development. In fact, current developmental follow-up practices may not identify developmental challenges in a timely manner and important opportunities for interventions may be lost. This study aimed to explore the perspectives of parents of children and adolescents with CHD with respect to developmental follow-up in Canada.

Interpretive description was used as a methodological approach for this qualitative study. Parents of children aged 5–15 years with complex CHD were eligible. Semi-structured interviews that aimed to explore their perspectives regarding their child’s developmental follow-up were conducted.

Fifteen parents of children with CHD were recruited for this study. They expressed that the lack of systematic and responsive developmental follow-up services and limited access to resources to support their child’s development placed an undue burden on their families, and as a result, they needed to assume new roles as case managers or advocates to address these limitations. This additional burden resulted in a high level of parental stress, which, in turn, affected the parent–child relationship and siblings.

The limitations of the current Canadian developmental follow-up practices put undue pressure on the parents of children with complex CHD. The parents stressed the importance of implementing a universal and systematic approach to developmental follow-up to allow for the timely identification of challenges, enabling the initiation of interventions and supports and promoting more positive parent–child relationships.