There are a few number of case reports and small-scale case series reporting dilated cardiomyopathy due to vitamin D-deficient rickets. The present study evaluates the clinical, biochemical, and echocardiographic features of neonates with vitamin D deficiency.

In this prospective single-arm observational study, echocardiographic evaluation was performed on all patients before vitamin D3 and calcium replacement. Following remission of biochemical features of vitamin D deficiency, control echocardiography was performed. Biochemical and echocardiographic characteristics of the present cohort were compared with those of 27 previously published cases with dilated cardiomyopathy due to vitamin D deficiency.

The study included 148 cases (95 males). In the echocardiographic evaluation, none of the patients had dilated cardiomyopathy. All of the mothers were also vitamin D deficient and treated accordingly. Comparison of patients with normocalcaemia and hypocalcaemia at presentation revealed no statistically significant difference between the ejection fraction and shortening fraction, while left ventricle end-diastolic diameter and left ventricle end-systolic diameter were higher in patients with hypocalcaemia. Previously published historical cases were older and had more severe biochemical features of vitamin D deficiency.

To the best of our knowledge, in this first and largest cohort of neonates with vitamin D deficiency, we did not detect dilated cardiomyopathy. Early recognition and detection before developing actual rickets and preventing prolonged hypocalcaemia are critically important to alleviate cardiac complications.

Accessory pathways are commonly seen due to delamination of tricuspid valve leaflets. In addition to accessory pathways, an enlarged right atrium due to tricuspid regurgitation and incisional scars creates substrates for atrial re-entries and ectopic tachycardia. We sought to describe our experience with catheter ablation in children with Ebstein’s anomaly.

During the study period, of 89 patients diagnosed with Ebstein’s anomaly, 26 (30.9%) of them who underwent 33 ablation procedures were included in the study. Accessory pathways were observed in the majority of procedures (n = 27), whereas atrial flutter was observed in five, atrioventricular nodal reentrant tachycardia in five, and atrial tachycardia in two procedures. Accessory pathways were commonly localised in the right posteroseptal (n = 10 patients), right posterolateral (n = 14 patients), septal (n = two patients), and left posteroseptal (n = one patient) areas. Multiple accessory pathways and coexistent arrhythmia were observed in six procedures. All ablation attempts related to the accessory pathways were successful, but recurrence was observed in five (19%) of the ablations. Ablation for atrial flutter was performed in five patients; two of them were ablated successfully. One of the atrial tachycardia cases was ablated successfully.

Ablation in patients with Ebstein’s anomaly is challenging, and due to nature of the disease, it is not a rare occasion in this group of patients. Ablation of accessory pathways has high success, but also relatively high recurrence rates, whereas ablation of atrial arrhythmias has lower success rates, especially in operated patients.

CT is an accepted non-invasive imaging tool to assess the coronary arteries in adults; however, its utilisation in children is limited by high heart rate and lack of standardised protocol. We sought to assess diagnostic quality and factors that affect image quality of CT in assessing coronary artery lesions in Kawasaki patients less than 18 years of age.

CT coronary angiography was performed on patients with Kawasaki disease diagnosed with coronary aneurysm or suspected to have coronary stenosis. Studies were performed using electrocardiogram-gated protocols. General anaesthesia was used in patients who were not cooperative for breathing control. Heart rate, image quality, and effective radiation dose were documented.

Fifty-two Kawasaki patients underwent CT coronary angiography to assess coronary artery lesions. Median heart rate was 88 beats per minute (range 50–165 beats/minute). Image quality was graded as excellent in 34 (65%) patients, good in 17 (32%), satisfactory in 1, and poor in 1 patient. Coronary artery aneurysm was found in 25 (bilateral = 6, unilateral = 19, multiple = 11). Thrombus was found in 11 patients resulting in partial and total occlusion in 8 and 3 patients, respectively. Coronary stenosis was noted in 2 patients. The effective radiation dose was 1.296 millisievert (median 0.81 millisievert). Better diagnostic imaging quality was significantly related to lower heart rate (p = 0.007).

Electrocardiogram-triggered CT coronary angiography provides a good diagnostic assessment of coronary artery lesions in children with Kawasaki disease.

Ceraflex septal occluder and the Figulla atrial septal defect occluder have the advantage of a pivoting mechanism and softer device architecture. This study sought to examine the safety and efficacy of these occluders compared to the Amplatzer septal occluder.

This was a retrospective study. Between January, 2013 and April, 2020, patients with at least 6 months of follow-up were included. Early and late-onset outcomes were examined.

Four hundred seven patients (range: 0.17–70.72 years; 53.1% >18 years; male: 29.2%) underwent atrial septal defect occlusion using Amplatzer septal occluder (n = 313), Ceraflex septal occluder (n = 36) and FSO (n = 58). A longer procedure time was observed in the Amplatzer septal occluder group. Early-onset complication rates in Amplatzer septal occluder, Ceraflex septal occluder and Figulla atrial septal defect occluder were 3.83%, 5.56% and 0%. Ten (2.46%) patients developed delayed complications (2.56%, 0% and 1.72% in the Amplatzer septal occluder, Ceraflex septal occluder and Figulla atrial septal defect occluder groups). Device erosion rate was not different between groups. The occlusion rates were comparable among all the devices.

There is no significant difference in safety and efficacies between the novel atrial septal defect occluding devices compared to Amplatzer septal occluder.

Diagnosis of sinus venosus defects, not infrequently associated with complex anomalous pulmonary venous drainage, may be delayed requiring multimodality imaging.

Retrospective review of all patients from February 2008 to January 2019.

Thirty-seven children were diagnosed at a median age of 4.2 years (range 0.5−15.5 years). In 32 of 37 (86%) patients, diagnosis was achieved on transthoracic echocardiography, but five patients (14%) had complex variants (four had high insertion of anomalous vein into the superior caval vein and three had multiple anomalous veins draining to different sites, two of whom had drainage of one vein into the high superior caval vein). In these five patients, the final diagnosis was achieved by multimodality imaging and intra-operative findings. The median age at surgery was 5.2 years (range 1.6−15.8 years). Thirty-one patients underwent double patch repair, four patients a Warden repair, and two patients a single-patch repair. Of the four Warden repairs, two patients had a high insertion of right-sided anomalous pulmonary vein into the superior caval vein, one patient had bilateral superior caval veins, and one patient had right lower pulmonary vein insertion into the right atrium/superior caval vein junction. There was no post-operative mortality, reoperation, residual shunt or pulmonary venous obstruction. One patient developed superior caval vein obstruction and one patient developed atrial flutter.

Complementary cardiac imaging modalities improve diagnosis of complex sinus venosus defects associated with a wide variation in the pattern of anomalous pulmonary venous connection. Nonetheless, surgical treatment is associated with excellent outcomes.

We aimed to characterise the impact of Down syndrome on myocardial performance and loading conditions in infants with Down syndrome and CHD over the peri-operative period by comparing them with infants matched for cardiac lesion with a normal microarray.

Left ventricular global longitudinal strain, right ventricular free wall longitudinal strain, left ventricular end-systolic wall stress, and right ventricular systolic pressure were measured in the two groups over the peri-operative period.

Fifty-five infants had a diagnosis of Down syndrome and these were compared with 29 control infants. Left ventricular global longitudinal strain decreased in both groups post-operatively with the Down syndrome group demonstrating some recovery pre-discharge (18 ± 3 versus 16 ± 3 %, p = 0.01). Right ventricular longitudinal strain significantly decreased in both groups post-operatively with the control group demonstrating better recovery by hospital discharge (14 ± 4 versus 18 ± 6 %, p < 0.01). End-systolic wall stress was lower and right ventricular systolic pressure was higher in the Down syndrome group throughout the study period (all p < 0.05). Down syndrome was an independent predictor of the duration of ventilation, post-operative use of inotropes, and intensive care stay. Right ventricular longitudinal strain was an independent predictor of duration of intensive care stay.

This study demonstrates the difference between the two groups in relation to left and right ventricular function, particularly prior to discharge, and outlines the additional impact a diagnosis of Down syndrome has on myocardial performance during the peri-operative period.

The diagnosis of hypertension in adolescents aged ≥13 and <16 years is based on the percentile according to age, gender, and height in the European Society of Hypertension guidelines guideline; whereas, the American Academy of Pediatrics guideline uses blood pressure above 130/80 mmHg as a single criterion. Therefore, this study aimed to evaluate the compatibility of these two guidelines in adolescents aged ≥13 and <16 years.

This study was designed by retrospectively screening the records of 395 adolescents with both office and 24-hour ambulatory blood pressure measurements. Each blood pressure measurement was classified according to both the ESGH2016 and AAP2017 guidelines. Patients were divided into three subgroups according to body mass index. Cohen’s kappa analysis was used to evaluate the agreement between the two guidelines.

The majority of adolescents were normotensive according to both guidelines, 55.9% by ESHG2016 and 43.1% by AAP2017. For the whole group, the frequency of hypertension was 32.4% with ESHG2016 and 34.4% with AAP2017; while, in obese patients, hypertension frequencies were 38.8% and 43.3%, respectively. The diagnosis of hypertension was demonstrated with the two guidelines, and there was significant agreement at a substantial level, both for the obese subgroup and the whole study group (kappa value = 0.738 and 0.785, respectively). The frequency of white-coat hypertension was higher with the AAP2017 guideline (28.1% versus 16.2%, p < 0.001).

With our experience in this single-centre study, it seems that both the AAP2017 and the ESHG2016 guidelines can be used in the diagnosis of hypertension in adolescents.

Pericardiocentesis is the invasive percutaneous procedure for acute and chronic excessive accumulation of pericardial fluid. There is a paucity of data on the effectiveness and safety of pericardiocentesis in children.

To evaluate the effectiveness and safety of pericardiocentesis and factors associated with acute procedural failure and adverse events.

This was a single-centered retrospective study to describe all the children aged ≤20 years who underwent pericardiocentesis. Data on demographics, etiologies of pericardial effusion, and repeat intervention at follow-up were collected.

A total of 127 patients underwent 153 pericardiocentesis. The median age was 6.5 years (1 day–20 years) with weight of 17 kg (0.5–125). Most common etiology was post-pericardiotomy syndrome (n = 56, 44%), followed by infectious (12%), malignant (10%), and iatrogenic (9%). Pericardiocentesis was performed more commonly in the catheterisation laboratory (n = 86, 59%). Concurrent pericardial drain placement was performed in 67 patients (53%). Acute procedural success was 92% (141/153). Repeat intervention was performed in 33 patients (22%). The incidence of adverse events was 4.6% (7/153): hemopericardium requiring emergent surgery (n = 2); hemopericardium with hypotension (n = 2); seizure with anesthesia induction (n = 1); and right ventricle puncture with needle (n = 2). Pericardiocentesis at the bedside had a higher rate of acute procedural failure than that in the catheterisation lab (17 versus 1%, p < 0.01). No identifiable risk factors were associated with adverse events.

Pericardiocentesis was life-saving in children with its high effectiveness and safety even in urgent situations. Although initial pericardiocentesis was effective, one of five patients required re-intervention for recurrent pericardial effusion.

Obesity increases the risk of post-operative arrhythmias in adults undergoing cardiac surgery, but little is known regarding the impact of obesity on post-operative arrhythmias after CHD surgery.

Patients undergoing CHD surgery from 2007 to 2019 were prospectively enrolled in the parent study. Telemetry was assessed daily, with documentation of all arrhythmias. Patients aged 2–20 years were categorised by body mass index percentile for age and sex (underweight <5, normal 5–85, overweight 85–95, and obese >95). Patients aged >20 years were categorised using absolute body mass index. We investigated the impact of body mass index category on arrhythmias using univariate and multivariate analysis.

There were 1250 operative cases: 12% underweight, 65% normal weight, 12% overweight, and 11% obese. Post-operative arrhythmias were observed in 38%. Body mass index was significantly higher in those with arrhythmias (18.8 versus 17.8, p = 0.003). There was a linear relationship between body mass index category and incidence of arrhythmias: underweight 33%, normal 38%, overweight 42%, and obese 45% (p = 0.017 for trend). In multivariate analysis, body mass index category was independently associated with post-operative arrhythmias (p = 0.021), with odds ratio 1.64 in obese patients as compared to normal-weight patients (p = 0.036). In addition, aortic cross-clamp time (OR 1.007, p = 0.002) and maximal vasoactive–inotropic score in the first 48 hours (OR 1.03, p = 0.04) were associated with post-operative arrhythmias.

Body mass index is independently associated with incidence of post-operative arrhythmias in children after CHD surgery.

Intracardiac echocardiography Doppler-derived gradients have previously been shown to correlate with post-procedure echocardiographic evaluations when compared with invasive gradients measured during percutaneous pulmonary valve implantation, suggesting that intracardiac echocardiography could offer an accurate and predictable starting point to estimate valve function after percutaneous pulmonary valve implantation.

We performed a retrospective chart review of 51 patients who underwent percutaneous pulmonary valve implantation between September 2018 and December 2019 in whom intracardiac echocardiography was performed immediately after valve implantation. We evaluated the correlation between intracardiac echocardiography gradients and post-procedural Doppler-derived gradients. Among the parameters assessed, those which demonstrated the strongest correlation were used to create a predictive model of expected echo-derived gradients after percutaneous pulmonary valve implantation. The equation was validated on the same sample data along with a subsequent cohort of 25 consecutive patients collected between January 2020 and July 2020.

All the assessed correlation models between intracardiac echocardiography evaluation and post-procedure transthoracic echocardiographic assessments were statistically significant, presenting moderate to strong correlations. The strongest relationship was found between intracardiac echocardiography mean gradients and post-procedural transthoracic echocardiographic mean gradients. Therefore, an equation was created based on the intracardiac echocardiography-derived mean gradient, to allow prediction of the post-procedural and follow-up transthoracic echocardiographic-derived mean gradients within a range of ±5 mmHg from the observed value in more than 80% of cases.

There is a strong correlation between intracardiac echocardiography and post-procedure transthoracic echocardiographic. This allowed us to derive a predictive equation that defines the expected transthoracic echocardiographic Doppler-derived gradient following the procedure and at out-patient follow-up after percutaneous pulmonary valve implantation.

Systemic ventricular end-diastolic pressure is an important haemodynamic variable in adult patients with Fontan circulation. Risk factors associated with elevated end-diastolic pressure have not been clearly identified in this population.

All patients > 18 years with Fontan circulation who underwent cardiac catheterisation at our centre between 1/08 and 3/19 were included. Relevant patient variables were extracted. Univariate and multivariate general linear models were analysed to identify variables associated with end-diastolic pressure.

Forty-two patients were included. Median age was 24.0 years (20.9–29.0) with a body mass index of 23.7 kg/m2 (21.5–29.7). 10 (23.8%) patients had a systemic right ventricle. The median (Interquartile range) and mean pulmonary artery pressure were 11.0 mmHg (9.0–12.0) and 16.0 mmHg (13.0–18.0), respectively. On univariate analysis, end-diastolic pressure was positively associated with body mass index (p < 0.01), age > 25 years (p = 0.04), symptoms of heart failure (p < 0.01), systemic ventricular systolic pressure (p = 0.03), pulmonary artery mean pressure (p < 0.01), and taking diuretics (p < 0.01) or sildenafil (p < 0.01). End-diastolic pressure was negatively associated with aortic saturation (p < 0.01). On multivariate analysis, end-diastolic pressure was positively associated with age ≥ 25 years (p < 0.01), and body mass index (p = 0.04).

In a cohort of adult patients with Fontan circulation undergoing catheterisation, end-diastolic pressure was positively associated with age ≥ 25 years and body mass index on multivariate analysis. Maintaining a healthy body mass index may offer haemodynamic benefit in adults with Fontan physiology.

Hypotension is an adverse event that may be related to systemic exposure of milrinone; however, the true exposure–safety relationship is unknown.

Using the Pediatric Trials Network multicentre repository, we identified children ≤17 years treated with milrinone. Hypotension was defined according to age, using the Pediatric Advanced Life Support guidelines. Clinically significant hypotension was defined as hypotension with concomitant lactate >3 mg/dl. A prior population pharmacokinetic model was used to simulate milrinone exposures to evaluate exposure–safety relationships.

We included 399 children with a median (quarter 1, quarter 3) age of 1 year (0,5) who received 428 intravenous doses of milrinone (median infusion rate 0.31 mcg/kg/min [0.29,0.5]). Median maximum plasma milrinone concentration was 110.7 ng/ml (48.4,206.2). Median lowest systolic and diastolic blood pressures were 74 mmHg (60,85) and 35 mmHg (25,42), respectively. At least 1 episode of hypotension occurred in 178 (45%) subjects; clinically significant hypotension occurred in 10 (2%). The maximum simulated milrinone plasma concentrations were higher in subjects with clinically significant hypotension (251 ng/ml [129,329]) versus with hypotension alone (86 ng/ml [44, 173]) versus without hypotension (122 ng/ml [57, 208], p = 0.002); however, this relationship was not retained on multivariable analysis (odds ratio 1.01; 95% confidence interval 0.998, 1.01).

We successfully leveraged a population pharmacokinetic model and electronic health record data to evaluate the relationship between simulated plasma concentration of milrinone and systemic hypotension occurrence, respectively, supporting the broader applicability of our novel, efficient, and cost-effective study design for examining drug exposure–response and –safety relationships.

The coronary flow velocity reserve of the left anterior descending artery was examined by transthoracic Doppler echocardiography in 189 patients (male 125, female 64) with a history of Kawasaki disease and 10 volunteers (control). Their ages ranged from 6 to 40 years (median 22 years). The patients were divided into nine groups based on the left anterior descending artery lesions. The coronary flow velocity reserve was measured by intravenous administration of adenosine triphosphate (0.15 mg/kg/minute) while fasting. The coronary flow velocity reserve was calculated as the ratio of hyperaemic to basal mean diastolic flow velocities. The respective groups were as follows: control, no coronary artery lesions (n = 39), no coronary artery lesions in the right coronary artery (n = 29), regression (n = 11), aneurysm at the bifurcation of the left coronary artery (n = 26), aneurysm of the left anterior descending artery (n = 15), localised stenosis <75% (n = 12), localised stenosis ≥75% (n = 17), segmental stenosis (n = 5) and coronary artery bypass grafting (n = 36). One-factor ANOVA followed by Tukey’s test was used to compare the coronary flow velocity reserve among the groups.

The coronary flow velocity reserve was significantly lower in the localised stenosis ≥75%, segmental stenosis and coronary artery grafting groups than in the other groups (p < 0.05).

The endothelial function in the epicoronary artery was preserved in patients with a history of Kawasaki disease and dilated coronary artery lesions.

To investigate the safety and feasibility of midazolam for conscious sedation in transcatheter device closure of atrial septal defects guided solely by transthoracic echocardiography.

A retrospective analysis was performed on 55 patients who underwent transcatheter device closure of atrial septal defects from October, 2019 to May, 2020. All patients received intravenous midazolam and local anesthesia with lidocaine to maintain sedation. A group of previous patients with unpublished data who underwent the same procedure with general anesthesia was set as the control group. The relevant clinical parameters, the Ramsay sedation scores, the numerical rating scale, and the post-operative satisfaction questionnaire were recorded and analyzed.

In the midazolam group, the success rate of atrial septal defect closure was 98.2%. Hemodynamic stability was observed during the procedure. None of the patients needed additional endotracheal intubation for general anesthesia. Compared with the control group, the midazolam group had no statistically significant differences in the Ramsay sedation score and numerical rating scale scores. Patients in the midazolam group experienced more post-operative satisfaction than those in the control group.

Conscious sedation using midazolam is a safe and effective anesthetic technique for transcatheter device closure of atrial septal defects guided solely by transthoracic echocardiography.

To investigate the risk factors associated with prolonged ventilation after Fontan surgery.

Retrospective case series.

Tertiary childrens hospital.

We included 123 children who underwent Fontan surgery without delayed sternal closure or extracorporeal membrane oxygenation between 2011 and 2017.

Fontan surgery.

Prolonged ventilation was defined as intubation for more than 24 hours after surgery. Preoperative, intraoperative, and perioperative data were collected retrospectively from medical records. Multivariate logistic regression analysis was used to identify risk factors for prolonged ventilation. The median age and weight of patients were 2.2 years and 10.0 kg, respectively. Seventeen per cent of the patients (n = 21) received prolonged mechanical ventilation, and the median intubation period was 2.9 days. There were no 90-day or in-hospital deaths. The independent predictors of prolonged ventilation identified were fenestration (p < 0.01), low pulmonary artery index (p = 0.02), and advanced atrioventricular regurgitation (p < 0.01). The duration of ICU stay was significantly longer in the prolonged ventilation group than in the early extubation group (10 days versus 6 days, p < 0.01).

Fenestration, low pulmonary artery index, and significant atrioventricular regurgitation are risk factors for prolonged ventilation after Fontan surgery. Careful preoperative and perioperative management that considers the risk factors for prolonged ventilation in each individual is important.

To assess current demographics and duties of physicians as well as the structure of paediatric cardiac critical care in the United States.

REDCap surveys were sent by email from May till August 2019 to medical directors (“directors”) of critical care units at the 120 United States centres submitting data to the Society of Thoracic Surgeons Congenital Heart Surgery Database and to associated faculty from centres that provided email lists. Faculty and directors were asked about personal attributes and clinical duties. Directors were additionally asked about unit structure.

Responses were received from 66% (79/120) of directors and 62% (294/477) of contacted faculty. Seventy-six percent of directors and 54% of faculty were male, however, faculty <40 years old were predominantly women. The majority of both groups were white. Median bed count (n = 20) was similar in ICUs and multi-disciplinary paediatric ICUs. The median service expectation for one clinical full-time equivalent was 14 weeks of clinical service (interquartile range 12, 16), with the majority of programmes (86%) providing in-house attending night coverage. Work hours were high during service and non-service weeks with both directors (37%) and faculty (45%).

Racial and ethnic diversity is markedly deficient in the paediatric cardiac critical care workforce. Although the majority of faculty are male, females make up the majority of the workforce younger than 40 years old. Work hours across all age groups and unit types are high both on- and off-service, with most units providing attending in-house night coverage.

Iron deficiency is associated with worse outcomes in children and adults with systolic heart failure. While oral iron replacement has been shown to be ineffective in adults with heart failure, its efficacy in children with heart failure is unknown. We hypothesised that oral iron would be ineffective in replenishing iron stores in ≥50% of children with heart failure.

We performed a single-centre retrospective cohort study of patients aged ≤21 years with systolic heart failure and iron deficiency who received oral iron between 01/2013 and 04/2019. Iron deficiency was defined as ≥2 of the following: serum iron <50 mcg/dL, serum ferritin <20 ng/mL, transferrin >300 ng/mL, transferrin saturation <15%. Iron studies and haematologic indices pre- and post-iron therapy were compared using paired-samples Wilcoxon test.

Fifty-one children with systolic heart failure and iron deficiency (median age 11 years, 49% female) met inclusion criteria. Heart failure aetiologies included cardiomyopathy (51%), congenital heart disease (37%), and history of heart transplantation with graft dysfunction (12%). Median dose of oral iron therapy was 2.9 mg/kg/day of elemental iron, prescribed for a median duration of 96 days. Follow-up iron testing was available for 20 patients, of whom 55% (11/20) remained iron deficient despite oral iron therapy.

This is the first report on the efficacy of oral iron therapy in children with heart failure. Over half of the children with heart failure did not respond to oral iron and remained iron deficient.

Expert guidance from scientific societies and regulatory agencies recommend a framework of principles for frequency of in-person evaluations and remote monitoring for patients with cardiac implantable electronic devices. However, there are limited data regarding adherence to recommendations among paediatric electrophysiologists, and there are no data regarding cardiac implantable electronic device-related ancillary testing.

To assess current clinical practices for cardiac implantable electronic device in-person evaluation, remote monitoring, and cardiac implantable electronic device-related ancillary testing, the Paediatric and Congenital Electrophysiology Society members were surveyed. The main outcome measures were variations in frequency of in person evaluation, frequency of remote monitoring, and cardiac implantable electronic device-related ancillary testing.

All respondents performed in-person evaluation at least once a year, but <50% of respondents performed an in-person evaluation within 2 weeks of cardiac implantable electronic device implantation. Remote monitoring was performed every 3 months for pacemakers and implantable cardioverter defibrillators by 71 and 75% respondents, respectively. Follow-up echocardiography was performed every 2–3 years by 53% respondents for patients with >50% ventricular pacing. Majority of respondents (75%) did not perform either an exercise stress test or ambulatory Holter monitoring or chest X-ray (65%) after cardiac implantable electronic device implantation.

This survey identified significant practice variations in cardiac implantable electronic device in- person evaluation, remote monitoring, and ancillary testing practices among paediatric electrophysiologists. Cardiac implantable electronic device management may be optimised by development of a paediatric-specific guidelines for follow-up and ancillary testing.