In recent years, cardiac resynchronization therapy (CRT) has also started to be performed in the paediatric and CHD population. This study aimed to evaluate the efficacy of CRT in children with CHD.

Patients with CHD who underwent CRT treatment in our paediatric cardiology clinic between January, 2010 and January, 2020 were included in the study. Demographic findings, 12-lead electrocardiograms, echocardiograms, clinical characteristics, management strategies, and outcomes were reviewed systematically.

The study population consisted of 18 CHD patients who had been treated with CRT for 10 years in our institution. The median age was 11 years (2.2–18 years) and the median weight was 39 kg (10–81 kg). Systemic ventricle was left ventricle in 13 patients, right ventricle in 4 patients, and 1 patient had single-ventricle physiology. CRT implantation indications were as follows: dysfunction after permanent pacemaker in 11 patients, dysfunction after left bundle branch block in 4 patients, and systemic ventricular dysfunction in 3 patients. CRT implantation techniques were epicardial (n = 13), hybrid (n = 4), and transvenous (n = 1) methods. QRS duration significantly decreased after CRT implantation (160 versus 124 m/second, p < 0.05). Median systemic ventricle ejection fraction (EF) significantly increased after the procedure (30 versus 50%, p < 0.05). Fourteen patients (78%) were responders, two patients (11%) were superresponders, and two patients (11%) were non-responders after the CRT treatment. One patient deceased during follow-up. Median follow-up duration was 40 months (6–117 months).

When electromechanical dyssynchrony occurs in paediatric cases with CHD and developing heart failure, patients should be evaluated in terms of CRT to improve ventricular function. Alternative CRT therapy will be beneficial in these cases that do not improve clinically despite optimal medical treatment.

Shape-memory abnormalities are seen in some nitinol atrial septal occluders. Variably described as cobra-head, tulip, and others, their incidence, mechanisms, clinical impact, and outcome have not been systematically analysed.

We retrospectively reviewed all consecutive device closures in the last 6 years for deformations. Type and size of the occluder, deployment technique, size, and angulation/kinking of the delivery sheath were analysed. Procedural success, duration, and other complications were studied.

A total of 112 devices (11.8%) among 950 occluders used in 936 patients showed deformities. Fourteen of 936 received 2 devices. Deformities were transient and self-correcting in 40%. Multivariate analysis showed significant associations with oversized sheaths (p = 0.004), kinked/angulated sheaths (p < 0.001), special deployment techniques (p < 0.001), and twist in the device waist (p = 0.011). Despite more frequent deformities with Figulla (15.6%) and Amplatzer (13.9%) occluders than Cera occluders (6.6%) and larger devices (>24 mm – 14.6%) than smaller devices (less than or equal to 24 mm – 9.7%), they were not significant on multivariate analysis. In vivo manipulations corrected most deformities; nineteen needed in vitro reformations and four needed a change of device. Despite prolongation of the procedure, repeated attempts (mean 2.76 ± 1.7 attempts, with a range from 1 to 9 attempts), and supraventricular tachycardia in two patients, there were no serious adverse effects.

Deformations were frequent in 11.8% of atrial septal occluders on a targeted search. Oversized and angulated/kinked sheaths, special techniques like pulmonary vein deployment and twist in device waist during procedure predisposed to deformities. While most deformities were corrected with manipulations, removal of the device was infrequently needed and change of device was rarely required. Long procedural time and multiple attempts for deployment did not affect procedural success.

Amongst patients with CHD, the time of transition to adulthood is associated with lapses in care leading to significant morbidity. The purpose of this study was to identify differences in perceptions between parents and teens in regard to transition readiness.

Responses were collected from 175 teen–parent pairs via the validated CHD Transition Readiness survey and an information request checklist. The survey was distributed via an electronic tablet at a routine clinic visit.

Parents reported a perceived knowledge gap of 29.2% (the percentage of survey items in which a parent believes their teen does not know), compared to teens self-reporting an average of 25.9% of survey items in which they feel deficient (p = 0.01). Agreement was lowest for long-term medical needs, physical activities allowed, insurance, and education. In regard to self-management behaviours, agreement between parent and teen was slight to moderate (weighted κ statistic = 0.18 to 0.51). For self-efficacy, agreement ranged from slight to fair (weighted κ = 0.16 to 0.28). Teens were more likely to request information than their parents (79% versus 65% requesting at least one item) particularly in regard to pregnancy/contraception and insurance.

Parents and teens differ in several key perceptions regarding knowledge, behaviours, and feelings related to the management of heart disease. Specifically, parents perceive a higher knowledge deficit, teens perceive higher self-efficacy, and parents and teens agree that self-management is low.

The prevalence of attention deficit/hyperactivity disorder in the general population is common and is now diagnosed in 4%–12% of children. Children with CHD have been shown to be at increased risk for attention deficit/hyperactivity disorder. Case reports have led to concern regarding the use of attention deficit/hyperactivity disorder medications in children with underlying CHD. We hypothesised that medical therapy for patients with CHD and attention deficit/hyperactivity disorder is safe.

A single-centre, retrospective chart review was performed evaluating for adverse events in patients aged 4–21 years with CHD who received attention deficit/hyperactivity disorder therapy over a 5-year span. Inclusion criteria were a diagnosis of CHD and concomitant medical therapy with amphetamines, methylphenidate, or atomoxetine. Patients with trivial or spontaneously resolved CHD were excluded from analysis.

In 831 patients with CHD who received stimulants with a mean age of 12.9 years, there was only one adverse cardiovascular event identified. Using sensitivity analysis, our median follow-up time was 686 days and a prevalence rate of 0.21% of adverse events. This episode consisted of increased frequency of supraventricular tachycardia in a patient who had this condition prior to initiation of medical therapy; the condition improved with discontinuation of attention deficit/hyperactivity disorder therapy.

The incidence of significant adverse cardiovascular events in our population was similar to the prevalence of supraventricular tachycardia in the general population. Our single-centre experience demonstrated no increased risk in adverse events related to medical therapy for children with attention deficit/hyperactivity disorder and underlying CHD. Further population-based studies are indicated to validate these findings.

Haematopoietic stem cell transplantation is an important and effective treatment strategy for many malignancies, marrow failure syndromes, and immunodeficiencies in children, adolescents, and young adults. Despite advances in supportive care, patients undergoing transplant are at increased risk to develop cardiovascular co-morbidities.

This study was performed as a feasibility study of a rapid cardiac MRI protocol to substitute for echocardiography in the assessment of left ventricular size and function, pericardial effusion, and right ventricular hypertension.

A total of 13 patients were enrolled for the study (age 17.5 ± 7.7 years, 77% male, 77% white). Mean study time was 13.2 ± 5.6 minutes for MRI and 18.8 ± 5.7 minutes for echocardiogram (p = 0.064). Correlation between left ventricular ejection fraction by MRI and echocardiogram was good (ICC 0.76; 95% CI 0.47, 0.92). None of the patients had documented right ventricular hypertension. Patients were given a survey regarding their experiences, with the majority both perceiving that the echocardiogram took longer (7/13) and indicating they would prefer the MRI if given a choice (10/13).

A rapid cardiac MRI protocol was shown feasible to substitute for echocardiogram in the assessment of key factors prior to or in follow-up after haematopoietic stem cell transplantation.

The objectives of the present study were to determine whether there was any association between the grade of heart failure, as expressed by preoperative levels of brain natriuretic peptide and Ross score, and the preoperative coagulation status in patients with non-restrictive ventricular shunts and determine whether there were any postoperative disturbances of the coagulation system in these patients, as measured by thromboelastometry and standard laboratory analyses of coagulation.

Perioperative coagulation was analysed with laboratory-based coagulation tests and thromboelastometry before, 8 hours after, and 18 hours after cardiac surgery. In addition, brain natriuretic peptide was analysed before and 18 hours after surgery.

40 children less than 12 months old with non-restrictive congenital ventricular or atrio-ventricular shunts scheduled for elective repair of their heart defects.

All coagulation parameters measured were within normal ranges preoperatively. There was a significant correlation between brain natriuretic peptide and plasma fibrinogen concentration preoperatively. There was no statistically significant correlation between brain natriuretic peptide and INTEM-MCF, FIBTEM-MCF, plasma fibrinogen, activated partial thromboplastin time, prothrombin time, or platelet count at any other time point, either preoperatively or postoperatively. Postoperatively, fibrinogen plasma concentration and FIBTEM-MCF decreased significantly at 8 hours, followed by a large increase at 18 hours to higher levels than preoperatively.

There was no evidence of children with non-restrictive shunts having coagulation abnormalities before cardiac surgery. Brain natriuretic peptide levels or Ross score did not correlate with coagulation parameters in any clinically significant way.

The aim of this study is to report on the short-term and mid-term outcomes of preterm infants who underwent patent ductus arteriosus ligation through anterior mini-thoracotomy.

Data for 103 preterm infants who underwent patent ductus arteriosus clipping through an anterior mini-thoracotomy at the 2nd intercostal space between 2009 and 2019 were retrospectively reviewed. The patients were divided into two groups according to their weight at the time of surgery. The complications, morbidity, and mortality rates of each group were compared at postoperative day 30 and at the end of 1 year after surgery.

During the operation, the median weight of the patients was 900 g (IQR 800–1125 g), the median age was 21 days (IQR 14.5–29 days). The lowest body weight was 460 g. In three patients (3%), there was intraoperative bleeding from the patent ductus arteriosus that required transition to median sternotomy. In one patient (1%) a residual patent ductus arteriosus that required reoperation was observed. Twelve patients (12%) died in the first 30 days postoperatively. Six patients (6%) died between the postoperative day 30 and 1 year. There was no statistically significant difference in the rates of mortality, morbidity, and complication between the groups.

Based on our observations of over a hundred preterm infants with patent ductus arteriosus over a decade, ligation through anterior mini-thoracotomy is the main surgical procedure of choice for this patient group in our clinic. Our findings demonstrate the safety of this approach and we believe that it can be successfully replicated in other institutions.

Ascending aortic dilation is a feared complication in adults with repaired coarctation of the aorta, as the condition is associated with life-threatening complications such as aortic dissection and rupture. However, the data are currently limited regarding factors associated with ascending aortic dilation in these patients.

From the national register of congenital heart disease, 165 adult patients (≥ 18 years old) with repaired coarctation of the aorta, and echocardiographic data on aortic dimensions, were identified (61.2% male, mean age 35.8 ± 14.5 years). Aortic dilation (aortic diameters > 2 SD above reference mean) was found in 55 (33.3%) of the 165 included patients, and was associated with manifest aortic valve disease in univariable logistic regression analysis (OR 2.44, 95% CI [1.23, 4.83]).

Aortic dilation is common post-repair of coarctation of the aorta, and is associated with manifest aortic valve disease and thus indirectly with the presence of a bicuspid aortic valve. However, no association was found between aortic dilation and age or blood pressure.

To evaluate whether Kawasaki disease predisposes to premature atherosclerosis and to assess status of coronary artery abnormalities at least 10 years after diagnosis.

A prospective study was carried out on 21 patients who were diagnosed with Kawasaki disease at least 10 years back and are on regular follow-up. The study was conducted on 128 Slice Dual Source computed tomography scanner with electrocardiography-triggered radiation optimised protocols for assessment of coronary artery abnormalities and calcifications.

Study cohort had 21 subjects – 15 males and 6 females (age range: 11–23 years; mean: 15.76 + 3.72 years). Mean age at time of diagnosis was 3.21 + 2.48 years. Mean time interval from diagnosis of Kawasaki disease to computed tomography coronary angiography was 12.59 + 2.89 years. Four children had evidence of coronary artery abnormalities on transthoracic echocardiography at time of diagnosis. Of these, two had persistent abnormalities on computed tomography coronary angiography. One subject (4.76%) had coronary calcification that was localised to abnormal coronary artery segment. Four coronary artery abnormalities (one saccular; three fusiform aneurysms) were noted in two subjects.

Prevalence of coronary artery calcification is low and, if present, is localised to abnormal segments. This calcification is likely dystrophic rather than atherosclerotic. It appears that coronary artery abnormalities can persist for several years after acute episode of Kawasaki disease. Periodic follow-up by computed tomography coronary angiography is now a feasible non-invasive imaging modality for long term surveillance of patients with Kawasaki disease who had coronary artery abnormalities at time of diagnosis.

It is difficult to predict the complications and prognosis of ECMO, which is gaining widespread use in patients with pediatric surgery. Platelet-to-lymphocyte ratio (PLR) and neutrophil-to-lymphocyte ratio (NLR) are emerging inflammatory markers that can be calculated from complete blood count, which is a cheap and easily accessible laboratory analysis. The ratios between cellular elements in peripheral blood have been demonstrated to provide information on inflammation, infection, and immune response.

Sixty-seven patients who needed ECMO application after undergoing pediatric cardiovascular surgery in our clinic, between May 2005 and April 2020, were included in this study. The age of patients varied between 4 days and 17 years with a mean of 30.59 ± 147.26 months.

The relationships between PLR or NLR values and various blood parameters and blood gas results were found to be statistically nonsignificant in our group of pediatric ECMO recipients. Even if the effect of PLR and NLR values on mortality and prognosis is statistically nonsignificant in patients who need ECMO after congenital heart surgery, PLR and NLR are typically elevated in the postoperative period. An increase in these values above a certain threshold may be a statistically significant indicator for the prediction of mortality.

There are few studies in the literature concerning PLR and NLR values in patients with pediatric heart surgery. We consider this study will make way for new studies in the future.

Acute kidney injury is a risk factor for chronic kidney disease and mortality after congenital heart surgery under cardiopulmonary bypass. The neutrophil–lymphocyte ratio is an inexpensive and easy to measure biomarker for predicting outcomes in children with congenital heart disease undergoing surgical correction.

To identify children at high risk of acute kidney injury after tetralogy of Fallot repair using the neutrophil–lymphocyte ratio.

This single-centre retrospective analysis included consecutive patients aged < 18 years who underwent tetralogy of Fallot repair between January 2014 and December 2018. The pre-operative neutrophil–lymphocyte ratio was measured using the last pre-operative complete blood count test. We used the Acute Kidney Injury Network definition.

A total of 116 patients were included, of whom 39 (33.6%) presented with acute kidney injury: 20 (51.3%) had grade I acute kidney injury, nine had grade II acute kidney injury (23.1%), and 10 (25.6%) had grade III acute kidney injury. A high pre-operative neutrophil–lymphocyte ratio was associated with grade III acute kidney injury in the post-operative period (p = 0.04). Patients with acute kidney injury had longer mechanical ventilation time (p = 0.023), intensive care unit stay (p < 0.001), and hospital length of stay (p = 0.002).

Our results suggest that the pre-operative neutrophil–lymphocyte ratio can be used to identify patients at risk of developing grade III acute kidney injury after tetralogy of Fallot repair.