Patients with univentricular hearts can only be palliated by a staged surgical procedure that carries a high morbidity and mortality risk. The aim of this study was to examine the emotional demands, psychosocial burden, and quality of life of parents with children with univentricular hearts compared to parents of children with a simple heart defect, those with no heart defect and children with chronic diseases.

An anonymous questionnaire was created to interview parents about their quality of life, stressors, needs, strategies for coping with illness, and partnership satisfaction.

73 families participated in the study. Parents of children with univentricular hearts experience a significantly higher psychosocial burden, limitations in daily life, and distress in family interactions, as well as greater emotional distress compared to the other study groups. When comparing the families of children with other chronic diseases (e.g. cystic fibrosis, chronic arthritis and diabetes), these differences remained significant.

The study confirms a higher psychosocial burden, restrictions in daily life and a lower quality of life of parents with children with univentricular hearts, compared to parents of children with simple heart defects and parents of heart-healthy children or those with other chronic diseases. Since this condition persists until adolescence and adulthood, the families are exposed to special challenges and stresses throughout their lives. This has yet to be adequately addressed in the management of these families.

Congenital heart patients undergoing congenital heart surgery in the first year of life are at high risk of having a neurodevelopmental disorder. The most common difficulties are related to executive functioning. The following questions were assessed in the current project: Are patients having congenital heart surgery after one year of life at lower risk for neurodevelopmental disorders? At what age do executive function deficits manifest?

We evaluated executive function in four groups of congenital heart patients who had undergone congenital heart surgery. These groups were high-risk patients with and without a genetic syndrome associated with a neurodevelopmental disorder and low-risk patients with and without a genetic syndrome associated with a neurodevelopmental disorder. We evaluated executive function using the Behavior Rating Inventory of Executive Function – Preschool Version, Behavior Rating Inventory of Executive Function-2, and Minnesota Executive Function Scale at various ages. We compared the rates of executive function deficits in the high- and low-risk groups as well as compared that to the published norms for age. We also assessed at what age these deficits become apparent.

We found that both high- and low-risk groups had higher levels of executive functioning deficits compared to the norms for age. The low-risk group’s degree of executive function deficits appeared a little lower than the high-risk group. However, it was difficult to comment on the statistical significance. We also saw that executive function deficits often do not become apparent for many years after surgery. This finding highlights the need for continued evaluation of functioning as these kids mature.

It is unclear how extracorporeal membrane oxygenation use varies across paediatric cardiac surgical programmes and how it relates to post-operative mortality. We aimed to determine hospital-level variation in post-operative extracorporeal membrane oxygenation use and its association with case-mix adjusted mortality.

Retrospective analysis of 37 hospitals contributing to the Pediatric Cardiac Critical Care Consortium clinical registry from 1 August 2014 to 31 December 2019. Hospitalisations including cardiothoracic surgery and post-operative admission to paediatric cardiac ICUs were included. Two-level multivariable logistic regression with hospital random effect was used to determine case-mix adjusted post-operative extracorporeal membrane oxygenation use rates and in-hospital mortality. Hospitals were grouped into extracorporeal membrane oxygenation use tertiles, and mortality was compared across tertiles.

There were 43,640 eligible surgical hospitalisations; 1397 (3.2%) included at least one post-operative extracorporeal membrane oxygenation run. Case-mix adjusted extracorporeal membrane oxygenation rates varied more than sevenfold (0.9–6.9%) across hospitals, and adjusted mortality varied 10-fold (0–5.5%). Extracorporeal membrane oxygenation rates were 2.0%, 3.5%, and 5.2%, respectively, for low, middle, and high extracorporeal membrane oxygenation use tertiles (P < 0.0001), and mortality rates were 1.9%, 3.0%, and 3.1% (p < 0.0001), respectively. High extracorporeal membrane oxygenation use hospitals were more likely to initiate extracorporeal membrane oxygenation support intraoperatively (1.6% vs. 0.6% low and 1.1% middle, p < 0.0001). Extracorporeal membrane oxygenation indications were similar across hospital tertiles. When extracorporeal cardiopulmonary resuscitation was excluded, variation in extracorporeal membrane oxygenation use rates persisted (1.5%, 2.6%, 3.8%, p < 0.001).

There is hospital variation in adjusted post-operative extracorporeal membrane oxygenation use after paediatric cardiac surgery and a significant association with adjusted post-operative mortality. These findings suggest that post-operative extracorporeal membrane oxygenation use could be a complementary quality metric to mortality to assess performance of cardiac surgical programmes.

Transcatheter patent ductus arteriosus closure in premature infants is gaining attention with rapidly growing experience. The KA micro plug device poses many favourable features for ductus arteriosus closure in premature infants. The study aimed to describe the technique and outcomes of a KA micro plug in closing the ductus arteriosus in premature infants weighing less than 1500 g.

This is a single-centre retrospective study in premature infants with patent ductus arteriosus weighing less than 1500 g who underwent off-label use of a KA micro plug at the Stead Family Children’s Hospital from February 2022 to December 2023.

Eighteen premature infants weighing less than 1500 g underwent attempted transcatheter ductus arteriosus closure using a KA micro plug device. Median weight, age, and corrected gestational age at the procedure were 943 g (682–1225), 26 days (9–79), and 28.5 weeks (25.6–32), respectively. The procedure was successful with complete closure achieved in all patients. No major haemodynamic instabilities were encountered. No catheterization-related complications were encountered. On a median follow-up of 10.9 months (0.1–19), all patients are alive with the device in a good position without residual shunt or abnormal findings.

KA micro plug device for patent ductus arteriosus in infants weighing less than 1500 g is feasible, safe, and effective. We recommend that manufacturers of devices used to close the ductus arteriosus in small infants enhance both the devices and their delivery systems to simplify the procedure and mitigate the risk of haemodynamic instability.

Airway problems emerging after congenital cardiac surgery operations may have an impact on mortality and morbidity. Recently, to improve alveolar gas exchange and reduce respiratory effort, high-flow nasal cannula (HFNC) has started to be used in paediatric cases. This study aimed to evaluate the potential effects of high-flow nasal oxygen therapy on postoperative atelectasis development and reintubation rate in paediatric cardiac surgery patients.

This study was conducted retrospectively in term newborns and infants younger than six months of age who underwent congenital cardiac surgery operation from 1 November 2022 to 1 November 2023 and were followed in the paediatric cardiac ICU. Patients who were receiving mechanical ventilator support at least 12 hours postoperatively were evaluated for the development of postoperative atelectasis and reintubation in the first 3 days of extubation. The patients were grouped as HFNC and non-HFNC users. Demographic characteristics, surgery type, and ICU clinical follow-up data were obtained from medical records. The results were statistically evaluated.

A total of 40 patients who did not use HFNC in the early postoperative period and 40 patients with HFNC in the late period during the study period were included in the study. The median age was 1 month (IQR 15 days–2 months) with equal gender distribution. Among patients, 70% of them were in the neonatal age group. Reintubation rates in the first 72 hours in HFNC users and non-HFNC users were 2.5% and 12.5%, respectively (p < 0.05). The median postoperative atelectasis scores at 24, 48, and 72 hours of extubation were 2 versus 2.5 (p > 0.05), 1.5 versus 3.5 (p < 0.05), and 1 versus 3 (p < 0.05) in HFNC users and non-HFNC users, respectively.

HFNC therapy may have a positive effect on preventing atelectasis and reducing the reintubation rate in the early postoperative period.

Failing Fontan poses a significant clinical challenge. This study aims to improve patients’ outcomes by comprehensively understanding the incidence, pathophysiology, risk factors, and treatment of failing Fontan after total cavopulmonary connection.

We performed a retrospective analysis of patients who underwent total cavopulmonary connection at the German Heart Center Munich between 1994 and 2022. The onset of failing Fontan was defined as: protein-losing enteropathy, plastic bronchitis, NYHA class IV, NYHA class III for > one year, unscheduled hospital admissions for heart failure symptoms, and evaluation for heart transplantation.

Among 634 patients, 76 patients presented with failing Fontan, and the incidence was 1.48 per 100 patient-years. Manifestations included protein-losing enteropathy (n = 34), hospital readmission (n = 28), NYHA III (n = 18), plastic bronchitis (n = 16), evaluation for heart transplantation (n = 14), and NYHA IV (n = 4). Risk factors for the onset of failing Fontan were dominant right ventricle (p = 0.010) and higher pulmonary artery pressure before total cavopulmonary connection (p = 0.004). A total of 72 interventions were performed in 59 patients, including balloon dilatation/stent implantation in the total cavopulmonary connection pathway (n = 49) and embolization of collaterals (n = 24). Heart transplantation was performed in four patients. The survival after the onset of Fontan failure was 77% at 10 years. Patients with failing Fontan revealed significantly higher zlog-NT-proBNP levels after onset compared to those without (p = 0.021)

The incidence of Fontan failure was 1.5 per 100 patient years. Dominant right ventricle and higher pulmonary artery pressure before total cavopulmonary connection were significant risks for the onset of failing Fontan. Zlog-NT-proBNP is only a late marker of Fontan failure.

Certain rhythmic arterial pressure waves in humans and animals have been noticed for over one century. We found the novel and slowest arterial pressure waves in children following surgical repair for CHD, and examined their characteristics and clinical implications.

We enrolled 212 children with 22 types of CHD within postoperative 48 h. We monitored haemodynamics (blood pressure, cardiac cycle efficiency, dP/dTmax), cerebral (ScO2), and renal (SrO2) oxygen saturation every 6 s. Electroencephalogram was continuously monitored. Mean blood flow velocity (Vm) of the middle cerebral artery was measured at 24 h.

We found the waves with a frequency of ∼ 90 s immediately following surgical repair in 46 patients in 12 types of CHD (21.7%), being most prevalent in patients with aortic arch abnormalities (Aorta Group, n = 24, 42.3%) or ventricular septal defect (Ventricular Septal Defect Group, n = 12, 23.5%). In Aorta and Ventricular Septal Defect Groups, the occurrence of the waves was associated with lower blood pressures, dP/dTmax, cardiac cycle efficiency, ScO2, SrO2, Vm, worse electroencephalogram background abnormalities, higher number of electroencephalogram sharp waves, and serum lactate (Ps <0.0001–0.07), and were accompanied with fluctuations of ScO2 and SrO2 in 80.6% and 69.6% of patients, respectively.

The waves observed in children following cardiovascular surgery are the slowest ever reported, occurring most frequently in patients with aortic arch abnormalities or ventricular septal defect. While the occurrence of the waves was associated with statistically worse and fluctuated ScO2 and SrO2, worse systemic haemodynamics, and electroencephalogram abnormalities, at present these waves have no known clinical relevance.

Transthoracic echocardiography is the gold standard method for screening and confirmation of acute rheumatic fever and subclinical rheumatic heart disease. Secondary antibiotic prophylaxis that is regularly employed in subclinical rheumatic heart disease may help to reverse mild rheumatic carditis lesions, delay the progression of the disease, reduce morbidity and mortality, and improve patients’ quality of life.

We retrospectively evaluated the outcomes of 180 patients with subclinical rheumatic heart disease who were followed up for a mean of 4.92 ± 2.0 (3.5–6.5) years.

Between 1 March 2015 and 31 December 2023, 180 patients diagnosed with subclinical rheumatic heart disease with a mean follow-up of 4.92 ± 2.0 (3.5–6.5) years were included in the study. Of the patients, 50.6% were male, 49.4% were female, mean age at diagnosis was 11.74 ± 3.18 (9.68–13.65) years, and mean follow-up period was 4.92 ± 2.0 (3.5–6.5) years. Further, 87.2 % of the patients had mitral valve regurgitation, 38.3% had aortic valve regurgitation, and 27.2% had both valve (aortic and mitral valve) regurgitation. Moreover, Sydenham chorea was also diagnosed in 7.8% the patients. Of the patients, 90% had mild rheumatic heart disease, 7.8% had moderate rheumatic heart disease, and 2.2% had severe rheumatic heart disease. After the diagnosis of rheumatic heart disease, 76.7% patients received regular and 23.3% irregular secondary benzathine penicillin G prophylaxis.

We believe that echocardiography demonstrates its efficacy and safety profile in reducing the risk of rheumatic heart disease in patients diagnosed with subclinical rheumatic carditis and complying with regular secondary antibiotic prophylaxis.

Cardiac catheterisation is crucial for diagnosing and treating paediatric heart diseases, but it is poorly tolerated by small children, infants, and newborns without sedation. This study investigated whether maternal voice during sedation could lower stress and pain in children undergoing cardiac catheterisation and also assessed mothers’ stress levels before and after the procedure.

This was a prospective, monocentric, randomised, controlled interventional study at the University Hospital Bonn. Children aged 4 years or younger scheduled for elective cardiac catheterisation under procedural sedation and American Society of Anaesthesiologists class between 1 and 3 were eligible.

At the end of cardiac catheterisation, the intervention group showed a higher Newborn Infant Parasympathetic Evaluation index with an adjusted mean difference of 9.5 (± 4.2) (p = 0.026) and a lower median Children’s and Infants Postoperative Pain Scale score of 2.0 (IQR: 0.0–5.0) versus 4.5 (IQR: 3.0–6.0) than the control group (p = 0.027). No difference in the children’s cortisol level was found (p = 0.424). The mothers in the intervention group had a lower cortisol level than those in the control group before cardiac catheterisation (adjusted mean difference: −4.5 nmol/l (± 1.8 nmol/l), p = 0.011).

Listening to the maternal voice during cardiac catheterisation could lead to less postoperative pain and significantly lower stress and discomfort level in children. Less pain could reduce the incidence of postoperative delirium.

Additionally, mothers perceived involvement as positive. A reduced stress level of mothers can positively influence children and possibly reduce pain and anxiety.

Marfan syndrome is a rare genetic connective tissue disorder. Research on health-related quality of life in Swedish patients is lacking. We aimed to examine health-related quality of life in patients with Marfan syndrome with respect to reference values, sex, and age.

Using the registry for adult CHD, Sahlgrenska University Hospital/Östra Hospital, between 1 April 2009 and 31 January 2023, we identified 1916 patients. Of these, we included 33 patients aged ≥18 years who were diagnosed with Marfan syndrome and had completed the 36-item Short-Form Health Survey.

The median age was 32 years (interquartile range 25.5–47.0) and 22 (66.7%) were men. Patients with Marfan syndrome had significantly lower values than reference values for all scales in the Short-Form Health Survey except bodily pain, role-emotional, and the physical component summary score. For both men and women with Marfan syndrome, vitality was the subscale with the greatest percentage difference in comparison with healthy reference values (82% in women and 73% in men). Furthermore, men reported significantly higher vitality levels than women (62.5 points, interquartile range 43.8–75.0 vs. 35 points, interquartile range 10.0–65.0, p = 0.026).

Adults with Marfan syndrome in Sweden showed lower health-related quality of life levels in comparison with reference values for most Short-Form Health Survey scales, and there were differences between patients with Marfan syndrome in terms of sex and age.

To investigate functional outcomes in children who survived extracorporeal life support at 12 months follow-up post-discharge.

Some patients who require extracorporeal life support acquire significant morbidity during their hospitalisation. The Functional Status Scale is a validated tool that allows quantification of paediatric function.

A retrospective study that included children placed on extracorporeal life support at a quaternary children’s hospital between March 2020 and October 2021 and had follow-up encounter within 12 months post-discharge.

Forty-two patients met inclusion criteria: 33% female, 93% veno-arterial extracorporeal membrane oxygenation (VA ECMO), and 12% with single ventricle anatomy. Median age was 1.7 years (interquartile range 10 days–11.9 years). Median hospital stay was 51 days (interquartile range 34–91 days), and median extracorporeal life support duration was 94 hours (interquartile range 56–142 hours). The median Functional Status Scale at discharge was 8.0 (interquartile range 6.3–8.8). The mean change in Functional Status Scale from discharge to follow-up at 9 months (n = 37) was −0.8 [95% confidence interval (CI) −1.3 to −0.4, p < 0.001] and at 12 months (n = 34) was −1 (95% confidence interval −1.5 to −0.4, p < 0.001); the most improvement was in the feeding score. New morbidity (Functional Status Scale increase of ≥3) occurred in 10 children (24%) from admission to discharge. Children with new morbidity were more likely to be younger (p = 0.01), have an underlying genetic syndrome (p = 0.02), and demonstrate evidence of neurologic injury by electroencephalogram or imaging (p = 0.05).

In survivors of extracorporeal life support, the Functional Status Scale improved from discharge to 12-month follow-up, with the most improvement demonstrated in the feeding score.