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.

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.

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.

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.

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.

Determine whether weight gain velocity (g/day) 30 days after initiating feeds following cardiac surgery and other clinical outcomes improve in infants with single-ventricle physiology fed an exclusive human milk (EHM) diet with early fortification compared to non-protocolised “standard of care.”

This retrospective cohort study compares term infants with single-ventricle physiology who underwent neonatal surgical palliation. The retrospective control group (RCG) was fed according to non-protocolised standard of care at a single centre and was compared with infants in a previous protocolised multi-site randomised controlled trial assigned to either an EHM group or a control group (TCG). The primary outcome measure is weight gain velocity. Secondary outcomes include change in weight z-score, and incidence of feeding intolerance and necrotising enterocolitis.

We evaluated 45 surgically palliated neonates with single-ventricle physiology compared to the prior trial patients (EHM = 55, TCG = 52). Baseline demographics were similar between groups, except the RCG had fewer patients with hypoplastic left heart syndrome (51% vs. 77% vs. 84%, p = 0.0009). The RCG grew similarly to the TCG (7.5 g/day vs. 8.2 g/day), and both groups had significantly lower growth than the EHM group (12 g/day). Necrotising enterocolitis/suspected necrotising enterocolitis were similar in the RCG versus TCG but significantly higher in the RCG compared to the EHM group (20.5% vs. 3.6%, p = 0.033). Incidences of other morbidities were similar.

Neonates with single-ventricle physiology have improved short-term growth and decreased risk of necrotising enterocolitis or suspected necrotising enterocolitis when receiving an EHM diet after surgical palliation compared to non-protocolised feeding with bovine formula.

This study aimed to explore relationships between parental stress, coping, and outcomes for parents of infants with CHD, via observational approach reflecting domains of the Parental Stress and Resilience in CHD (PSRCHD) model.

Fifty-five parents of 45 infants with CHD completed questionnaires with measures of parental stress, Problem-Focused Coping (PFC), Emotion-Focused Coping (EFC), Avoidant Coping (AC), mental health (symptoms of anxiety and symptoms of depression), post-traumatic growth (PTG) and quality of life (QoL). Demographic and infant clinical data were obtained.

Parental stress showed significant small to medium positive correlations with MH and PTG, but no significant correlations with QoL. EFC and AC showed significant small to medium positive correlations with MH, and medium negative correlations with parental QoL. EFC and PFC had significant small to medium correlations with PTG. PFC and AC had significant small to medium correlations with infant QoL. Hierarchical multiple regression analyses indicated that parental symptoms of anxiety, PTG, parental QoL, infant QoL were significantly predicted by models comprising of parental stress, coping styles, and clinical controls (adjusted R2 = 13.0–47.9%, p range < 0.001–.048), with results for parental symptoms of depression falling marginally above significance (adjusted R2 = 12.3%, p = .056).

Parental stress, coping styles, and length of hospital stay are related to psychological outcomes in parents of infants with CHD. Future research may use the PSRCHD framework to assess mechanisms underlying CHD parents’ stress and coping experiences and investigate longitudinal relationships between parental factors and parent and child outcomes.

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.

The Duke Activity Status Index is used to assess an individual patient’s perception of their fitness abilities. It has been validated and shown to predict actual fitness in adults but has been studied less in the paediatric population, specifically those with heart disease. This study aims to assess if the Duke Activity Status Index is associated with measured markers of physical fitness in adolescents and young adults with heart disease.

This retrospective single-centre cohort study includes patients who completed a minimum of 12 weeks of cardiac rehabilitation between 2016 and 2022. Cardiac rehabilitation outcomes included physical, performance, and psychosocial measures. A comparison between serial testing was performed using a paired t-test. Univariable and multivariable analyses for Duke Activity Status Index were performed. Data are reported as median [interquartile range].

Of the 118 participants (20 years-old [13.9–22.5], 53% male), 33 (28%) completed at least 12 weeks of cardiac rehabilitation. Median peak oxygen consumption was 60.1% predicted [49–72.8%], and Duke Activity Status Index was 32.6 [21.5–48.8]. On Pearson’s correlation assessing the Duke Activity Status Index, there were significant associations with % predicted peak oxygen consumption (r = 0.49, p < 0.0001), 6-minute walk distance (r = 0.45, p < 0.0001), Duke Activity Status Index metabolic equivalents (r = 0.45, p < 0.0001), and dominant hand grip (r = 0.48, p < 0.0001). In multivariable analysis, the % predicted peak oxygen consumption (r = 0.40, p = 0.005) and dominant hand grip (r = 0.37, p = 0.005) remained statistically significant.

Duke Activity Status Index is associated with measures of physical fitness in paediatric and young adults with heart disease who complete a cardiac rehabilitation program.