Haemodynamic instability is common after surgical repair of CHDs in infants and children. Monitoring cardiac output in addition to traditional circulation parameters could improve the postoperative care of these patients. Echocardiography and transpulmonary thermodilution are the two most common methods for measuring cardiac output in infants.

To compare the results of cardiac output measurements using echocardiography and a transpulmonary thermodilution setup after paediatric cardiac surgery.

Forty children, scheduled for elective repair of a ventricular septal defect or of an atrio-ventricular septal defect using cardiopulmonary bypass, were enrolled in this prospective, observational study. Cardiac output was simultaneously measured using echocardiography and a commercially available transpulmonary thermodilution method (PiCCO™) at 18 h after the end of surgery.

At 18 h after surgery, PiCCO™ gave a mean of 3.0% higher cardiac output than echocardiography. This difference was not statistically significant. 95% of the observations fell within –50.0 to 82.6%.

The methods were found to have a good agreement on average, with no statistically significant difference between them. However, the spread of the results was large. It is questionable whether the methods can be used interchangeably in clinical practice.

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.

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.

Pain management for infants undergoing cardiothoracic surgery primarily utilises opioid analgesics. There is a paucity of data available for the use of non-steroidal anti-inflammatory medications such as ketorolac in this patient population.

This retrospective study evaluated patients between 30 days and 6 months undergoing cardiothoracic surgery. The primary endpoint evaluates ketorolac on reducing post-operative opioid use.

Of 243 evaluated patient, 145 met inclusion. Baseline demographics were similar amongst the cohorts. Patients administered ketorolac used less cumulative opiates, in morphine milligram equivalents, for post-op days (POD) 1–3 after surgery compared to patients not receiving ketorolac (9.47 versus 12.68; p = 0.002). The no-ketorolac group required more opiates on POD 1 (10.9 versus 5; p < 0.001) and POD 2 (4.2 versus 2.5; p = 0.006) with no difference found on POD 3 (2 versus 1.6; p = 0.2). There was a mean increase from baseline to highest serum creatinine level on POD 1–3 in the no-ketorolac group compared to the ketorolac group (0.15 versus 0.09 mg/dL; p < 0.014), with no difference in stage 1 or stage 2 acute kidney injury. There were no differences in average chest tube output in mL/kg/day (0.24 versus 0.32; p = 0.569) or need for transfusion (36% versus 24%; p = 0.125), respectively.

Scheduled administration of ketorolac after cardiothoracic surgery resulted in a significant reduction in opioid exposure, with no difference in rates of acute kidney injury or bleeding.

CHD is a unique group of medical pathologies. Literature worldwide reports significant decrements in the case volume of patients with these conditions due to the recent global pandemic of coronavirus disease 2019. The only centre providing congenital cardiac care for Lithuanian population is in a hospital which was the main medical institution for the sickest coronavirus disease 2019 patients. Hence, this centre had to maintain its service alongside the mobilisation of resources to tackle the crisis.

To evaluate the effect of the pandemic on the service of congenital heart surgery in Lithuania.

The activity of a single centre providing congenital heart care working in a main coronavirus 2019 pandemic hospital during the pandemic was analysed and compared to a matched period of pre-pandemic activity.

The number of admitted patients was similar during both pre-pandemic and pandemic periods. During the pandemic period, younger patients were more often operated as urgent cases. Their postoperative length of stay was longer. However, there were no differences in early postoperative mortality between the two groups.

It was possible to maintain an accessible and high-quality specialised congenital cardiac care for various age patients during global pandemic events, while working in the main pandemic hospital.

Diaphragm paralysis is a well-known complication following surgery for CHDs, which increases morbidity, mortality, and length of hospital stay as well as costs. Herein, we present our experience with diaphragm plication following paralysis of the phrenic nerve encountered after paediatric cardiac surgery.

This study retrospectively reviewed the medical records of 23 diaphragm plications in 20 patients who underwent paediatric cardiac surgery between January 2012 and January 2022. The patients were carefully selected based on aetiology and a combination of clinical manifestation and chest imaging characteristics including chest X-ray, ultrasonography, and fluoroscopy.

Twenty-three successful plications were performed in 20 patients (15 males and 5 females) out of a total of 1938 operations performed in our centre. Mean age and body weight were 18.2 ± 17.1 months and 8.3 ± 3.7 kg, respectively. The period between the cardiac surgery and diaphragmatic plication was 18.7 ± 15.1 days. The highest incidence of diaphragm paralysis was encountered in systemic to pulmonary artery shunt patients with 7 out of 152 patients (4.6%). Any mortality was not encountered during a mean follow-up period of 4.3 ± 2.6 years.

Early results of plication of the diaphragm following phrenic nerve palsy in symptomatic patients who underwent paediatric cardiac surgery are encouraging. Evaluation of the diaphragmatic function should be a routine part of post-operative echocardiography. Diaphragm paralysis may be a consequence of dissection, contusion, stretching, and thermal injury both in terms of hypothermia and hyperthermia.

We aimed to identify factors independently associated with the need for inotropic support for low cardiac output or haemodynamic instability after pulmonary artery banding surgery for CHD.

We performed a retrospective chart review of all neonates and infants who underwent pulmonary banding between January 2016 and June 2019 at our institution. Bivariate and multivariable analyses were performed to identify factors independently associated with the use of post-operative inotropic support, defined as the initiation of inotropic infusion(s) for depressed myocardial function, hypotension, or compromised perfusion within 24 hours of pulmonary artery banding.

We reviewed 61 patients. Median age at surgery was 10 days (25%,75%:7,30). Cardiac anatomy was biventricular in 38 patients (62%), hypoplastic right ventricle in 14 patients (23%), and hypoplastic left ventricle in 9 patients (15%). Inotropic support was implemented in 30 patients (49%). Baseline characteristics of patients who received inotropic support, including ventricular anatomy and pre-operative ventricular function, were not statistically different from the rest of the cohort. Patients who received inotropic support, however, were exposed to larger cumulative doses of ketamine intraoperatively – median 4.0 mg/kg (25%,75%:2.8,5.9) versus 1.8 mg/kg (25%,75%:0.9,4.5), p < 0.001. In a multivariable model, cumulative ketamine dose greater than 2.5mg/kg was associated with post-operative inotropic support (odds ratio 5.5; 95% confidence interval: 1.7,17.8), independent of total surgery time.

Inotropic support was administered in approximately half of patients who underwent pulmonary artery banding and more commonly occurred in patients who received higher cumulative doses of ketamine intraoperatively, independent of the duration of surgery.

Despite advances in medical care, we still come across pregnancy in Eisenmenger syndrome. Eisenmenger syndrome represents the severe end of the spectrum for disease in pulmonary artery hypertension associated with CHD. Due to very high maternal and perinatal morbidity and mortality, pregnancy is contraindicated among these women. Current guidelines also recommend that the women who become pregnant should opt for early termination of pregnancy. Here, we present a case series of 11 women of Eisenmenger syndrome and their pregnancy outcome.

It was a retrospective analysis of 12 pregnancies among 11 women with Eisenmenger syndrome who were managed in a tertiary care referral centre of Northern India.

The mean age of these women was 28 ± 4 years (range 22 to 36 years). Almost 80% of them (9/11) were diagnosed with Eisenmenger syndrome during pregnancy. The commonest cardiac lesion was Ventricular Septal defect (54.5%) followed by Atrial Septal defect (27.3%) and Patent Ductus arteriosus (9.1%). Only three women opted for medical termination of pregnancy, rest eight continued the pregnancy or presented late. Pregnancy complications found include pre-eclampsia (50%), abruption (22%), and fetal growth retardation (62.5%). There were three maternal deaths (mortality rate 27%) in postpartum period.

This case series highlights the delay in diagnosis and treatment of CHD despite improvement in medical care. Women with Eisenmenger syndrome require effective contraception, preconceptional counselling, early termination of pregnancy, and multidisciplinary care.

This study investigates the hygiene standards in the context of the COVID-19 pandemic and their impact on the perioperative incidence of human metapneumovirus as well as the typical symptom burden of human metapneumovirus-infected children with CHDs.

Between March 2018 and July 2021, all patients of a cardiac paediatric ICU of a German university hospital were included in this retrospective cohort analysis.

A total of 589 patients with CHD were included in the analysis. Three hundred and fifty-two patients (148 females and 204 males) were admitted before the introduction of social distancing and face masks between March 2018 and 15 April 2020 (cohort A). Two hundred and thirty-seven patients (118 females and 119 males) were admitted after the introduction between April 16 and July 2021 (cohort B). In cohort A, human metapneumovirus was detected in 11 out of 352 patients (3.1%) during their stay at cardiac paediatric ICU. In cohort B, one patient out of 237 (0.4%) tested positive for human metapneumovirus. Patients who tested positive for human metapneumovirus stayed in cardiac paediatric ICU for a median of 17.5 days (range, 2–45 days). Patients without a detected human metapneumovirus infection stayed in the cardiac paediatric ICU for a median of 4 days (range, 0.5–114 days). Nine out of 12 (75%) human metapneumovirus-positive patients showed atelectasis.

Perioperative human metapneumovirus infections prolong cardiac paediatric ICU stay in children with CHD. In affected patients, pulmonary impairment with typical symptoms appears. Under certain circumstances, a complication-rich perioperative infection with human metapneumovirus could be prevented in paediatric cardiac high-risk patients by prophylactic hygiene intervention.

Parents of infants with complex CHDs often describe their infants as especially fussy, irritable, and difficult to sooth, which together with the illness caretaking demands add to their stress. Little is known about how the behavioural style or temperament in the early months after discharge relates to parental quality of life. This study aimed to explore the associations between early infant temperament characteristics and parental quality of life in parents of infants with complex CHD.

This descriptive, cross-sectional study, utilised data collected in a previously described multisite randomised clinical trial in the United States. Multivariable linear regression models were used to examine the associations of interest.

Results demonstrated negative significant associations between most infant temperament subscales and parental quality of life. Higher scores on the Activity (β = −3.03, p = 0.021), Approach (β = −1.05, p = 0.021), Adaptability (β = −3.47, p = 0.004), Intensity (β = −2.78, p = 0.008), Mood (β = −4.65, p < 0.001), and Distractibility (β = −3.36, p = 0.007 were all significantly associated with lower parental quality of life scores, adjusting for parental dyadic adjustment, insurance type, number of medications, and number of unscheduled cardiologist visits.

Parental perceptions of infant’s difficult behavioural style or temperament characteristics appear to be associated with poorer quality of life in parents of infants with complex CHD post-cardiac surgery. Findings can be used in the screening process of families at potential risk of increased stress and poor illness adaptation and in the design of interventions to target parental mental health in this vulnerable patient population.

The effects of alpha-blockade on haemodynamics during and following congenital heart surgery are well documented, but data on patient outcomes, mortality, and hospital charges are limited. The purpose of this study was to characterise the use of alpha-blockade during congenital heart surgery admissions and to determine its association with common clinical outcomes.

A cross-sectional study was conducted using the Pediatric Health Information System database. De-identified data for patients under 18 years of age with a cardiac diagnosis who underwent congenital heart surgery were obtained from 2004 to 2015. Patients were subdivided on the basis of receiving alpha-blockade with either phenoxybenzamine or phentolamine during admission or not. Continuous and categorical variables were analysed using Mann−Whitney U-tests and Fisher exact tests, respectively. Characteristics between subgroups were compared using univariate analysis. Regression analyses were conducted to determine the impact of alpha-blockade on ICU length of stay, hospital length of stay, billed charges, and mortality.

Of the 81,313 admissions, 4309 (5.3%) utilised alpha-blockade. Phentolamine was utilised in 4290 admissions. In univariate analysis, ICU length of stay, total length of stay, inpatient mortality, and billed charges were all significantly higher in the alpha-blockade admissions. However, regression analyses demonstrated that other factors were behind these increased. Alpha-blockade was significantly, independently associated with a 1.5 days reduction in ICU length of stay (p < 0.01) and a 3.5 days reduction in total length of stay (p < 0.01). Alpha-blockade was significantly, independently associated with a reduction in mortality (odds ratio 0.8, 95% confidence interval 0.7−0.9). Alpha-blockade was not independently associated with any significant change in billed charges.

Alpha-blockade is used in a subset of paediatric cardiac surgeries and is independently associated with significant reductions in ICU length of stay, hospital length of stay, and mortality without significantly altering billed charges.

In patients with right ventricular diastolic dysfunction after complete repair of tetralogy of Fallot, some employ the use of beta-blockade. The theoretical benefit of this therapy is felt to be one of the two: 1) reduction in heart rate with subsequent increase in diastolic filling time and stroke volume; 2) halting or reversal of right ventricular remodelling. This study aimed to characterise the use of beta-blockade in paediatric admissions with complete repair of tetralogy of Fallot and characterise the effects of beta-blockade on admission characteristics.

Admissions from 2004 to 2015 in the Pediatric Health Information System database with complete repair of tetralogy of Fallot were identified. Characteristics between admissions with and without beta-blockade were compared by univariate analysis. Next, regression analyses were conducted to determine the independent association of beta-blockade on length of admission, billed charges, cardiac arrest, and inpatient mortality while controlling for demographic variables and comorbidities.

A total of 3594 admissions were included in the final analyses. Of these, 371 employed beta-blockade. Admissions with beta-blockade were more likely to have heart failure and tachyarrhythmias. These admissions also tended to be longer by univariate analysis. Regression analyses demonstrated that beta-blockade was independently associated with a 2.8-day increase in length of stay and no statistically significant change in billed charges, cardiac arrest, or inpatient mortality.

Beta-blockade after complete repair of tetralogy of Fallot is associated with a longer length of stay but did not statistically significantly impact billed charges, cardiac arrest, or inpatient mortality.

Young adults with heart disease constitute a growing group with the risk of cognitive and physical impairment. The knowledge of their academic performance and mental and physical health is, however, scant. This study aimed to compare young adults with CHDs or arrhythmia with their peers.

Information on physical health (Somatic Symptom Scale-8), mental health problems (Hopkins Symptoms Checklist-25), quality of life (Satisfaction With Life Scale), physical activity, and academic performance was collected online in a national cross-sectional survey in Norway among students in higher education (the SHoT2018 study).

Among 50,054 students, 172 (0.34%) reported CHD and 132 (0.26%) arrhythmias. Students reporting arrhythmias scored significantly higher than the control group on somatic symptoms (OR = 2.3 (95% CI: 1.62–3.27)), anxiety (OR = 1.60 (1.08–2.37)), depression (OR = 1.49 (1.05–2.11)), self-harm, and suicide attempt (OR = 2.72 (1.56–4.75)), and lower quality of life (OR 1.64 (1.16–2.32)) and more loneliness (OR = 1.99 (1.28–3.10)) compared to participants without heart disease. Participants with CHD reported an increased somatic symptom burden (OR = 1.58 (1.16–2.16)). Despite a tendency to a higher score, this group did not differ significantly from the control group on anxiety or depression, quality of life, or loneliness. However, the risk of self-harm thoughts and suicidality was significantly increased (OR for suicide attempt 2.22 (1.3–3.77)). There was no difference between the groups on academic performance.

Although Norwegian students with heart disease reported more somatic symptoms, their academic progress was not reduced compared to students without heart disease. Students with CHD or arrhythmias showed an increased risk of self-harm thoughts and suicidality.

Parents of infants with CHDs experience increased parenting stress compared to the general population, potentially interfering with parenting practices and bear adverse family outcomes. The changes in stress over the critical period of infancy have yet to be studied. The current study aimed to compare parenting stress changes over time between parents of infants with CHDs and parents of healthy infants during the first year of infants’ life.

Data from a larger prospective cohort study were longitudinally analysed using mixed-effects multivariable regression modelling. Sample included mothers of 129 infants with complex cardiac defects and healthy infants, recruited from the cardiac ICU of a large cardiac centre and outpatient paediatric practices in Northeastern America. Outcome was measured over four visits via the Parenting Stress Index Long Form.

Stress in the cardiac group has significantly decreased over time on the Parent Domain (p = 0.025), and stress in the healthy group has significantly increased over time on the Child Domain (p = 0.033). Parenting stress trajectories demonstrated significant differences between groups on the Parent Domain (p = 0.026) and on the Total Stress (p = 0.039) subscales.

Parenting stress in the paediatric cardiac population changes over time and differs from stress experienced by parents of healthy infants. Findings highlight stressful periods that may be potentially risky for parents of infants with CHDs and introduce additional illness-related and psychosocial/familial aspects to the parenting stress concept.

Cardiac dysfunction, arrhythmia, and hepatic fibrosis are well-known complications after right heart bypass surgery in patients with single-ventricle physiology. However, little is known about coronary arterial fistulae, and only a few reports have been published. This study aimed to elucidate the clinical characteristics of these rare coronary arterial fistulae that developed as complications in cases of single-ventricle physiology after right heart bypass surgery.

We retrospectively investigated the clinical features and courses of patients who developed acquired and progressive coronary arterial fistulae after right heart bypass surgery in our hospital.

We identified three cases of coronary arterial fistulae out of 21 patients who underwent right heart bypass surgery. All three cases underwent cardiac catheterisation for post-operative evaluation and were administered pulmonary vasodilators of phosphodiesterase type V inhibitors, antiplatelet, anticoagulation, and diuretics. Moreover, they had common clinical features such as right-dominant single ventricle and long-term exposure to chronic hypoxia. Serial angiograms revealed acquired and progressive coronary arterial fistulae. In addition, coronary arterial fistulae contributed to their symptoms of heart failure.

Patients with chronic hypoxia and dominant right ventricle, who are treated with phosphodiesterase type V inhibitors, should be followed up after right heart bypass surgery to monitor the possible development of coronary arterial fistulae. Moreover, the indication for pulmonary vasodilators in single-ventricle physiology after right heart bypass surgery should be optimised to avoid adverse effects.

CHDs can be complicated by renal injury which worsens morbidity and mortality. Urinary neutrophil gelatinase-associated lipocalin, a sensitive and specific biomarker of renal tubular injury, has not been studied in children with uncorrected CHDs. This study evaluated renal injury in children with uncorrected CHDs using this biomarker.

The patients were children with uncorrected CHDs with significant shunt confirmed on echocardiogram with normal renal ultrasound scan, in the paediatric cardiology clinic of a tertiary hospital. The controls were age-matched healthy children recruited from general practice clinics. Information on bio-data and socio-demographics were collected and urine was obtained for measurement of urinary neutrophil gelatinase-associated lipocalin levels.

A total of 65 children with uncorrected CHDs aged 2 to 204 months were recruited. Thirty-one (47.7%) were males while 36 (55.4%) had acyanotic CHDs. The median urinary neutrophil gelatinase-associated lipocalin level of patients of 26.10 ng/ml was significantly higher than controls of 16.90 ng/ml (U = 1624.50, p = 0.023). The median urinary neutrophil gelatinase-associated lipocalin level of patients with cyanotic and acyanotic CHDs were 30.2 ng/ml and 22.60 ng/ml respectively; (Mann–Whitney U = 368.50, p = 0.116). The prevalence of renal injury using 95th percentile cut-off value of urinary neutrophil gelatinase-associated lipocalin was 16.9%. Median age of patients with renalinjury was 16 (4–44) months.

Children with uncorrected CHDs have renal injury detected as early as infancy. The use of urinary neutrophil gelatinase-associated lipocalin in early detection of renal injury in these children may enhance early intervention and resultant prevention of morbidity and reduction in mortality.