In resource limited settings, children with cardiac disease present late, have poor outcomes and access to paediatric cardiology programmes is limited. Cardiac point of care ultrasound was introduced at several Médecins Sans Frontières sites to facilitate cardiopulmonary assessment. We describe the spectrum of disease, case management and outcomes of cases reviewed over the Telemedicine platform.

Previously ultrasound naïve, remotely placed clinical teams received ultrasound training on focussed image acquisition. The Médecins Sans Frontières Telemedicine platform was utilised for remote case and imaging review to diagnose congenital and acquired heart disease and guide management supported by a remotely situated paediatric cardiologist.

Two-hundred thirty-three cases were reviewed between 2016 and 2018. Of 191 who underwent focussed cardiac ultrasound, diagnoses included atrial and ventricular septal defects 11%, atrioventricular septal defects 7%, Tetralogy of Fallot 9%, cardiomyopathy/myocarditis 8%, rheumatic heart disease 8%, isolated pericardiac effusion 6%, complex congenital heart disease 4% and multiple other diagnoses in 15%. In 17%, there was no identifiable abnormality while 15% had inadequate imaging to make a diagnosis. Cardiologist involvement led to management changes in 75% of cases with a diagnosis. Mortality in the entire group was disproportionately higher among neonates (38%, 11/29) and infants (20%, 16/81). There was good agreement on independent review of selected cases between two independent paediatric cardiologists.

Cardiac point of care ultrasound performed by remote clinical teams facilitated diagnosis and influenced management in cases reviewed over a Telemedicine platform. This is a feasible method to support clinical care in resource limited settings.

Neonatal coarctation has to be diagnosed and treated urgently. Actually, the surgical treatment is the main option. The coarctation dilatation is usually achieved under fluoroscopy guidance whenever indicated. Balloon angioplasty could be an alternative approach or transient measure in difficult cases with cardiogenic shock or severe cardiac insufficiency.

In the reported case, we prove and discuss the major role of transthoracic echocardiography, which is used solely to guide the coarctation dilatation in neonate environment.

The reported case aims to assess the safety and the efficiency of two-dimensional TEE to guide the dilatation of aortic coarctation in neonate.

We describe successful dilatation of neonatal coarctation done exclusively using echocardiography in neonatal ICU at the bed. The procedure duration was 40 minutes (from the puncture to sheath removal). The coarctation was diagnosed easily and well described using TTE with good image quality obtained from supra-sternal plane and upper and left lateral view. TayShak balloon measuring 6 and 8 mm were used with a 0.018 French guided exchange wire.

Complete relief of the coarctation was checked by TTE without recording any complication. The follow-up in the third month (the submission time of this manuscript) showed very good results without requiring any surgical intervention or additional restenosis.

Our initial experience confirmed the safety and efficiency of coarctation dilatation using TTE as the only guidance tool at the bed in neonatal stage, especially in a case presenting severe metabolic and cardiac failure. This report suggests and encourages other potential applications in neonatology intensive care.

The prevalence of congenital heart disease (CHD) in adults is rising necessitating a greater understanding of acquired diseases such as community-acquired pneumonia, which remains a leading cause of age-related mortality and morbidity in the general population. We hypothesise that the CHD population, given cardiopulmonary mechanics and altered immune function, bears a uniquely high risk for pneumonia-related hospitalisations and mortality.

A countrywide cohort study was performed to calculate the relative risk and cumulative incidence of pneumonia hospitalisations and resultant 30-day mortality amongst the adult CHD population, matched 1:10 with non-CHD persons by gender, age, and adjusted for comorbidities. Cox proportional hazard regression quantified the impact of CHD severity and extracardiac defects.

The CHD cohort includes 17,162 adults. The majority demonstrate mild/moderate CHD complexity. The cumulative incidence of pneumonia hospitalisation was higher for adults with CHD (hazard ratio 1.90; 95% confidence interval: 1.74–2.06) than the comparison cohort. This risk was increased for those with extracardiac defects or a syndrome (hazard ratio: 4.34; 95% confidence interval: 3.39–5.54). Additionally, CHD individuals with severe/univentricular subtypes demonstrate a heightened risk compared to the non-CHD cohort (hazard ratio: 2.35; 95% confidence interval: 1.94–2.84), as well as compared to those with mild/moderate CHD (hazard ratio: 1.28; 95% confidence interval: 1.07–1.53). In addition, pneumonia hospitalisation mortality was elevated above the comparison population with a 30-day mortality rate ratio of 1.31 (95% confidence interval: 1.00–1.73).

Adults with CHD are at elevated risk of pneumonia hospitalisations and pneumonia-associated mortality. This risk is further elevated in those with severe CHD and extracardiac defects.

Treatment of hypoplastic left heart syndrome varies across institutions. This study examined the impact of introducing a standardised programme.

This retrospective cohort study evaluated the effects of a comprehensive strategy on 1-year transplant-free survival with preserved ventricular and atrioventricular valve (AVV) function following a Norwood operation. This strategy included standardised operative and perioperative management and dedicated interstage monitoring. The post-implementation cohort (C2) was compared to historic controls (C1). Outcomes were assessed using logistic regression and Kaplan–Meier analysis.

The study included 105 patients, 76 in C1 and 29 in C2. Groups had similar baseline characteristics, including percentage with preserved ventricular (96% C1 versus 100% C2, p = 0.28) and AVV function (97% C1 versus 93% C2, p = 0.31). Perioperatively, C2 had higher indexed oxygen delivery (348 ± 67 ml/minute/m2 C1 versus 402 ± 102ml/minute/m2 C2, p = 0.015) and lower renal injury (47% C1 versus 3% C2, p = 0.004). The primary outcome was similar in both groups (49% C1 and 52% C2, p = 0.78), with comparable rates of death and transplantation (36% C1 versus 38% C2, p = 0.89) and ventricular (2% C1 versus 0% C2, p = 0.53) and AVV dysfunction (11% C1 versus 11% C2, p = 0.96) at 1-year. When accounting for cohort and 100-day freedom from hospitalisation, female gender (OR 3.7, p = 0.01) increased and ventricular dysfunction (OR 0.21, p = 0.02) and CPR (OR 0.11, p = 0.002) or ECMO use (OR 0.15, p = 001) decreased the likelihood of 1-year transplant-free survival.

Standardised perioperative management was not associated with improved 1-year transplant-free survival. Post-operative ventricular or AVV dysfunction was the strongest predictor of 1-year mortality.

To assess the role of right ventricular outflow tract stenting as the primary intervention in Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries.

The management of a subset of infants with Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries requires a staged approach including rehabilitation of diminutive native pulmonary arteries, conventionally using an aortopulmonary shunt. We share our experience of pulmonary artery rehabilitation with right ventricular outflow tract stenting.

Retrospective review of all patients with Tetralogy of Fallot with pulmonary stenosis who underwent right ventricular outflow tract stenting as primary intervention over an 8-year period.

Ten patients underwent right ventricular outflow tract stent insertion at a median age of 61 days (interquartile range (IQR) 8.3–155 days). Median weight at stent deployment was 4.2 kg (IQR 3.2–5.7 kg). Oxygen saturations improved from a median of 79% (IQR 76–80%) to 92% (IQR 90–95%), p = 0.012. The median right and left pulmonary artery z score increased from −3.51 (IQR −4.59 to −2.80) and −2.07 (IQR −3.72 to 0.15) to a median of −1.17 (IQR −2.26 to 0.16) p < 0.05, and 0.24 (IQR −1.09 to 1.84) p < 0.05, respectively, at subsequent angiogram. Nine patients underwent further catheterisation. Four patients underwent complete anatomical repair. Only one patient required unifocalisation, as most patients had a native supply to all-important lung segments.

Right ventricular outflow tract stenting is a useful procedure in the subset of patients with Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries, where native pulmonary arterial growth is required to facilitate repair.

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.

After congenital heart surgery, some patients may need long-term mechanical ventilation because of chronic respiratory failure. In this study, we analysed outcomes of the patients who need tracheostomy and home mechanical ventilation.

Amongst 1343 patients who underwent congenital heart surgery between January, 2014 and June, 2018, 45 needed tracheostomy and HMV. The median age of these patients was 6.4 months (12 days–6.5 years). Nineteen patients underwent palliation while 26 patients underwent total repair. Post-operative diaphragm plication was performed in five patients (11%). Median duration of mechanical ventilation before tracheostomy was 32 days (8–154 days). The patients were followed up with their home ventilators in ward and at home. Mean follow-up time was 36.24 ± 11.61 months.

The median duration of ICU stay after tracheostomy was 27 days (range 2–93 days). Follow-up time in ward was median 30 days (2–156 days). A total of 12 patients (26.6%) were separated from the ventilator and underwent decannulation during hospital stay. Thirty-two patients (71.1%) were discharged home with home ventilator support. Of them, 15 patients (46.9%) were separated from the respiratory support in median of 6 weeks (1 week–11 months) and decannulations were performed. Total mortality was 31.1%. in which four patients are still HMV dependent. There was no significant difference for decannulation between total repair and palliation patients.

HMV via tracheostomy is a useful option for the treatment of children who are dependent on long-term ventilation after congenital heart surgery although there are potential risks.

Vasovagal syncope is the most common cause of syncope in childhood and its treatment is not at a satisfactory level yet. We aimed to investigate patients who were diagnosed with vasovagal syncope, did not benefit from conventional treatment, received midodrine treatment, and to evaluate their response to midodrine treatment.

Files of 24 patients who were diagnosed with recurrent vasovagal syncope, did not benefit from non-pharmacological treatments, and received midodrine treatment during June 2017–October 2019 were retrospectively analysed.

In total, 24 patients received a treatment dose of midodrine at 5 mg/day (2.5 mg BID) included in the study. The mean number of syncope was 5.75 ± 2.67 prior to treatment. Following treatment, the mean number of syncope was 0.42 ± 0.89. It was observed that syncope episodes did not recur in 17 patients, but it recurred in 4 out of 7 patients in the first 3 months of the treatment and did not recur in the following months. The episodes improved in two patients with an increase in the treatment dose, but the syncope episodes continued in only one patient.

It was concluded that midodrine treatment was effective and safe in adolescents with recurrent vasovagal syncope.

Young adults with congenital heart disease (CHD) are increasing in number with an increased risk for acute kidney injury. Little is known concerning the impact of non-recovery of kidney function for these patients. Therefore, we sought to explore the rates of acute kidney disease, persistent renal dysfunction, and their associations with adverse outcomes in young adults with CHD.

This is a single-centre retrospective study including all patients at the ages of 18–40 with CHD who were admitted to an intensive care unit between 2010 and 2014. Patients with a creatinine ≥ 1.5 times the baseline at the time of hospital discharge were deemed to have persistent renal dysfunction, while acute kidney disease was defined as a creatinine ≥ 1.5 times the baseline 7–28 days after a diagnosis of acute kidney injury. Outcomes of death at 5 years and length of hospital stay were examined using multivariable logistic regression and negative binomial regression, respectively.

Of the (89/195) 45.6% of patients with acute kidney injury, 33.7% had persistent renal dysfunction and 23.6% met the criteria for acute kidney disease. Persistent renal dysfunction [odds ratio (OR), 3.27; 95% confidence interval (CI): 1.15–9.29] and acute kidney disease (OR: 11.79; 95% CI: 3.75–39.09) were independently associated with mortality at 5 years. Persistent renal dysfunction was associated with a longer duration of hospital stay (Incidence Rate Ratio: 1.96; 95% CI: 1.53–2.51).

In young adults with CHD, acute kidney injury was common and persistent renal dysfunction, as well as acute kidney disease, were associated with increased mortality and length of hospitalisation.

Understanding the impact of the COVID-19 pandemic on paediatric non-COVID-19-related care, as well as patient and caregiver concerns and stressors, is critical for informing healthcare delivery. It was hypothesised that high care disruptions and psychological stress would be observed among paediatric and adult CHD patients in the early phase of the pandemic.

A cross-sectional, international, electronic survey study was completed. Eligible participants included parents of children with acquired or CHD, adults with CHD, or caregivers of adults with CHD.

A total of 1220 participants from 25 countries completed the survey from 16 April to 4 May, 2020. Cardiac care disruption was significant with 38% reporting delays in pre-pandemic scheduled cardiac surgeries and 46% experiencing postponed cardiac clinic visits. The majority of respondents (75%) endorsed moderate to high concern about the patient with heart disease becoming ill from COVID-19. Worry about returning for in-person care was significantly greater than worry of harm to patient due to postponed care. Clinically significant psychological stress was high across the sample including children (50%), adults with CHD (42%), and caregivers (42%).

The early phase of the COVID-19 pandemic contributed to considerable disruptions in cardiac care for patients with paediatric and adult CHD. COVID-19-related fears are notable with potential to impact willingness to return to in-person care. Psychological stress is also very high necessitating intervention. Further study of the impact of delays in care on clinical outcomes is warranted.

There is limited data describing the characteristics of paediatric post-operative cardiac surgery patients who develop pneumothoraces after chest tube removal. Patient management after chest tube removal is not standardised across paediatric cardiac surgery programmes. The purposes of this study were to describe the frequency of pneumothorax after chest tube removal in paediatric post-operative cardiac surgical patients and to describe the patient and clinical characteristics of those patients who developed a clinically significant pneumothorax requiring intervention.

A single-institution retrospective descriptive study (1 January, 2010–31 December, 2018) was utilised to review 11,651 paediatric post-operative cardiac surgical patients from newborn to 18 years old.

Twenty-five patients were diagnosed with a pneumothorax by chest radiograph following chest tube removal (0.2%). Of these 25 patients, 15 (1.6%) had a clinically significant pneumothorax and 8 (53%) did not demonstrate a change in baseline clinical status or require an increase in supplemental oxygen, 14 (93%) required an intervention, 9 (60%) were <1 year of age, 4 (27%) had single-ventricle physiology, and 5 (33%) had other non-cardiac anomalies/genetic syndromes.

In our cohort of patients, we confirmed the incidence of pneumothorax after chest tube removal is low in paediatric post-operative cardiac surgery patients. This population does not always exhibit changes in clinical status despite having clinically significant pneumothoraces. We suggest the development of criteria, based on clinical characteristics, for patients who are at increased risk of developing a pneumothorax and would require a routine chest radiograph following chest tube removal.