The clinical effects of CHD can occur during the neonatal period, childhood, adolescent, and even adulthood. Some CHD in the adult population have indications for surgical management.

The objective of this study was to review the role of humanitarian cardiac surgery missions in the surgical management of CHD in the adult population in a developing country.

Over a 5.5-year period – June, 2003, February, 2013–October, 2017 – five different humanitarian cardiac surgery teams visited National Cardiothoracic Center of Excellence, Nigeria. During the period, they operated on adults with CHD. A retrospective study of the patients treated was performed using data obtained from our Hospital Information Technology Department. The demography of the patients, types of CHD, operative modalities, as well as the outcome was analysed using Microsoft Excel. The results were presented in arithmetic of percentages using tables.

During the period, a total of 18 CHD patients were treated.

A 22q11 chromosome deletion is common in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals. We sought to determine whether 22q11 chromosome deletion is associated with increased postoperative morbidity after unifocalisation surgery.

We included all patients with this diagnosis undergoing primary or revision unifocalisation ± ventricular septal defect closure at our institution from 2008 to 2016, and we excluded patients with unknown 22q11 status. Demographic and surgical data were collected. We compared outcomes between those with 22q11 chromosome deletion and those without using non-parametric analysis.

We included 180 patients, 41% of whom were documented to have a chromosome 22q11 deletion. Complete unifocalisation was performed in all patients, and intracardiac repair was performed with similar frequency regardless of 22q11 chromosome status. Duration of mechanical ventilation was longer in 22q11 deletion patients. This difference remained significant after adjustment for delayed sternal closure and/or intracardiac repair. Duration of ICU stay was longer in patients with 22q11 deletion, although no longer significant when adjusted for delayed sternal closure and intracardiac repair. Finally, length of hospital stay was longer in 22q11-deleted patients, but this difference was not significant on unadjusted or adjusted analysis.

Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals and 22q11 deletion are at risk for greater prolonged mechanical ventilation after unifocalisation surgery. Careful attention should be given to the co-morbidities of this population in the perioperative period to mitigate risks that may complicate the postoperative course.

Aspirin resistance has been reported in up to 80% of children with cardiovascular defects undergoing surgery. Because of a patient who had embolic stroke while on therapeutic aspirin dose but in whom aspirin resistance was present on his thromboelastography platelet mapping, we chose to obtain thromboelastography platelet mapping on cardiac patients on aspirin to assess their risk.

This study evaluates aspirin resistance noted in these patients and their characteristics.

This is a retrospective study of 25 patients taking aspirin for a month at therapeutic dose. In total, 11 female patients were enrolled. Ages in all subjects were 5 months to 27 years. A total of 19 patients had a Fontan surgery. Three had a cavopulomanary anastomosis, one had a hybrid procedure, and two had coronary anomalies. Compliance was assessed at the time of the clinic visit. Aspirin resistance was defined as platelet inhibition below 50%. Variables evaluated were level of platelet inhibition, age, body mass index, and gender.

In countries with ample resources, no debate exists as to whether heart surgery should be provided. However, where funding is limited, what responsibility exists to care for children with congenital heart defects? If children have a “right” to surgical treatment, to whom is the “duty” to provide it assigned? These questions are subjected to ethical analysis.

Examination is initially based on the four principles of medical ethics: autonomy, beneficence, non-maleficence, and justice. Consideration of beneficence and justice is expanded using a consequentialist approach.

Social structures, including governments, exist to foster the common good. Society, whether by means of government funding or otherwise, has the responsibility, according to the means available, to assure health care for all based on the principles of beneficence, non-maleficence, and justice. In wealthy countries, adequate resources exist to fund appropriate treatment; hence it should be provided to all based on distributive justice. In resource-limited countries, however, decisions regarding provision of care for expensive or complex health problems must be made with consideration for broader effects on the general public. Preliminary data from cost-effectiveness analysis indicate that many surgical interventions, including cardiac surgery, may be resource-efficient. Given that information, utilitarian ethical analysis supports dedication of resources to congenital heart surgery in many low-income countries. In the poorest countries, where access to drinking water and basic nutrition is problematic, it will often be more appropriate to focus on these issues first.

Ethical analysis supports dedication of resources to congenital heart surgery in all but the poorest countries.

Veno-arterial extracorporeal membrane oxygenation is frequently used in patients with cardiac disease. We evaluated short-term outcomes and identified factors associated with hospital mortality in cardiac patients supported with veno-arterial extracorporeal membrane oxygenation.

A retrospective review of patients supported with veno-arterial extracorporeal membrane oxygenation at a university-affiliated children’s hospital was performed.

A total of 253 patients with cardiac disease managed with extracorporeal membrane oxygenation were identified; survival to discharge was 48%, which significantly improved from 39% in an earlier era (1995–2001) (p=0.01). Patients were categorised into surgical versus non-surgical groups on the basis of whether they had undergone cardiac surgery before or not, respectively. The most common indication for extracorporeal membrane oxygenation was extracorporeal cardiopulmonary resuscitation: 96 (51%) in the surgical group and 45 (68%) in the non-surgical group. In a multiple covariate analysis, single-ventricle physiology (p=0.01), duration of extracorporeal membrane oxygenation (p<0.01), and length of hospital stay (p=0.03) were associated with hospital mortality. Weekend or night shift cannulation was associated with mortality in non-surgical patients (p=0.05).

We report improvement in survival compared with an earlier era in cardiac patients supported with extracorporeal membrane oxygenation. Single-ventricle physiology continues to negatively impact survival, along with evidence of organ dysfunction during extracorporeal membrane oxygenation, duration of extracorporeal membrane oxygenation, and length of stay.

Neuroprotective developmental care is paramount for neonates with CHD. Although several developmental care scales exist, either they have not been psychometrically tested or were not designed for the needs of neonates with CHD. The purpose of this study is to describe item development and content validity testing of the developmental care scale for neonates with CHD, which measures five domains of the developmental care provided by bedside nurses to neonates in the cardiac ICU: sleep, pain and stress management, activities of daily living, family-centred care, and environment.

For this cross-sectional study, items were developed based on clinical expertise and the core measures for developmental care. In this study, seven experts provided content validity ratings of items for total scale and subscale fit and relevance. A content validity index was used to determine item retention. Item modifications and additions were based on expert feedback.

Expert ratings provided evidence of content validity on 24 of 53 items within the five domains of developmental care. A total of 24 items were deleted, and five items with low content validity ratings were retained, because of conceptual importance, and revised. An additional 11 items were added based on expert qualitative feedback.

This study provided evidence of content validity of the developmental care scale for neonates with CHD by researchers and bedside nurses caring for these neonates. Further psychometric testing is warranted to provide evidence of internal consistency reliability, construct validity, and to identify variables that influence quality of the developmental care.

Ductal patency is mandatory to manage patients with ductal-dependent pulmonary circulation. The aim of this study is to elucidate the morphological and haemodynamic features of ductus arteriosus with right ventricular outflow tract obstruction, and investigate the appropriate perinatal management.

Patients with prenatal diagnosis of right ventricular outflow tract obstruction at our institution between 2010 and 2015 were included in the study. Reverse orientation of the ductus arteriosus is defined as an inferior angle of <90° at the aortic junction, and normal orientation of the ductus arteriosus as an angle of >90°. We retrospectively reviewed the shape and flow pattern of ductus arteriosus and the clinical characteristics of the cases.

A total of 39 patients were enrolled. The shape was divided into normal orientation (n=15) and reverse orientation (n=24) of the ductus arteriosus. There was no significant difference in the type of oxygen saturation at birth and age at shunt operation between both the groups. However, the median narrowest diameter of ductus arteriosus in the normal orientation group was significantly smaller than that in the reverse orientation group (2.0 [1.0–5.4] versus 3.0 [1.3–4.4] mm, p<0.05). In two patients of the normal orientation group, ductus arteriosus had closed at birth, and one of whom died because of severe cyanosis.

Normal orientation pattern might have high incidence of an early narrowing or closure of ductus arteriosus at birth. The critical patients need careful evaluation by repeated foetal echocardiography and further maternal interventions.

The American College of Cardiology Quality Network enables national benchmarking and collaborative quality improvement through vetted metrics. We describe here our initial experience with the Quality Network.

Quarterly data for metrics pertaining to chest pain, Kawasaki disease, tetralogy of Fallot, elevated body mass index, and others were shared with the collaboratives for benchmarking. National improvement efforts focussed on counselling for elevated body mass index and 22q11.2 testing in tetralogy of Fallot. Improvement strategies included developing multi-disciplinary workgroups, educational materials, and electronic health record advances.

Chest pain metric performance was high compared with national means: obtaining family history (90–100% versus 51–77%), electrocardiogram (100% versus 89–99%), and echocardiogram for exertional complaints (95–100% versus 74–96%). Kawasaki metric performance was high, including obtaining coronary measurements (100% versus 85–97%), prescribing aspirin (100% versus 86–99%), follow-up with imaging (100% versus 85–98%), and documenting no activity restriction without coronary aneurysms (83–100% versus 64–93%). Counselling for elevated body mass index was variable (25–75% versus 31–50%) throughout quality improvement efforts. Testing for 22q11.2 deletion in tetralogy of Fallot patients was consistently above the national mean (60–85% versus 54–68%) with improved genetics data capture.

The Quality Network promotes meaningful benchmarking and collaborative quality improvement. Our high performance for chest pain and Kawasaki metrics is likely related to previous improvement efforts in chest pain management and a dedicated Kawasaki team. Uptake of counselling for elevated body mass index is variable; stronger engagement among numerous providers is needed. Recommendations for 22q11.2 testing in tetralogy of Fallot were widely recognised and implemented.

A wide variety of surgical strategies are used in tetralogy of Fallot repair. We sought to describe the international contemporary practice patterns for surgical management of tetralogy of Fallot.

Surgeons from 18 international paediatric cardiac surgery centres (representing over 1800 tetralogy of Fallot cases/year) completed a Research Electronic Data Capture-based survey. Participating countries include: China (4), India (2), Nepal (1), Korea (1), Indonesia (1), Saudi Arabia (3), Japan (1), Turkey (1), Australia (1), United States of America (2), and Canada (1). Summary measures were reported as means and counts (percentages). Responses were weighted based on case volume/centre.

Primary repair is the prevalent strategy (83%) with variation in age at elective repair (range). Approximately 47% of sites use patient age as a factor in determining the strategy, with age <3 months being the common cut-off for staged repair. In addition, patient weight of <3 kg is an indication for staged repair in 80% of participating institutions. Trans-atrial ventricular septal defect closure is the preferred approach in 62% of sites. Approximately 70% of responders reported using pulmonary valve z-score to guide right ventricular outflow tract management technique with the most prevalent cut-off for annulus preservation being a z-score of −3. Estimated incidence of annulus preservation is 53%. Minimal trans-annular incision is performed in >90% of all trans-annular repairs.

In this cohort representing 11 countries, there is variation in tetralogy of Fallot surgical management with no consensus on standard of practice. A large international prospective cohort study would allow analysis of impact of underlying anatomy and repair strategy on early and late outcomes.

Although sleep apnoea is an important disorder associated with cardiac events, data regarding its prevalence and risk factors in adult patients with CHD are limited.

In this study, patients underwent a sleep study in the hospital. Indications for admission were classified as heart failure, diagnostic catheterisation, interventional catheterisation, or arrhythmia. The prevalence, characteristics, and risk factors of sleep apnoea using a type 3 portable overnight polygraph in adult patients with CHD were evaluated.

This study comprised 104 patients of median age 36 years with interquartile range of 28–48 years, admitted for heart failure 34% (n = 36), diagnostic catheterisation 26% (n = 27), interventional catheterisation 18% (n = 19), or arrhythmia 22% (n = 23). The prevalence of sleep apnoea, defined as a respiratory disturbance index ≥5, was 63% (n = 63), with a distribution of 37, 16, and 10% for mild (5≤ respiratory disturbance index <15), moderate (15⩽ respiratory disturbance index <30), and severe (respiratory disturbance index ≥30) sleep apnoea, respectively. A large majority of the sleep apnoea cases were categorised as obstructive sleep apnoea (92%, n = 58). The respiratory disturbance index ≥15 group had a significantly higher proportion of male patients and higher body mass index, noradrenaline level, and aortic blood pressure than the group without sleep apnoea (respiratory disturbance index <5). Multi-variable analysis showed that NYHA class ≥II, whose odds ratio 4.36, 95% confidence interval 1.09–20.87, and body mass index ≥25, whose odds ratio 4.29, 95% confidence interval 1.32–15.23, were independent risk factors for a respiratory disturbance index ≥15.

Our results showed a high prevalence of sleep apnoea in adult patients with CHD. Its unique haemodynamics may be associated with a high prevalence of sleep apnoea. Congestive heart failure and being overweight are important risk factors for sleep apnoea. Management of heart failure and general lifestyle improvements are important for controlling sleep apnoea symptoms in these patients.