Pulmonary vasodilators improve the functional capacity of some patients with pulmonary arterial hypertension. However, pulmonary vasodilators frequently fail to improve unequivocal endpoints of efficacy in patients with lower pulmonary arterial pressures who have been palliated with a Fontan procedure.

Haemodynamic measurements and the results of acute vasodilator testing in a subset of patients were reviewed to determine whether some patients acutely respond more favourably to sildenafil and might be candidates for precision care with a phosphodiesterase V inhibitor long term.

Heart catheterisation was performed in 11 patients with a Fontan procedure. Haemodynamic measurements were performed before and after treatment with intravenous sildenafil (mean 0.14, range 0.05–0.20 mg/kg). Results (mean ± standard deviation) were compared by paired and unpaired t-tests to identify statistically significant changes.

Sildenafil was acutely associated with changes in mean pulmonary arterial pressure, transpulmonary gradient, indexed blood flow, and indexed vascular resistance. Changes in mean pulmonary arterial pressure were greater for patients with a mean pulmonary arterial pressure greater than 14 mmHg compared to patients with a lower mean pulmonary arterial pressure. Changes in transpulmonary gradient were greater for patients with a transpulmonary gradient greater than 5 mmHg compared to patients with a lower transpulmonary gradient.

Sildenafil acutely decreases mean pulmonary arterial pressure and transpulmonary gradient and causes greater acute changes in patients with higher mean pulmonary arterial pressures and transpulmonary gradients. Haemodynamic measurements and vasodilator testing might help to guide precision care following Fontan palliation.

Hospital readmission is an important driver of costs among patients with CHD. We assessed predictors of 30-day rehospitalisation following cardiac surgery in CHD patients across the lifespan.

This was a retrospective analysis of 981 patients with CHD who had cardiac surgery between January 2011 and December 2012. A multivariate logistic regression model was used to identify demographic, clinical, and surgical predictors of 30-day readmission. Receiver operating curves derived from multivariate logistic modelling were utilised to discriminate between patients who were readmitted and not-readmitted at 30 days. Model goodness of fit was assessed using the Hosmer–Lemeshow test statistic.

Readmission in the 30 days following congenital heart surgery is common (14.0%). Among 981 patients risk factors associated with increased odds of 30-day readmission after congenital heart surgery through multivariate analysis included a history of previous cardiac surgery (p < 0.001), longer post-operative length of stay (p < 0.001), as well as nutritional (p < 0.001), haematologic (p < 0.02), and endocrine (p = 0.04) co-morbidities. Patients who underwent septal defect repair had reduced odds of readmission (p < 0.001), as did children (p = 0.04) and adult (p = 0.005) patients relative to neonates.

Risk factors for readmission include a history of cardiac surgery, longer length of stay, and co-morbid conditions. This information may serve to guide efforts to prevent readmission and inform resource allocation in the transition of care to the outpatient setting. This study also demonstrated the feasibility of linking a national subspecialty registry to a clinical and administrative data repository to follow longitudinal outcomes of interest.

Familial hypercholesterolemia is a genetic disease with plasma total cholesterol especially low-density lipoprotein-cholesterol elevation. In this study, we aimed to examine the changes in the electrocardiographies of children with familial hypercholesterolemia.

Electrocardiography of 85 patients with a diagnosis of familial hypercholesterolemia, followed up from the Pediatric Metabolism and Pediatric Cardiology outpatient clinic was examined. Electrocardiography of 83 children from the control group who did not have hypercholesterolemia in a similar gender and age range were examined. Heart rate, P wave, PR interval, P wave dispersion, QRS wave, QT interval, corrected QT (calculated with Bazett formula), Tpeak-end interval, QT dispersion, corrected QT dispersion, JT interval, corrected JT (calculated with Bazett formula) were statistically compared.

P wave, PR interval, and P wave dispersion values were significantly higher (p < 0.05) in the children with familial hypercholesterolemia. Corrected QT, QT dispersion, corrected QT dispersion, JT interval, corrected JT, Tpeak-end interval were significantly higher than the control group (p < 0.05) in children with familial hypercholesterolemia. These statistical differences in electrocardiography parameters support the risk of atrial and/or ventricular arrhythmia in children with familial hypercholesterolemia.

We found that high total cholesterol and low-density lipoprotein-cholesterol variables are associated with an increased risk of cardiac atrial and/or ventricular arrhythmia. The findings suggest that total cholesterol and low-density lipoprotein-cholesterol variability can be used as a new marker for the risk of cardiac arrhythmia. In this case, decreasing total cholesterol and low-density lipoprotein-cholesterol variability below certain thresholds may decrease the risk of cardiac arrhythmia.

Hypoplastic left heart syndrome is a single ventricle defect. While staged surgical palliative treatments have revolutionised care, patients with hypoplastic left heart syndrome continue to have significant morbidity and mortality. In 2017, the National Pediatric Cardiology Quality Improvement Collaborative recommended all single ventricle patients to receive a prenatal palliative care consult. This study aimed to elucidate provider perspectives on the implementation of prenatal palliative care consults for families expecting a child with hypoplastic left heart syndrome.

An online survey was administered to obstetric and paediatric providers of relevant disciplines to assess their experience with palliative care involvement in hypoplastic left heart syndrome cases.

Nearly, all physicians (97%) and most registered nurses (79%) agreed that the initial palliative care consult for patients with hypoplastic left heart syndrome should occur during the prenatal period. Respondents also indicated that prenatal palliative care consults should also be offered in a variety of other CHD conditions. Participants believed positive aspects of this new referral protocol included an expanded support network for families, decreased family stress during the postnatal period, increased patient education about what to expect during the postnatal period, and continuity of care.

Multidisciplinary healthcare professionals believe that prenatal palliative care consults provide a variety of benefits for patients and families with hypoplastic left heart syndrome. Additional, multi-centre research is necessary to evaluate whether prenatal palliative care consults should become standard of care for families expecting a child with a single ventricle defect.

Cardiovascular morbidity is high in patients with coarctation of aorta even after successful repair. This study aimed to assess the frequency of late hypertension and the relationship between ambulatory hypertension and cardiovascular target organ damage in children and adolescents after early and successful repair of coarctation of the aorta.

Twenty-five children and adolescents (mean age 13.5 ± 3.43 years) with repaired coarctation of the aorta (median age at repair 4 months, arm–leg gradient <20 mmHg) and 16 healthy controls were included. Office and ambulatory blood pressure, pulse wave velocity, and left ventricular mass index were assessed.

Both day- and night-time systolic blood pressure standard deviation score and left ventricular mass index were significantly higher in patients compared to controls (p ≤ 0.001 for all), whereas pulse wave velocity did not differ. The prevalence of masked hypertension, isolated nocturnal hypertension, and left ventricular hypertrophy were 40, 28, and 24%, respectively. Left ventricular mass index was higher in patients with sustained hypertension, masked hypertension, and normotension compared to controls (p < 0.05). In multivariate analysis, higher night-time systolic blood pressure standard deviation score was the only independent predictor of left ventricular mass index.

The present study reveals a high prevalence of masked hypertension, isolated nocturnal hypertension, and left ventricular hypertrophy in children and adolescents with coarctation of the aorta even after early and successful repair. Ambulatory blood pressure monitoring should be considered to diagnose hypertension. All coarctation of aorta patients should be followed up lifelong and encouraged to establish a healthy lifestyle starting from childhood.

Catheterization in small children should be performed with the lowest diameter introducer sheaths to prevent permanent vessel damage. The objective of this study is to evaluate the clinical safety and efficacy of the Glidesheath Slender in small children.

We present a group of 52 patients (male: n = 36) with median age 118.5 days (min. 3; max. 1302), median weight: 5.3 kg (min. 1.4; max. 14.0), median height: 60.5 cm (min. 39; max. 102), and median body surface area 0.28 m2 (min. 0.12; max. 0.63) in whom percutaneous catheter interventions (n = 55) were performed via a Glidesheath Slender. In 49 children, the intervention was performed from femoral access (artery n = 35; vein n = 14) in 2 from the axillary arterial access and in 1 from the jugular venous access. In all patients, the vessel access was obtained under ultrasound guidance. After the catheterization, the pulse on the peripheral arteries (posterior tibial artery or radial artery) was palpable in all patients, and no signs for vessel dysfunction were present.

The Glidesheath Slender effectively reduces the outer sheath diameter for various types of interventions in small children by one French, reducing the risk of vessel complications (stenosis, occlusion). Interventions via Glidesheath Slender in small patients are safe and feasible and extend the transcatheter possibilities in small children with congenital heart diseases.

Cardiac catheterisations for CHD produce anxiety for patients and families. Current strategies to mitigate anxiety and explain complex anatomy include pre-procedure meetings and educational tools (cardiac diagrams, echocardiograms, imaging, and angiography). More recently, three-dimensionally printed patient-specific models can be added to the armamentarium. The purpose of this study was to evaluate the efficacy of pre-procedure meetings and of different educational tools to reduce patient and parent anxiety before a catheterisation.

Prospective study of patients ≥18 and parents of patients <18 scheduled for clinically indicated catheterisations. Patients completed online surveys before and after meeting with the interventional cardiologist, who was blinded to study participation. Both the pre- and post-meeting surveys measured anxiety using the State-Trait Anxiety Inventory. In addition, the post-meeting survey evaluated the subjective value (from 1 to 4) of individual educational tools: physician discussion, cardiac diagrams, echocardiograms, prior imaging, angiograms and three-dimensionally printed cardiac models. Data were compared using paired t-tests.

Twenty-three patients consented to participate, 16 had complete data for evaluation. Mean State-Trait Anxiety Inventory scores were abnormally elevated at baseline and decreased into the normal range after the pre-procedure meeting (39.8 versus 31, p = 0.008). Physician discussion, angiograms, and three-dimensional models were reported to be most effective at increasing understanding and reducing anxiety.

In this pilot study, we have found that pre-catheterisation meetings produce a measurable decrease in patient and family anxiety before a procedure. Discussions of the procedure, angiograms, and three-dimensionally printed cardiac models were the most effective educational tools.

To determine the false-positive rate of pulse oximetry screening at moderate altitude, presumed to be elevated compared with sea level values and assess change in false-positive rate with time.

We retrospectively analysed 3548 infants in the newborn nursery in Albuquerque, New Mexico, (elevation 5400 ft) from July 2012 to October 2013. Universal pulse oximetry screening guidelines were employed after 24 hours of life but before discharge. Newborn babies between 36 and 36 6/7 weeks of gestation, weighing >2 kg and babies >37 weeks weighing >1.7 kg were included in the study. Log-binomial regression was used to assess change in the probability of false positives over time.

Of the 3548 patients analysed, there was one true positive with a posteriorly-malaligned ventricular septal defect and an interrupted aortic arch. Of the 93 false positives, the mean pre- and post-ductal saturations were lower, 92 and 90%, respectively. The false-positive rate before April 2013 was 3.5% and after April 2013, decreased to 1.5%. There was a significant decrease in false-positive rate (p = 0.003, slope coefficient = −0.082, standard error of coefficient = 0.023) with the relative risk of a false positive decreasing at 0.92 (95% CI 0.88–0.97) per month.

This is the first study in Albuquerque, New Mexico, reporting a high false-positive rate of 1.5% at moderate altitude at the end of the study in comparison to the false-positive rate of 0.035% at sea level. Implementation of the nationally recommended universal pulse oximetry screening was associated with a high false-positive rate in the initial period, thought to be from the combination of both learning curve and altitude. After the initial decline, it remained steadily elevated above sea level, indicating the dominant effect of moderate altitude.

Giant coronary aneurysms are the most severe complications of Kawasaki disease. There are few reports of outcomes from China. Most previous studies were based only on absolute aneurysmal dimensions. The aim of the present study was to catalog the outcomes of Kawasaki disease with giant coronary aneurysms in southwest China based on absolute dimensions and the z-score adjusted for body surface area.

All patients diagnosed with giant coronary aneurysms (z-score ≥ 10 or absolute dimension ≥ 8 mm) between December, 2002 and December, 2018 were included. We retrospectively analysed patient characteristics and clinical data from 38 patients with giant coronary aneurysms. Over a median follow-up period of 30.5 months (range from 1.7 months to 22.3 years), including patients in chronic phase who had been diagnosed prior to 2002, eight patients had myocardial infarction, including two deaths and one patient with coronary artery bypass grafting. The 1-, 2-, and 5-year event-free rates were 0.63, 0.63, and 0.53 for thrombosis, respectively, and 0.86, 0.81, and 0.81 for major adverse cardiac events, respectively. The 1-, 2-, and 5-year regression-free rates were 0.94, 0.85, and 0.67, respectively. A total of 73.7% of patients remained active.

In the early stages of Kawasaki disease, patients with giant coronary aneurysms often experience major cardiovascular events; however, they are also likely to have normalisation of the coronary internal luminal diameter. With long-term anticoagulation, close cardiologic monitoring, and prompt thrombolytic therapy, most patients can achieve disease-free periods.

Macitentan is an orally active, potent, dual endothelin receptor antagonist and is the only registered treatment for pulmonary arterial hypertension that significantly reduced morbidity and mortality in a long-term study.

We have recently reported that switch from bosentan to macitentan significantly improved exercise capacity in children and young adults with pulmonary arterial hypertension in a 24-week prospective study and well tolerated without adverse events. We now aimed to evaluate clinical efficacy, safety of switch in a larger patient population, in a 24-month prospective study.

This is a single-institution, 24-month prospective study. Patients ≥12 years with idiopathic/heritable, pulmonary arterial hypertension, or related to CHD or residual pulmonary arterial hypertension due to repaired congenital systemic-to-pulmonary shunts and on bosentan treatment were included. Concomitant treatment with oral phosphodiesterase type 5 inhibitors/inhaled prostanoids was allowed. Outcome measures included change from baseline to 24 months, in the 6-minute walk distance, functional class, oxygen saturation at rest/after walk distance test, and natriuretic peptide levels. Safety end points included adverse events, laboratory abnormalities.

Twenty-seven patients (19 adults/8 children, mean age: 21.1 ± 6.3 years (12–36), weight: 53.1 ± 15.7 kgs (26–87)) were included. Mean duration of macitentan treatment: 22.3 ± 3.9 months (9–24). Six-minute walk distance significantly improved from baseline (mean: 458 ± 79 m (300–620)) at 6 months (mean: 501 ± 73 m (325–616) + 43 m) (p < 0.05), at 12 months (mean: 514 ± 82 m (330–626) + 56 m) (p < 0.05), and at 24 months (mean: 532 ± 85 m (330–682) + 74 m) (p < 0.05). We observed a significant improvement during the first 6 months but no incremental improvement after 6 months (p > 0.05). Macitentan did not significantly change functional class, oxygen saturation, and natriuretic levels (p > 0.05). None of the patients had anaemia, hepatotoxicity, and peripheral edema.

Our study is the first study which showed that switch from bosentan to macitentan improved exercise capacity in children and young adults with pulmonary arterial hypertension significantly in the first 6 months and compared to baseline in 24 months and well tolerated without adverse events.

Neonates are at high risk of bleeding after open-heart surgery. We sought to determine pre-operative and intra-operative risk factors for increased bleeding after neonatal open-heart surgery with cardiopulmonary bypass.

We conducted a retrospective cohort study of neonates (0–30 days old) who underwent open-heart surgery with cardiopulmonary bypass from January, 2009, to March, 2013. Cardiac diagnosis; demographic and surgical data; and blood products, haemostatic agents, and anti-thrombotic agents administered before, during, and within 24 hours after surgery were abstracted from the electronic health record and anaesthesia records. The outcome of interest was chest tube output (in ml/kg body weight) within 24 hours. Relationships between chest tube output and putative associated factors were evaluated by unadjusted and adjusted linear regression.

The cohort consisted of 107 neonates, of whom 79% had a Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) Mortality Category of 4 or 5. Median chest tube output was 37 ml/kg (range 9–655 ml/kg). Age, African-American race, and longer durations of surgery and cardiopulmonary bypass each had statistically significant associations with increased chest tube output in unadjusted analyses. In multivariable analysis, African-American race retained an independent, statistically significant association with increased chest tube output; the geometric mean of chest tube output among African-American neonates was 71% higher than that of Caucasians (95% confidence interval, 29–125%; p = 0.001).

Among neonates with CHD undergoing open-heart surgery with cardiopulmonary bypass, African-American race is independently associated with greater chest tube output over the first 24 hours post-operatively.

Infants with single ventricle following stage I palliation are at risk for poor nutrition and growth failure. We hypothesise a standardised enteral feeding protocol for these infants that will result in a more rapid attainment of nutritional goals without an increased incidence of gastrointestinal co-morbidities.

Single-centre cardiac ICU, prospective case series with historical comparisons. Feeding cohort consisted of consecutive patients with a single ventricle admitted to cardiac ICU over 18 months following stage I palliation (n = 33). Data were compared with a control cohort and admitted to the cardiac ICU over 18 months before feeding protocol implementation (n = 30). Feeding protocol patients were randomised: (1) protocol with cerebro-somatic near-infrared spectroscopy feeding advancement criteria (n = 17) or (2) protocol without cerebro-somatic near-infrared spectroscopy feeding advancement criteria (n = 16).

Median time to achieve goal enteral volume was significantly higher in the control compared to feeding cohort. There were no significant differences in enteral feeds being held for feeding intolerance or necrotising enterocolitis between cohorts. Feeding cohort had significant improvements in discharge nutritional status (weight, difference admit to discharge weight, weight-for-age z score, volume, and caloric enteral nutrition) and late mortality compared to the control cohort. No infants in the feeding group with cerebro-somatic near-infrared spectroscopy developed necrotising enterocolitis versus 4/16 (25%) in the feeding cohort without cerebro-somatic near-infrared spectroscopy (p = 0.04).

A feeding protocol is a safe and effective means of initiating and advancing enteral nutrition in infants following stage I palliation and resulted in improved nutrition delivery, weight gain, and nourishment status at discharge without increased incidence of gastrointestinal co-morbidities.

Clinical studies have revealed decreased exercise capacity in adults with small, unrepaired ventricular septal defects. Increasing shunt ratio and growing incompetence of the aortic and pulmonary valve with retrograde flow during exercise have been proposed as reasons for the previously found reduced exercise parameters. With MRI, haemodynamic shunt properties were measured during exercise in ventricular septal defects.

Patients with small, unrepaired ventricular septal defects and healthy peers were examined with MRI during exercise. Quantitative flow scans measured blood flow through ascending aorta and pulmonary artery. Scans were analysed post hoc where cardiac index, retrograde flows, and shunt ratio were determined.

In total, 32 patients (26 ± 6 years) and 28 controls (27 ± 5 years) were included. The shunt ratio was 1.2 ± 0.2 at rest and decreased to 1.0 ± 0.2 at peak exercise, p < 0.01. Aortic cardiac index was lower at peak exercise in patients (7.5 ± 2 L/minute/m2) compared with controls (9.0±2L l/minute/m2), p<0.01. Aortic and pulmonary retrograde flow was larger in patients during exercise, p < 0.01. Positive correlation was demonstrated between aortic cardiac index at peak exercise and previously established exercise capacity for all patients (r = 0.5, p < 0.01).

Small, unrepaired ventricular septal defects revealed declining shunt ratio with increasing exercise and lower aortic cardiac index. Patients demonstrated larger retrograde flow both through the pulmonary artery and the aorta during exercise compared with controls. In conclusion, adults with unrepaired ventricular septal defects redistribute blood flow during exercise probably secondary to a more fixed pulmonary vascular resistance compared with age-matched peers.

For CHD patients undergoing corrective surgery utilising cardiopulmonary bypass, post-operative inhaled nitric oxide has been administered to alleviate pulmonary hypertension. We performed a systematic review and meta-analyses to determine the effect of inhaled nitric oxide on haemodynamics, gas exchange, and hospitalisation characteristics in children immediately after cardiopulmonary bypass.

A systematic review of the literature was performed to identify full-text manuscripts in English. PubMed, EMBASE, and the Cochrane databases were queried. Once manuscripts were identified for inclusion, a list of all the endpoints in each manuscript was created. Endpoints with data present from two or more studies were then kept for pooled analyses. All endpoints included were continuous variables and so mean and standard deviation were utilised as the effect data for comparison.

A total of eight studies were deemed appropriate for inclusion. There were significant differences with decreases in mean pulmonary artery pressure of −6.82 mmHg, left atrial pressure of −1.16 mmHg, arteriovenous oxygen difference of −1.63, arterial carbon dioxide concentration of −2.41 mmHg, mechanical ventilation duration of −8.56 hours, and length of cardiac ICU stay duration of −0.91 days. All significant variables achieved p < 0.001.

Inhaled nitric oxide in children immediately after cardiopulmonary bypass decreases mean pulmonary artery pressure significantly and decreases the arterial carbon dioxide concentration significantly without significantly altering other haemodynamic parameters. This results in a statistically shorter duration of mechanical ventilation and cardiac ICU length of stay without altering overall hospital length of stay.

To evaluate the diagnostic and predictive values of plasma connective tissue growth factor in children with pulmonary hypertension (PH)-related CHD.

Forty patients with PH-related CHD were enrolled as group I, and 40 patients with CHD and no PH served as group II. Forty healthy children of matched age and sex served as a control group. Echocardiographic examinations and plasma connective tissue growth factor levels were performed for all included children. Cardiac catheterisation was performed for children with CHD only.

Plasma connective tissue growth factor levels were significantly higher in children with PH-related CHD compared to CHD-only patients and to control group and this elevation went with the severity of PH. There was a significant positive correlation between connective tissue growth factor levels and mean pulmonary pressure, pulmonary vascular resistance, and right ventricular diameter. A significant negative correlation was noticed between connective tissue growth factor levels, oxygen saturation, and right ventricular diastolic function. The sensitivity of plasma connective tissue growth factor as a diagnostic biomarker for PH was 95%, and the specificity was 90% at a cut-off value ≥650 pg/mL. The predictive value of plasma connective tissue growth factor for adverse outcome had a sensitivity of 88% and a specificity of 83% at a cut-off value ≥1900 pg/mL.

Connective tissue growth factor is a promising biomarker with good diagnostic and predictive values in children with PH-related CHD.

To evaluate delivery management and outcomes in fetuses prenatally diagnosed with CHD.

A retrospective cohort study was conducted on 6194 fetuses (born between 2013 and 2016), comparing prenatally diagnosed with CHD (170) to those with non-cardiac (234) and no anomalies (5790). Primary outcomes included the incidence of preterm delivery and mode of delivery.

Gestational age at delivery was significantly lower between the CHD and non-anomalous cohorts (38.6 and 39.1 weeks, respectively). Neonates with CHD had a significantly lower birth weights (p < 0.001). There was an approximately 1.5-fold increase in the rate of primary cesarean sections associated with prenatally diagnosed CHD with an odds ratio of 1.49 (95% CI 1.06–2.10).

Our study provides additional evidence that the prenatal diagnosis of CHD is associated with a lower birth weight, preterm delivery, and with an increased risk of delivery by primary cesarean section.

The perioperative complications rate in paediatric cardiac surgery, as well as the failure-to-rescue impact, is less known in low- and middle-income countries.

To evaluate perioperative complications rate, mortality related to complications, different patients’ demographics, and procedural risk factors for perioperative complication and post-operative death.

Risk factors for perioperative complications and operative mortality were assessed in a retrospective single-centre study which included 296 consecutive children undergoing cardiac surgery.

Overall mortality was 5.7%. Seventy-three patients (24.7%) developed 145 perioperative complications and had 17 operative mortalities (23.3%). There was a strong association between the number of perioperative complications and mortality – 8.1% among patients with only 1 perioperative complication, 35.3% – with 2 perioperative complications, and 42.1% – with 3 or more perioperative complications (p = 0.007). Risk factors of perioperative complications were younger age (odds ratio 0.76; (95% confidence interval 0.61, 0.93), previous cardiac surgery (odds ratio 3.5; confidence interval 1.33, 9.20), extracardiac structural anomalies (odds ratio 3.03; confidence interval 1.27, 7.26), concomitant diseases (odds ratio 3.23; confidence interval 1.34, 7.72), and cardiopulmonary bypass (odds ratio 6.33; confidence interval 2.45, 16.4), whereas the total number of perioperative complications per patient was the only predictor of operative death (odds ratio 1.89; confidence interval 1.06, 3.37).

In a program with limited systemic resources, failure-to-rescue is a major contributor to operative mortality in paediatric cardiac surgery. Despite the comparable crude mortality, the operative mortality among patients with perioperative complications in our series was significantly higher than in the developed world. A number of initiatives are needed in order to improve failure-to-rescue rates in low- and middle-income countries.