Infants with moderate-to-severe CHD frequently undergo cardiopulmonary bypass surgery in childhood. Morbidity and mortality are highest in those who develop post-operative low cardiac output syndrome. Vasoactive and inotropic medications are mainstays of treatment for these children, despite limited evidence supporting their use.

To help inform clinical practice, as well as the conduct of future trials, we performed a systematic review of existing literature on inotropes and vasoactives in children after cardiac surgery using the PubMed and EMBASE databases. We included studies from 2000 to 2020, and the patient population was defined as birth – 18 years of age. Two reviewers independently reviewed studies to determine final eligibility.

The final analysis included 37 papers. Collectively, selected studies reported on 12 different vasoactive and inotropic medications in 2856 children. Overall evidence supporting the use of these drugs in children after cardiopulmonary bypass was limited. The majority of studies were small with 30/37 (81%) enrolling less than 100 patients, 29/37 (78%) were not randomised, and safety and efficacy endpoints differed widely, limiting the ability to combine data for meta-analyses.

Vasoactive and inotropic support remain critical parts of post-operative care for children after cardiopulmonary bypass surgery. There is a paucity of data for the selection and dosing of vasoactives and inotropes for these patients. Despite the knowledge gaps that remain, numerous recent innovations create opportunities to rethink the conduct of clinical trials in this high-risk population.

We aimed to determine the early and midterm outcomes of ductal stenting in neonates with ductal-dependent pulmonary blood flow.

Between January, 2014 and July, 2018, 102 patients who underwent 115 cardiac catheterisation procedures for ductal stent implantation in our department were retrospectively reviewed. The age of the neonates ranged from 3 to 30 days (median: 11 days) and their weights ranged from 1.8 to 5.8 kg (mean, 2.8 ± 0.53 kg). Fifty-two patients had functional single ventricle and 50 had biventricular physiology. Thirty-one patients’ weights were <2,500 g (30.3%). The patent ductus arteriosus was vertical in 60 patients (58.8%). The mean ductal length was 12.4 ± 4.1 mm (range, 7.8–23 mm), and the mean narrowest ductal diameter was 2.1 ± 0.7 mm (range, 1.2–3.4 mm).

The technical success rate was 85.2%. Procedure-related mortality occurred in three patients (2.9%). After the procedure, the aortic oxygen saturation increased from a mean of 73.1 ± 6.2% to a mean of 90.4 ± 4.3% (p < 0.001), and the ductus diameter increased from a mean of 2.1 ± 0.7 mm to a mean of 4.2 ± 0.9 mm (p < 0.001). Either transcatheter or surgical reinterventions were required in 35 patients (34.3%) during the follow-up period after a median of 101 days (2–356 days). Thirty-three patients (32.3%) were bridged to surgical repair after a median of 288 days (163–650 days). The median duration of palliation with ductal stents was 210 days (range, 2–525 days).

Ductus arteriosus stenting may be a reasonable and effective alternative to surgery for the initial palliation procedure in neonates with ductus-dependent pulmonary flow.

Infective endocarditis remains a severe complication associated with a high morbidity and mortality in patients after heart valve replacement. Exploration of the pathogenesis is of high demand and we, therefore, present a competent model that allows studying bacterial adherence and the role of plasma fibrinogen in this process using a new in-house designed low-volume flow chamber. Three cardiac graft tissues used for pulmonary valve replacement have been tested under shear conditions to investigate the impact of surface composition on the adhesion events.

Tissue pieces of cryopreserved homograft (non-decellularised), decellularised homograft and bovine pericardium patch were investigated for fibrinogen binding. Adherence of Staphylococcus aureus to these graft tissues was studied quantitatively under flow conditions in our newly fabricated chamber based on a parallel plates’ modality. The method of counting colony-forming units was reliable and reproducible to assess the propensity of different graft materials for bacterial attachment under shear.

Bacterial perfusions over all plasma-precoated tissues identified cryopreserved homograft with the lowest affinity for S. aureus compared to decellularised homograft presenting a significantly higher bacterial adhesion (p < 0.05), which was linked to a more avid fibrinogen binding (p < 0.01). Bovine pericardial patch, as a reference tissue in this study, was confirmed to be the most susceptible tissue graft for the bacterial adhesion, which was in line with our previous work.

The two studied homograft tissues showed different levels of bacterial attachment, which might be postulated by the involvement of fibrinogen in the adhesion mechanism(s) shown previously for bovine tissues.

Children with congenital heart disease (CHD) have complex unique post-operative care needs. Limited data assess parents’ hospital discharge preparedness and education quality following cardiac surgery. The goals were to identify knowledge gaps in discharge preparedness after congenital heart surgery and to assess the acceptability of an educational mobile application to improve discharge preparedness.

Telephonic interviews with parents of children with two-ventricle physiology who underwent cardiac surgery 5–7 days post-discharge and in-person interviews with clinicians were conducted. We collected parent and clinician demographics, parent health literacy information and patient clinical data. We analysed interview transcripts using summative content analysis.

We interviewed 26 parents and 6 clinicians. Twenty-two of the 26 (85%) parents felt ready for discharge; 4 of the 6 (67%) clinicians did not feel most parents were ready for discharge. Fifteen of the 26 parents (58%) reported receiving the majority of discharge teaching on the day of discharge. Eight parents did not feel like all of their questions were answered. Most parents (14/26, 54%) preferred visual educational learning aids and could accurately describe important aspects of care. Most parents (23/26, 88%) and all 6 clinicians felt a mobile application for post-operative care education would be helpful.

Most parents received education on the day of discharge and could describe the information they received prior to discharge, although there were some preparedness gaps identified after discharge. Clinicians and parents varied in their perceptions of the readiness for discharge. Most responses suggest that a mobile application for discharge education may be helpful for transition to home.

An examination of invasive procedure cancellations found that the lack of pre-procedural oral screening was a preventable cause, for children with congenital heart disease. The purpose of this study was to implement an oral screening tool within the paediatric cardiology clinic, with referral to paediatric dental providers for positive screens. The target population were children aged ≥6 months to <18 years old, being referred for cardiac procedures.

The quality implementation framework method was used for this study design. The multi-modal intervention included education, audit and feedback, screening guidelines, environmental support, and interdisciplinary collaboration. Baseline rates for oral screenings were determined by retrospective chart audit from January 2018 to January 2019 (n = 211). Provider adherence to the oral screening tool was the outcome measure. Positive oral screens, resulting in referral to the paediatric dental clinic, were measured as a secondary outcome. Provider adherence rates were used as a process measure.

Data collected over 14 weeks showed a 29% increase in documentation of oral screenings prior to referral, as compared to the retrospective chart audit. During the study period, 13% of completed screenings were positive (n = 5). Provider compliance for the period was averaged at 70% adherence.

A substantial increase in pre-procedural oral screenings by paediatric cardiologists was achieved using the quality implementation framework and targeted interventions.

Congenital heart disease (CHD) is multifactorial in origin, resulting from an interaction between environmental and genetic factors. Multifactorial growth delay is common in infants with CHD. The impact of a genetic abnormality and CHD on the growth of an infant is lacking in the literature. The aim of this study is to compare the growth and method of feeding following neonatal cardiac surgery in infants with normal versus abnormal genetic testing.

A retrospective chart review of neonates who underwent a Risk Adjustment in Congenital Heart Surgery IV–VI procedure between 1 January, 2006 and 22 September, 2016 was performed at our institution. Weight, length, head circumference measurements, and feeding method were collected at birth, time of neonatal surgery, and monthly up to 6 months of age.

A total of 53 infants met inclusion criteria, of which 22 had abnormal genetic testing. Approximately 90% of infants were discharged following neonatal cardiac surgery with supplemental tube feeds. At each monthly follow-up visit, more infants were exclusively fed orally: 80% of infants with normal genetics at 5 months post-operative follow-up versus 60% of infants with abnormal genetic testing, although statistically insignificant. Growth was not different among the two groups.

Infants with critical CHD with or without genetic abnormalities are at risk for growth delays and many need supplemental tube feeds post-operatively and throughout follow-up. Infants with genetic abnormalities are slower to achieve oral feeds and more likely to require tube feedings. It is important to have a systematic protocol for managing these high-risk infants.

The diagnosis of Postural Orthostatic Tachycardia syndrome traditionally involves orthostatic vitals evaluation. The Compensatory Reserve Index is a non-invasive, FDA-cleared algorithm that analyses photoplethysmogram waveforms in real time to trend subtle waveform features associated with varying degrees of central volume loss, from normovolemia to decompensation. We hypothesised that patients who met physiologic criteria for Postural Orthostatic Tachycardia syndrome would have greater changes in Compensatory Reserve Index with orthostatic vitals.

Orthostatic vitals and Compensatory Reserve Index values were assessed in individuals previously diagnosed with Postural Orthostatic Tachycardia syndrome and healthy controls aged 12–21 years. Adolescents were grouped for comparison based on whether they met heart rate criteria for Postural Orthostatic Tachycardia syndrome (physiologic Postural Orthostatic Tachycardia syndrome).

Sixty-one patients were included. Eighteen percent of patients with an existing Postural Orthostatic Tachycardia syndrome diagnosis met heart rate criteria, and these patients had significantly greater supine to standing change in Compensatory Reserve Index (0.67 vs. 0.51; p<0.001). The optimal change in Compensatory Reserve Index for physiologic Postural Orthostatic Tachycardia syndrome was 0.60. Patients with physiologic Postural Orthostatic Tachycardia syndrome were more likely to report previous diagnoses of anxiety or depression (p = 0.054, 0.042).

An accurate diagnosis of Postural Orthostatic Tachycardia syndrome may be confounded by related comorbidities. Only 18% (8/44) of previously diagnosed Postural Orthostatic Tachycardia syndrome patients met heart rate criteria. Findings support the utility of objective physiologic measures, such as the Compensatory Reserve Index, to more accurately identify patients with true autonomic dysfunction.

Brucellosis is an important systemic infectious disease, especially in developing countries. Every organ and system of the human body can be affected; however, cardiovascular complications of brucellosis are rare.

To assess cardiac functions in patients with acute brucellosis without overt cardiac involvement and to answer the following question: Is there any cardiac dysfunction despite the absence of endocarditis in these patients?

This cross-sectional study included 67 children with brucellosis and 40 healthy children. We performed a detailed echocardiographic examination in individuals at the beginning of the treatment. Patients with infective endocarditis were excluded from the study.

Echocardiography revealed no difference of ejection fraction, mitral and tricuspid annular plane systolic excursion, pulsed-wave Doppler-derived early diastolic peak velocity (E)/late diastolic peak velocity (A) ratios in mitral and tricuspid valves between the two groups. The deceleration time of early mitral inflow was longer in patients with brucellosis. Early diastolic peak velocity of the mitral and tricuspid annuluses obtained by tissue Doppler imaging (Ea) was significantly lower in children with brucellosis. The peak velocity obtained by tissue Doppler imaging during late diastole (Aa), Ea and Ea/Aa ratios in the interventricular septum, left ventricle posterior wall and right ventricle free wall was lower in patients with brucellosis than in the control group. The E/Ea ratio, isovolumic relaxation time, right ventricle and left ventricle myocardial performance indices were higher in patients with brucellosis.

Patients with acute brucellosis may have diastolic dysfunction without overt cardiac involvement and infective endocarditis.

Care of children with functionally univentricular hearts is resource-intensive.

To analyse pregnancy and early post-natal outcomes of fetuses with functionally univentricular hearts in the setting of a low–middle-income country.

A retrospective study was conducted during the period of January 2008–October 2019. Study variables analysed included gestational age at diagnosis, maternal and fetal comorbidities and cardiac diagnosis including morphologic type of single ventricle. Outcomes analysed included pregnancy outcomes, type of post-natal care and survival status on the last follow-up.

A total of 504 fetuses were included. Mean maternal age was 27.5 ± 4.8 years and mean gestational age at diagnosis was 25.6 ± 5.7 weeks. Pregnancy outcomes included non-continued pregnancies (54%), live births (42.7%) and loss to follow-up (3.3%). Gestational age at diagnosis was the only factor that impacted pregnancy outcomes (non-continued pregnancies 22.5 ± 3.5 vs. live births 29.7 ± 5.7 weeks; p < 0.001). Of the 215 live births, intention-to-treat was reported in 119 (55.3%) cases; of these 103 (86.6%) underwent cardiac procedures. Seventy-nine patients (36.7%) opted for comfort care. On follow-up (median 10 (1–120) months), 106 patients (21%) were alive. Parental choice of intention-to-.treat or comfort care was the only factor that impacted survival on follow-up.

Prenatal diagnosis of functionally univentricular hearts was associated with overall low survival status on follow-up due to parental decisions on not to continue pregnancy or non-intention-to-treat after birth. Early detection of these complex defects by improved prenatal screening can enhance parental options and reduce resource impact in low-and-middle-income countries.

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.

Acute respiratory infection is one of the main causes of morbidity in children. Some studies have suggested that pulmonary hypertension and congenital heart disease with haemodynamic repercussion increase the severity of respiratory infections, but there are few publications in developing countries.

This was a prospective cohort study evaluating the impact of pulmonary hypertension and congenital heart disease (CHD) with haemodynamic repercussion as predictors of severity in children under 5 years of age hospitalised for acute respiratory infection.

Altogether, 217 children hospitalised for a respiratory infection who underwent an echocardiogram were evaluated; 62 children were diagnosed with CHD with haemodynamic repercussion or pulmonary hypertension. Independent predictors of admission to intensive care included: pulmonary hypertension (RR 2.14; 95% CI 1.06–4.35, p = 0.034), respiratory syncytial virus (RR 2.52; 95% CI 1.29–4.92, p = 0.006), and bacterial pneumonia (RR 3.09; 95% CI 1.65–5.81, p = 0.000). A significant difference was found in average length of hospital stay in children with the cardiovascular conditions studied (p = 0.000).

Pulmonary hypertension and CHD with haemodynamic repercussion as well as respiratory syncytial virus and bacterial pneumonia were predictors of severity in children with respiratory infections in this study. Early recognition of cardiovascular risks in paediatric populations is necessary to lessen the impact on respiratory infections.

This study aimed to evaluate the early outcomes of patients who underwent a concomitant therapeutic maze procedure for congenital heart surgery.

Between 2019 and 2020, eight patients underwent surgical cryoablation by using the same type of cryoablation probe.

Three patients had atrial flutter, two had Wolf–Parkinson–White syndrome, two intra-atrial reentrant tachycardia, and one had atrial fibrillation. Four patients underwent electrophysiological study. Preoperatively, one patient was on 3, two were on 2, five were on 1 antiarrhythmic drug. Six patients underwent right atrial maze and two underwent bilateral atrial maze. Five out of six right atrial maze patients underwent right atrial reduction. Nine different lesion sets were used. Some of the lesions were combined and applied as one lesion. In Ebstein’s anomaly patients, the lesion from coronary sinus to displaced tricuspid annulus was delicately performed. The single ventricle patient with heterotaxy had junctional rhythm at the time of discharge and was the only patient who experienced atrial extrasystoles 2 months after discharge. Seven of the eight patients were on sinus rhythm. No patient needed permanent pacemaker placement.

Cryomaze procedure can be applied in congenital heart diseases with acceptable arrhythmia-free rates by selecting the appropriate materials and suitable lesion sets. The application of cryomaze in heterotaxy patients can be challenging due to differences in the conduction system and complex anatomy. Consensus with the electrophysiology team about the choice of the right–left or biatrial maze procedure is mandatory for operational success.

Efficacy of sildenafil in treating paediatric pulmonary arterial hypertension is controversial. This systematic review aimed to explore the safety and effect of sildenafil on treating paediatric pulmonary arterial hypertension (PAH) through meta-analysis.

In this study, the electronic databases, including the Cochran Library database, EMBASE, and MEDLINE were systemically retrieved to identify the related randomised controlled trials (RCTs). Two reviewers had independently completed study selection, data collection, and assessment of the bias risk. Amongst 938 articles researched according to our retrieval strategy, 15 papers that involved 673 cases had been screened. Relative to control group, the sildenafil group had markedly reduced mortality (RR = 0.25, 95% CI: 0.12–0.51; p < 0.0001), but difference within the mortality was not statistically significant between high- and low-dose sildenafil groups (p = 0.152). Nonetheless, difference of the mean pulmonary arterial pressure between sildenafil as well as control group was of no statistical significance. Differences in the length of hospital stay and the incidences of pulmonary hypertensive crisis between children with PAH and controls were of no statistical significance. However, the summary estimate favoured that sildenafil reduced the duration of mechanical ventilation time, as well as the length of ICU stay and inotropic support.

Sildenafil therapy reduces the mortality of PAH patients, but its effects on the haemodynamic outcomes and other clinical outcomes are still unclear.

Rapid right ventricular pacing during balloon aortic valvuloplasty is commonly used to achieve balloon stability in children and adults. There is no consensus for the use of the technique in neonates and infants. We sought to review our institutional experience with rapid right ventricular pacing-assisted balloon aortic valvuloplasty across all age groups and evaluate the safety and effectiveness of the technique in the sub-group of neonates and infants <12months.

Retrospective study between February, 2011 and February, 2020.

A total of 37 patients (Group I: 21 neonates/infants <12months and Group II: 16 children 12 months–16 years) were analysed. Catheter-measured left ventricular to aortic gradient reduced from median of 66 mmHg (with a range from 30 to 125 mmHg) to 14 mmHg (with a range from 5 to 44 mmHg) in Group I and 44 mmHg (with a range from 28 to 93 mmHg) to 18 mmHg (with a range from 2 to 65 mmHg) in Group II (p < 0.001). Procedure and fluoroscopy times were identical in the two groups. Balloon:annulus ratio was 0.94 and 0.88 in Groups I and II, respectively. Freedom from reintervention was 100% for Group I at a median time of 3.2 years and 81% at 2.7 years for Group II. Reinterventions in Group II (3/16 pts) were performed predominantly for complex left ventricular outflow tract stenosis. At follow-up echocardiogram, 45% of patients in Group I had no aortic regurgitation, 30% trace-mild, 20% mild-moderate, and 5% moderate aortic regurgitation, whereas in Group II, 50% of patients had no aortic regurgitation, 32% had mild aortic regurgitation, and 18% mild-moderate aortic regurgitation. Unicuspid valves were only encountered in Group 1 (2/21 pts, 10%) and they were predictive of mild-aortic regurgitation during follow-up (p = 0.003). Ventricular fibrillation occurred in three neonates with suspicion of myocardial ischemia on the pre-procedure echocardiogram. All were successfully defibrillated.

Rapid right ventricular pacing can be expanded in neonates and infants to potentially decrease the incidence of aortic regurgitation and reintervention rates, hence avoiding high-risk surgical bail-out procedures for severe aortic regurgitation in the first year of life. Myocardial ischemia may predispose to ventricular dysrhythmias during rapid right ventricular pacing.

Thrombocytopenia occasionally occurs following the closure of some giant patent ductus arteriosus cases. Unfortunately, there is no associated research describing the associated risk factors for thrombocytopenia post-procedure.

We reviewed all patients who received occluders with sizes ≥10/12 mm between January 2013 and June 2019. All the data and information on the characteristics of the patients and their follow-up were recorded. Univariate analysis, receiver operating characteristic curves, and linear regression were used to analyse the risk factors for thrombocytopenia and the predictors of hospitalisation stay.

Finally, 32 patients (17.5%) suffered from thrombocytopenia. Univariate analysis revealed the ratio between occluder disc size (mm) and body weight (kg) (1.71 ± 0.51 versus 1.35 ± 0.53) as an independent predictive factor for thrombocytopenia, and the area under the curve of the ratio of occluder size and body weight for predicting thrombocytopenia post-closure was 0.691 (95% confidence interval: 0.589–0.792, p = 0.001). The best cut-off value for the ratio of occluder size and weight was 1.5895, with a sensitivity and specificity of 68.8 and 66.9%, respectively. Each unit of the ratio of occluder size and body weight predicted an average hospitalisation stay of 2.856 days (95% confidence interval: 1.380–4.332). Treatment with medication did not reduce the hospitalisation stay or benefit platelet restoration.

Once the ratio of occluder size and body weight is greater than 1.6, thrombocytopenia always exists. Every unit of the ratio of occluder size and body weight represents an additional 3 days of hospitalisation. Treatment does not reduce the duration of hospitalisation.