Sickle cell anaemia is characterised by frequent, sometimes serious events referred to as “crisis”. Cardiopulmonary consequences such as pulmonary hypertension and myocardial ischaemia may accompany a serious crisis.

To determine the cardiovascular changes that occur during a severe sickle cell crisis.

A cross-sectional comparative study of sickle cell anaemia in children (5–17 years) admitted during a severe crisis (cases) and those in steady state (controls) was conducted over a 2-year period. Effects of the crisis on the cardiopulmonary system were assessed. The diagnosis of myocardial ischaemia was made using electrocardiography and serological cardiac biomarkers, while cardiac dysfunction and the presence of pulmonary hypertension were determined using echocardiography. The presence of systemic hypertension and tachycardia was also evaluated.

A total of 176 patients were recruited, 92 in steady state (male:female ratio, 1.2:1) and 84 in severe crisis (male:female ratio, 1.3:1). The mean age was 10.4 ± 3.2 years for steady state and 10.5 ± 3.4 years for those in crisis. The mean heart rate in crisis was higher than in steady state (p < 0.0001). The blood pressures (systolic, p < 0.0001, diastolic, p < 0.0001, mean, p < 0.0001) as well as myocardial ischaemia scores (p < 0.0001) were higher in patients with crisis than in those in steady state. Similarly, conduction abnormalities, pulmonary hypertension, and ventricular dysfunction were more prevalent in the crisis than in the steady state.

The present data suggest that sickle cell crisis results in a derangement of clinical, electrocardiographical, and echocardiographical parameters in children with sickle cell anaemia. Further research on these cardiovascular events may improve the overall care of these patients.

Duchenne muscular dystrophy is associated with progressive cardiorespiratory failure, including left ventricular dysfunction.

Males with probable or definite diagnosis of Duchenne muscular dystrophy, diagnosed between 1 January, 1982 and 31 December, 2011, were identified from the Muscular Dystrophy Surveillance Tracking and Research Network database. Two non-mutually exclusive groups were created: patients with ≥2 echocardiograms and non-invasive positive pressure ventilation-compliant patients with ≥1 recorded ejection fraction. Quantitative left ventricular dysfunction was defined as an ejection fraction <55%. Qualitative dysfunction was defined as mild, moderate, or severe. Progression of quantitative left ventricular dysfunction was modelled as a continuous time-varying outcome. Change in qualitative left ventricle function was assessed by the percentage of patients within each category at each age. Forty-one percent (n = 403) had ≥2 ejection fractions containing 998 qualitative assessments with a mean age at first echo of 10.8 ± 4.6 years, with an average first ejection fraction of 63.1 ± 12.6%. Mean age at first echo with an ejection fraction <55 was 15.2 ± 3.9 years. Thirty-five percent (140/403) were non-invasive positive pressure ventilation-compliant and had ejection fraction information. The estimated rate of decline in ejection fraction from first ejection fraction was 1.6% per year and initiation of non-invasive positive pressure ventilation did not change this rate.

In our cohort, we observed that left ventricle function in patients with Duchenne muscular dystrophy declined over time, independent of non-invasive positive pressure ventilation use. Future studies are needed to examine the impact of respiratory support on cardiac function.

Children with complex CHD are at risk for psychopathology such as severe attention-deficit/hyperactivity disorder symptoms after congenital heart surgery.

The aim of this study was to investigate if children with Ventricular Septal Defect, Transposition of Great Arteries, or Tetralogy of Fallot have an increased occurrence of attention-deficit/hyperactivity disorder symptoms compared with the background population and to investigate differences between the three CHDs in terms of occurrence and appearance of attention-deficit/hyperactivity disorder symptoms.

A national register-based survey was conducted, including children aged 10–16 years with surgically corrected CHDs without genetic abnormalities and syndromes. The Attention-Deficit/Hyperactivity Disorder-Rating Scale questionnaires were filled in by parents and school teachers.

In total, 159 out of 283 questionnaires were completed among children with CHDs and compared with age- and sex-matched controls. Children with CHDs had significantly increased inattention scores (p = 0.009) and total attention-deficit/hyperactivity disorder scores (p = 0.008) compared with controls. Post hoc analyses revealed that children with Tetralogy of Fallot had significantly higher inattention scores compared with children both with Ventricular Septal Defect (p = 0.043) and controls (p = 0.004).

Attention-deficit/hyperactivity disorder symptoms and inattention symptoms were significantly more frequent among children aged 10–16 years with CHDs, in particular in children with corrected Tetralogy of Fallot.

Little is known about emotional quality-of-life in paediatric heart disease in low- and middle-income countries where the prevalence of uncorrected lesions is high. Research on emotional quality-of-life and its predictors in these settings is key to planning interventions.

Ten-year retrospective cross-sectional study of children aged 6–17 years with uncorrected congenital or acquired heart disease in 12 low- and middle-income countries was conducted. Emotional functioning score of the PedsQL TM 4.0 generic core scale and data on patient-reported limitation in sports participation were collected via in-person interview and analysed using regression analyses.

Ninety-four children reported mean emotional functioning scores of 71.94 (SD 25.32) [95% CI 66.75–77.13] with lower scores independently associated with having a parent with a chronic illness or who had died (p = 0.005), having less than three siblings (p = 0.007), and reporting a subjective limitation in carrying an item equivalent to a 4 lb load (p = 0.021). Patient-reported limitation in sports participation at least “sometimes” was present in 69% and was independently associated with experiencing symptoms at least once a month (p < 0.001).

Some of the factors which were associated with better emotional quality-of-life were similar to those identified in previous studies in patients with corrected defects. Patient-reported limitation in sports participation is common. In addition to corrective surgery and exercise, numerous other interventions which are practicable during surgical missions might improve emotional quality-of-life.

The standard transthoracic echocardiography has some limitations in emergent and community-based situations. The emergence of pocket-sized ultrasound has led to influential advancements.

In this prospective study, in the hospital-based phase, children with suspected structural heart diseases were enrolled. In the school-based phase, healthy children were randomly selected from six schools. All individuals were examined by experienced operators using both the standard and the pocket-sized echocardiography.

A total of 73 individuals with a mean age of 9.9 ± 3.2 years in the hospital-based cohort and 143 individuals with a mean age of 12.8 ± 2.9 years in the school-based cohort were examined. The agreements between the standard and the pocket-sized echocardiography were good or excellent for major CHDs in both cohorts (κ statistics > 0.61). Among valvular pathologies, agreements for tricuspid and pulmonary valves’ regurgitation were moderate among school-based cohorts (0.56 [95% confidence interval 0.12–1] and 0.6 [95% confidence interval 0.28–0.91], respectively). The agreements for tricuspid and pulmonary valves’ regurgitation were excellent (>0.9) among hospital-based population. Other values for valvular findings were good or excellent. The overall sensitivity and specificity were 87.5% (95% confidence interval 47.3–99.7) and 93.8% (95% confidence interval 85–98.3) among the hospital-based individuals, respectively, and those were 88% (95% confidence interval 77.8–94.7) and 68.4% (95% confidence interval 56.7–78.6) among the school-based individuals, respectively. The cost of examination was reduced by approximately 70% for an individual using the pocket-sized device.

When interpreted by experienced operators, the pocket-sized echocardiography can be used as screening tool among school-aged population.

Ventilation-associated changes in blood carbon dioxide levels are associated with various physiological changes in infants undergoing surgery. Studies on the effects of mechanical ventilation on cerebral haemodynamics especially for infants with CHD are scarce.

This study was done to compare the changes in regional cerebral oxygen saturation and cerebral blood flow velocity when the end-tidal carbon dioxide partial pressure changed during different minute ventilation settings in infants undergoing ventricular septal defect repair.

A total of 67 patients less than 1 year old with ventricular septal defect were enrolled, and 65 patients (age: 6.7 ± 3.4 months, weight: 6.4 ± 1.5 kg) were studied. After anaesthesia induction and endotracheal intubation, the same mechanical ventilation mode (The fraction of inspired oxygen was 50%, and the inspiratory-to-expiratory ratio was 1:1.5.) was adopted. The end-tidal carbon dioxide partial pressure of 30 mmHg (T1), 35 mmHg (T2), 40 mmHg (T3), or 45 mmHg (T4) were obtained, respectively, by adjusting tidal volume and respiratory rate. Minute ventilation per kilogram was calculated by the formula: minute ventilation per kilogram = tidal volume * respiratory rate/kg. Regional cerebral oxygen saturation was monitored by real-time near-infrared spectroscopy. Cerebral blood flow velocity (systolic flow velocity, end-diastolic flow velocity, and mean flow velocity), pulsatility index, and resistance index were measured intermittently by transcranial Doppler. Systolic pressure, diastolic pressure, stroke volume index, and cardiac index were recorded using the pressure recording analytical method.

As the end-tidal carbon dioxide partial pressure increased from 30 to 45 mmHg, regional cerebral oxygen saturation increased significantly from 69 ± 5% to 79 ± 4% (p < 0.001). Cerebral blood flow velocity (systolic flow velocity, end-diastolic flow velocity, and mean flow velocity) increased linearly, while pulsatility index and resistance index decreased linearly from T1 (systolic flow velocity, 84 ± 19 cm/second; end-diastolic flow velocity, 14 ± 4 cm/second; mean flow velocity, 36 ± 10 cm/second; pulsatility index, 2.13 ± 0.59; resistance index, 0.84 ± 0.12) to T4 (systolic flow velocity, 113 ± 22 cm/second; end-diastolic flow velocity, 31 ± 6 cm/second; mean flow velocity, 58 ± 11 cm/second; pulsatility index, 1.44 ± 0.34; resistance index, 0.72 ± 0.07) (p < 0.001). There were significant differences in changes of systolic flow velocity, end-diastolic flow velocity, mean flow velocity, pulsatility index, and resistance index as the end-tidal carbon dioxide partial pressure increased from 30 to 45 mmHg between subgroups of infants ≤6 and infants >6 months, while the changes of regional cerebral oxygen saturation between subgroups were not statistically different. Regional cerebral oxygen saturation and cerebral blood flow velocity (systolic flow velocity, end-diastolic flow velocity, and mean flow velocity) were negatively correlated with minute ventilation per kilogram (r = −0.538, r = −0.379, r = −0.504, r = −0.505, p < 0.001). Pulsatility index and resistance index were positively related to minute ventilation per kilogram (r = 0.464, r = 0.439, p < 0.001). The diastolic pressure was significantly reduced from T1 (41 ± 7 mmHg) to T4 (37 ± 6 mmHg) (p < 0.001). There were no significant differences in systolic pressure, stroke volume index, and cardiac index with the change of end-tidal carbon dioxide partial pressure from T1 to T4 (p = 0.063, p = 0.382, p = 0.165, p > 0.05).

A relative low minute ventilation strategy increases regional cerebral oxygen saturation and cerebral blood flow, which may improve cerebral oxygenation and brain perfusion in infants undergoing ventricular septal defect repair.

Congenital heart disease is the most frequent malformation in newborns. The postoperative mortality of these patients can be assessed with the Risk Adjustment in Congenital Heart Surgery-1 (RACHS-1) score. This study evaluates whether the RACHS-1 score can also be used as a predictor for the length of postoperative ventilation and what is the influence of age.

In a retrospective study over the period from 2007 to 2013, all patient records were evaluated: 598 children with congenital heart disease and cardiac surgery were identified and 39 patients have been excluded because of additional comorbidities. For evaluation of mortality, 559 patients could be analysed, after exclusion of 39 deceased patients, 520 cases remained for analysis of postoperative ventilation.

Overall mortality was 7% with a dependency on RACHS-1 categories. The median length of postoperative ventilation rose according to the RACHS-1 categories: RACHS-1 category 1: 9 hours (interquartile range (IQR) 7–13 hours), category 2: 30 hours (IQR 12–85 hours), category 4: 58 hours (IQR 13–135 hours), category 4: 71 hours (IQR 29–165 hours), and category 6: 189 hours (IQR 127–277 hours). Some of the RACHS-1 subgroups differed significantly from the categories, especially the repair of tetralogy of Fallot with a longer ventilation time and strong variability. Younger age was an independent factor for longer postoperative ventilation.

RACHS-1 is a good predictor for the length of postoperative ventilation after cardiac surgery with the exception of some subgroups. Younger age is another independent factor for longer postoperative ventilation. These data provide better insight into ventilation times and allow better planning of operations in terms of available intensive care beds.

Development of pulmonary arteriovenous fistulas in patients with cavopulmonary anastomosis may result in a significant morbidity. Although the use of bubble contrast echocardiography with selective injection into both the branch pulmonary arteries in identifying pulmonary arteriovenous fistulas has been increasing, the actual efficacy of this diagnostic modality has not been properly evaluated. Thus, this study aimed to assess the efficacy of bubble contrast echocardiography in detecting pulmonary arteriovenous fistulas in children with total cavopulmonary connection.

A total of 140 patients were included. All patients underwent cardiac catheterisation. Bubble contrast echocardiographic studies were performed by injecting agitated saline solution into the branch pulmonary arteries. Transthoracic echocardiograms that use an apical view were conducted to assess the appearance of bubble contrast in the systemic ventricles. Then, the contrast echocardiogram results and other cardiac parameters were compared.

No correlation was found between contrast echocardiogram grade and other cardiac parameters, such as pulmonary capillary wedge saturation and pulmonary artery resistance. Moreover, only 13 patients had negative results on both the right and left contrast echocardiograms, and 127 of the 140 patients had positive results on contrast echocardiograms even though they had normal pulmonary capillary wedge saturation. Results showed that bubble contrast echocardiography was a highly sensitive method and was likely to obtain false-positive results.

Bubble contrast echocardiography might be highly false positive in detecting pulmonary arteriovenous fistulas in patients with cavopulmonary anastomosis. We have to consider how we make use of this method. Further standardisation of techniques is required.

Cardiac surgical interventions for children with trisomy 18 and trisomy 13 remain controversial, despite growing evidence that definitive cardiac repair prolongs survival. Understanding quality of life for survivors and their families therefore becomes crucial. Study objective was to generate a descriptive summary of parental perspectives on quality of life, family impact, functional status, and hopes for children with trisomy 18 and trisomy 13 who have undergone heart surgery.

A concurrent mixed method approach utilising PedsQL™ 4.0 Generic Core Parent Report for Toddlers or the PedsQL™ Infant Scale, PedsQL™ 2.0 Family Impact Module, Functional Status Scale, quality of life visual analogue scale, and narrative responses for 10 children whose families travelled out of state to access cardiac surgery denied to them in their home state due to genetic diagnoses.

Parents rated their child’s quality of life as 80/100, and their own quality of life as 78/100 using validated scales. Functional status was rated 11 by parents and 11.6 by providers (correlation 0.89). On quality of life visual analogue scale, all parents rated their child’s quality of life as “high” with mean response 92.7/100. Parental hopes were informed by realistic perspective on prognosis while striving to ensure their children had access to reaching their full potential. Qualitative analysis revealed a profound sense of the child’s relationality and valued life meaning.

Understanding parental motivations and perceptions on the child’s quality of life has potential to inform care teams in considering cardiac interventions for children with trisomy 18 and trisomy 13.

To evaluate the feasibility, efficacy, and safety of Amplatzer vascular plug II in large and elongated ducts in infants.

Patent arterial duct device closure is technically challenging in infants with large and elongated ducts because Amplatzer duct occluder and Amplatzer duct occluder II have high chances of causing aortic coarctation and left pulmonary artery stenosis, respectively. The Amplatzer vascular plug II being soft with no retention discs on either sides helps in mitigating these problems.

This is a prospective, observational study involving infants with clinical, echocardiographic and angiographic evidence of large left to right shunt. All the children underwent duct closure using Amplatzer vascular plug II.

Eighteen infants qualified for the study. Mean age and weight were 8.63 ± 3.84 months and 6.3 ± 1.7 kg, respectively. The angiographic mean duct diameter at the pulmonary artery end was 4.66 ± 0.92 mm, and the mean duct length was 9.4 ± 2.48 mm. The size of Amplatzer vascular plug II used varied from 6 mm to 10 mm. Technical success was achieved in 16/18 cases. One patient had device embolisation, and in the other, the device was found to be unstable. The ratio of Amplatzer vascular plug II size to the duct diameter was 1.65 ± 0.27, while the ratio of ductal length to device length was 1.48 ± 0.46 in those with successful outcome.

Amplatzer vascular plug II is a safe and effective option in appropriately selected infants with elongated ducts. Diameter and length of Amplatzer vascular plug II vis-a-vis those of the ductus are important determinants of the successful outcome.

There is a paucity of functional data on mid-to-late preterm infants between 30+0 and 34+6 weeks gestation. We aimed to characterise transitional cardiopulmonary and haemodynamic changes during the first 48 hours in asymptomatic mid-to-late preterm infants.

Forty-five healthy preterm newborns (mean ± standard deviation) gestation of 32.7 ± 1.2 weeks) underwent echocardiography on Days 1 and 2. Ventricular mechanics were assessed by speckle tracking-derived deformation, rotational mechanics, tissue Doppler imaging, and right ventricle-focused measures (tricuspid annular plane systolic excursion, fractional area change). Continuous haemodynamics were assessed using the NICOM™ system to obtain left ventricular output, stroke volume, heart rate, and total peripheral resistance by non-invasive cardiac output monitoring.

Right ventricular function increased (all measures p < 0.005) with mostly stable left ventricular performance between Day 1 and Day 2. NICOM-derived left ventricular output [mean 34%, 95% confidence interval 21–47%] and stroke volume [29%, 16–42%] increased with no change in heart rate [5%, −2 to 12%]. There was a rise in mean blood pressure [11%, 1–21%], but a decline in total peripheral resistance [−14%, −25 to −3%].

Left ventricular mechanics remained persevered in mid-to-late premature infants, but right ventricular function increased. Non-invasive cardiac output monitoring is feasible in preterm infants with an increase in left ventricular output driven by an improvement in stroke volume during the transitional period.

Stenting of ostial pulmonary artery stenosis presents several unique challenges. These include difficulty in defining anatomy and need for precise stent placement in order to avoid missing the ostial stenosis or jailing either the contralateral branch pulmonary artery or the ipsilateral upper lobe branch.

A retrospective review of outcomes was conducted in 1.5 or 2-ventricle patients who underwent stent placement for ostial branch pulmonary artery stenosis. Specific catheterisation lab techniques were reviewed.

Forty-seven branch pulmonary arteries underwent stent placement for ostial stenosis in 43 patients. The median age and weight were 3.7 (0.3–18.1) years and 14.2 (5.6–70.0) kg, respectively. Three (2–8) angiographic projections were needed to profile the ostial stenosis. Open-cell stents were used in 23 and stents were modified in 5 cases. Following stent implantation, the minimum diameter improved from 3.6 (0.8–10.5) to 8.1 (4.2–16.5) mm (p < 0.001). The gradient improved from 21 (0–66) to 4 (0–27) mmHg (p < 0.001). Stent malposition occurred in eight (17%) of the stents placed. Five migrated distally causing suboptimal ostial coverage necessitating placement of a second stent in four. Three migrated proximally and partially jailed the contralateral pulmonary artery. Intentional jailing of the upper lobe branch occurred in four additional cases. At a follow-up of 2.4 (0.3–4.9) years, 15 stents underwent further dilation and 1 had a second stent placed within the exiting stent.

Ostial branch pulmonary artery stenosis may require additional angiography to accurately define the ostial stenosis. Treatment with stents is effective but carries high rates of stent malposition.