To save this undefined to your undefined account, please select one or more formats and confirm that you agree to abide by our usage policies. If this is the first time you used this feature, you will be asked to authorise Cambridge Core to connect with your undefined account.
Find out more about saving content to .
To save this article to your Kindle, first ensure firstname.lastname@example.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below.
Find out more about saving to your Kindle.
Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.
Early surgical intervention in infants with complex CHD results in significant disruptions to their respiratory, gastrointestinal, and nervous systems, which are all instrumental to the development of safe and efficient oral feeding skills. Standardised assessments or treatment protocols are not currently available for this unique population, requiring the clinician to rely on knowledge based on neonatal literature. Clinicians need to be skilled at evaluating and analysing these systems to develop an appropriate treatment plan to improve oral feeding skill and safety, while considering post-operative recovery in the infant with complex CHD. Supporting the family to re-establish their parental role during the hospitalisation and upon discharge is critical to reducing parental stress and oral feeding success.
The paediatric post-cardiac catheterisation Wrap (Wrap), an innovative medical safety device, swaddles young paediatric patients in a supine position aiding in immobilisation post-cardiac catheterisation. This pilot study investigated the feasibility and safety of using the Wrap on young paediatric patients during their bed rest period following cardiac catheterisation with femoral access.
Boston Children’s Hospital Cardiac Catheterization Lab.
20 patients, ages 1–5 years and weighing 3–25 kg.
Investigator-developed tools used to collect data included the Demographic and Outcome Measures Data Tool, the Parent/Caregiver Satisfaction, and Provider Ease of Use tools. They measured:
1. The feasibility of using the Wrap
2. Wrap ease of use from the nurse providers’ perspective
3. Parent satisfaction related to the Wrap
4. Frequency of Wrap non-bleeding-related adverse events
5. Frequency of rebleeding at femoral groin access sites
The Wrap was feasible and safe; increased nurse provider satisfaction by allowing visualisation of the groin access sites while minimising the need for hands-on care; and increased parent satisfaction by allowing parents to hold and provide comfort while their child was on bed rest.
Implications for Research:
The Wrap is a safe alternative to the current practice of swaddling with a bath blanket. Further studies are warranted to assess the Wrap’s effectiveness in reducing the incidence of rebleeding events in the post-cardiac catheterisation period and explore clinical use outside of the Cardiac Catheterization Lab.
Pulmonary insufficiency requiring reintervention frequently occurs after primary tetralogy of Fallot repair. Repeat interventions present a challenge for both the surgeon and patient. We compare a minimally invasive, 5 cm left anterior mini-incision to redo median sternotomy for pulmonary valve replacement in tetralogy of Fallot patients.
Following Internal Review Board approval, we conducted a single institution retrospective review of patients with tetralogy of Fallot who underwent pulmonary valve replacement via redo median sternotomy or left anterior mini-incision between 13 July, 2016 and 6 March, 2020.
Twenty-three patients underwent pulmonary valve replacement following primary tetralogy of Fallot repair between March 2016 and March 2020. Twelve patients received a redo-median sternotomy from March 2016 to August 2018. Left anterior mini-incision was first offered in August of 2018 and was chosen by all eleven patients thereafter. The two groups had similar baseline characteristics including preoperative pulmonary valve dysfunction. Early trends suggest a longer cardiopulmonary bypass time for patients who received left anterior mini-incisions. Other outcomes were comparable, including operative times, blood product requirements, residual pulmonary valve dysfunction, postoperative pain, narcotic requirements, ICU length of stay, total length of stay, and postoperative complications.
In patients who have previously undergone primary repairs of tetralogy of Fallot, outcomes for pulmonary valve replacement via left anterior mini-incision are comparable to those via redo median sternotomy.
Two-dimensional speckle tracking echocardiography-derived left ventricular longitudinal systolic strain is an important myocardial deformation parameter for assessing the systolic function of the left ventricle. Strain values differ according to the vendor machine and software. This study aimed to provide normal reference values for global and regional left ventricular longitudinal systolic strain in Egyptian children using automated functional imaging software integrated into the General Electric healthcare machine and to study the correlation between the global longitudinal left ventricular systolic strain and age, body size, vital data, and some echocardiographic parameters.
Healthy children (250) aged from 1 to 16 years were included. Conventional echocardiography was done to measure the left ventricular dimensions and function. Automated functional imaging was performed to measure the global and regional peak longitudinal systolic strain.
The global longitudinal strain was −21.224 ± 1.862%. The regional strain was −20.68 ± 2.11%, −21.06 ± 1.84%, and −21.86 ± 2.71% at the basal, mid, and apical segments, respectively. The mean values of the systolic longitudinal strain become significantly more negative from base to apex. Age differences were found as regard to global and regional longitudinal strain parameters but no gender differences. The global peak longitudinal systolic strain correlated positively with age. No correlations were found with either the anthropometric parameters or the vital data.
Age-specific normal values for two-dimensional speckle tracking-derived left ventricular longitudinal regional and global systolic strain are established using automated functional imaging.
Owing to its obvious cosmetic appeal, minimal invasive repair of congenital heart defects (CHDs) through the mini right axillary thoracotomy is becoming routine in many centres. Besides cosmesis, and before becoming a new norm, it is important to establish its outcomes as safe compared to repairs through traditional median sternotomy.
Between 2013 and 2021, 116 consecutive patients underwent defect repairs through mini right axillary thoracotomy. Patient, operative data, and hospital outcomes were compared to contemporary mini right axillary thoracotomy and sternotomy series.
There was no mortality or need for approach conversion (mean age 4.3 years, range 0.17–17, mean weight 18.6 kg, range 4.8–74.4) in 118 repairs for atrial septal defect, ventricular septal defect, partial anomalous pulmonary venous return, partial atrioventricular canal with mitral cleft, scimitar syndrome, double-chambered right ventricle, cor triatriatum, and tricuspid valve repair. Protocol included on-table extubation, achieved in 97 children, with 23 outliers leading to 0.7 average hours of mechanical ventilation (range 0–66 hours), indwelling chest drain time of 2.6 days (range 1–9 days), intensive care stay of 1.8 days (range 1–10 days), and hospital stay of 3.9 days (range 2–18 days). Late revisions were required in one patient after scimitar repair for scimitar vein stenosis at 2 weeks, and in another for repair of superior caval vein stenosis after a Warden operation at 2 months; reoperations (5/116 = 4.3%) were successfully performed through the same mini right axillary incision.
While providing obvious cosmetic advantages, the minimally invasive right axillary thoracotomy approach for the surgical repair of common CHDs yields excellent results and is safe compared to the benchmark median sternotomy approach.
Survival after paediatric in-hospital cardiac arrest is worse on nights and weekends without demonstration of disparity in cardiopulmonary resuscitation quality. It is unknown whether these findings differ in children with CHD. This study aimed to determine whether cardiopulmonary resuscitation quality might explain the hypothesised worse outcomes of children with CHD during nights and weekends.
In-hospital cardiac arrest data collected by the Pediatric Resuscitation Quality Collaborative for children with CHD. Chest compression quality metrics and survival outcomes were compared between events that occurred during day versus night, and during weekday versus weekend using multivariable logistic regression.
We evaluated 3614 sixty-second epochs of chest compression data from 132 subjects between 2015 and 2020. There was no difference in chest compression quality metrics during day versus night or weekday versus weekend. Weekday versus weekend was associated with improved survival to hospital discharge (adjusted odds ratio 4.56 [1.29,16.11]; p = 0.02] and survival to hospital discharge with favourable neurological outcomes (adjusted odds ratio 6.35 [1.36,29.6]; p = 0.02), but no difference with rate of return of spontaneous circulation or return of circulation. There was no difference in outcomes for day versus night.
For children with CHD and in-hospital cardiac arrest, there was no difference in chest compression quality metrics by time of day or day of week. Although there was no difference in outcomes for events during days versus nights, there was improved survival to hospital discharge and survival to hospital discharge with favourable neurological outcome for events occurring on weekdays compared to weekends.
Paediatric ICUs have shared the burden of the COVID-19 pandemic, including subspecialty cardiac ICUs. We sought to address knowledge gaps regarding patient characteristics, acuity, and sequelae of COVID-19 in the paediatric cardiac ICU setting.
Retrospective review of paediatric cardiac ICU admissions with COVID-19-related disease.
Single centre tertiary care paediatric cardiac ICU.
All patients with PCR/antibody evidence of primary COVID-19 infection, and/or Multisystem Inflammatory Syndrome in Children, were admitted between 26 March, 2020 and 31 March, 2021.
Main outcomes measures:
Patient-level demographics, pre-existing conditions, clinical symptoms, and outcomes related to ICU admission were captured from medical records.
Among 1064 patients hospitalised with COVID-19/Multisystem Inflammatory Syndrome in Children, 102 patients (9.5%) were admitted to cardiac ICU, 76 of which were symptomatic (median age 12.5 years [IQR 7.5–16.0]). The primary system involved at presentation was cardiovascular in 48 (63%). Vasoactive infusions were required in 62% (n = 47), with eight patients (11%) requiring VA ECMO. Severity of disease was categorised as mild/moderate in 16 (21%) and severe/critical in 60 patients (79%). On univariate analysis, African-American race, presentation with gastrointestinal symptoms or elevated inflammatory markers were associated with risk for severe disease. All-cause death was observed in five patients (7%, n = 5/72) with four patients remaining hospitalised at the time of data query.
COVID-19 and its cardiovascular sequelae were associated with important morbidity and significant mortality in a notable minority of paediatric patients admitted to a paediatric cardiac ICU. Further study is required to quantify the risk of morbidity and mortality for COVID-19 and sequelae.
This study aimed to examine the effects of theophylline use in preventing renal dysfunction in patients undergoing CHD surgery.
A total of 94 patients aged 1–60 months were included in the study. Patients in the theophylline group were enrolled according to a pre-defined protocol for treatment administration, while controls were selected retrospectively from patients without theophylline treatment during the same period – who were matched according to critical baseline characteristics.
The incidence of acute kidney injury was similar between the two groups (p = 1.000). Higher urinary output and lower fluid balance were found intraoperatively and also postoperatively in the theophylline group (all, p < 0.050). Postoperative decrease in urinary output and estimated glomerular filtration rate were higher in the theophylline group (p < 0.050). Lower postoperative urea and creatinine levels were shown in theophylline recipients (p < 0.050). Urea levels increased significantly in the non-theophylline group during surgery (p < 0.001), and no significant change was observed in theophylline group (p = 0.136). Postoperative increase in creatinine and lactate levels was demonstrated in theophylline group (p < 0.050), and lactate levels were higher in the non-theophylline group during and after cardiopulmonary bypass (p = 0.010). Multiple linear regression analysis revealed less reduction in estimated glomerular filtration rate with higher age and in the presence of theophylline use (p < 0.050).
Although we demonstrated a similar incidence of acute kidney injury in the both groups, we revealed an important decrease in serum creatinine, urea and lactate levels, accompanied by improved estimated glomerular filtration rate, increased urine output and decreased fluid overload, with theophylline treatment, suggesting that renal functions significantly improved with the use of theophylline.
First-year cardiology fellows must quickly learn basic competency in echocardiography during fellowship orientation. This educational process was disrupted in 2020 due to the coronavirus pandemic, as our hands-on echocardiography teaching transitioned from practice on paediatric volunteers to simulation-based training. We previously described an improvement in echocardiographic completeness after implementation of a standardised imaging protocol for the performance of acute assessments of ventricular function. Herein, we assessed whether this improvement could be sustained over the two subsequent years, including the fellowship year affected by the pandemic. Echocardiograms performed by first-year paediatric cardiology fellows to assess ventricular function were reviewed for completeness. The frequency with which each requested component was included was measured. A total demographic score (out of 7) and total imaging score (out of 23) were calculated. The pre-protocol years (2015–2017) were compared to the post-protocol years (2018–2020), and the pre-COVID years (2018–2019) were compared to the year affected by COVID (2020). There was a sustained improvement in completeness after protocol implementation with improvement in the demographic score (median increasing from 6 to 7, p < 0.001) and imaging score (median increasing from 13 to 16, p < 0.001). More individual components showed a statistically significant increase in frequency compared to our prior publication. The COVID pandemic resulted in very few differences in completeness. Demographic reporting improved modestly (p = 0.04); the imaging score was unchanged (p = 0.59). The only view obtained less frequently was the apical two-chamber view. A standardised imaging protocol allowed sustained improvements in echocardiographic completeness despite the disruption of fellowship orientation by COVID-19.
Constrictive pericarditis is uncommon in children. It results from scarring and consequent loss of the normal elasticity of the pericardial sac and is most commonly seen as a late sequelae of idiopathic or viral pericarditis. Here, we report a case of protein losing enteropathy as a complication of constrictive pericarditis in a 2-year-old child. Pericardial thickening was demonstrated by cardiac MRI and subsequent pericardiectomy led to remarkable improvement and resolution of protein losing enteropathy.
To characterise the use of peripherally inserted central catheters in paediatric cardiac patients and to identify risk factors associated with their complications.
Materials and Methods:
Observational retrospective cohort study in paediatric cardiac patients who underwent peripherally inserted central catheter placement in a tertiary children’s hospital from January 2000 to June 2018.
1822 cardiac patients underwent 2952 peripherally inserted central catheter placements in the study period. Median age was 29 days, with survival to hospital discharge of 96.4%. Successful placement achieved 94.5% of attempts, with a median line duration of 12 days. Factors associated with successful placement were the use of general anaesthesia (odds ratio 7.52, p < 0.001) and year of placement (odds ratio 1.08, p < 0.001). The incidence of complications was 28.6%, with thrombosis/occlusion being the most frequent (33%). Thrombosis/occlusion were associated with two and three lumens (odds ratio 1.96, p < 0.001 and 4.63, p = 0.037, respectively). Lines placed by interventional radiology had decreased infiltration (odds ratio 0.20, p = 0.002) and lower migration/malposition (odds ratio 0.36, p < 0.001). The use of maintenance intravenous fluids (odds ratio 3.98, p = 0.008) and peripheral tip position (odds ratio 3.82, p = 0.001) were associated with increased infiltration. The probability of infection decreased over time (odds ratio 0.79, p < 0.001).
Peripherally inserted central catheters in paediatric cardiac patients have complication rates similar to other paediatric populations. A prospective assessment of the factors associated with their complications in this patient population may be beneficial in improving outcomes.
Propionic acidemia is a rare autosomal recessive inborn error of metabolism. It is relatively common in Middle East. Dilated cardiomyopathy is one of the leading causes of morbidity and mortality for patients with propionic acidemia. Liver transplantation has been used for patient with frequent metabolic decompensations and was shown to be beneficial in propionic acidemia-related dilated cardiomyopathy. Up to our knowledge, there has been one reported case of recurrent dilated cardiomyopathy 3 years after liver transplantation. We report the first case, from Middle East, of recurrent dilated cardiomyopathy, 6 years after liver transplantation.
The timing of pulmonary valve replacement in patients with pulmonary regurgitation following treatment of pulmonary stenosis is undefined. Although cardiac magnetic resonance-based right ventricular volumes in tetralogy of Fallot patients have been used as a guide in pulmonary stenosis patients, anatomic differences between tetralogy of Fallot and pulmonary stenosis patients complicate their application to pulmonary stenosis patients and could result in late referral for pulmonary valve replacement. We sought to determine if pulmonary stenosis patients referred for pulmonary valve replacement were at greater risk for morbidity or need for tricuspid valve intervention at the time of pulmonary valve replacement. A retrospective cohort study was performed on all adult patients with a diagnosis of pulmonary stenosis or tetralogy of Fallot followed at our centre. Clinical and imaging-based exposures were collected. Pre-specified endpoints included need for concomitant tricuspid valve repair or replacement and pre- and post-pulmonary valve replacement cardiac magnetic resonance-based volumetric measurements. Between 1/1999 and 1/2020, 235 patients underwent pulmonary valve replacement for pulmonary regurgitation (52 with pulmonary stenosis, 183 with tetralogy of Fallot). Pulmonary stenosis patients were more likely to have at least moderate tricuspid regurgitation (p = 0.010), undergo concomitant tricuspid valve intervention (p = 0.003), and require tricuspid valve repair or replacement secondary to annular dilation (p = 0.027) compared to tetralogy of Fallot patients. There was no difference in pre-pulmonary valve replacement right ventricular size between pulmonary stenosis and tetralogy of Fallot patients. These findings suggest that referral for pulmonary valve replacement may be occurring later in the disease course for pulmonary stenosis patients.
Anomalous right coronary artery from pulmonary artery (ARCAPA) is a rare congenital heart disease that can lead to abnormal coronary perfusion and a need for surgical repair. Here, we report the outcomes of patients who underwent ARCAPA surgery within the Pediatric Cardiac Care Consortium (PCCC), a North American registry of interventions for paediatric heart diseases. We queried the PCCC for patients undergoing surgical repair for ARCAPA at <18 years of age between 1982 and 2003. Outcomes were obtained from the PCCC and after linkage with the National Death Index (NDI) and the Organ Procurement and Transplantation Network (OPTN) through 2019. Twenty-four patients (males: 15) were identified having surgery for ARCAPA at a median age of 5.8 (IQR 2.7–10.3) years. Of them, 23 cases were considered “simple” (without major intracardiac disease) and one “complex” (co-existing with tetralogy of Fallot). Five patients presented with symptoms [chest pain (1), dyspnoea on exertion (2) or history of syncope (2)]; while the remaining 19 patients were referred for evaluation of either murmur or co-existing CHD. There was no in-hospital mortality after the surgical repair. Fourteen patients had sufficient identifiers for NDI/OPTN linkage; among them, only one death occurred from unrelated non-cardiac causes within a median period of 19.4 years of follow-up (IQR: 18–24.6). Outcomes were excellent after reimplantation up to 25 years later and further longitudinal monitoring is important to understand the interaction of pre-existing coronary pathology with the effects of ageing.
CHD is an important phenotypic feature of chromosome 22q11.2 copy number variants. Biventricular repair is usually possible, however there are rare reports of patients with chromosome 22q copy number variants and functional single ventricle cardiac disease.
This is a single centre retrospective review of patients with chromosome 22q copy number variants who underwent staged single ventricle reconstructive surgery between 1 July, 1984 and 31 December, 2020.
Seventeen patients met inclusion criteria. The most common diagnosis was hypoplastic left heart syndrome (n = 8) and vascular anomalies were present in 13 patients. A microdeletion of the chromosome 22 A-D low-copy repeat was present in 13 patients, and the remaining had a duplication. About half of the patients had documented craniofacial abnormalities and/or hypocalcaemia, and developmental delay was very common. Fifteen patients had a Norwood operation, 10 patients had a superior cavopulmonary anastomosis, and 7 patients had a Fontan. Two patients had cardiac transplantation after Fontan. Overall survival is 64% at 1 year, and 58% at 5 and 10 years. Most deaths occurred following Norwood operation (n = 5).
CHD necessitating single ventricle reconstruction associated with chromosome 22q copy number variants is not common, but typically occurs as a variant of hypoplastic left heart syndrome with the usual cytogenetic microdeletion. The most common neonatal surgical intervention performed is the Norwood, where most of the mortality burden occurs. Associated anomalies and medical issues may cause additional morbidity after cardiac surgery, but survival is similar to infants with other types of single ventricle disease.
This study aimed to provide baseline information on the potential role of salivary cortisol in reflecting the stress response in children undergoing congenital heart surgery.
Patients and methods:
Children underwent congenital cardiac surgery, aged between one and seventeen years were included. Saliva samples were collected pre- and postoperatively by the health caregiver immediately after the children woke up (07:00–09:00 am) and at 06:00 pm in the evening. Salivary cortisol levels were compared with the reference index values from a large database.
Median baseline preoperative morning salivary cortisol levels were significantly lower than the reference values in both < 5-year-old females (p = 0.01) and males (p = 0.04) and in males between 11 and 20 years of age (p = 0.01). Median baseline preoperative evening salivary cortisol levels were significantly higher than the reference value in < 5-year-old females (p = 0.01) and between 5 and 10 years of age (p = 0.04) and in between 11- and20-year-old males (p = 0.01). Median postoperative morning salivary cortisol levels were significantly lower than the reference value in both < 5-year-old females (p = 0.01) and males (p = 0.04) and females between 5 and 10 year of age (p = 0.04). Median postoperative evening salivary cortisol levels were significantly higher than the reference value in < 5-year-old females (p = 0.01) and between 5- and 10-year-old females (p = 0.04).
Diurnal variability of salivary cortisol levels in children undergoing congenital heart surgery may be different from normal reference values both in preoperative and postoperative periods that can be a predictive indicator of anxiety on pre- and postoperative period for children that undergoing cardiac surgery.
Patent ductus arteriosus is an important cause of morbidity and mortality, especially in very low birth weight infants. The aim of the study is to report our single-centre short-term results of preterm patients who underwent ligation through left anterior mini-thoracotomy .
Data of 27 preterm infants operated by the same surgeon who underwent Patent ductus arteriosus (PDA) closure with left anterior mini-thoracotomy technique between November 2020 and January 2022 at a single institution were reviewed. The patients were divided into two groups according to their weight at the time of surgery. Data on early postoperative outcomes and survival rates after discharge were collected.
Twenty-seven patients with a mean (±SD) gestational age of 25.8 (±2.0) weeks and a mean birth weight of 1027 (±423) g were operated using left anterior mini-thoracotomy technique. The lowest body weight was 480 g. Complications such as bleeding, abnormal healing of incision, or pneumothorax were not seen. There were 8 mortalities after the operation (29,6 %). The causes of the deaths were sepsis, necrotising enterocolitis, hydrops fetalis, hepatoblastoma, and intracranial bleeding. There was no statistically significant difference in the rates of complication between the groups.
Left anterior mini-thoracotomy technique can be performed as the first choice when transcatheter intervention cannot be applied in preterm infants. It provides easy access to the PDA, a good exposure, minimal contact with the lungs, good cosmetic results in early and mid-term and shortens the operation time, especially in very low birth weight preterm babies. However, early ligation may be helpful to minimise the complications related to PDA.
We reported a case of ischaemic stroke with moyamoya disease with simultaneous occurrence of patent foramen ovale. The patient underwent percutaneous closure of patent foramen ovale and was scheduled for follow-up.
Scimitar syndrome is a congenital anomaly in which some or all of right pulmonary veins drain into inferior caval vein. It is associated with anomalous systemic arteries arising from descending aorta supplying to right lung. Transcatheter embolisation of this artery prevents complications. We present a 2.5-year-old girl in which anomalous artery was embolised using Amplatzer PiccoloTM Occluder, for first time.