Review
Neurodevelopment outcomes in the first 5 years of the life of children with transposition of the great arteries surgically corrected in the neonatal period: systematic review and meta-analysis
- Catarina Soares, Rafael José Vieira, Sandra Costa, Rita Moita, Mariana Andrade, Hercília Guimarães
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- Published online by Cambridge University Press:
- 15 November 2023, pp. 2471-2480
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Congenital Heart Defects are the most common abnormalities at birth, resulting in many short- and long-term consequences.
Objectives:In patients with transposition of the great arteries, surgical correction may achieve definitive treatment, so a thorough knowledge of the long-term outcomes, particularly neurodevelopment outcomes, is essential. Therefore, we conducted a systematic review and meta-analysis to study the neurodevelopment outcomes in the first 5 years of the life of children submitted to corrective surgery for transposition of the great arteries in the neonatal period.
Methods:A total of 17 studies from 18 reports were included, assessing 809 individuals with surgically corrected transposition of the great arteries. The neurodevelopmental outcomes were assessed with the Bayley Scales of Infant and Toddler Development (BSID) and the Wechsler Intelligence Scale for Children (WISC).
Results:Mean Mental Development Index (MDI) and Psychomotor Development Index (PDI) were within the average values from 1 to 3 years of age, although the proportion of children scoring more than 1 standard deviation below the mean in PDI, MDI, motor, and language composite scores was significantly higher than in the general population. From 4 to 5 years, mean full-scale global intelligence quotient (IQ), verbal IQ, and performance IQ scores did not differ significantly from the general population.
Conclusion:This study revealed neurodevelopment scores within the normal range at 5 years of age in children submitted to corrective surgery for transposition of the great arteries in the neonatal period. However, these early outcomes may not adequately predict long-term outcomes. Further studies are needed to identify specific risk factors and early markers of later impairment to guide the establishment of early interventions.
Original Article
Serum-soluble suppression of tumourigenicity-2 as a biomarker in children with congestive heart failure
- Eslam Amer, Doaa El Amrousy, Sahar Hazaa, Amr Zoair
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- Published online by Cambridge University Press:
- 13 March 2023, pp. 2481-2486
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Background:
We aimed to evaluate serum soluble suppression of tumorigenicity-2 in children with congestive heart failure, to assess the diagnostic and prognostic values of soluble suppression of tumorigenicity-2 in these patients, and to correlate its levels with various clinical and echocardiographic data.
Methods:We included 60 children with congestive heart failure as the patient group. Sixty healthy children of matched age and sex served as the control group. Patients were evaluated clinically and by echocardiography. Serum level of suppression of tumorigenicity-2 was measured for patients at admission. All patients were followed up for death or readmission for a period of one year.
Results:Soluble suppression of tumorigenicity-2 was significantly higher in children with congestive heart failure as compared to the control group. Soluble suppression of tumorigenicity-2 was significantly increased in patients with higher severity of congestive heart failure. There was a significant increase in soluble suppression of tumorigenicity-2 in patients with bad prognosis compared to those with good prognosis. There was a significant positive correlation between soluble suppression of tumorigenicity-2 and respiratory rate, heart rate, and clinical stage of congenital heart failure, while there was a significant negative correlation between soluble suppression of tumorigenicity-2 and left ventricular systolic and diastolic function. The best cut-off of soluble suppression of tumorigenicity-2 to diagnose congestive heart failure was > 3.6 with 87% sensitivity and 79% specificity. The cut-off point of soluble suppression of tumorigenicity-2 to diagnose congestive heart failure in children was ≥ 31.56 ng/ml, with 95% sensitivity and 91.37% specificity. Moreover, the cut-off point of soluble suppression of tumorigenicity-2 to predict bad prognosis in children with congestive heart failure was ≥ 255.5 ng/ml, with 92% sensitivity and 89.0% specificity.
Conclusion:Soluble suppression of tumorigenicity-2 is a good diagnostic and predictive biomarker in children with congestive heart failure.
Risk factors for long post-operative hospital stays after cardiopulmonary bypass surgery in full-term neonates
- Ahmed Asfari, Santiago Borasino, Erika Mendoza, Kristal M. Hock, Jordan L. Huskey, A.K.M. Fazlur Rahman, Hayden Zaccagni, Jonathan W. Byrnes
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- Published online by Cambridge University Press:
- 16 March 2023, pp. 2487-2492
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Background:
Long hospital stays for neonates following cardiac surgery can be detrimental to short- and long-term outcomes. Furthermore, it can impact resource allocation within heart centres' daily operations. We aimed to explore multiple clinical variables and complications that can influence and predict the post-operative hospital length of stay.
Methods:We conducted a retrospective observational review of the full-term neonates (<30 days old) who had cardiac surgery in a tertiary paediatric cardiac surgery centre – assessment of multiple clinical variables and their association with post-operative hospital length of stay.
Results:A total of 273 neonates were screened with a mortality rate of 8%. The survivors (number = 251) were analysed; 83% had at least one complication. The median post-operative hospital length of stay was 19.5 days (interquartile range 10.5, 31.6 days). The median post-operative hospital length of stay was significantly different among patients with complications (21.5 days, 10.5, 34.6 days) versus the no-complication group (14 days, 9.6, 19.5 days), p < 0.01. Among the non-modifiable variables, gastrostomy, tracheostomy, syndromes, and single ventricle physiology are significantly associated with longer post-operative hospital length of stay. Among the modifiable variables, deep vein thrombosis and cardiac arrest were associated with extended post-operative hospital length of stay.
Conclusions:Complications following cardiac surgery can be associated with longer hospital stay. Some complications are modifiable. Deep vein thrombosis and cardiac arrest are among the complications that were associated with longer hospital stay and offer a direct opportunity for prevention which may be reflected in better outcomes and shorter hospital stay.
Neutrophil-to-lymphocyte ratio as a predictive and prognostic marker in children with dilated cardiomyopathy
- Mai Ahmed, Doaa El Amrousy, Hossam Hodeib, Shimaa Elnemr
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- Published online by Cambridge University Press:
- 17 March 2023, pp. 2493-2497
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Objectives:
We aimed to evaluate neutrophil-to-lymphocyte ratio in children with acute heart failure due to dilated cardiomyopathy, to assess the predictive and prognostic values of neutrophil-to-lymphocyte ratio, and to correlate its levels with brain natriuretic peptide and other various data in these patients.
Method:We included 50 children with acute heart failure due to dilated cardiomyopathy as the patient group. Fifty healthy children of matched age and sex served as the control group. Patients were evaluated clinically and by echocardiography. A complete blood count with differentiation to evaluate neutrophil-to-lymphocyte ratio was done, and the serum level of brain natriuretic peptide was also measured. All patients were followed up for death or readmission for a period of one year.
Results:Neutrophil-to-lymphocyte ratio was significantly higher in patient group as compared to the control group. Neutrophil-to-lymphocyte ratio was significantly increased in patients with higher severity of heart failure. There was a significant increase in neutrophil-to-lymphocyte ratio in patients with bad prognoses compared to those with good prognoses. There was a significant positive correlation between neutrophil-to-lymphocyte ratio and both brain natriuretic peptide and clinical stage of heart failure while there was a significant negative correlation between neutrophil-to-lymphocyte ratio and left ventricular systolic function. The best cut-off of neutrophil-to-lymphocyte ratio to predict adverse outcomes in children with dilated cardiomyopathy was >3.6 with 87% sensitivity and 79% specificity. The cut-off of neutrophil-to-lymphocyte ratio to predict patients who will not respond to conventional treatment was ≥3.85 with 85% sensitivity and 100% specificity.
Conclusion:Neutrophil-to-lymphocyte ratio is a cheap good predictive and prognostic biomarker in children with dilated cardiomyopathy.
Comparison of the effects of conventional method and primary sutureless techniques on early postoperative rhythm problems in patients with total abnormal pulmonary venous return anomaly
- Erkut Ozturk, Sezen Gulumser Sisko, Gulhan Tunca Sahin, Ibrahim Cansaran Tanıdır, Alper Guzeltas, Sertac Haydin, Ali Can Hatemi, Yakup Ergul
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- Published online by Cambridge University Press:
- 21 March 2023, pp. 2498-2503
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Background:
Total abnormal pulmonary venous return anomaly is a CHD characterised by abnormal pulmonary venous flow directed to the right atrium. In this study, we aimed to compare the effects of these techniques on early rhythm problems in total abnormal pulmonary venous return anomaly cases operated with conventional or primary sutureless techniques.
Method:Seventy consecutive cases (median age 1 month, median weight 4 kg) who underwent total abnormal pulmonary venous return anomaly repair with conventional or primary sutureless technique between May 1 2020 and May 1 2022 were evaluated. The rate, diagnosis, and possible risk factors of postoperative arrhythmias were investigated. The results were evaluated statistically.
Results:When the total abnormal pulmonary venous return anomaly subgroup of 70 cases was evaluated, 40 cases were supracardiac, 18 cases were infracardiac, 7 cases were cardiac, and 5 cases were mixed type. Twenty-eight (40%) cases had a pulmonary venous obstruction. Primary sutureless technique (57%, supracardiac n = 24, mixed = 3, infracardiac = 13) was used in 40 patients. Median cardiopulmonary bypass time (110 versus 95 minutes) and median aortic clamp time (70 versus 60 minutes), median peak lactate (4.7 versus 4.8 mmol/l) in the first 72 hours, and median peak vasoactive inotropic score in the first 72 hours of the primary sutureless and conventional technique used cases value (8 versus 10) were similar. The total incidence of arrhythmias in the conventional group was significantly higher than in the primary sutureless group (46.7% versus 22.5%, p = 0.04). Supraventricular early beat was observed in 3 (7.5%), sinus tachycardia was seen in 6 (15%), junctional ectopic tachycardia was seen in 1 (2.5%), intra-atrial reentry tachycardia was seen in 1 (2.5%), usual supraventricular tachyarrhythmia was seen in 2 cases (5%) in the primary sutureless group. In the conventional group, supraventricular early beat was observed in six of the cases (20%), sinus tachycardia in five (16.7%), junctional ectopic tachycardia in four (13.3%), intra-atrial reentry tachycardia (10%) in three, and supraventricular tachyarrhythmia in seven cases (23.3%). In the first 30 days, there was a similar mortality rate (10% versus 10%), with four patients in the primary sutureless group and three in the conventional group. The median follow-up period of the cases was 8 months (interquartile range (IQR) 6–10 months). In the follow-up, arrhythmias were detected in two cases (one supraventricular tachyarrhythmia and one intra-atrial reentry tachycardia) in the primary sutureless group and three cases (two supraventricular tachyarrhythmia, one intra-atrial reentry tachycardia) in the conventional technique. All cases were converted to normal sinus rhythm with cardioversion and combined antiarrhythmic therapy.
Conclusion:Different arrhythmias can be observed in the early period in patients with operated total abnormal pulmonary venous return anomaly. Although a higher rate of rhythm problems was observed in the early period in the conventional method compared to the primary sutureless technique, no significant effect was found on mortality and morbidity between the groups.
Haemodynamic effects of prophylactic post-operative hydrocortisone following cardiopulmonary bypass in neonates undergoing cardiac surgery
- Lily M. Landry, Viswanath Gajula, Jarrod D. Knudson, Christopher L. Jenks
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- Published online by Cambridge University Press:
- 23 March 2023, pp. 2504-2510
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Multiple studies have endeavoured to define the role of steroids in paediatric congenital heart surgery; however, steroid utilisation remains haphazard. In September, 2017, our institution implemented a protocol requiring that all neonates undergoing cardiac surgery with the use of cardiopulmonary bypass receive a five-day post-operative hydrocortisone taper. This single-centre retrospective study was designed to test the hypothesis that routine post-operative hydrocortisone administration reduces the incidence of capillary leak syndrome, leads to favourable postoperative fluid balance, and less inotropic support in the early post-operative period. Data were gathered on all term neonates who underwent cardiac surgery with the use of bypass between September, 2015 and 2019. Subjects who were unable to separate from bypass, required long-term dialysis, or long-term mechanical ventilation were excluded. Seventy-five patients met eligibility criteria (non-hydrocortisone group = 52; hydrocortisone group = 23). For post-operative days 0–4, we did not observe a significant difference in net fluid balance or vasoactive inotropic score between study groups. Similarly, we saw no major difference in secondary clinical outcomes (post-operative duration of mechanical ventilation, ICU/hospital length of stay, and time from surgery to initiation of enteral feeds). In contrast to prior analyses, our study was unable to demonstrate a significant difference in net fluid balance or vasoactive inotropic score with the administration of a tapered post-operative hydrocortisone regimen. Similarly, we saw no effect on secondary clinical outcomes. Further long-term randomised control studies are necessary to validate the potential clinical benefit of utilising steroids in paediatric cardiac surgery, especially in the more fragile neonatal population.
Qualitative assessment of nurse satisfaction in the paediatric cardiac ICU
- Julie E. Stark, Kim Steanson, Emily R. Cooperstein, Robert E. Harper, M. Lynne Smith
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- Published online by Cambridge University Press:
- 27 March 2023, pp. 2511-2517
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The objective of this qualitative assessment, utilising the constant comparative method, was to identify satisfiers and dissatisfiers that influence paediatric cardiac ICU nurse retention and recognise areas for improvement. Interviews for this study were performed in a single, large academic children’s hospital from March of 2020 through July of 2020. Each bedside paediatric cardiac ICU nurse underwent a single semi-structured interview. Among 12 interviews, four satisfiers were identified: paediatric cardiac ICU patient population, paediatric cardiac ICU care team, personal accomplishment, and respect. Four dissatisfiers were identified: moral distress, fear, poor team dynamics, and disrespect. Through this process of inquiry, grounded theory was developed regarding strategies to improve paediatric cardiac ICU nurse retention. Tactics outlined here should be used to support retention in the unique environment of the paediatric cardiac ICU.
Successful modified implantation of insertable cardiac monitor in a very young paediatric patient
- Namun S. Srivastava, Janine Arruda, Caroline Al Haddadin, Jessica Ries, Christopher Snyder
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- Published online by Cambridge University Press:
- 27 March 2023, pp. 2518-2520
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Insertable cardiac monitors are minimally invasive devices designed for implantation in the chest wall of patients to record heart rhythms and relate them to symptoms over prolonged periods. The Jot Dx™ (Abbott Laboratories, Abbott Park, IL, USA) is the latest Food and Drug Administration-cleared insertable cardiac monitor that is Bluetooth™ enabled allowing for near-immediate transmission of data from patients to physicians. We report on the first paediatric patient, in a patient weighing 11.7 kg, to undergo a modified, vertical, parasternal implantation of a Jot Dx™.
Developmental care pathway for hospitalised infants with CHD: on behalf of the Cardiac Newborn Neuroprotective Network, a Special Interest Group of the Cardiac Neurodevelopmental Outcome Collaborative
- Amy J. Lisanti, Dorothy J. Vittner, Jennifer Peterson, Andrew H. Van Bergen, Thomas A. Miller, Erin E. Gordon, Karli A. Negrin, Hema Desai, Suzie Willette, Melissa B. Jones, Sherrill D. Caprarola, Anna J. Jones, Stephanie M. Helman, Jodi Smith, Corinne M. Anton, Laurel M. Bear, Lauren Malik, Sarah K. Russell, Dana J. Mieczkowski, Bridy O. Hamilton, Meghan McCoy, Yvette Feldman, Michelle Steltzer, Melanie L. Savoca, Diane L. Spatz, Samantha C. Butler
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- Published online by Cambridge University Press:
- 30 March 2023, pp. 2521-2538
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Infants and children born with CHD are at significant risk for neurodevelopmental delays and abnormalities. Individualised developmental care is widely recognised as best practice to support early neurodevelopment for medically fragile infants born premature or requiring surgical intervention after birth. However, wide variability in clinical practice is consistently demonstrated in units caring for infants with CHD. The Cardiac Newborn Neuroprotective Network, a Special Interest Group of the Cardiac Neurodevelopmental Outcome Collaborative, formed a working group of experts to create an evidence-based developmental care pathway to guide clinical practice in hospital settings caring for infants with CHD. The clinical pathway, “Developmental Care Pathway for Hospitalized Infants with Congenital Heart Disease,” includes recommendations for standardised developmental assessment, parent mental health screening, and the implementation of a daily developmental care bundle, which incorporates individualised assessments and interventions tailored to meet the needs of this unique infant population and their families. Hospitals caring for infants with CHD are encouraged to adopt this developmental care pathway and track metrics and outcomes using a quality improvement framework.
Racial and ethnic disparities in socio-economic status, access to care, and healthcare utilisation among children with heart conditions, National Survey of Children’s Health 2016–2019
- Ashley S. Judge, Karrie F. Downing, Wendy N. Nembhard, Matthew E. Oster, Sherry L. Farr
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- 31 March 2023, pp. 2539-2547
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Among children with and without heart conditions of different race/ethnicities, upstream social determinants of health, such as socio-economic status, access to care, and healthcare utilisation, may vary. Using caregiver-reported data from the 2016–19 National Survey of Children’s Health, we calculated the prevalence of caregiver employment and education, child’s health insurance, usual place of medical care in the past 12 months, problems paying for child’s care, ≥2 emergency room visits, and unmet healthcare needs by heart condition status and race/ethnicity (Hispanic, non-Hispanic Black, and non-Hispanic White). For each outcome, we used multivariable logistic regression to generate adjusted prevalence ratios controlling for child’s age and sex. Of 2632 children with heart conditions and 104,841 without, 65.4% and 58.0% were non-Hispanic White and 52.0% and 51.1% were male, respectively. Children with heart conditions, compared to those without, were 1.7–2.6 times more likely to have problems paying for healthcare, have ≥2 emergency room visits, and have unmet healthcare needs. Hispanic and non-Hispanic Black children with heart conditions, compared to non-Hispanic White, were 1.5–3.2 times as likely to have caregivers employed <50 weeks in the past year and caregivers with ≤ high school education, public or no health insurance, no usual place of care, and ≥2 emergency room visits. Children with heart conditions, compared to those without, may have greater healthcare needs that more commonly go unmet. Among children with heart conditions, Hispanic and non-Hispanic Black children may experience lower socio-economic status and greater barriers to healthcare than non-Hispanic White children.
The prevalence of abnormal spirometry in children with CHD
- Geena Y. Zhou, Daniel Cerrone, Katherine Lewinter, Imran Masood, Jon Detterich, Sasha Singh, Payal Shah, Thomas Keens, Arash Sabati, Roberta Kato
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- Published online by Cambridge University Press:
- 31 March 2023, pp. 2548-2552
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Background:
The burden of pulmonary disease in children with CHD remains under-recognised. Studies have examined children with single ventricle and two ventricle heart disease and documented a decreased forced vital capacity. Our study sought to further explore the pulmonary function of children with CHD.
Methods:A retrospective review was performed of spirometry in CHD patients over a 3-year period. Spirometry data were corrected for size, age, and gender and analysed using z-scores.
Results:The spirometry of 260 patients was analysed. About 31% had single ventricle (n = 80, 13.6 years (interquartile range 11.5-16.8)) and 69% had two ventricle circulation (n = 180, 14.4 years (interquartile range 12.0-17.3)). Single ventricle patients were found to have a lower median forced vital capacity z-score compared to two ventricle patients (p = 0.0133). The prevalence of an abnormal forced vital capacity was 41% in single ventricle patients and 29% in two ventricle patients. Two ventricle patients with tetralogy of Fallot and truncus arteriosus had similar low forced vital capacity comparable to single ventricle patients. The number of cardiac surgeries predicted an abnormal forced vital capacity in two ventricle patients except tetralogy of Fallot patients.
Conclusion:Pulmonary morbidity in patients with CHD is common with a decreased forced vital capacity noted in single ventricle and two ventricle patients. Forced vital capacity is lower in patients with single ventricle circulation; however, two ventricle patients with tetralogy of Fallot or truncus arteriosus have similar lung function in comparison to the single ventricle group. The number of surgical interventions was predictive of forced vital capacity z-score in some but not all two ventricle patients and not predictive in single ventricle patients suggesting a multifactorial to pulmonary disease in children with CHD.
Experiences of parents of children with hypoplastic left heart syndrome during their treatment: a qualitative study
- Agustín Silberberg, Josefina Castro Méndez, Silvio F. Torres, Rafael Fraire, Alejandro Siaba Serrate, Christian Kreutzer, Mariana López Daneri, Thomas Iolster
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- Published online by Cambridge University Press:
- 03 April 2023, pp. 2553-2558
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Aim:
To investigate the opinion and personal experience of parents of children born with Hypoplastic Left Heart Syndrome and what advice they would give to other parents who have to decide between treatment options.
Methods:We conducted a qualitative, descriptive and retrospective study by means of a survey directed to parents of children born with Hypoplastic Left Heart Syndrome in a tertiary hospital in Buenos Aires (Argentina). Their answers and data regarding medical procedures were analysed.
Results:Parents of thirteen out of sixteen patients with Hypoplastic Left Heart Syndrome were surveyed. Norwood surgery had been performed in all the patients, many had received other procedures, and five had died. In relation to the decision-making process, sixty-one percent of parents would recommend other parents to remain at peace after having done everything possible and 54% would suggest to not feel guilt despite the final result. None of the parents would recommend rejecting surgical treatment and choosing comfort care.
Conclusion:The majority of parents of children with Hypoplastic Left Heart Syndrome would recommend continuing with the therapeutic effort in order to feel at peace and reduce feelings of guilt.
Surgical strategies to address re-operative complex left ventricular outflow tract and thoracic aortic pathology: Cleveland Clinic children’s experience
- Dominique L. Tucker, Leah J. Lee, Munir Ahmad, Naseeb Shaheen, Sohini Gupta, Hani K. Najm, Miza Salim Hammoud, Justin T. Tretter, Tara Karamlou
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- Published online by Cambridge University Press:
- 04 April 2023, pp. 2559-2566
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Background:
Complex patients requiring operations on the left ventricular outflow tract, aortic valve, or thoracic aorta after previous repair of aortopathy constitute a challenging group, with limited information guiding decision-making. We aimed to use our institutional experience to highlight management challenges and describe surgical pearls to address them.
Methods:Forty-one complex patients with surgery on the left ventricular outflow tract, aortic valve, or aorta at Cleveland Clinic Children’s between 2016 and 2021 following previous repair of aortic pathology were retrospectively reviewed. Patients with known connective tissue disease or single ventricle circulation were excluded.
Results:Median age at index procedure was 23 years (range 0.25–48) with median of 2 prior sternotomies. Previous aortic operations included subvalvular (n = 9), valvular (n = 6), supravalvular (n = 13), and multi-level surgeries (n = 13). Four deaths occurred in median follow-up of 2.5 years. Mean left ventricular outflow tract gradients improved significantly for patients with obstruction (34.9 ± 17.5 mmHg versus 12.6 ± 6.0 mmHg; p < 0.001). Technical pearls include the following: 1) liberal use of anterior aortoventriculoplasty with valve replacement; 2) primarily anterior aortoventriculoplasty following the subpulmonary conus in contrast to more vertical incision for post-arterial switch operation patients; 3) pre-operative imaging of mediastinum and peripheral vasculature for cannulation and sternal re-entry; and 4) proactive use of multi-site peripheral cannulation.
Conclusions:Operation to address the left ventricular outflow tract, aortic valve, or aorta following prior congenital aortic repair can be accomplished with excellent outcomes despite high complexity. These procedures commonly include multiple components, including concomitant valve interventions. Cannulation strategies and anterior aortoventriculoplasty in specific patients require modifications.
Plasma mid-regional proadrenomedullin level in children with pulmonary hypertension associated with CHD
- Shimaa A. Elesawy, Hesham A. El-Serogy, Esraa A. Sorour, Amr M. Zoair
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- Published online by Cambridge University Press:
- 04 April 2023, pp. 2567-2573
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Adrenomedullin has been shown to inhibit proliferation in pulmonary artery smooth muscle cells and to alleviate pulmonary artery collagen accumulation in pulmonary hypertension. We aimed to assess mid-regional proadrenomedullin level in children with pulmonary hypertension due to CHDs. The current study was conducted in the Pediatric Cardiology Unit, Tanta University Hospital, on 50 children with CHDs: twenty-five patients had a complication of pulmonary hypertension and the other 25 patients without pulmonary hypertension. Another 25 children without CHDs were concluded as a control group. We performed complete history taking, full clinical assessment, chest X-ray, electrocardiogram, and echocardiographic assessment. Plasma level of mid-regional proadrenomedullin was assessed using a sandwich enzyme-linked immunosorbent assay test. Our results showed that the mean plasma level of mid-regional proadrenomedullin was significantly increased in patients with pulmonary hypertension. Significant positive correlation was found between mid-regional proadrenomedullin and mean pulmonary artery pressure. The best cut-off point of mid-regional proadrenomedullin as a diagnostic biomarker to discriminate patients with CHDs complicated with pulmonary hypertension was 199.22 nmol/l. Mid-regional proadrenomedullin significantly increased in patients with pulmonary hypertension who died as compared to patients who survived, with the best cut-off point was 428,8 nmol/l. We concluded that plasma levels of mid-regional proadrenomedullin were significantly elevated in children with pulmonary hypertension complicated by the CHDs. It could be used as a cardiac biomarker in these patients, with good diagnostic and prognostic value.
Mid-term follow-up after COVID-19 vaccination in adults with CHD: a prospective study
- Flavia Fusco, Giancarlo Scognamiglio, Anna Selvaggia Roma, Massimiliana Abbate, Giovanni Papaccioli, Assunta Merola, Michela Palma, Nunzia Borrelli, Rosaria Barracano, Anna Correra, Nicola Grimaldi, Giovanni Domenico Ciriello, Maurizio D’Abbraccio, Cristina Scavone, Annalisa Capuano, Berardo Sarubbi
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- Published online by Cambridge University Press:
- 11 April 2023, pp. 2574-2580
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Background:
Long-term data on COVID-19 vaccine safety, immunogenicity, and acceptance in adults with CHD are lacking.
Methods:This is a prospective study including adults with CHD patients undergoing COVID-19 vaccination from January 2021 to June 2022. Data on adverse events, antispike IgG titre, previous or subsequent COVID-19 infection, booster doses, and patients’ attitude towards vaccination were collected.
Results:Four hundred and ninety CHD patients (36 ± 13 years, 53% male, 94% with moderate/complex defects) were prospectively included: 433 (88%) received a Pfizer–BioNTech mRNA vaccine, 31 (6%) Moderna mRNA vaccine, 23 (5%) AstraZeneca–Oxford ChAdOx1 nCov-19 vaccine, and 3 (0.6%) Janssen Vaccine; 310 (63%) received a booster dose. Median follow-up after vaccination was 1.53 [1.41–1.58] years. No major adverse event was reported. Eighty-two fully vaccinated patients contracted COVID-19 during follow-up after a median of 5.4 [4.3–6.5] months from the last dose. One patient with Ebstein’s disease died from severe COVID-19. Symptoms’ duration in patients who tested positive after vaccination was significantly shorter than in the group tested positive before vaccination (5.5 [3–8] versus 9 [2.2–15] days, p = 0.04). Median antispike IgG titre measured in 280 individuals (57%) at a median of 1.4 [0.7–3.3] months from the last dose was 2381 [901–8307] BAU/ml. Sixty patients (12%) also showed positive antinucleocapsid antibodies, demonstrating previous SARS-COV2 exposure. Twenty-nine percent appeared to have concerns regarding vaccine safety and 42% reported fearing potential effects of the vaccine on their cardiac disease before discussing with their CHD cardiologist.
Conclusion:COVID-19 vaccines appear safe in the mid-term follow-up in adults with CHD with satisfactory immunogenicity and reduction of symptoms’ duration in case of infection.
Implementation of the Cardiac Inpatient Neurodevelopmental Care Optimization (CINCO) programme: an interdisciplinary, generalisable approach to inpatient neurodevelopmental care
- Kelly R. Wolfe, Sherrill D. Caprarola, Caelah Clark, Jesse Davidson, Melanie D. Everitt, Laura Faul, Colton Hageman, Sarah L. Kelly, Emily Maloney, Hilary Patteson, Sarah Scott, Alyse Talbot, Suhong Tong, Kimberly L. DiMaria
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- Published online by Cambridge University Press:
- 12 April 2023, pp. 2581-2588
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Background:
Children with CHD are at risk for neurodevelopmental delays, and length of hospitalisation is a predictor of poorer long-term outcomes. Multiple aspects of hospitalisation impact neurodevelopment, including sleep interruptions, limited holding, and reduced developmental stimulation. We aimed to address modifiable factors by creating and implementing an interdisciplinary inpatient neurodevelopmental care programme in our Heart Institute.
Methods:In this quality improvement study, we developed an empirically supported approach to neurodevelopmental care across the continuum of hospitalisation for patients with CHD using three plan-do-study-act cycles. With input from multi-level stakeholders including parents/caregivers, we co-designed interventions that comprised the Cardiac Inpatient Neurodevelopmental Care Optimization (CINCO) programme. These included medical/nursing orders for developmental care practices, developmental kits for patients, bedside developmental plans, caregiver education and support, developmental care rounds, and a specialised volunteer programme. We obtained data from the electronic health record for patients aged 0–2 years admitted for at least 7 days to track implementation.
Results:There were 619 admissions in 18 months. Utilisation of CINCO interventions increased over time, particularly for the medical/nursing orders and caregiver handouts. The volunteer programme launch was delayed but grew rapidly and within six months, provided over 500 hours of developmental interaction with patients.
Conclusions:We created and implemented a low-cost programme that systematised and expanded upon existing neurodevelopmental care practices in the cardiac inpatient units. Feasibility was demonstrated through increasing implementation rates over time. Key takeaways include the importance of multi-level stakeholder buy-in and embedding processes in existing clinical workflows.
Pre-Glenn aorto-pulmonary collaterals in single-ventricle patients
- Varun J. Sharma, Laura Carlson, Jesse Esch, Mallika Gopal, Kimberlee Gauvreau, Isaac Wamala, Angelika Muter, Diego Porras, Meena Nathan
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- Published online by Cambridge University Press:
- 17 April 2023, pp. 2589-2596
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Background:
In single-ventricle patients undergoing staged-bidirectional Glenn, 36–59% have aorto-pulmonary collateral flow, but risk factors and clinical outcomes are unknown. We hypothesise that shunt type and catheter haemodynamics may predict pre-bidirectional Glenn aorto-pulmonary collateral burden, which may predict death/transplantation, pulmonary artery or aorto-pulmonary collateral intervention.
Methods:Retrospective cohort study of patients undergoing a Norwood procedure for single-ventricle anatomy. Covariates included clinical and haemodynamic characteristics up to/including pre-bidirectional Glenn catheterisation and aorto-pulmonary collateral burden at pre-bidirectional Glenn catheterisation. Multivariable models used to evaluate relationships between risk factors and outcomes.
Results:From January 2011 to March 2016, 104 patients underwent Norwood intervention. Male sex (odds ratio 3.36, 95% confidence interval 1.17–11.4), age at pre-bidirectional Glenn assessment (2.12, 1.33–3.39 per month), and pulmonary to systemic flow ratio (1.23, 1.08–1.41 per 0.1 unit) were associated with aorto-pulmonary collateral burden. Aorto-pulmonary collateral burden was not associated with death/transplantation (hazard ratio 1.19, 95% confidence interval 0.37–3.85), pulmonary artery (sub-hazard ratio 1.38, 0.32–2.61), or aorto-pulmonary collateral interventions (sub-hazard ratio 1.11, 0.21–5.76). Longer post-Norwood length of stay was associated with greater risk of death/transplantation (hazard ratio 1.22 per week, 95% confidence interval 1.08–1.38), but lower risk of aorto-pulmonary collateral intervention (sub-hazard ratio 0.86 per week, 95% confidence interval 0.75–0.98). Time to pre-bidirectional Glenn catheterisation was associated with lower risk of pulmonary artery (sub-hazard ratio 0.80 per month, 95% confidence interval 0.65–0.98) and aorto-pulmonary collateral intervention (sub-hazard ratio 0.79, 0.63–0.99). Probability of moderate/severe aorto-pulmonary collateral burden increased with left-to-right shunt (22.5% at <1.0, 57.6% at >1.4) and the age at pre-bidirectional Glenn catheterisation (10.6% at <2 months, 56.9% at >5 months).
Conclusions:Aorto-pulmonary collateral burden is common after Norwood procedure and increases as age at bidirectional Glenn increases. As expected, higher pulmonary to systemic flow ratio is a marker for greater aorto-pulmonary collateral burden pre-bi-directional Glenn; aorto-pulmonary collateral burden does not confer risk of death/transplantation or pulmonary artery intervention.
Failure to post-dilate BeSmooth peripheral stents to adult vessel size diameters during benchside tests
- Raymond N. Haddad, Damien Bonnet, Sophie Malekzadeh-Milani
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- Published online by Cambridge University Press:
- 19 April 2023, pp. 2597-2603
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Background:
Low-profile stent implantation remains a rescue treatment for aortic coarctation and branch pulmonary arteries stenosis in small children. Stent re-expansion to cope with vascular growth remains problematic.
Objectives:To evaluate ex vivo feasibility and mechanical behaviour of over-dilating BeSmooth peripheral stents (Bentley InnoMed, Germany).
Methods:Three BeSmooth peripheral stents in diameters of 7, 8, and 10 mm were dilated to nominal pressure and then 13 atm. BeSmooth Ø7 × 23 mm was sequentially post-dilated using 12, 14, and 16 mm high-pressure balloons. BeSmooth Ø10 × 57 mm was post-dilated with a 14 mm balloon and then with a 48 mm bare-metal Optimus XXL stent hand-mounted on a 14 mm balloon (stent-in-stent). BeSmooth Ø8 × 57 mm was directly post-dilated with a 48 mm bare-metal Optimus XXL stent hand-mounted on a 16 mm balloon (stent-in-stent). The stents’ diameter and length were measured. Digital inflation pressure was noted. Balloon rupture and stent fracture patterns were closely evaluated.
Results:At 20atm pressure, BeSmooth Ø7 × 23 mm shortened to 2 mm forming a 12 mm diameter solid ring circle and the woven balloon ruptured radially. At 10 atm pressure, BeSmooth Ø10 × 57 mm fractured longitudinally in various dispatched breaking points at a diameter of 13 mm without shortening and ruptured the balloon with multiple pinholes. At 10 atm pressure, BeSmooth Ø8 × 57 mm fractured centrally at three different points at a diameter of 11.5 mm without shortening and the balloon broke radially in half.
Conclusions:In our benchmark tests, extreme shortening, severe balloon rupture, or unpredictable stent fracture patterns at small balloon diameters limits safe post-dilation of BeSmooth stents beyond 13 mm. BeSmooth stents are not ideal candidates for off-label stent interventions in smaller patients.
NT-proBNP levels as a predictor of higher left ventricular end-diastolic pressure in children with small perimembranous ventricular septal defect
- Putria R. Apandi, Mulyadi M. Djer, Rahmat B. Kuswiyanto, Elrika A. Wijaya, Bagus Artiko, Sri E. Rahayuningsih
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- Published online by Cambridge University Press:
- 20 April 2023, pp. 2604-2609
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Introduction:
The decision to close small ventricular septal defect is still controversial. Previous study showed that ventricular dysfunction in adulthood is correlated with small perimembranous ventricular septal defect. N terminal pro B-type natriuretic peptide (NT-proBNP) is a neurohormone secreted primarily from the ventricles in response to increased left and right ventricular pressure and volume load. The left ventricular end-diastolic pressure will reflect ventricular performance. This study aimed to evaluate the correlation between left ventricular end-diastolic pressure and the NT-proBNP in children with small perimembranous ventricular septal defect.
Materials and Methods:Level of NT-proBNP in 41 patients with small perimembranous ventricular septal defect was measured before transcatheter closure procedure. We also measured the left ventricular end-diastolic pressure in each patients during catheterisation. We investigated the value of NT-proBNP in patients with small perimembranous ventricular septal defect and its correlation with the level of left ventricular end-diastolic pressure
Result:We found positive correlation between NT-proBNP and left ventricular end-diastolic pressure (r = 0.278, p = 0.046). The median of NT-proBNP at left ventricular end-diastolic pressure < 10 was lower than at left ventricular end-diastolic pressure ≥ 10 (0.87 ng/ml versus 1.83 ng/ml, p = 0.023). The results of the NT-proBNP diagnostic test for predicting left ventricular end-diastolic pressure ≥ 10 using Receiver Operating Characteristic (ROC) analysis showed the area under the curve value of 0.715 (95% CI: 0.546–0.849). The cut-off value >0.99 ng/ml of NT-proBNP has 75.0% sensitivity and 72.2% specifity.
Conclusion:Level of NT-proBNP higher than 0.99 ng/ml was correlated significantly with left ventricular end-diastolic pressure ≥10 in children with small perimembranous ventricular septal defect.
Coronary artery dilation associated with bicuspid and unicuspid aortic valve disease in children: a series of 17 patients
- David L. Robinson, L. LuAnn Minich, Shaji C. Menon, Zhining Ou, Aaron W. Eckhauser, Adam L. Ware
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- Published online by Cambridge University Press:
- 20 April 2023, pp. 2610-2615
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Introduction:
Coronary artery dilation associated with bicuspid/unicuspid aortic valves is described in adults with limited data in children. We aimed to describe the clinical course of children with bicuspid/unicuspid aortic valves and coronary dilation including coronary Z-score changes over time, association of coronary changes with aortic valve anatomy/function, and complications.
Materials and methods:Institutional databases were searched for children ≤18 years with both bicuspid/unicuspid aortic valves and coronary dilation (1/2006-6/2021). Kawasaki disease and isolated supra-/subvalvar aortic stenosis were excluded. Statistics were descriptive with associations measured by Fisher’s exact test and overlapping 83.7% confidence intervals.
Results:Of 17 children, bicuspid/unicuspid aortic valve was diagnosed at birth in 14 (82%). Median age at coronary dilation diagnosis was 6.4 years (range: 0-17.0). Aortic stenosis was present in 14 (82%) [2 (14%) moderate, 8 (57%) severe]; 10 (59%) had aortic regurgitation; 8 (47%) had aortic dilation. The right coronary was dilated in 15 (88%), left main in 6 (35%), and left anterior descending in 1 (6%) with no relationship between leaflet fusion pattern or severity of aortic regurgitation/stenosis on coronary Z-score. Follow-up evaluations were available for 11 (mean 9.3 years, range 1.1–14.8) with coronary Z-scores increasing in 9/11 (82%). Aspirin was used in 10 (59%). There were no deaths or coronary artery thrombosis.
Discussion:In children with bicuspid/unicuspid aortic valves and coronary dilation, the right coronary artery was most frequently involved. Coronary dilation was observed in early childhood and frequently progressed. Antiplatelet medication use was inconsistent, but no child died nor developed thrombosis.