Review
Patient-reported outcome measures in congenital heart surgery: a systematic review
- Jeevan Francis, Sneha Prothasis, Joseph George, Serban Stoica
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- Published online by Cambridge University Press:
- 18 January 2023, pp. 337-341
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Background:
Patient-reported outcome measures are commonly used to evaluate the effectiveness of treatments. CHD remains the most common congenital malformation. There has been a gradual shift in evaluating the outcome of surgery for CHD from mortality to morbidity and now to self-reported outcomes.
Aims:We aimed to review studies assessing patient-reported outcome measures as a useful marker of outcome for patients, both children and adults, who underwent surgery for CHD.
Methods:A systematic database search was conducted of original articles that explored the application of patient-reported outcome measures in the CHD surgical setting in PubMed and SCOPUS from inception to February 2022.
Results:Our search yielded 1511 papers, of which six studies were included in this review after screening abstract and full-text, with a total sample size of 5734 patients. The main areas of discussion were the utility of patient-reported outcome measures, determinants of patient-reported outcome measures, and the need for a congenital cardiac surgery-specific patient-reported outcome measure for paediatric patients and their parents/guardians and adult patients.
Conclusion:This systematic review reports the use of patient-reported outcome measures to be a useful indicator to gain insight into the patients’ perspective to provide holistic and patient-centred management. However, further studies are required to assess the utility of patient-reported outcome measures in a congenital cardiac surgical setting.
Original Article
TGA + IVS + LVOTO: patterns of practice and outcomes
- Husain Esmaeil, Jeffrey P. Jacobs, Christo I. Tchervenkov, Andrzej Kansy, Bohdan Maruszewski, Zdzislaw Tobota, James D. St. Louis, James K. Kirklin, David M. Overman, Vladimiro Vida, Claudia Herbst, Awais Ashfaq, Zohair Al-Halees, George E. Sarris
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- Published online by Cambridge University Press:
- 17 February 2023, pp. 342-348
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Purpose:
Transposition of great arteries with intact ventricular septum and left ventricular outflow tract obstruction (TGA + IVS + LVOTO) is uncommon. We reviewed operations performed in patients with TGA + IVS + LVOTO in the European Congenital Heart Surgeons Association Congenital Database (ECHSA-CD).
Methods:All 109 patients with a diagnosis of TGA + IVS + LVOTO in ECHSA-CD who underwent cardiac surgery during a 21-year period (01/2000-02/2021, inclusive) were included. Preoperative variables, operative data, and postoperative outcomes were collected.
Results:These 109 patients underwent 176 operations, including 37 (21.0%) arterial switch operations (ASO), 26 (14.2%) modified Blalock-Taussig-Thomas shunts (MBTTS), 11 (6.2%) Rastelli operations, and 13 (7.3%) other palliative operations (8 superior cavopulmonary anastomosis[es], 4 Fontan, and 1 other palliative procedure). Of 37 patients undergoing ASO, 22 had a concomitant procedure.
There were 68 (38.6%) reoperations, including 11 pacemaker procedures and 8 conduit operations. After a systemic-to-pulmonary artery shunt, reoperations included shunt reoperation (n = 4), Rastelli (n = 4), and superior cavopulmonary anastomosis (n = 3).
Overall Operative Mortality was 8.2% (9 deaths), including three following ASO, two following “Nikaidoh, Kawashima, or LV-PA conduit” procedures, and two following Rastelli. Postoperative complications occurred after 36 operations (20.4%). The most common complications were delayed sternal closure (n = 11), postoperative respiratory insufficiency requiring mechanical ventilation >7 days (n = 9), and renal failure requiring temporary dialysis (n = 8).
Conclusion:TGA + IVS + LVOTO is rare (109 patients in ECHSA-CD over 21 years). ASO, MBTTS, and Rastelli are the most common operations performed for TGA + IVS + LVOTO. Larger international studies with long-term follow-up are needed to better define the anatomy of the LVOTO and to determine the optimal surgical strategy.
Early postnatal metabolic profile in neonates with critical CHDs
- Osman Guvenc, Serdar Beken, Saygin Abali, Neslihan Yildirim Saral, Gizem Yalcin, Eda Albayrak, Burcu Bulum, Ozlem Demirelce, Serdar Basgoze, Ender Odemis, Ersin Erek, Mustafa Serteser, Ayse Korkmaz Toygar
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- 04 October 2022, pp. 349-353
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Background:
Cyanotic CHD is a life-threatening condition that presents with low oxygen saturation in the newborn period. Hypoxemia might cause alterations in the metabolic pathways. In the present study, we aimed to evaluate the early postnatal amino acid and carnitine/acylcarnitine profiles of newborn infants with cyanotic CHD.
Methods:A single centre case-control study was conducted. Twenty-seven patients with cyanotic CHD and 54 healthy newborn controls were enrolled. As part of the neonatal screening programme, results of amino acid and carnitine/acylcarnitine were recorded and compared between groups.
Results:Twenty-seven neonates with cyanotic CHD and 54 healthy newborns as controls were enrolled in the study. Cyanotic CHD neonates had higher levels of alanine, phenylalanine, leucine/isoleucine, citrulline, ornithine, C5, C5-OH; but lower levels of C3, C10, C12, C14, C14:1, C16, C16.1, C18, C5-DC, C6-DC, C16-OH, C16:1-OH when compared with the healthy controls.
Conclusion:This study showed that there are differences between patients with cyanotic CHD and healthy controls in terms of postnatal amino acid and carnitine/acylcarnitine profiles.
Early outcomes of the treatment of aortic coarctation with BeGraft aortic stent in children and young adults
- Ahmet V. Kavurt, Hazım A. Gürsu, Gökçe Kaş, Nevin Özdemiroğlu, Denizhan Bağrul, Ayşe E. K. Gül, İbrahim Ece, İbrahim İ Çetin
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- 19 October 2022, pp. 354-361
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We report our experience and early outcomes of using the BeGraft aortic stent in children, adolescents, and young adults. BeGraft aortic stent (Bentley InnoMed, Hechingen, Germany) requires a smaller long sheath compared to other covered stents, and it has a low profile and adequate radial power. With these features, it can overcome some limitations in the treatment of coarctation, especially in children. This is a single centre retrospective analysis of 11 implanted BeGraft aortic stents in coarctation of the aorta between July 2020 and November 2021. The eleven stents were successfully implanted in 11 patients (10 males). The median age of the patients was 13.7 years (interquartile range 12–16 years), and the median weight was 43 kg (interquartile range 35–62 kg). In five patients, after the stents were opened completely by the first balloon, they were exchanged with a Z-MED II™ balloon, 1–3 mm larger in diameter, and the stents were redilated. The median catheter-derived systolic peak-to-peak pressure gradient was 23 mm Hg (interquartile range 16–37 mmHg) before the procedure and 3 mm Hg (interquartile range 1–5 mm Hg) after the procedure. Except for the partial femoral artery thrombosis in two patients, no other procedural complications were observed in our study. The median follow-up duration was 5 months (interquartile range 2–12 months). During follow-up, only one patient (9%) had stent narrowing that required dilation. Our initial results and short-term follow-up showed that the BeGraft aortic stent implantation and redilation can be performed effectively, safely, and successfully in the treatment of coarctation of the aorta.
Transcatheter resolution of an aneurism of the pulmonary trunk with residual ductus arteriosus after thoracoscopic clipping: a new approach
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- Sylvia K. Sánchez González, José Luis Colín Ortíz, Rigoberto Zamudio Meneses, Hugo Cabrera González, Roberto Maldonado Alonso
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- 18 March 2022, pp. 362-365
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Patent ductus arteriosus is the most common cardiac anomaly in our country. In the last few decades, there has been a lot of interest in developing less invasive techniques like video-assisted thoracoscopic clipping; nevertheless, this also has some complications. We present an 8-year-old female, which had been treated with video-assisted thoracoscopic clipping of patent ductus arteriosus. Five years later, she presented with a large aneurysm of the ductus arteriosus extending to the pulmonary trunk and a residual patent ductus arteriosus. A Cardia ASD occluder of 24 mm was placed in the aneurysm, and the residual ductus arteriosus was then closed with an Amplatzer Plug vascular II device of 10 mm, with a good outcome. The development of an aneurysm after video-assisted patent ductus arteriosus closure is apparently a non-reported complication; therefore, there are also no reports for its treatment. That is why we present this case as an option for its resolution.
High variability in cardiac education and experiences during United States paediatric critical care fellowships
- Amy E. Hanson, Christopher W. Mastropietro, Lee D. Murphy, Courtney M. Rowan
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- 04 March 2022, pp. 366-370
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Background:
Paediatric cardiac critical care continues to become more sub-specialised, and many institutions have transitioned to dedicated cardiac ICUs. Literature regarding the effects of these changes on paediatric critical care medicine fellowship training is limited.
Objective:To describe the current landscape of cardiac critical care education during paediatric critical care medicine fellowship in the United States and demonstrate its variability.
Methods:A review of publicly available information in 2021 was completed. A supplemental REDCap survey focusing on cardiac ICU experiences during paediatric critical care medicine fellowships was e-mailed to all United States Accreditation Council of Graduate Medical Education-accredited paediatric critical care medicine fellowship programme coordinators/directors. Results are reported using inferential statistics.
Results:Data from 71 paediatric critical care medicine fellowship programme websites and 41 leadership responses were included. Median fellow complement was 8 (interquartile range: 6, 12). The majority (76%, 31/41) of programmes had a designated cardiac ICU. Median percentage of paediatric critical care medicine attending physicians with cardiac training was 25% (interquartile range: 0%, 69%). Mandatory cardiac ICU time was 16 weeks (interquartile range: 13, 20) with variability in night coverage and number of other learners present. A minority of programmes (29%, 12/41) mandated other cardiac experiences. Median CHD surgical cases per year were 215 (interquartile range: 132, 338). When considering the number of annual cases per fellow, programmes with higher case volume were not always associated with the highest case number per fellow.
Conclusions:There is a continued trend toward dedicated cardiac ICUs in the United States, with significant variability in cardiac training during paediatric critical care medicine fellowship. As the trend toward dedicated cardiac ICUs continues and practices become more standardised, so should the education.
Short to midterm follow-up of multi-system inflammatory syndrome in children with special reference to cardiac involvement
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- Omeir A. Aziz, Masood Sadiq, Ahmad U. Qureshi, Najam Hyder, Uzma Kazmi, Afsheen Batool, Samia Naz, Asma Mushtaq, Attia Bari, Junaid Rashid
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- Published online by Cambridge University Press:
- 24 March 2022, pp. 371-379
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Objectives:
We aim to describe the early and upto 16 months follow-up of post-coronavirus disease (COVID), multi-system inflammatory syndrome in children (MIS-C), with special reference to cardiac involvement.
Study design:This cohort non-interventional descriptive study included patients <18 years admitted between May, 2020 and April, 2021. Based on underlying similarities, children were classified as post-COVID MIS-C with overlapping Kawasaki Disease, MIS-C with no overlapping Kawasaki Disease, and MIS-C with shock. Post-discharge, patients were followed at 1, 3, 6, 12, and 16 months.
Results:Forty-one patients predominantly males (73%), at median age of 7 years (range 0.2–16 years) fulfilled the World Health Organisation criteria for MIS-C. Cardiac involvement was seen in 15 (36.5%); impaired left ventricle (LV) function in 5 (12.2%), coronary artery involvement in 10 (24.4%), pericardial effusion in 6 (14.6%) patients, and no arrhythmias. There were two hospital deaths (4.9%), both in MIS-C shock subgroup (2/10, 20%). At 1 month, there was persistent LV dysfunction in 2/5, coronary artery abnormalities in 7/10, and pericardial effusion resolved completely in all patients. By 6 months, LV function returned to normal in all but coronary abnormalities persisted in two patients. At last follow-up (median 9.8 months, interquartile range 2–16 months), in 36/38 (94.7%) patients, coronary artery dilatation was persistent in 2 (20%) patients.
Conclusions:Children with MIS-C have a good early outcome, though MIS-C with shock can be life-threatening subgroup in a resource-constrained country setting. On midterm follow-up, there is normalisation of LV function in all and recovery of coronary abnormalities in 80% of patients.
Effect of treadmill exercise stress testing on troponin levels in children and adolescents
- Anthony G. Pompa, Gaurav Arora, Tyler H. Harris
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- 18 March 2022, pp. 380-382
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Background:
Chest pain is a common complaint among paediatric patients and cardiac troponin (cTn) level is often part of the initial emergency department evaluation. It is well known that after intense endurance exercise cTn levels can be elevated in patients with otherwise healthy hearts, however the effect shorter duration exercise has on cTn levels in this population is not known.
Objective:Determine the behaviour of cTn levels in healthy children and adolescent patients after short burst, high-intensity aerobic exercise.
Methods:Patients without haemodynamically significant heart disease referred for a treadmill exercise stress test (EST) were recruited over a 6-month period. cTn levels were measured prior to exercise and 4 hours after exercise.
Results:Thirteen patients enrolled. Indications for EST were exertional syncope (six), chest pain (four), and long QT syndrome (three). The median exercise time was 12.9 (9.9–13.7) minutes with an average endurance at the fiftieth percentile for age and maximum heart rate rose to an average of 92 (74–98)% of the predicted peak for age. cTn levels prior to exercise were undetectable in all patients. There was no cTn rise in any patient after exercise. There were no ischaemic changes or arrhythmias on exercise electrocardiograms.
Conclusion:Serum cTn levels do not rise significantly in healthy children after short duration, high-intensity aerobic exercise. Physicians evaluating paediatric patients with an elevated cTn level after less than prolonged strenuous activity likely cannot attribute this lab value solely to exercise and may need to undertake further cardiac investigation.
Characteristics and long-term outcome for congenital left main coronary artery atresia
- Etsuko Tsuda, Toshikatsu Yagihara, Shinya Tsukano, Hajime Ichikawa, Soichiro Kitamura
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- 30 March 2022, pp. 383-387
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The prevalence of congenital left main coronary artery atresia is very low. We report the characteristics and long-term outcomes of four children with left main coronary artery atresia. Three patients had heart murmurs due to mitral regurgitation at less than 1 year old. Their myocardial ischaemia worsened on exercise with aging. In the fourth patient, hypertrophic cardiomyopathy and Noonan syndrome were suspected at 1 year old. The development of communicating arteries between the conus branch and the left anterior descending artery was detected at 7 years old. The left main coronary artery atresia was confirmed by a selective coronary angiogram at 15 years old. Congenital left main coronary artery atresia could not be diagnosed by two-dimensional echocardiography; however, the left coronary arteries were small. Two patients underwent coronary artery bypass grafting of the left anterior descending artery using the left internal thoracic artery at 3 years and 6 years old, respectively. Two patients had an angioplasty with a cut back at the orifice of the left coronary artery at 2 years old and 17 years old, respectively. Two patients had no cardiac events without medication for more than 30 years after the operation. We must differentiate the diagnosis of left main coronary artery atresia in the small left coronary arteries with mitral regurgitation during the first year. Coronary artery revascularisation and mitral annuloplasty are needed. The long-term outcome of both coronary artery bypass grafting and angioplasty were good. The degree of mitral regurgitation after surgery may affect the prognosis.
Machine learning predicts blood lactate levels in children after cardiac surgery in paediatric ICU
- Koichi Sughimoto, Jacob Levman, Fazleem Baig, Derek Berger, Yoshihiro Oshima, Hiroshi Kurosawa, Kazunori Aoki, Yusuke Seino, Tetsuya Ueda, Hao Liu, Kagami Miyaji
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- Published online by Cambridge University Press:
- 04 April 2022, pp. 388-395
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Background:
Although serum lactate levels are widely accepted markers of haemodynamic instability, an alternative method to evaluate haemodynamic stability/instability continuously and non-invasively may assist in improving the standard of patient care. We hypothesise that blood lactate in paediatric ICU patients can be predicted using machine learning applied to arterial waveforms and perioperative characteristics.
Methods:Forty-eight post-operative children, median age 4 months (2.9–11.8 interquartile range), mean baseline heart rate of 131 beats per minute (range 33–197), mean lactate level at admission of 22.3 mg/dL (range 6.3–71.1), were included. Morphological arterial waveform characteristics were acquired and analysed. Predicting lactate levels was accomplished using regression-based supervised learning algorithms, evaluated with hold-out cross-validation, including, basing prediction on the currently acquired physiological measurements along with those acquired at admission, as well as adding the most recent lactate measurement and the time since that measurement as prediction parameters. Algorithms were assessed with mean absolute error, the average of the absolute differences between actual and predicted lactate concentrations. Low values represent superior model performance.
Results:The best performing algorithm was the tuned random forest, which yielded a mean absolute error of 3.38 mg/dL when predicting blood lactate with updated ground truth from the most recent blood draw.
Conclusions:The random forest is capable of predicting serum lactate levels by analysing perioperative variables, including the arterial pressure waveform. Thus, machine learning can predict patient blood lactate levels, a proxy for haemodynamic instability, non-invasively, continuously and with accuracy that may demonstrate clinical utility.
“The only thing I wonder is when I will have surgery again”: everyday life for children with right ventricle outflow tract anomalies during assessment for heart surgery
- Birgitta Svensson, Petru Liuba, Anne Wennick, Malin Berghammer
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- Published online by Cambridge University Press:
- 30 March 2022, pp. 396-401
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Background:
Children with right ventricle outflow tract anomalies require repeated heart surgeries, thereby needing regular preoperative assessments throughout their lifetime. This situation puts a heavy burden on these children. Thus, the aim of this study was to explore how children diagnosed with right ventricle outflow tract anomalies experience their heart disease and their everyday life during the preoperative assessment and after the decision on whether to perform a new cardiac surgery.
Methods:Individual interviews were conducted with nine children between 9 to 17 years of age on three occasions from 2014 to 2016. In total there were 27 interviews which all were analyzed with thematic analysis.
Results:The analysis yielded three themes and eight subthemes. The theme Me and my heart disease concerns children’s experiences of the heart disease. Almost all described symptoms and how they adapt in their everyday life. The theme Being me concerns the children’s sense of self, where their heart disease was not prominent. The theme Being placed in someone else’s hands describes how the assessment was more of a safety net at least until the decision of heart surgery.
Conclusion:The children’s symptoms, their experiences during the assessment, their future surgeries and how the heart disease affects their everyday life could be better understood as elements of their adaptation to the heart disease. In order to achieve individualized support based on the child’s experiences and to ensure that these children are involved in their own care a child-centered approach is recommended.
Corrigendum
“The only thing I wonder is when I will have surgery again” – Everyday life for children with right ventricle outflow tract anomalies during assessment for heart surgery – CORRIGENDUM
- Birgitta Svensson, Petru Liuba, Anne Wennick, Malin Berghammer
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- 12 May 2022, p. 402
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Original Article
Life satisfaction, health-related quality of life and physical activity after treatment for valvular aortic stenosis
- Cecilia Kjellberg Olofsson, Pia Skovdahl, Jonatan Fridolfsson, Daniel Arvidsson, Mats Börjesson, Jan Sunnegårdh, Sandra Buratti
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- 30 March 2022, pp. 403-409
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Objective:
To investigate health-related quality of life and life satisfaction in children and adolescents treated for isolated congenital valvular aortic stenosis compared to healthy peers. Our second aim was to investigate the relationship between objectively measured physical activity, health-related quality of life and life satisfaction in the same group.
Methods:Forty-eight patients, 8–18 years of age, were recruited, as well as 43 healthy peers matched for age, gender and residential area. Health-related quality of life was assessed by the KIDSCREEN-52 self-report and parent proxy report, and life satisfaction was evaluated with the Satisfaction With Life Scale. Physical activity was measured with an accelerometer for 7 days. Sports participation was self-reported.
Results:No differences in the health-related quality of life domains were found between patients and controls in the self-reports. In the proxy reports, parents of the adolescents rated their child’s autonomy lower than did the parents of the healthy controls. A negative relationship was found between moderate to vigorous physical activity, sports participation, life satisfaction and the psychological well-being domain in adolescent patients. In children there was a positive relationship between moderate physical activity and the physical and psychological well-being domains.
Conclusion:Overall, children and adolescents treated for valvular aortic stenosis reported similar life satisfaction and health-related quality of life as their healthy peers. The negative relationships between intense physical activity and sports participation with health-related quality of life and life satisfaction in adolescent patients might be explained by both physical and psychological factors in these teenagers with complex, lifelong heart disease.
Virtual reality curriculum increases paediatric residents’ knowledge of CHDs
- Tiffany R. Lim, Hunter C. Wilson, David M. Axelrod, David K. Werho, Stephanie S. Handler, Sunkyung Yu, Katherine Afton, Ray Lowery, Patricia B. Mullan, James Cooke, Sonal T. Owens
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- 25 April 2022, pp. 410-414
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Objectives:
Virtual reality has emerged as a unique educational modality for medical trainees. However, incorporation of virtual reality curricula into formal training programmes has been limited. We describe a multi-centre effort to develop, implement, and evaluate the efficacy of a virtual reality curriculum for residents participating in paediatric cardiology rotations.
Methods:A virtual reality software program (“The Stanford Virtual Heart”) was utilised. Users are placed “inside the heart” and explore non-traditional views of cardiac anatomy. Modules for six common congenital heart lesions were developed, including narrative scripts. A prospective case–control study was performed involving three large paediatric residency programmes. From July 2018 to June 2019, trainees participating in an outpatient cardiology rotation completed a 27-question, validated assessment tool. From July 2019 to February 2020, trainees completed the virtual reality curriculum and assessment tool during their cardiology rotation. Qualitative feedback on the virtual reality experience was also gathered. Intervention and control group performances were compared using univariate analyses.
Results:There were 80 trainees in the control group and 52 in the intervention group. Trainees in the intervention group achieved higher scores on the assessment (20.4 ± 2.9 versus 18.8 ± 3.8 out of 27 questions answered correctly, p = 0.01). Further analysis showed significant improvement in the intervention group for questions specifically testing visuospatial concepts. In total, 100% of users recommended integration of the programme into the residency curriculum.
Conclusions:Virtual reality is an effective and well-received adjunct to clinical curricula for residents participating in paediatric cardiology rotations. Our results support continued virtual reality use and expansion to include other trainees.
Application of probability theory to neonatal cardiac evaluation
- Javier O. Rodríguez Velásquez, Sandra C. Correa Herrera, Ana M. Bertolotto Cepeda
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- 06 May 2022, pp. 415-419
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Based on probability theory, a methodology that allows diagnosing neonatal cardiac dynamics was previously developed; however, diagnostic applications of this method are required to validate it to the neonatal cardiac dynamics was conducted, allowing to differentiate normal from pathological dynamics. The hourly maximum and minimum heart rate values from 39 continuous and ambulatory electrocardiographic records with a minimum length of 21 hours were taken, from newborns between 0 and 10 days of life, 9 clinically within normality limits and 30 with cardiac pathologies. The probability of occurrence of heart rates in ranges of 5 beats/minute was calculated. The distributions of probability were analysed, and finally the diagnosis was determined by the physical-mathematical methodology. Then, a statistical validation of sensitivity, specificity, and diagnostic agreement was performed. Normal registries showed probability distributions with absent or minimal presence of heart rates of the ranges between 125 and 135 beats/minute, while the abnormal ones had values within these ranges, as well as absence or minimal presence of heart rates from 75 beats/minute to 85 beats/minute. The sensitivity and specificity were 100%, and the Kappa coefficient had a value of 1. Hereby, it is concluded that through an application of a physical–mathematical methodology of neonatal cardiac diagnosis, it is possible to differentiate normality from disease.
Variation in hospital costs and resource utilisation after congenital heart surgery
- Joshua J. Blinder, Yuan-Shung Huang, Joseph W. Rossano, Andrew T Costarino, Yimei Li
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- 04 April 2022, pp. 420-431
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Background:
Children undergoing cardiac surgery have overall improving survival, though they consume substantial resources. Nationwide inpatient cost estimates and costs at longitudinal follow-up are lacking.
Methods:Retrospective cohort study of children <19 years of age admitted to Pediatric Health Information System administrative database with an International Classification of Diseases diagnosis code undergoing cardiac surgery. Patients were grouped into neonates (≤30 days of age), infants (31–365 days of age), and children (>1 year) at index procedure. Primary and secondary outcomes included hospital stay and hospital costs at index surgical admission and 1- and 5-year follow-up.
Results:Of the 99,670 cohort patients, neonates comprised 27% and had the highest total hospital costs, though daily hospital costs were lower. Mortality declined (5.6% in 2004 versus 2.5% in 2015, p < 0.0001) while inpatient costs rose (5% increase/year, p < 0.0001). Neonates had greater index diagnosis complexity, greater inpatient costs, required the greatest ICU resources, pharmacotherapy, and respiratory therapy. We found no relationship between hospital surgical volume, mortality, and hospital costs. Neonates had higher cumulative hospital costs at 1- and 5-year follow-up compared to infants and children.
Conclusions:Inpatient hospital costs rose during the study period, driven primarily by longer stay. Neonates had greater complexity index diagnosis, required greater hospital resources, and have higher hospital costs at 1 and 5 years compared to older children. Surgical volume and in-hospital mortality were not associated with costs. Further analyses comprising merged clinical and administrative data are necessary to identify longer stay and cost drivers after paediatric cardiac surgery.
Radiographic and histologic characterisation of white matter injury in a sheep model of CHD
- Kendall M. Lawrence, Enrico Radaelli, Patrick E. McGovern, Daniel J. Licht, Marcus G. Davey, Alan W. Flake, J. William Gaynor, Arastoo Vossough
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- 19 April 2022, pp. 432-436
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Nearly one in five children with CHD is born with white matter injury that can be recognised on postnatal MRI by the presence of T1 hyperintense lesions. This pattern of white matter injury is known to portend poor neurodevelopmental outcomes, but the exact aetiology and histologic characterisation of these lesions have never been described. A fetal sheep was cannulated at gestational age 110 days onto a pumpless extracorporeal oxygenator via the umbilical vessels and supported in a fluid environment for 14.5 days. The fetus was supported under hypoxic conditions (mean oxygen delivery 16 ml/kg/day) to simulate the in utero conditions of CHD. At necropsy, the brain was fixed, imaged with MRI, and then stained to histologically identify areas of injury. Under hypoxemic in utero conditions, the fetus developed a T1 hyperintense lesion in its right frontal lobe. Histologically, this lesion was characterised by microvascular proliferation and astrocytosis without gliosis. These findings may provide valuable insight into the aetiology of white matter injury in neonates with CHD.
Safety of drug-eluting stents for stenting patent arterial duct in neonates
- Harikrishnan K.N. Kurup, Arun Gopalakrishnan, Deepa Sasikumar, Kavassery M. Krishnamoorthy
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- 05 May 2022, pp. 437-443
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Objectives:
The primary objective was to evaluate the trend of blood sirolimus concentrations in neonates following ductal stenting. The long-term outcomes and incidence of infections were also evaluated.
Methods:Prospective open-label observational study in a tertiary referral centre over a 1-year period. Serum sirolimus levels were estimated at 1 hour and 24hrs post-stent insertion followed by 7 days in neonates who underwent ductal stenting. The trend in sirolimus levels, incidence of infections, complications and outcomes following ductal stenting were studied.
Results:Seven neonates with duct-dependent pulmonary circulation underwent ductal stenting at median age of 8.5 days and weight of 2.83kg. The average stent size was 3.5±0.4 mm, and average stent length was 16.3±5.1 mm. The mean sirolimus concentrations at 1 hour, 24 hours and 7 days were 41.3±6.9ng/ml, 15.4±7.1ng/ml and 3.1±0.85ng/ml respectively. Levels fell below therapeutic range for all patients by 7 days. Three patients had sepsis or necrotising enterocolitis, but responded well to antibiotics; 1 patient had aspiration related sudden death. There were no further events at a mean follow-up of 207 days, and 4 patients underwent elective surgery at 238 ± 81 days after ductal stenting.
Conclusions:This study demonstrates applicability of drug-eluting stents for ductal stenting in newborns. Drug-eluting stents with abluminal drug delivery are associated with high sirolimus levels in initial hours but rapidly taper to negligible levels within a week of implantation. Neonates with high pre-procedure likelihood of infection developed sepsis but responded well to conservative management. The patency of drug-eluting ductal stents is preserved over long-term follow-up.
A multimedia paediatric cardiology assessment tool for medical students and general paediatric trainees: development and validation
- Hunter C. Wilson, Tiffany R. Lim, David M. Axelrod, David K. Werho, Stephanie S. Handler, Patricia B. Mullan, James M. Cooke, Sonal T. Owens
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- 12 April 2022, pp. 444-448
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Background:
Understanding how cardiovascular structure and physiology guide management is critically important in paediatric cardiology. However, few validated educational tools are available to assess trainee knowledge. To address this deficit, paediatric cardiologists and fellows from four institutions collaborated to develop a multimedia assessment tool for use with medical students and paediatric residents. This tool was developed in support of a novel 3-dimensional virtual reality curriculum created by our group.
Methods:Educational domains were identified, and questions were iteratively developed by a group of clinicians from multiple centres to assess understanding of key concepts. To evaluate content validity, content experts completed the assessment and reviewed items, rating item relevance to educational domains using a 4-point Likert scale. An item-level content validity index was calculated for each question, and a scale-level content validity index was calculated for the assessment tool, with scores of ≥0.78 and ≥0.90, respectively, representing excellent content validity.
Results:The mean content expert assessment score was 92% (range 88–97%). Two questions yielded ≤50% correct content expert answers. The item-level content validity index for 29 out of 32 questions was ≥0.78, and the scale-level content validity index was 0.92. Qualitative feedback included suggestions for future improvement. Questions with ≤50% content expert agreement and item-level content validity index scores <0.78 were removed, yielding a 27-question assessment tool.
Conclusions:We describe a multi-centre effort to create and validate a multimedia assessment tool which may be implemented within paediatric trainee cardiology curricula. Future efforts may focus on content refinement and expansion to include additional educational domains.
Echocardiographic changes and impact on clinical management in pregnant women with heart disease
- Karishma Patel, Lidija McGrath, Paola Roldan, Marshall Taunton, Kathleen Brookfield, Emmanuelle Paré, Abigail Khan
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- Published online by Cambridge University Press:
- 21 June 2022, pp. 449-456
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Background:
While guidelines recommend echocardiography for pregnant women with heart disease, there are limited data on its effect on clinical practice. In this study, we investigated pregnancy-associated echocardiographic changes and their impact on management.
Methods:This was a retrospective study of pregnant women with heart disease followed at an academic medical centre from 2016 to 2020. Data on maternal intrapartum and postpartum echocardiograms were collected and the impact on management analysed.
Results:421 echocardiograms in 232 pregnancies were included in the study. The most common cardiac diagnosis was CHD (60.8% of pregnancies), followed by cardiomyopathy (9.9%). The frequency of baseline echocardiographic abnormalities varied by diagnosis, with abnormal right ventricular systolic pressure being the most common (15.0% of pregnancies in CHD and 23.1% of pregnancies with cardiomyopathy). 39.2% of the 189 follow-up echocardiograms had a significant change from the prior study, with the most common changes being declines in right ventricular function (4.2%) or left ventricular function (3.7%), and increases in right ventricular systolic pressure (5.3%) and aortic size (21.2%). 17.8% of echocardiograms resulted in a clinical management change, with the most common change being shorter interval follow-up.
Conclusions:Echocardiographic changes in pregnant women with heart disease are common, in particular increases in aortic size. Echocardiography results in changes in management in a small but significant proportion of patients. Further studies are needed to determine how other factors, including patient access and resource allocation, factor into the use of echocardiography during pregnancy.