Original Article
The association between feeding protocol compliance and weight gain following high-risk neonatal cardiac surgery
- Jamie M. Furlong-Dillard, Benjamin J. Miller, Kathy A. Sward, Alaina I. Neary, Trudy L. Hardin-Reynolds, Grace Jeffers, Bonnie A. Clay, Dongngan T. Truong, Thomas A. Miller, Courtney E. Jones, Linda M. Lambert, David K. Bailly
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- Published online by Cambridge University Press:
- 24 May 2019, pp. 594-601
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Background:
Children with congenital heart disease are at high risk for malnutrition. Standardisation of feeding protocols has shown promise in decreasing some of this risk. With little standardisation between institutions’ feeding protocols and no understanding of protocol adherence, it is important to analyse the efficacy of individual aspects of the protocols.
Methods:Adherence to and deviation from a feeding protocol in high-risk congenital heart disease patients between December 2015 and March 2017 were analysed. Associations between adherence to and deviation from the protocol and clinical outcomes were also assessed. The primary outcome was change in weight-for-age z score between time intervals.
Results:Increased adherence to and decreased deviation from individual instructions of a feeding protocol improves patients change in weight-for-age z score between birth and hospital discharge (p = 0.031). Secondary outcomes such as markers of clinical severity and nutritional delivery were not statistically different between groups with high or low adherence or deviation rates.
Conclusions:High-risk feeding protocol adherence and fewer deviations are associated with weight gain independent of their influence on nutritional delivery and caloric intake. Future studies assessing the efficacy of feeding protocols should include the measures of adherence and deviations that are not merely limited to caloric delivery and illness severity.
Development of feeding information for infants with CHD
- Luise V. Marino, Mark J. Johnson, Natalie J. Davies, Catherine Kidd, Trevor Richens, Tara Bharucha, Anne-Sophie Darlington
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- Published online by Cambridge University Press:
- 05 August 2019, pp. 1165-1171
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Introduction:
Infants with CHD often experience growth failure. Ensuring optimal growth before surgery is associated with improved outcomes and has emerged as a significant cause of parental stress. Parents have reported a perceived lack of accessible feeding information for infants with CHD. To address this gap, the aim of this study was to develop feeding information to better support parents.
Materials and methods:A search for existing material on six electronic databases and an internet search for unpublished (grey) literature on feeding information for infants with CHD were carried out. Following the development of feeding information, semi-structured interview(s) with parents/health-care professionals were completed, focusing on whether the information was easy to understand, relevant, provided sufficient information around feeding/feeding difficulties, and whether there were any information gaps. Iterative changes were made to the information following each interview. The process was completed until thematic saturation was achieved.
Results:A total of 23 unique articles were identified of which 5 studies were included. From the grey literature, four web pages were reviewed. A total of 22 parents and 25 health-care professionals were interviewed. All parents/health-care professionals felt that the feeding information developed provided sufficient information; however, many wanted information on how to introduce complementary food, particularly if weaning was delayed.
Conclusions:This study describes the development of feeding information for infants with CHD. From parent interviews, gaps identified focused on the introduction of complementary foods and uncertainty regarding the feeding journey beyond surgery.
Creation of a novel algorithm to identify patients with Becker and Duchenne muscular dystrophy within an administrative database and application of the algorithm to assess cardiovascular morbidity
- Jonathan H Soslow, Matthew Hall, W Bryan Burnette, Kan Hor, Joanne Chisolm, Christopher Spurney, Justin Godown, Meng Xu, James C Slaughter, Larry W Markham
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- Published online by Cambridge University Press:
- 26 January 2019, pp. 290-296
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Background
Outcome analyses in large administrative databases are ideal for rare diseases such as Becker and Duchenne muscular dystrophy. Unfortunately, Becker and Duchenne do not yet have specific International Classification of Disease-9/-10 codes. We hypothesised that an algorithm could accurately identify these patients within administrative data and improve assessment of cardiovascular morbidity.
MethodsHospital discharges (n=13,189) for patients with muscular dystrophy classified by International Classification of Disease-9 code: 359.1 were identified from the Pediatric Health Information System database. An identification algorithm was created and then validated at three institutions. Multi-variable generalised linear mixed-effects models were used to estimate the associations of length of stay, hospitalisation cost, and 14-day readmission with age, encounter severity, and respiratory disease accounting for clustering within the hospital.
ResultsThe identification algorithm improved identification of patients with Becker and Duchenne from 55% (code 359.1 alone) to 77%. On bi-variate analysis, left ventricular dysfunction and arrhythmia were associated with increased cost of hospitalisation, length of stay, and mortality (p<0.001). After adjustment, Becker and Duchenne patients with left ventricular dysfunction and arrhythmia had increased length of stay with rate ratio 1.4 and 1.2 (p<0.001 and p=0.004) and increased cost of hospitalization with rate ratio 1.4 and 1.4 (both p<0.001).
ConclusionsOur algorithm accurately identifies patients with Becker and Duchenne and can be used for future analysis of administrative data. Our analysis demonstrates the significant effects of cardiovascular disease on length of stay and hospitalisation cost in patients with Becker and Duchenne. Better recognition of the contribution of cardiovascular disease during hospitalisation with earlier more intensive evaluation and therapy may help improve outcomes in this patient population.
CT evaluation of aberrant right subclavian artery: anatomy and clinical implications
- Maciej Krupiński, Małgorzata Irzyk, Zbigniew Moczulski, Robert Banyś, Ireneusz Dwojak, Małgorzata Urbańczyk-Zawadzka
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- Published online by Cambridge University Press:
- 23 November 2018, pp. 128-132
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Purpose
The aim of the study was to perform CT angiography-based evaluation of aberrant right subclavian artery prevalence, anatomy, and its influence on clinical symptoms.
MethodsA total of 6833 patients who underwent 64-slice or dual-source CT angiography and those who revealed aberrant right subclavian artery underwent evaluation of its anatomy and were interviewed for the presence of clinical symptoms.
ResultsAberrant right subclavian artery was found in 32 (0.47%) patients consisting of 13 males and 19 females, with mean age of 60.8±13.4 years. Among the interviewed 30 (94%) patients, oesophageal compression was observed in 14 cases (47%) and tracheal compression in three cases (10%). None of the patients underwent surgery related to aberrant right subclavian artery. Dysphagia was the most common clinical symptom in nine cases (30%), and in those patients the median distance between aberrant right subclavian artery and trachea was lower (4 mm) than in individuals without dysphagia (7.5 mm) (p = 0.009). The median lumen area of the aberrant right subclavian artery at the level of oesophagus was higher in patients with dysphagia (208 mm2) compared with individuals without dysphagia (108 mm2) (p = 0.01).
ConclusionsAberrant right subclavian artery is a rare occurring abnormality in CT angiography. In the evaluated adult population, the most common symptom was dysphagia, which occurred in patients with decreased distance between aberrant right subclavian artery and trachea and increased lumen area of the aberrant artery at the level of compressed oesophagus.
Ruptured mitral valves chordae tendineae around a convalescent infant with acute Kawasaki disease
- Etsuko Tsuda, Jun Negishi, Hikari Miike, Kenichi Kurosaki
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- 26 October 2018, pp. 30-35
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Severe valvulitis owing to acute Kawasaki disease leading to severe mitral regurgitation is a rare event in infants. Further, there is less information about underlying ruptured mitral chordae tendineae causing severe mitral regurgitation. We encountered ruptured mitral chordae tendineae in three female patients after Kawasaki disease. The age at the onset of Kawasaki disease ranged from 3 to 8 months, and detection of ruptured mitral chordae tendineae was from 24 to 90 days. Two patients had acute heart failure, and one was asymptomatic. One patient underwent mitral annuloplasty, and the others responded to medication. These ruptured mitral chordae tendineae occurred after the remission of the initial acute Kawasaki disease, in the early course and the convalescent of acute Kawasaki disease. Further, the recurrent fever was also detected in them. The ruptured mitral chordae tendineae in an infant within 6 months can be detected by systolic heart murmur around the convalescent stage of acute Kawasaki disease, although the prevalence is very low.
Red cell transfusion practices after stage 1 palliation: a survey of practitioners from the Pediatric Cardiac Intensive Care Society
- Aditya Badheka, Priscilla Yu, Felina Mille, Dennis Durbin, Okan Elci, Joshua Blinder
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- 14 November 2019, pp. 1452-1458
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Introduction:
Neonates may require increased red cell mass to optimise oxygen content after stage 1 palliation; however, data informing transfusion practices are limited. We hypothesise there is a patient-, provider-, and institution-based heterogeneity in red cell transfusion decision-making after stage 1 palliation.
Methods:We conducted an online survey of Pediatric Cardiac Intensive Care Society practitioners in 2016. Respondents answered scenario-based questions that defined transfusion indications and identified haematocrit transfusion thresholds. Respondents were divided into restrictive and liberal groups based on a haematocrit score. Fisher’s exact test was used to determine the associations between transfusion likelihood and patient, provider, and institutional characteristics. Bonferroni correction was applied to adjust the p-value to 0.004 for multiple comparisons.
Results:There was a 21% response rate (116 responses). Most were male (58.6%), attending physicians (85.3%) with >5 year of intensive care experience (88.7%) and subspeciality training in critical care medicine (47.4%). The majority of institutions were academic (96.6%), with a separate cardiac ICU (86.2%), and performed >10 stage 1 palliation cases annually (68.1%). After Bonferroni correction, there were no significant patient, respondent, or institutional differences between the restrictive and liberal groups. No respondent or institutional characteristics influenced transfusion decision-making after stage 1 palliation.
Conclusions:Decision-making around red cell transfusion after stage 1 palliation is heterogeneous. We found no clear relationships between patient, respondent, or institutional characteristics and transfusion decision-making among surveyed respondents. Given the lack of existing data informing red cell transfusion after stage 1 palliation, further studies are necessary to inform evidence-based guidelines.
A single centre experience with an evolving approach for the repair of coarctation of the aorta
- Kelly Costopoulos, Joseph Philip, Dalia Lopez-Colon, Giridhar Kaliki, Arun Chandran, Mark Bleiweis
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- Published online by Cambridge University Press:
- 12 July 2019, pp. 885-887
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Background:
Isolated coarctation of the aorta can be repaired by either lateral thoracotomy or sternotomy approach with end-to-end anastomosis. Most commonly, neonates with coarctation of the aorta also have hypoplasia of the arch, requiring median sternotomy and extended end-to-side anastomosis with arch augmentation. The aim of this study was to describe our experience as the institution adopted the median sternotomy approach for repair, by reviewing complications, mortality, and reintervention.
Methods:Retrospective chart review of 66 patients aged 0–1 year who had arch repair performed by a single surgeon over an 8-year period was performed. Median age at surgery was 22 days (4–232) and median weight was 3.08 kg (1.25–8.0). Forty-one (62%) patients underwent median sternotomy.
Results:There was 1 death from a noncardiac cause. Eighteen per cent of our patients were ≤2.5 kg. Vocal cord paresis occurred in 16% of patients under 2.5 kg and 9.5% of patients 2.5 kg or above at the time of surgery. Hypertension at 6-month follow-up was greater in patients under 2.5 kg (44%) than patients 2.5 kg or above (15%). Total surgical reintervention rate was 6%. For patients above 2.5 kg, the surgical reintervention rate was 5.4% and for patients below 2.5 kg, the surgical reintervention rate was 8.3%.
Conclusion:We concluded that for neonates with coarctation of the aorta and hypoplastic arch, median sternotomy is a safe surgical approach with low morbidity and mortality with the possible advantage of reduced surgical re-intervention and mortality in the population below 2.5 kg.
Learning strategies among adult CHD fellows
- Jouke P. Bokma, Joshua A. Daily, Adrienne H. Kovacs, Erwin N. Oechslin, Helmut Baumgartner, Paul Khairy, Barbara J.M. Mulder, Gruschen R. Veldtman
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- Published online by Cambridge University Press:
- 10 September 2019, pp. 1356-1360
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Objective:
Subspecialisation is increasingly a fundamental part of the contemporary practice of medicine. However, little is known about how medical trainees learn in the modern era, and particularly in growing and relatively new subspecialties, such as adult CHD. The purpose of this study was to assess institutional-led and self-directed learning strategies of adult CHD fellows.
Methods:This international, cross-sectional online survey was conducted by the International Society for Adult Congenital Heart Disease and consisted primarily of categorical questions and Likert rating scales. All current or recent (i.e., those within 2 years of training) fellows who reported training in adult CHD (within adult/paediatric cardiology training or within subspecialty fellowships) were eligible.
Results:A total of 75 fellows participated in the survey: mean age: 34 ± 5; 35 (47%) female. Most adult CHD subspecialty fellows considered case-based teaching (58%) as “very helpful”, while topic-based teaching was considered “helpful” (67%); p = 0.003 (favouring case-based). When facing a non-urgent clinical dilemma, fellows reported that they were more likely to search for information online (58%) than consult a faculty member (29%) or textbook (3%). Many (69%) fellows use their smartphones at least once daily to search for information during regular clinical work.
Conclusions:Fellows receiving adult CHD training reported a preference for case-based learning and frequent use of online material and smartphones. These findings may be incorporated into the design and enhancement of fellowships and development of online training resources.
Early conversion of classic Fontan conversion may decrease term morbidity: single centre outcomes
- David Blitzer, Asma S. Habib, John W. Brown, Adam C. Kean, Jiuann-Huey I. Lin, Mark W. Turrentine, Mark D. Rodefeld, Jeremy L. Herrmann, William Aaron Kay
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- Published online by Cambridge University Press:
- 28 June 2019, pp. 1045-1050
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Background:
The initial classic Fontan utilising a direct right atrial appendage to pulmonary artery anastomosis led to numerous complications. Adults with such complications may benefit from conversion to a total cavo-pulmonary connection, the current standard palliation for children with univentricular hearts.
Methods:A single institution, retrospective chart review was conducted for all Fontan conversion procedures performed from July, 1999 through January, 2017. Variables analysed included age, sex, reason for Fontan conversion, age at Fontan conversion, and early mortality or heart transplant within 1 year after Fontan conversion.
Results:A total of 41 Fontan conversion patients were identified. Average age at Fontan conversion was 24.5 ± 9.2 years. Dominant left ventricular physiology was present in 37/41 (90.2%) patients. Right-sided heart failure occurred in 39/41 (95.1%) patients and right atrial dilation was present in 33/41 (80.5%) patients. The most common causes for Fontan conversion included atrial arrhythmia in 37/41 (90.2%), NYHA class II HF or greater in 31/41 (75.6%), ventricular dysfunction in 23/41 (56.1%), and cirrhosis or fibrosis in 7/41 (17.1%) patients. Median post-surgical follow-up was 6.2 ± 4.9 years. Survival rates at 30 days, 1 year, and greater than 1-year post-Fontan conversion were 95.1, 92.7, and 87.8%, respectively. Two patients underwent heart transplant: the first within 1 year of Fontan conversion for heart failure and the second at 5.3 years for liver failure.
Conclusions:Fontan conversion should be considered early when atrial arrhythmias become common rather than waiting for severe heart failure to ensue, and Fontan conversion can be accomplished with an acceptable risk profile.
Tissue characterisation and myocardial mechanics using cardiac MRI in children with hypertrophic cardiomyopathy
- Sudeep Sunthankar, David A. Parra, Kristen George-Durrett, Kimberly Crum, Joshua D. Chew, Jason Christensen, Frank J. Raucci, Jr, Meng Xu, James C. Slaughter, Jonathan H. Soslow
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- Published online by Cambridge University Press:
- 26 November 2019, pp. 1459-1467
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Introduction:
Distinguishing between hypertrophic cardiomyopathy and other causes ofleft ventricular hypertrophy can be difficult in children. We hypothesised that cardiac MRI T1 mapping could improve diagnosis of paediatric hypertrophic cardiomyopathy and that measures of myocardial function would correlate with T1 times and extracellular volume fraction.
Methods:Thirty patients with hypertrophic cardiomyopathy completed MRI with tissue tagging, T1-mapping, and late gadolinium enhancement. Left ventricular circumferential strain was calculated from tagged images. T1, partition coefficient, and synthetic extracellular volume were measured at base, mid, apex, and thickest area of myocardial hypertrophy. MRI measures compared to cohort of 19 healthy children and young adults. Mann–Whitney U, Spearman’s rho, and multivariable logistic regression were used for statistical analysis.
Results:Hypertrophic cardiomyopathy patients had increased left ventricular ejection fraction and indexed mass. Hypertrophic cardiomyopathy patients had decreased global strain and increased native T1 (−14.3% interquartile range [−16.0, −12.1] versus −17.3% [−19.0, −15.7], p < 0.001 and 1015 ms [991, 1026] versus 990 ms [972, 1001], p = 0.019). Partition coefficient and synthetic extracellular volume were not increased in hypertrophic cardiomyopathy. Global native T1 correlated inversely with ejection fraction (ρ = −0.63, p = 0.002) and directly with global strain (ρ = 0.51, p = 0.019). A logistic regression model using ejection fraction and native T1 distinguished between hypertrophic cardiomyopathy and control with an area under the receiver operating characteristic curve of 0.91.
Conclusion:In this cohort of paediatric hypertrophic cardiomyopathy, strain was decreased and native T1 was increased compared with controls. Native T1 correlated with both ejection fraction and strain, and a model using native T1 and ejection fraction differentiated patients with and without hypertrophic cardiomyopathy.
Pulmonary effects on exercise testing in tetralogy of Fallot patients repaired with a transannular patch
- Adam W. Powell, Wayne A. Mays, Sandra K. Knecht, Clifford Chin
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- 26 November 2018, pp. 133-139
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Background
A transannular patch is often used in the contemporary surgical repair of tetralogy of Fallot. This can lead to significant pulmonary insufficiency and increased right ventricular volumes and ultimately pulmonary valve replacement. Cardiopulmonary exercise testing is used to assess exercise capacity in tetralogy of Fallot patients before pulmonary valve replacement. There is only few published literatures on how lung function affects functional capacity in tetralogy of Fallot patients repaired with a transannular patch.
MethodsA retrospective chart review was done from 2015 to 2017 on patients with tetralogy of Fallot who underwent maximal effort cardiopulmonary exercise testing with cycle ergometry and with concurrent pulmonary function testing. Tetralogy of Fallot patients repaired with a transannular patch without pulmonary valve replacement were compared with age, gender, and size-matched normal controls.
ResultsIn the tetralogy of Fallot group, 24 out of 57 patients underwent primary repair with a transannular patch. When compared to the normal controls, they demonstrated abnormal predicted forced expiratory volume in one second (79 ± 23.1% versus 90.7 ± 14.1%, p<0.05), predicted maximal voluntary ventilation (74 ± 18% versus 90.5 ± 16.2%, p<0.05) while having low-normal predicted forced vital capacity (80.5 ± 17.2% versus 90.2 ± 12.4%, p<0.05) and normal breathing reserve percentage (50.3 ± 11.3% versus 47.5 ± 17.3%, p = 0.52). Cardiopulmonary exercise testing abnormalities included significantly lower percent predicted oxygen consumption (63.2 ± 12.2% versus 87 ± 12.1%, p<0.05), maximal heart rate (171.8 ± 18.9 versus 184.6 ± 13.6, p<0.05), and percent predicted maximum workload (61.7 ± 15.9% versus 88.3 ± 21.5%, p<0.05).
ConclusionsTetralogy of Fallot patients repaired with a transannular patch can have abnormal pulmonary function testing with poor exercise capacity in addition to chronotropic incompetence and impaired muscular power.
CHIP-Family intervention to improve the psychosocial well-being of young children with congenital heart disease and their families: results of a randomised controlled trial
- Malindi van der Mheen, Maya G. Meentken, Ingrid M. van Beynum, Jan van der Ende, Eugène van Galen, Anne Zirar, Elisabeth W.C. Aendekerk, Tabitha P.L. van den Adel, Ad J.J.C. Bogers, Christopher G. McCusker, Manon H.J. Hillegers, Willem A. Helbing, Elisabeth M.W.J. Utens
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- Published online by Cambridge University Press:
- 05 August 2019, pp. 1172-1182
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Objective:
Children with congenital heart disease and their families are at risk of psychosocial problems. Emotional and behavioural problems, impaired school functioning, and reduced exercise capacity often occur. To prevent and decrease these problems, we modified and extended the previously established Congenital Heart Disease Intervention Program (CHIP)–School, thereby creating CHIP-Family. CHIP-Family is the first psychosocial intervention with a module for children with congenital heart disease. Through a randomised controlled trial, we examined the effectiveness of CHIP-Family.
Methods:Ninety-three children with congenital heart disease (age M = 5.34 years, SD = 1.27) were randomised to CHIP-Family (n = 49) or care as usual (no psychosocial care; n = 44). CHIP-Family consisted of a 1-day group workshop for parents, children, and siblings and an individual follow-up session for parents. CHIP-Family was delivered by psychologists, paediatric cardiologists, and physiotherapists. At baseline and 6-month follow-up, mothers, fathers, teachers, and the child completed questionnaires to assess psychosocial problems, school functioning, and sports enjoyment. Moreover, at 6-month follow-up, parents completed program satisfaction assessments.
Results:Although small improvements in child outcomes were observed in the CHIP-Family group, no statistically significant differences were found between outcomes of the CHIP-Family and care-as-usual group. Mean parent satisfaction ratings ranged from 7.4 to 8.1 (range 0–10).
Conclusions:CHIP-Family yielded high program acceptability ratings. However, compared to care as usual, CHIP-Family did not find the same extent of statistically significant outcomes as CHIP-School. Replication of promising psychological interventions, and examination of when different outcomes are found, is recommended for refining interventions in the future.
Trial registryDutch Trial Registry number NTR6063, https://www.trialregister.nl/trial/5780.
Congenital cardiac surgery and parental perception of risk: a qualitative study
- Robyn R. Lotto, Ian D. Jones, Rafael Guerrero, Ram Dhannapuneni, Attilio A. Lotto
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- 13 September 2019, pp. 1361-1367
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Introduction:
The way risk is interpreted by parents of children undergoing congenital cardiac surgery is poorly documented. Literature suggests clinicians have concerns that parents may not understand the complexity of procedures. Conversely, some parents perceive an unnecessary over-emphasis of risks.
Aim:To explore how risk is encountered by parents of children who are undergoing cardiac surgery, in order to deliver effective and compassionate care.
Methods:A qualitative approach was adopted. Interviews were undertaken with 18 parents (mothers n = 10; fathers n = 8). Recordings were transcribed verbatim and analysed using a constant comparative-based approach.
Findings:Three themes emerged from the data: the nature of risk, reflecting the complexity of parental perception of risk and the influence of the doctor–parent relationship; presenting risk, highlighting the way in which risk is presented to and interpreted by parents; and risk and responsibility, examining the way in which parents engaged with risk and the impact of this on their relationship.
Conclusions:The way in which risk is perceived by parents is complex and multi-factorial. The doctor–parent relationship is key to parental engagement. However, parents manage risk and uncertainty through a number of mechanisms, including those perceived as being not rational. This can cause tension, particularly when required to engage in informed decision-making.
Anxiety, depression, and behavioural rating scales in children with non-cardiac chest pain
- Aytaç Kenar, Utku Arman Örün, Tamer Yoldaş, Şeyma Kayalı, Şahin Bodur, Selmin Karademir
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- 30 August 2019, pp. 1268-1271
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Introduction:
Chest pain is the second most common reason for referral to paediatric cardiologists after benign heart murmurs. Aetiology frequently depends on non-cardiac reasons. In addition, individuals may experience non-cardiac chest pain which is idiopathic or of unknown origin. The aim of this study is to examine psychological symptoms in children and adolescents with medically unexplained chest pain.
Methods:A total of 76 patients (ages 8–18 years) were included in the study, who were referred to the paediatric cardiology department with the complaint of chest pain but did not have any detected cardiac aetiology or any other organic causes of chest pain. The control group was composed of 51 healthy volunteers. Self-evaluation scales were given to both groups which included Beck Anxiety Inventory and Children’s Depression Inventory. Also parents of both groups completed the Conner’s Parent Rating Scale for assessment of Attention-deficit/hyperactivity disorder.
Results:Anxiety scores of the non-cardiac chest pain group were significantly higher compared to controls. No significant differences were found between patients and controls in terms of attention-deficit/hyperactivity disorder and depression scores. In patient group, patterns were similar for boys and girls and for children and adolescents; except girls scored significantly higher than boys in children’s depression inventory.
Conclusions:In children and adolescents, non-cardiac chest pain is associated with increased levels of anxiety. These results show the importance of psychiatric evaluation in non-cardiac chest pain patients. Larger controlled studies are needed to determine the prevalence and impact of attention-deficit/hyperactivity disorder and depression in children and adolescents with non-cardiac chest pain.
Ethics in humanitarian efforts: when should resources be allocated to paediatric heart surgery?
- Kathleen N. Fenton, Marcelo Cardarelli, Frank Molloy, William M. Novick
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- 18 October 2018, pp. 36-39
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Background
In countries with ample resources, no debate exists as to whether heart surgery should be provided. However, where funding is limited, what responsibility exists to care for children with congenital heart defects? If children have a “right” to surgical treatment, to whom is the “duty” to provide it assigned? These questions are subjected to ethical analysis.
MethodsExamination is initially based on the four principles of medical ethics: autonomy, beneficence, non-maleficence, and justice. Consideration of beneficence and justice is expanded using a consequentialist approach.
ResultsSocial structures, including governments, exist to foster the common good. Society, whether by means of government funding or otherwise, has the responsibility, according to the means available, to assure health care for all based on the principles of beneficence, non-maleficence, and justice. In wealthy countries, adequate resources exist to fund appropriate treatment; hence it should be provided to all based on distributive justice. In resource-limited countries, however, decisions regarding provision of care for expensive or complex health problems must be made with consideration for broader effects on the general public. Preliminary data from cost-effectiveness analysis indicate that many surgical interventions, including cardiac surgery, may be resource-efficient. Given that information, utilitarian ethical analysis supports dedication of resources to congenital heart surgery in many low-income countries. In the poorest countries, where access to drinking water and basic nutrition is problematic, it will often be more appropriate to focus on these issues first.
ConclusionEthical analysis supports dedication of resources to congenital heart surgery in all but the poorest countries.
Near-infrared spectroscopy for prediction of extubation success after neonatal cardiac surgery
- Eleanor A. Gradidge, Lisa M. Grimaldi, Katherine Cashen, Keshava M. N. Gowda, Kurt D. Piggott, Michael Wilhelm, John M. Costello, Christopher W. Mastropietro
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- Published online by Cambridge University Press:
- 06 June 2019, pp. 787-792
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Introduction:
Reliable predictors of extubation readiness are needed and may reduce morbidity related to extubation failure. We aimed to examine the relationship between changes in pre-extubation near-infrared spectroscopy measurements from baseline and extubation outcomes after neonatal cardiac surgery.
Materials and Methods:In this retrospective cross-sectional multi-centre study, a secondary analysis of prospectively collected data from neonates who underwent cardiac surgery at seven tertiary-care children’s hospitals in 2015 was performed. Extubation failure was defined as need for re-intubation within 72 hours of the first planned extubation attempt. Near-infrared spectroscopy measurements obtained before surgery and before extubation in patients who failed extubation were compared to those of patients who extubated successfully using t-tests.
Results:Near-infrared spectroscopy measurements were available for 159 neonates, including 52 with single ventricle physiology. Median age at surgery was 6 days (range: 1–29 days). A total of 15 patients (9.4 %) failed extubation. Baseline cerebral and renal near-infrared spectroscopy measurements were not statistically different between those who were successfully extubated and those who failed, but pre-extubation cerebral and renal values were significantly higher in neonates who extubated successfully. An increase from baseline to time of extubation values in cerebral oximetry saturation by ≥ 5 % had a positive predictive value for extubation success of 98.6 % (95%CI: 91.1–99.8 %).
Conclusion:Pre-extubation cerebral near-infrared spectroscopy measurements, when compared to baseline, were significantly associated with extubation outcomes. These findings demonstrate the potential of this tool as a valuable adjunct in assessing extubation readiness after paediatric cardiac surgery and warrant further evaluation in a larger prospective study.
Clinical aspects of splenomegaly as a possible predictive factor of coronary artery changes in Kawasaki disease
- Dong Wan Kang, Sung Hoon Kim
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- Published online by Cambridge University Press:
- 21 December 2018, pp. 297-302
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Background
Although many clinical features that are not typically included in the diagnostic criteria for Kawasaki disease, such as gall bladder hydrops, are known to occur with Kawasaki disease, splenomegaly is not concerned. We investigated the relationship of splenomegaly with the development of coronary artery lesions in Kawasaki disease.
Methods and resultsThis retrospective descriptive study was conducted through a review of medical records of children with Kawasaki disease from March 2011 to February 2017. We analysed information regarding clinical presentation, treatment, hospital stay, and outcome. A total of 396 patients during this 6-year period met the enrolment criteria. Of these, 77 (23.4%) underwent abdominal ultrasonography during the treatment period. The patients included 46 males and 31 females with an average age of 35.8 ± 26.1 months. Gallbladder hydrops were detected in 32 patients, and acute acalculous cholecystitis was not found. Splenomegaly was detected in 21 patients. Kawasaki disease patients with gallbladder hydrops had no statistical difference in clinical or laboratory findings or in development of coronary artery lesions compared to patients without gallbladder hydrops. However, patients with splenomegaly belonged more to incomplete Kawasaki disease, had longer fever duration, had more frequent cervical lymphadenopathy and polymorphous rash, had higher neutrophil percentage, N-terminal fragment of pro-brain natriuretic peptide, and alanine aminotransferase levels, and a higher incidence of coronary artery lesions than patients without splenomegaly.
ConclusionSplenomegaly belongs to incomplete Kawasaki disease patients mainly with a higher incidence of coronary artery lesions than that of patients without it.
Determination of dynamic thiol/disulphide homeostasis in children with tetralogy of Fallot and ventricular septal defect
- Münevver T. Temel, Seniz Demiryürek, Ahmet Saracaloglu, Celal H. Ozer, Hale N. Kahraman, Mehmet E. Coskun, Osman Başpınar, Abdullah T. Demiryürek
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- Published online by Cambridge University Press:
- 01 April 2019, pp. 499-504
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Oxidative stress may contribute to the pathogenesis of congenital heart defects, but the role of dynamic thiol/disulphide homeostasis has not been evaluated. The objective of this study was to assess whether there are changes in thiol/disulphide homeostasis and nitric oxide levels in children with tetralogy of Fallot (TOF) and ventricular septal defect (VSD). A total of 47 children with congenital heart defects (24 TOF and 23 VSD) and 47 healthy age- and sex-matched controls were included in this study. Serum total thiol and native thiol levels were measured using a novel automatic spectrophotometric method. The amount of dynamic disulphide bonds and related ratios were calculated from these values. Serum nitric oxide levels were detected using a chemiluminescence assay. We found that the average native thiol, total thiol, and disulphide levels were decreased in patients with VSD when compared with healthy individuals (p < 0.001, p < 0.001, and p < 0.01, respectively). While native thiol levels were decreased (p < 0.01), disulphide levels were elevated in the TOF group (p < 0.05). We observed marked augmentation of disulphide/native thiol (p < 0.001) and disulphide/total thiol ratios (p < 0.01) in the TOF group. However, there was a significant decrease in native thiol/total thiol ratio in patients with TOF. No significant changes in these ratios were noted in the VSD group. We detected significant elevations in serum nitric oxide levels in children with TOF and VSD (p < 0.001 for all). These results are the first to demonstrate that thiol/disulphide homeostasis and nitric oxide are associated with TOF and VSD in children.
Prediction of heart failure and death in an adult population of Fontan patients
- Jan Thorben Sieweke, Arash Haghikia, Christian Riehle, Carolin Klages, Muharrem Akin, Tobias König, Carolin Zwadlo, Jens Treptau, Andreas Schäfer, Johann Bauersachs, Mechthild Westhoff-Bleck
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- Published online by Cambridge University Press:
- 30 April 2019, pp. 602-609
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Background:
Late Fontan survivors are at high risk to experience heart failure and death. Therefore, the current study sought to investigate the role of non-invasive diagnostics as prognostic markers for failure of the systemic ventricle following Fontan procedure.
Methods:This monocentric, longitudinal observational study included 60 patients with a median age of 24.5 (19–29) years, who were subjected to cardiac magnetic resonance imaging, echocardiography, cardiopulmonary exercise testing, and blood analysis. The primary endpoint of this study was decompensated heart failure with symptoms at rest, peripheral and/or pulmonary edema, and/or death.
Results:During a follow-up of 24 months, 5 patients died and 5 patients suffered from decompensated heart failure. Clinical (NYHA class, initial surgery), functional (VO2 peak, ejection fraction, cardiac index), circulating biomarkers (N-terminal pro brain natriuretic peptide), and imaging parameters (end diastolic volume index, end systolic volume index, mass-index, contractility, afterload) were significantly related to the primary endpoint. Multi-variate regression analysis identified afterload as assessed by cardiac magnetic resonance imaging as an independent predictor of the primary endpoint (hazard ratio 1.98, 95% confidence interval 1.19–3.29, p = 0.009).
Conclusion:We identified distinct parameters of cardiopulmonary exercise testing, cardiac magnetic resonance imaging, and blood testing as markers for future decompensated heart failure and death in patients with Fontan circulation. Importantly, our data also identify increased afterload as an independent predictor for increased morbidity and mortality. This parameter is easy to assess by non-invasive cardiac magnetic resonance imaging. Its modulation may represent a potential therapeutic approach target in these high-risk patients.
Outcome for children following admission to hospital with a first episode of heart failure, due to heart muscle disease, in the ventricular assist device (VAD) era
- Andres Rico-Armada, David S. Crossland, Louise Coats, Zdenka Reinhardt, Anthony Hermuzi, Neil Seller, Asif Hasan, John J. O’Sullivan
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- Published online by Cambridge University Press:
- 12 July 2019, pp. 888-892
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Aims:
Most reports on the outcome of children who present with heart failure, due to heart muscle disease, are from an era when ventricular assist devices were not available. This study provides outcome data for the current era where prolonged circulatory support can be considered for most children.
Methods & Results:Data was retrieved on 100 consecutive children, who presented between 2010 – 2016, with a first diagnosis of unexplained heart failure. Hospital outcome was classified as either death, transplantation, recovery of function or persistent heart failure. Median age at presentation was 24 months and 58% were < 5 years old. Hospital mortality was 12% and 59% received a heart transplant. Most, 79%, of the transplants were carried out on patients with a device. Recovery of function was observed in 18% and 10% stabilised on oral therapy. Eighty-four percent of the deaths occurred in the <5 year old group. Shorter duration of support was associated with survival (34 days in survivors versus 106 in non-survivors, p = 0.01) and 72% were on an assist device at time of death.
Conclusion:Heart failure in children who require referral to a transplant unit is a serious illness with a high chance of either transplantation or death. Modifications in assist devices will be required to improve safety, especially for children < 5 years old where the donor wait may be prolonged. The identification of children who may recover function requires further study.