Original Article
Early and midterm results of ductal stent implantation in neonates with ductal-dependent pulmonary circulation: a single-centre experience
- Mehmet Akif Onalan, Ender Odemis, Murat Saygi, Bahar Temur, Selim Aydin, Ibrahim Halil Demir, Ersin Erek
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- Published online by Cambridge University Press:
- 03 September 2020, pp. 1772-1782
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Objective:
We aimed to determine the early and midterm outcomes of ductal stenting in neonates with ductal-dependent pulmonary blood flow.
Methods:Between January, 2014 and July, 2018, 102 patients who underwent 115 cardiac catheterisation procedures for ductal stent implantation in our department were retrospectively reviewed. The age of the neonates ranged from 3 to 30 days (median: 11 days) and their weights ranged from 1.8 to 5.8 kg (mean, 2.8 ± 0.53 kg). Fifty-two patients had functional single ventricle and 50 had biventricular physiology. Thirty-one patients’ weights were <2,500 g (30.3%). The patent ductus arteriosus was vertical in 60 patients (58.8%). The mean ductal length was 12.4 ± 4.1 mm (range, 7.8–23 mm), and the mean narrowest ductal diameter was 2.1 ± 0.7 mm (range, 1.2–3.4 mm).
Results:The technical success rate was 85.2%. Procedure-related mortality occurred in three patients (2.9%). After the procedure, the aortic oxygen saturation increased from a mean of 73.1 ± 6.2% to a mean of 90.4 ± 4.3% (p < 0.001), and the ductus diameter increased from a mean of 2.1 ± 0.7 mm to a mean of 4.2 ± 0.9 mm (p < 0.001). Either transcatheter or surgical reinterventions were required in 35 patients (34.3%) during the follow-up period after a median of 101 days (2–356 days). Thirty-three patients (32.3%) were bridged to surgical repair after a median of 288 days (163–650 days). The median duration of palliation with ductal stents was 210 days (range, 2–525 days).
Conclusion:Ductus arteriosus stenting may be a reasonable and effective alternative to surgery for the initial palliation procedure in neonates with ductus-dependent pulmonary flow.
Review Article
Insights into stent implantation for coronary artery lesions caused by Kawasaki disease
- Etsuko Tsuda
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- 09 June 2020, pp. 911-918
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Fifty years have passed since the first report of Kawasaki disease in 1967, and the prevalence of acute coronary syndrome in Kawasaki disease patients with coronary artery lesions exceeding 40 years old has increased. Primary coronary stent implantation is currently an acceptable method in ischaemic coronary heart disease in adults. However, it is unknown whether the stent implantation is effective or not in this population. As the clue to answer this question, I reviewed the references on Kawasaki disease patients who underwent the stent implantations between 1997 and 2019. Thirty-three patients underwent stent implantations for 34 coronary arteries. Adverse effects in the late period were found in 19 (68%) of 28 vessels with follow-up angiograms. There were complete occlusion 9, restenosis 8, and migration 2. A new aneurysm formation was found in 7 (37%) among the 19 vessels, and 6 (86%) of the 7 vessels were drug-eluting stent and 5 were found after the procedure for chronic total occlusion. The adverse effects free-rate at 1 year and 3 years were 57 and 25%, respectively. At present, the usefulness of stent implantation in the long-term results was scarce. Even if primary percutaneous coronary intervention without a stent implantation is performed for acute coronary syndrome, it can be expected to maintain the patency of the culprit lesion for several years. It is better to avoid a stent implantation as long as possible in this population. Knowing the long-term efficacy and complications of stent implantations is important for deciding the procedure.
Interview
Global Leadership in Paediatric and Congenital Cardiac Care: “Using data to improve outcomes – an interview with Jennifer S. Li, MD, MHS”
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- Justin T. Tretter, Jeffrey P. Jacobs
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- 14 September 2020, pp. 1226-1230
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Dr. Jennifer Li is the focus of our second in a planned series of interviews in Cardiology in the Young entitled, “Global Leadership in Paediatric and Congenital Cardiac Care”. Dr. Li was born in Boston, Massachusetts, United States of America, and moved to Indianapolis, Indiana where she completed her secondary education. She then attended Stanford University, majoring in Chemistry and English and graduating with distinction in 1983. Dr. Li then attended Duke University School of Medicine, graduating in 1987. She then completed her internship at Children’s Hospital of Philadelphia in 1987–1989, returning to Duke University Medical Center to complete both her residency in general paediatrics in 1989–1990 followed by her fellowship in paediatric cardiology in 1990–1993. She would later complete her Master’s Degree in Health Sciences at Duke University in 2005.
Dr. Li has spent her entire career as a paediatric cardiologist at Duke University Medical Center, where she was appointed a Professor of Pediatrics and Professor of Medicine in 2008 and has held the position as Beverly C. Morgan Endowed Professor of Pediatrics since 2012. She has served as the Chief of Paediatric Cardiology at Duke University Medical Center since 2006. She also was the Director of Paediatric Research at Duke Clinical Research Institute from 2001-2015. Dr. Li has played an instrumental role in evaluating the safety and efficacy of drugs in children, as well as in analysing and linking large multicentric databases to evaluate the outcomes, quality, and cost of paediatric and congenital cardiac care. Dr. Li has received funding from the National Institute of Health of the United States of America, as well as from industry and foundation grants. This article presents our interview with Dr. Li, an interview that covers her experience collaborating with governmental organizations and industry in the pursuit of common interests to design clinical drug trials, link and analyse large, multicentric databases, and improve paediatric health care.
Review Article
The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States
- Tarek Alsaied, Kiona Y. Allen, Jeffrey B. Anderson, Julia S. Anixt, David W. Brown, Frank Cetta, Rachael Cordina, Yves D’udekem, Meghan Didier, Salil Ginde, Michael V. Di Maria, Michelle Eversole, David Goldberg, Bryan H. Goldstein, Erin Hoffmann, Adrienne H. Kovacs, Carole Lannon, Stacey Lihn, Adam M. Lubert, Bradley S. Marino, Emily Mullen, Diane Pickles, Rahul H. Rathod, Jack Rychik, James S. Tweddell, Sharyl Wooton, Gail Wright, Adel Younoszai, Tom Glenn, Alicia Wilmoth, Kurt Schumacher
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- 08 July 2020, pp. 1070-1075
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The Fontan Outcomes Network was created to improve outcomes for children and adults with single ventricle CHD living with Fontan circulation. The network mission is to optimise longevity and quality of life by improving physical health, neurodevelopmental outcomes, resilience, and emotional health for these individuals and their families. This manuscript describes the systematic design of this new learning health network, including the initial steps in development of a national, lifespan registry, and pilot testing of data collection forms at 10 congenital heart centres.
Original Article
Paediatric cardiomyopathies: echocardiographic diagnosis, clinical profile, and demographic characteristics: the experience of a tertiary referral centre for Latin American paediatric cardiology
- Víctor M. Huertas-Quiñones, Camilo F. Mestra, Valeria Peña-Trujillo, Sebastián Gallo-Bernal, Mariana Villaveces, Laura C. Alarcón-Forero
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- 17 February 2020, pp. 462-467
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Background:
Although multiple studies have been conducted in the adult population, there is a vast knowledge gap regarding the epidemiologic characteristics of cardiomyopathies in the paediatric population. This issue is even more crucial when the precarious situation of medical research in Latin America is considered. Given the potential impact that these disorders could have on Latin American health systems, a comprehensive epidemiologic study regarding the clinical profile and sociodemographic characteristics of these patients will influence the way we approach paediatric cardiomyopathies.
Methods:An observational retrospective study was conducted at a tertiary referral centre for Colombian and Latin American paediatric cardiology. We analysed all cases of primary cardiomyopathies in children younger than 18 years of age who presented at our institution between 2010 and 2016. Cases of cardiomyopathies were classified according to World Health Organization guidelines.
Results:From a total of 29,533 children who attended our institution during the study period, 89 new cases of primary cardiomyopathies were identified. The median age at diagnosis was 11 years (interquartile range 4–9). Dilated cardiomyopathy accounted for 57.3% (n = 51) of cases; hypertrophic cardiomyopathy, 12.3% (n = 11); restrictive cardiomyopathy, 8.9% (n = 8); non-compacted cardiomyopathy, 7.8% (n = 7); arrhythmogenic ventricular cardiomyopathy, 6.7% (n = 6); and unspecified cardiomyopathy, 6.7% (n = 6). Heart failure was observed in 53.93% of the patients. The overall mortality was 12.36% (n = 11), which included two of eight patients who underwent cardiac transplantation.
Staphylococcus aureus adheres avidly to decellularised cardiac homograft tissue in vitro in the fibrinogen-dependent manner
- Bartosz Ditkowski, Kirsten Leeten, Ramadan Jashari, Elizabeth Jones, Ruth Heying
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- 21 September 2020, pp. 1783-1787
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Objective:
Infective endocarditis remains a severe complication associated with a high morbidity and mortality in patients after heart valve replacement. Exploration of the pathogenesis is of high demand and we, therefore, present a competent model that allows studying bacterial adherence and the role of plasma fibrinogen in this process using a new in-house designed low-volume flow chamber. Three cardiac graft tissues used for pulmonary valve replacement have been tested under shear conditions to investigate the impact of surface composition on the adhesion events.
Methods:Tissue pieces of cryopreserved homograft (non-decellularised), decellularised homograft and bovine pericardium patch were investigated for fibrinogen binding. Adherence of Staphylococcus aureus to these graft tissues was studied quantitatively under flow conditions in our newly fabricated chamber based on a parallel plates’ modality. The method of counting colony-forming units was reliable and reproducible to assess the propensity of different graft materials for bacterial attachment under shear.
Results:Bacterial perfusions over all plasma-precoated tissues identified cryopreserved homograft with the lowest affinity for S. aureus compared to decellularised homograft presenting a significantly higher bacterial adhesion (p < 0.05), which was linked to a more avid fibrinogen binding (p < 0.01). Bovine pericardial patch, as a reference tissue in this study, was confirmed to be the most susceptible tissue graft for the bacterial adhesion, which was in line with our previous work.
Conclusion:The two studied homograft tissues showed different levels of bacterial attachment, which might be postulated by the involvement of fibrinogen in the adhesion mechanism(s) shown previously for bovine tissues.
Aetiological evaluation of chest pain in childhood and adolescence
- Emre Aygun, Sibel Tugce Aygun, Taciser Uysal, Fatih Aygun, Hasan Dursun, Ahmet Irdem
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- 05 May 2020, pp. 617-623
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Background:
Chest pain, as a common cause of hospital admissions in childhood, necessitates detailed investigations due to a wide range of differential diagnoses. In this study, we aimed to determine the distribution of diseases causing chest pain in children and investigate the clinical characteristics of children with chest pain.
Methods:This study included 782 patients aged between 3 and 18 years who presented to a paediatric cardiology outpatient clinic with chest pain between April 2017 and March 2018. Aetiological causes and demographic features of the patients were analysed.
Results:Most prevalent causes of chest pain were musculoskeletal system (33%) and psychogenic (28.4%) causes. Chest pain due to cardiac reasons was seen in eight patients (1%). Diseases of musculoskeletal and gastrointestinal systems and psychogenic disorders were significantly more common in male and female patients, respectively (p < 0.001 for all). In winter, patients’ age and the number of patients with ≥12 years were higher than those in other seasons (p < 0.001). Most of the parents (70.8%) and patients (90.2%) thought that chest pain in their children was caused by cardiac causes.
Conclusion:Most of the diagnoses for chest pain in childhood period are benign and include the musculoskeletal system and psychogenic diseases. Although chest pain due to cardiac diseases is rare, a comprehensive analysis of medical history, detailed physical examination and cardiac imaging with echocardiography is needed to reach more accurate diagnoses.
Review Article
Interventions on patent ductus arteriosus and its impact on congenital heart disease
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- Mariam Zaidi, Nilofer Sorathia, Hannah Abbasi, Arwa Khashkhusha, Amer Harky
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- 23 November 2020, pp. 1566-1571
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The ductus arteriosus (DA) connects the pulmonary artery to the aorta to bypass the pulmonary circulation in utero. It normally closes within 24–72 hours after birth due to increased pulmonary resistance from an increase in oxygen partial pressure with the baby’s first breath. Medical treatment can help close the DA in certain situations where closure is delayed. However, in duct-dependent cardiac defects, the presence of the DA is crucial for survival and as such medical and surgical techniques have evolved to prevent closure. This review aims to outline the two main management options for keeping a ductus arteriosus patent. This includes stenting the PDA and shunting via a modified Blalock–Taussig shunt. Whilst both techniques exist, multicentre trials have found equal mortality end points but significantly reduced morbidity with stenting than shunting. This is also reflected by shorter recovery times, reduced requirement for extracorporeal membrane oxygenation (ECMO), and improved quality of life, although stent longevity remains a limiting factor.
Original Article
Left ventricular dysfunction in Duchenne muscular dystrophy
- Katherine A. James, Jane Gralla, Leslie A. Ridall, ThuyQuynh N. Do, Angela S. Czaja, Peter M. Mourani, Emma Ciafaloni, Christopher Cunniff, Jennifer Donnelly, Joyce Oleszek, Shree Pandya, Elinora Price, Michele L. Yang, Scott R. Auerbach
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- 22 January 2020, pp. 171-176
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Background:
Duchenne muscular dystrophy is associated with progressive cardiorespiratory failure, including left ventricular dysfunction.
Methods and Results:Males with probable or definite diagnosis of Duchenne muscular dystrophy, diagnosed between 1 January, 1982 and 31 December, 2011, were identified from the Muscular Dystrophy Surveillance Tracking and Research Network database. Two non-mutually exclusive groups were created: patients with ≥2 echocardiograms and non-invasive positive pressure ventilation-compliant patients with ≥1 recorded ejection fraction. Quantitative left ventricular dysfunction was defined as an ejection fraction <55%. Qualitative dysfunction was defined as mild, moderate, or severe. Progression of quantitative left ventricular dysfunction was modelled as a continuous time-varying outcome. Change in qualitative left ventricle function was assessed by the percentage of patients within each category at each age. Forty-one percent (n = 403) had ≥2 ejection fractions containing 998 qualitative assessments with a mean age at first echo of 10.8 ± 4.6 years, with an average first ejection fraction of 63.1 ± 12.6%. Mean age at first echo with an ejection fraction <55 was 15.2 ± 3.9 years. Thirty-five percent (140/403) were non-invasive positive pressure ventilation-compliant and had ejection fraction information. The estimated rate of decline in ejection fraction from first ejection fraction was 1.6% per year and initiation of non-invasive positive pressure ventilation did not change this rate.
Conclusions:In our cohort, we observed that left ventricle function in patients with Duchenne muscular dystrophy declined over time, independent of non-invasive positive pressure ventilation use. Future studies are needed to examine the impact of respiratory support on cardiac function.
Overfeeding and obesity in young children with positive pressure ventilation via tracheostomy following cardiac surgery
- Takeshi Tsuda, David A. Hehir, Deepika Thacker, Allison Lamma, Donna Tearl, Samuel S. Gidding
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- 03 August 2020, pp. 1397-1404
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Objectives:
Infants with CHD requiring positive pressure ventilation via tracheostomy are especially vulnerable to malnutrition following cardiac surgery. Current post-operative feeding recommendations may overestimate the caloric needs.
Design:We retrospectively studied infants requiring tracheostomy after cardiac surgery. Anthropometric and nutritional data were collected, including caloric goals, weight-for-age z score, length-for-age z score, and weight-for-length z score. Changes in anthropometrics over time were compared to ascertain the impact of nutritional interventions. Data were shown as mean ± standard deviation.
Results:Nineteen infants with CHD required tracheostomy at 160 ± 109 days (7–364 days), 13 had reparative surgery, and 6 had palliative surgery for single ventricle. The indications for tracheostomy consisted of airway abnormality/obstruction (n = 13), chronic respiratory failure (n = 7), and/or vocal cord paresis (n = 2). Initial maintenance nutritional target was set at 100–130 cal/kg per day. Fourteen patients (73.7%) became obese (maximum weight-for-length z score: 2.59 ± 0.47) under tracheostomy and gastrostomy feeding, whereas five patients did not (weight-for-length z score: 0.2 ± 0.83). Eight obese patients (weight-for-length z score: 2.44 ± 0.85) showed effective reduction of obesity within 6 months (weight-for-length z score: 0.10 ± 0.20; p < 0.05 compared with pre-adjustment) after appropriate feeding adjustment (40–90 cal/kg per day). Overall mortality was high (31.6%) in this population.
Conclusion:Standard nutritional management resulted in overfeeding and obesity in young children with CHD requiring positive pressure ventilation via tracheostomy. Optimal nutritional management in this high-risk population requires close individualised management by multidisciplinary teams.
Tricuspid valvuloplasty for isolated tricuspid regurgitation in children
- Guanxi Wang, Kai Ma, Kunjing Pang, Sen Zhang, Lei Qi, Yang Yang, Zicong Feng, Fengqun Mao, Jianhui Yuan, Hao Zhang, Shoujun Li
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- 25 June 2020, pp. 1076-1080
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Background:
Isolated congenital tricuspid regurgitation other than Ebstein’s anomaly was rare especially for children. The objective of this study was to investigate the clinical characteristics and to assess the results of tricuspid valvuloplasty for children with isolated tricuspid regurgitation.
Methods:From January 2010 to June 2019, 10 consecutive patients with isolated tricuspid regurgitation who were unresponsive to drug therapy underwent tricuspid valvuloplasty in our hospital. Patients’ clinical data were analysed retrospectively.
Results:Mean age at operation was 48.5 ± 31.0 (range: 9–106) months and mean weight at operation was 16.1 ± 6.9 (range: 8.6–33.0) kg. All patients presented severe isolated tricuspid regurgitation. According to pathological lesions, the main causes accounted for chordae tendinea rupture (3/10), leaflet cleft (2/10), mal-connected chordal tendinea to leaflets (2/10), elongated chordae (1/10) and chordae absent (1/10), and severe anterior leaflet dysplasia (1/10). Individualised tricuspid valvuloplasty was adapted to all of them successfully. Post-operative echocardiography showed no tricuspid regurgitation in two patients and mild regurgitation in eight patients. The cardiothoracic ratios on their chest roentgenograms decreased from 0.59 ± 0.05 to 0.54 ± 0.05. At the latest follow-up (50.4 ± 47.2 months), echocardiography showed that mild to moderate tricuspid regurgitation in seven patients, moderate tricuspid regurgitation in three patients, and no patient with severe tricuspid regurgitation. All patients were in NYHA functional class I.
Conclusions:For patients with isolated tricuspid regurgitation who were not well responsive to drug therapy, individualised tricuspid valve repair can achieve an excellent result.
Extensive scar modification for the treatment of intra-atrial re-entrant tachycardia in patients after congenital heart surgery
- Astrid A. Hendriks, Zsuzsanna Kis, Ferdi Akca, Sing-Chien Yap, Sip A. Wijchers, Rohit E. Bhagwandien, Tamas Szili-Torok
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- Published online by Cambridge University Press:
- 23 July 2020, pp. 1231-1237
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Background:
Catheter ablation is an important therapeutic option for atrial tachycardias in patients with CHD. As a result of extensive scarring and surgical repair, multiple intra-atrial re-entrant tachycardia circuits develop and serve as a substrate for arrhythmias. The best ablation approach for patients with multiple intra-atrial re-entrant tachycardias has not been investigated. Here, we compared substrate-based ablation using extensive scar modification to conventional ablation.
Methods:The present study included patients with surgically corrected CHD that underwent intra-atrial re-entrant tachycardia ablation. Extensive scar modification was defined as substrate ablation based on a dense voltage map, aimed to eliminate all potentials in the scar region. The control group had activation mapping-based ablation. A clinical composite endpoint was assessed. Points were given for type, number, and treatment of intra-atrial re-entrant tachycardia recurrence.
Results:In 40 patients, 63 (extensive scar modification 13) procedures were performed. Acute procedural success was achieved in 78%. Procedural duration was similar in both groups. Forty-nine percent had a recurrence within 1 year. During a 5-year follow-up (2.5–7.5 years), 46% required repeat catheter ablation. Compared to baseline, clinical composite endpoint significantly decreased by 46% after 12 months (p = 0.001). Acute procedural success, procedural parameters, recurrence and repeat ablation were similar between extensive scar modification and activation mapping-based ablation.
Conclusion:Catheter ablation using extensive scar modification for intra-atrial re-entrant tachycardias occurring after surgically corrected CHD illustrated similar short- and long-term outcomes and procedural efficiency compared to catheter ablation using activation mapping-based ablation. The choice of ablation approach for multiple intra-atrial re-entrant tachycardia should remain at the discretion of the operator.
Mature and immature platelets during the first week after birth and incidence of patent ductus arteriosus
- Hannes Sallmon, Boris Metze, Petra Koehne, Bernd Opgen-Rhein, Katja Weiss, Joachim C. Will, Christina Victoria Franke, Georg Hansmann, Martin Koestenberger, Christoph Bührer, Felix Berger, Sven C. Weber, Malte Cremer
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- Published online by Cambridge University Press:
- 28 April 2020, pp. 769-773
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Background:
Thrombocytopenia is a risk factor for patent ductus arteriosus. Immature and mature platelets exhibit distinct haemostatic properties; however, whether platelet maturity plays a role in postnatal, ductus arteriosus closure is unknown.
Methods:In this observational study, counts of immature and mature platelets (=total platelet count − immature platelet count) were assessed on days 1, 3, and 7 of life in very low birth weight infants (<1500 g birth weight). We performed echocardiographic screening for haemodynamically significant patent ductus arteriosus on day 7.
Results:Counts of mature platelets did not differ on day 1 in infants with (n = 24) and without (n = 45) haemodynamically significant patent ductus arteriosus, while infants with significant patent ductus arteriosus exhibited lower counts of mature platelet on postnatal days 3 and 7. Relative counts of immature platelets (fraction, in %) were higher in infants with patent ductus arteriosus on day 7 but not on days 1 and 3. Receiver operating characteristic curve analysis unraveled associations between both lower mature platelet counts and higher immature platelet fraction (percentage) values on days 3 and 7, with haemodynamically significant ductus arteriosus. Logistic regression analysis revealed that mature platelet counts, but not immature platelet fraction values, were independent predictors of haemodynamically significant patent ductus arteriosus.
Conclusion:During the first week of postnatal life, lower counts of mature platelets and higher immature platelet fraction values are associated with haemodynamically significant patent ductus arteriosus. Lower counts of mature platelet were found to be independent predictors of haemodynamically significant patent ductus arteriosus.
P wave dispersion, Tpeak–Tend interval, and Tp-e/QT ratio in children with psoriasis
- Mecnun Çetin, İbrahim H. Yavuz, Mehmet Gümüştaş, Göknur Ö. Yavuz
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- 08 January 2020, pp. 318-322
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Background:
Psoriasis is a chronic inflammatory, multi-system disease that often begins in childhood and characterised by inflammatory skin, nails, scalp, and joint manifestations. The inflammation in psoriasis may promote some effect on the cardiac conduction system.
Objective:The aim of this study is to investigate myocardial repolarisation anomaly on the conducting system in the paediatric psoriasis using P wave dispersion, Tpeak–Tend interval, and Tp-e/QT ratio.
Methods:Forty-two patients diagnosed with psoriasis and 37 age- and sex-matched healthy children were enrolled in the study. Electrocardiographic parameters in psoriasis and control group were recorded from an electrocardiogram for each patient.
Results:The results indicated that the parameters including Pdis, QTc dis, Tp-e dis interval, and Tp-e max/QTmax ratios, which are known to be key indicators for the prediction of severe atrial or ventricular arrhythmia and sudden cardiac death and also important parameters used as the indicators for the non-invasive evaluation of the transmural heterogeneity were significantly longer in the study group compared to the control group (p < 0.05).
Conclusions:This study includes the evidence linking psoriasis with increased myocardial repolarisation heterogeneity. These findings suggest that this patient population may be at an increased risk for arrhythmias. Our findings may be a basis for further studies.
Incidence and outcomes of prosthetic valve endocarditis in adults with tetralogy of Fallot
- Alexander C. Egbe, Srikanth Kothapalli, William R. Miranda, Raja Jadav, Keerthana Banala, Rahul Vojjini, Faizan Faizee, Fouad Khalil, Maria Najam, Mounika Angirekula, Daniel C. Desimone, Heidi M. Connolly
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- 08 January 2020, pp. 19-23
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Background:
The risk of endocarditis varies with CHD complexity and the presence of prosthetic valves. The purpose of the study was therefore to describe incidence and outcomes of prosthetic valve endocarditis in adults with repair tetralogy of Fallot.
Methods:Retrospective review of adult tetralogy of Fallot patients who underwent prosthetic valve implantation, 1990–2017. We defined prosthetic valve endocarditis-related complications as prosthetic valve dysfunction, perivalvular extension of infection such abscess/aneurysm/fistula, heart block, pulmonary/systemic embolic events, recurrent endocarditis, and death due to sepsis.
Results:A total of 338 patients (age: 37 ± 15 years) received 352 prosthetic valves (pulmonary [n = 308, 88%], tricuspid [n = 13, 4%], mitral [n = 9, 3%], and aortic position [n = 22, 6%]). The annual incidence of prosthetic valve endocarditis was 0.4%. There were 12 prosthetic valve endocarditis-related complications in six patients, and these complications were prosthetic valve dysfunction (n = 4), systemic/pulmonary embolic events (n = 2), heart block (n = 1), aortic root abscess (n = 1), recurrent endocarditis (n = 2), and death due to sepsis (n = 1). Three (50%) patients required surgery at 2 days, 6 weeks, and 23 weeks from the time of prosthetic valve endocarditis diagnosis. Altogether three of the six (50%) patients died, and one of these deaths was due to sepsis.
Conclusions:The incidence, complication rate, and outcomes of prosthetic valve endocarditis in tetralogy of Fallot patients underscore some of the risks of having a prosthetic valve. It is important to educate the patients on the need for early presentation if they develop systemic symptoms, have a high index of suspicion for prosthetic valve endocarditis, and adopt a multi-disciplinary care approach in this high-risk population.
Review Article
Idiopathic dilatation of the right atrium: a not so benign entity
- Carin Walter, Joaquim Bartrons, Olga Gómez, José M. Caffarena, Juan M. Carretero
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- 05 June 2020, pp. 919-922
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Idiopathic dilatation of the right atrium is an isolated enlargement of the right atrium in the absence of other cardiac lesions. This rare anomaly has a clinical spectrum ranging from asymptomatic to heart failure or even sudden death. It can be associated with atrial arrhythmias and thrombus formation. Antiplatelet therapy is prescribed in most cases reported in the literature, and reduction plasty is indicated when there is rapid growth of the right atrium, compression of adjacent structures, or refractory arrhythmias. We report four cases of idiopathic dilatation of the right atrium diagnosed during prenatal screening. We describe the intrauterine course and management in postnatal life until early childhood.
Original Article
The progression of an acceptable pulmonary stenosis immediately after total correction of tetralogy of Fallot
- Yi-Seul Kim, Jinyoung Song, June Huh, I-Seok Kang, Ji-Hyuk Yang, Tae-Gook Jun
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- 04 May 2020, pp. 774-778
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Background:
A certain degree of pulmonary stenosis after total correction of tetralogy of Fallot has been considered acceptable. But the long-term outcomes are not well understood. We observed the natural course of immediate pulmonary stenosis and investigated related factors for progression.
Methods:Fifty-two patients with acceptable pulmonary stenosis immediately after operation were enrolled. Acceptable pulmonary stenosis was defined as peak pressure gradient between 15 and 45 mmHg by Doppler echocardiography. Latent class linear mixed model was used to differentiate patients with progressed pulmonary stenosis, and the factors related to progression were analysed.
Results:Pulmonary stenosis progressed in 14 patients (27%). Between the progression group and no progression group, there were no significant differences in operative age, sex, and the use of the transannular patch technique. However, immediate gradient was higher in the progression group (32.1 mmHg versus 25.7 mmHg, p = 0.009), and the cut-off value was 26.8 mmHg (sensitivity = 65.3%, specificity = 65.8%). Main stenosis at the sub-valve was observed more frequently in the progression group (85.7% versus 52.6%, p = 0.027). Despite no difference in the preoperative pulmonary valve z value, the last follow-up pulmonary valve z value was significantly lower in the progression group (−1.15 versus 0.35, p = 0.002).
Conclusions:Pulmonary stenosis immediately after tetralogy of Fallot total correction might progress in patients with immediate pulmonary stenosis higher than ≥26.8 mmHg and the main site was sub-valve area.
Post-operative discharge education for parent caregivers of children with congenital heart disease: a needs assessment
- Candace N. Mannarino, Kelly Michelson, Lindsay Jackson, Erin Paquette, Mary E. McBride
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- 22 September 2020, pp. 1788-1796
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Objective:
Children with congenital heart disease (CHD) have complex unique post-operative care needs. Limited data assess parents’ hospital discharge preparedness and education quality following cardiac surgery. The goals were to identify knowledge gaps in discharge preparedness after congenital heart surgery and to assess the acceptability of an educational mobile application to improve discharge preparedness.
Methods:Telephonic interviews with parents of children with two-ventricle physiology who underwent cardiac surgery 5–7 days post-discharge and in-person interviews with clinicians were conducted. We collected parent and clinician demographics, parent health literacy information and patient clinical data. We analysed interview transcripts using summative content analysis.
Results:We interviewed 26 parents and 6 clinicians. Twenty-two of the 26 (85%) parents felt ready for discharge; 4 of the 6 (67%) clinicians did not feel most parents were ready for discharge. Fifteen of the 26 parents (58%) reported receiving the majority of discharge teaching on the day of discharge. Eight parents did not feel like all of their questions were answered. Most parents (14/26, 54%) preferred visual educational learning aids and could accurately describe important aspects of care. Most parents (23/26, 88%) and all 6 clinicians felt a mobile application for post-operative care education would be helpful.
Conclusions:Most parents received education on the day of discharge and could describe the information they received prior to discharge, although there were some preparedness gaps identified after discharge. Clinicians and parents varied in their perceptions of the readiness for discharge. Most responses suggest that a mobile application for discharge education may be helpful for transition to home.
Faecal calprotectin concentrations in neonates with CHD: pilot study
- Graeme O’Connor, Katherine L. Brown, Andrew M. Taylor
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- 03 April 2020, pp. 624-628
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Neonates with CHD are at increased risk of developing necrotising enterocolitis due to mesenteric hypoperfusion. Necrotising enterocolitis results in repeated feed interruptions contributing to poor growth during the early post-operative phase. Poor weight gain and longer hospital stay are risk factors for death in neonates with CHD. Abdominal radiography is used as a diagnostic tool for necrotising enterocolitis; however, its utility is limited in the early stages of necrotising enterocolitis when pneumatosis intestinalis is absent. Calprotectin is a neutrophil activation biomarker, and elevated levels are evident in inflammatory diseases such as necrotising enterocolitis. The aim of this study was to determine whether there is a correlation between faecal calprotectin concentration and gut inflammation in neonates with CHD. This prospective single-centre study recruited newly diagnosed term patients with duct-dependent CHD between March 2018 and March 2019. Faecal calprotectin concentrations were measured in post-surgical patients using enzyme-linked immunosorbent assay methods. A total of 30 patients were included in the analysis. Calprotectin concentration for patients who developed necrotising enterocolitis was 3528 µg/g compared with 390 µg/g without, compared with 1339 µg/g in patients with suspected necrotising enterocolitis (p = 0.0001). Patients with suspected necrotising enterocolitis had a significantly longer length of hospital stay, on average 18 days longer compared to patients without necrotising enterocolitis (p = 0.03). Faecal calprotectin concentrations may reflect severity of gut inflammation in neonates with CHD. Suspected necrotising enterocolitis contributes to longer days nil by mouth and an increase in length of hospital stay.
Clinical and socio-economic predictors of work participation in adult CHD patients
- Lauren A. Sarno, Lindsay Cortright, Tiara Stanley, Dmitry Tumin, Jennifer S. Li, Charlie J. Sang, Jr
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- Published online by Cambridge University Press:
- 02 July 2020, pp. 1081-1085
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Background:
Adults with CHD have reduced work participation rates compared to adults without CHD. We aimed to quantify employment rate among adult CHD patients in a population-based registry and to describe factors and barriers associated with work participation.
Methods:We retrospectively identified adults with employment information in the North Carolina Congenital Heart Defects Surveillance Network. Employment was defined as any paid work in a given year. Logistic regression was used to examine patients’ employment status during each year.
Results:The registry included 1,208 adult CHD patients with a health care encounter between 2009 and 2013, of whom 1,078 had ≥1 year of data with known employment status. Overall, 401 patients (37%) were employed in their most recent registry year. On multivariable analysis, the odds of employment decreased with older age and were lower for Black as compared to White patients (odds ratio = 0.78; 95% confidence interval: 0.62, 0.98; p = 0.030), and single as compared to married patients (odds ratio = 0.50; 95% confidence interval: 0.39, 0.63; p < 0.001).
Conclusion:In a registry where employment status was routinely captured, only 37% of adult CHD patients aged 18–64 years were employed, with older patients, Black patients, and single patients being less likely to be employed. Further work is needed to consider how enhancing cardiology follow-up for adults with CHD can integrate support for employment.