Original Article
Combining Congenital Heart Surgical and Interventional Cardiology Outcome Data in a Single Database: The Development of a Patient-Centered Collaboration of the European Congenital Heart Surgeons Association (ECHSA) and the Association for European Paediatric and Congenital Cardiology (AEPC)
- Jeffrey P. Jacobs, Thomas Krasemann, Claudia Herbst, Zdzislaw Tobota, Bohdan Maruszewski, Jose Fragata, Tjark Ebels, Vladimiro L. Vida, Ilkka Mattila, Andrzej Kansy, Boulos Asfour, Jürgen Hörer, Attilio A. Lotto, M. Sertaç Çiçek, Petru Liuba, Sven Dittrich, Massimo Chessa, Regina Bökenkamp, Gurleen Sharland, Katarina Hanséus, Nico A. Blom, George E. Sarris
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- Published online by Cambridge University Press:
- 22 August 2023, pp. 1043-1059
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The European Congenital Heart Surgeons Association (ECHSA) Congenital Database (CD) is the second largest clinical pediatric and congenital cardiac surgical database in the world and the largest in Europe, where various smaller national or regional databases exist. Despite the dramatic increase in interventional cardiology procedures over recent years, only scattered national or regional databases of such procedures exist in Europe. Most importantly, no congenital cardiac database exists in the world that seamlessly combines both surgical and interventional cardiology data on an international level; therefore, the outcomes of surgical and interventional procedures performed on the same or similar patients cannot easily be tracked, assessed, and analyzed. In order to fill this important gap in our capability to gather and analyze information on our common patients, ECHSA and The Association for European Paediatric and Congenital Cardiology (AEPC) have embarked on a collaborative effort to expand the ECHSA-CD with a new module designed to capture data about interventional cardiology procedures. The purpose of this manuscript is to describe the concept, the structure, and the function of the new AEPC Interventional Cardiology Part of the ECHSA-CD, as well as the potentially valuable synergies provided by the shared interventional and surgical analyses of outcomes of patients. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow centers to have access to robust surgical and transcatheter outcome data from their own center, as well as robust national and international aggregate outcome data for benchmarking. Each contributing center or department will have access to their own data, as well as aggregate data from the AEPC Interventional Cardiology Part of the ECHSA-CD. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow cardiology centers to have access to aggregate cardiology data, just as surgical centers already have access to aggregate surgical data. Comparison of surgical and catheter interventional outcomes could potentially strengthen decision processes. A study of the wealth of information collected in the database could potentially also contribute toward improved early and late survival, as well as enhanced quality of life of patients with pediatric and/or congenital heart disease treated with surgery and interventional cardiac catheterization across Europe and the world.
Expert Consensus Statement: Anatomy, Imaging, and Nomenclature of Congenital Aortic Root Malformations
- Justin T. Tretter, Diane E. Spicer, Rodney C. G. Franklin, Marie J. Béland, Vera D. Aiello, Andrew C. Cook, Adrian Crucean, Rohit S. Loomba, Shi-Joon Yoo, James A. Quintessenza, Christo I. Tchervenkov, Jeffrey P. Jacobs, Hani K. Najm, Robert H. Anderson
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- Published online by Cambridge University Press:
- 08 June 2023, pp. 1060-1068
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Over the past 2 decades, several categorizations have been proposed for the abnormalities of the aortic root. These schemes have mostly been devoid of input from specialists of congenital cardiac disease. The aim of this review is to provide a classification, from the perspective of these specialists, based on an understanding of normal and abnormal morphogenesis and anatomy, with emphasis placed on the features of clinical and surgical relevance. We contend that the description of the congenitally malformed aortic root is simplified when approached in a fashion that recognizes the normal root to be made up of 3 leaflets, supported by their own sinuses, with the sinuses themselves separated by the interleaflet triangles. The malformed root, usually found in the setting of 3 sinuses, can also be found with 2 sinuses, and very rarely with 4 sinuses. This permits description of trisinuate, bisinuate, and quadrisinuate variants, respectively. This feature then provides the basis for classification of the anatomical and functional number of leaflets present. By offering standardized terms and definitions, we submit that our classification will be suitable for those working in all cardiac specialties, whether pediatric or adult. It is of equal value in the settings of acquired or congenital cardiac disease. Our recommendations will serve to amend and/or add to the existing International Paediatric and Congenital Cardiac Code, along with the Eleventh iteration of the International Classification of Diseases provided by the World Health Organization.
In memoriam: Professor Abraham Rudolph (1924– April 9, 2023) - a tribute
- Norman H. Silverman, David F. Teitel
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- 13 June 2023, pp. 1069-1070
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Dr Abraham Rudolph, one of the foremost leaders in Pediatric Cardiology, passed away on April 9, 2023, aged 99. His entire career was marked by imagination, creativity, and a devotion to the care of children with heart disease. He will be sorely missed by the many who knew him personally and the countless physicians whose understanding of their specialty was enhanced by his discoveries and teachings.
Interview
Global leadership in paediatric and congenital cardiac care: education and empowerment to improve outcomes in low- and middle-income countries – an interview with Krishna Kumar, MD, DM FAHA
- Mayme E. Marshall, Jeffrey P. Jacobs, Justin T. Tretter
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- 21 July 2023, pp. 1071-1078
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Dr Krishna Kumar is the focus of our sixth in a series of interviews in Cardiology in the Young entitled, “Global Leadership in Paediatric and Congenital Cardiac Care.” Dr Kumar was born in Raurkela, India. He attended medical school at Maulana Azad Medical College in New Delhi, graduating in 1984. Dr Kumar then went on to complete internal medicine, emergency medicine, and adult cardiology training at All India Institute of Medical Sciences in 1988, 1989, and 1990, respectively. He then pursued paediatric cardiology training at Harvard Medical School in Boston, MA, USA. Dr Kumar began his clinical position as a paediatric cardiologist at Amrita Institute of Medical Sciences in Kochi, Kerala, India.
During his impressive career, Dr Kumar has made significant contributions to educational advancement, research and innovation, public health advocacy, and clinical care. Dr Kumar is credited for distinguishing paediatric cardiology as a distinct subspecialty in India. He was a founding member of the Pediatric Cardiology Society of India and the original editor of the society’s academic journal. Recognising the deficit of paediatric cardiology-trained physicians in low- and middle-income countries, Dr Kumar helped establish formal structured training programmes for paediatric cardiology in India. More recently, he established the Children’s HeartLink Fellowships in paediatric cardiac sciences at Amrita Institute of Medical Sciences in Kochi and Institut Jantung Negara in Malaysia. Through educational programmes, Dr Kumar has taught countless caregivers and paediatricians, in India and neighbouring countries, the early identification and management of children with CHD. Dr Kumar has established a premier paediatric heart programme at Amrita Institute of Medical Sciences. As department Chief, he emphasises the importance of teamwork, advocacy, and continuous quality improvement. He has developed numerous low-cost strategies for the management of CHD. He has established large community-based studies on rheumatic heart disease and CHD in South India. Dr Kumar’s focus on advocacy and policy change in India has made a substantial impact on early identification and treatment of CHD in the subcontinent. He has made a global impact on the care of paediatric cardiology patients through his educational programmes, research and innovation, large-scale research registries, and advocacy for public health policy changes. He is an incredibly humble and generous leader, and his patients and community are the source of his unending motivation.
Original Article
Quality analysis of publicly available information about hypoplastic left heart syndrome
- Zachary Brennan, Omar M. Sharaf, John A. Treffalls, Natalia Roa-Vidal, Douglas J. Weinstein, Jonah S. Bassuk, Yuriy Stukov, Giles J. Peek, Mark S. Bleiweis, Jeffrey P. Jacobs
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- Published online by Cambridge University Press:
- 22 August 2023, pp. 1079-1085
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Purpose:
Publicly available health information is increasingly important for patients and their families. While the average US citizen reads at an 8th-grade level, electronic educational materials for patients and families are often advanced. We assessed the quality and readability of publicly available resources regarding hypoplastic left heart syndrome (HLHS).
Methods:We queried four search engines for “hypoplastic left heart syndrome”, “HLHS”, and “hypoplastic left ventricle”. The top 30 websites from searches on Google, Yahoo!, Bing, and Dogpile were combined into a single list. Duplicates, commercial websites, physician-oriented resources, disability websites, and broken links were removed. Websites were graded for accountability, content, interactivity, and structure using a two-reviewer system. Nonparametric analysis of variance was performed.
Results:Fifty-two websites were analysed. Inter-rater agreement was high (Kappa = 0.874). Website types included 35 hospital/healthcare organisation (67.3%), 12 open access (23.1%), 4 governmental agency (7.7%), and 1 professional medical society (1.9%). Median total score was 19 of 39 (interquartile range = 15.8–25.3): accountability 5.5 of 17 (interquartile range = 2.0–9.3), content 8 of 12 (interquartile range = 6.4–10.0), interactivity 2 of 6 (interquartile range = 2.0–3.0), and structure 3 of 4 (interquartile range = 2.8–4.0). Accountability was low with 32.7% (n = 17) of sites disclosing authorship and 26.9% (n = 14) citing sources. Forty-two percent (n = 22) of websites were available in Spanish. Total score varied by website type (p = 0.03), with open access sites scoring highest (median = 26.5; interquartile range = 20.5–28.6) and hospital/healthcare organisation websites scoring lowest (median = 17.5; interquartile range = 13.5–21.5). Score differences were driven by differences in accountability (p = 0.001) – content scores were similar between groups (p = 0.25). Overall readability was low, with median Flesch–Kincaid Grade Level of 11th grade (interquartile range = 10th–12th grade).
Conclusions:Our evaluation of popular websites about HLHS identifies multiple opportunities for improvement, including increasing accountability by disclosing authorship and citing sources, enhancing readability by providing material that is understandable to readers with the full spectrum of educational background, and providing information in languages besides English, all of which would enhance health equity.
Evaluation of tumour necrosis factor alpha-stimulated gene-6 and fibroblast growth factor-2 levels in patients diagnosed with multi-system inflammatory syndrome in children
- Onur Tasci, Kubra Dogan
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- 15 March 2023, pp. 1086-1091
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Investigations are still ongoing about the pathophysiology of multi-system inflammatory syndrome in children, which can progress with serious morbidity and mortality after COVID-19 infection. In this study, we aimed to investigate whether fibroblast growth factor-2 and tumour necrosis factor alpha-stimulated gene-6 levels play a role in the diagnosis of the disease and on cardiac involvement. Twenty-three patients (11 girls, 12 boys) and 26 healthy controls (10 girls, 16 boys) were included in the study. The mean age of the patient and control group was 8.45 ± 2.43 and 10.73 ± 4.27 years, respectively. There was no difference between the fibroblast growth factor-2 and tumour necrosis factor alpha-stimulated gene-6 levels of the patient and control groups. When the patients with myocardial involvement in the patient group were compared with the patients without myocardial involvement in terms of fibroblast growth factor-2 and tumour necrosis factor alpha-stimulated gene-6 levels, no difference was found between these groups. The correlation of fibroblast growth factor-2 and tumour necrosis factor alpha-stimulated gene-6 levels with other laboratory parameters was investigated in the patient group. Fibroblast growth factor-2 was moderately inversely correlated with white blood cell count (r = -0.541, p = 0.008), absolute neutrophil count (r = −0.502, p = 0.015) and C-reactive protein (r = −0.528, p = 0.010). Fibroblast growth factor-2 was strongly inversely correlated with erythrocyte sedimentation rate (r = −0.694, p =<0.001). Our data show that fibroblast growth factor-2 and tumour necrosis factor alpha stimulated gene-6 do not provide sufficient information about diagnosis and cardiac involvement in multi-system inflammatory syndrome in children.
Perfusion index in newborns with CHD without clinical signs of hypoperfusion and heart failure: comparison with healthy newborns
- Mustafa Argun, Süleyman Sunkak, Ferhan Elmalı, Şuayip Keskin, Nazmi Narin
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- 17 July 2023, pp. 1092-1096
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Introduction:
Peripheral perfusion index has been proposed as a possible method for detecting circulatory impairment. We aimed to determine the normal range of peripheral perfusion index in healthy newborns and compare it with that of newborns with CHD.
Methods:Right-hand saturation and right-hand peripheral perfusion index levels were recorded, and physical examination and echocardiography were performed in newborns who were 0–28 days old and whom were evaluated in our paediatric cardiology outpatient clinic. The saturation and peripheral perfusion index levels of newborns with normal heart anatomy and function were compared with those of newborns with CHD.
Results:Out of 358 newborns (238 mature and 75 premature) enrolled in the study, 39 had CHD (20 mild CHD, 13 moderate CHD, and 6 severe CHD), of which 29 had CHD with left-to-right shunting, 5 had obstructive CHD, and 5 had cyanotic CHD. No newborn had clinical signs of hypoperfusion or heart failure, such as prolonged capillary refill, weakened pulses, or coldness of extremities. Peripheral perfusion index level was median (interquartile range) 1.7 (0.6) in healthy newborns, 1.8 (0.7) in newborns with mild CHD, and 1.8 (0.4) in newborns with moderate and severe CHD, and there was no significant difference between the groups regarding peripheral perfusion index level.
Conclusion:Peripheral perfusion index remains unchanged in newborns with CHD without the clinical signs of hypoperfusion or heart failure. Larger studies with repeated peripheral perfusion index measurements can determine how valuable this method will be in the follow-up of newborns with CHD.
Common arterial trunk in functionally univentricular hearts: a case series
- Huzeifa Elhedai, Sanfui Yong, Milind Chaudhari, Phil Botha, Paul Miller, Oliver Stumper
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- 11 July 2022, pp. 1097-1101
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Introduction:
The association of a univentricular heart defect with common arterial trunk is extremely rare. There is a lack of population-based outcome studies reported in the literature.
Methods:The hospital records, echocardiographic and other imaging modality data, outpatients’ records, operation notes, and other electronic data were reviewed. Patients were reviewed, and the final outcomes of surgery were observed.
Results:Six cases (two males) with common arterial trunk presented over a 30-year period. Five had a complete unbalanced atrioventricular septal defect (83%) and one (17%) had tricuspid atresia associated with common arterial trunk. All had antenatal diagnosis. Two cases (33%) were excluded from initial surgical palliation due to Trisomy 21 in one and severe truncal valve regurgitation in one. Initial surgical palliation was performed in four cases (67%) at median age of 31 days (2–60) and consisted of disconnection and reconstruction of the pulmonary arteries and establishing controlled pulmonary blood flow. There were no early deaths. Conversion to cavopulmonary shunt was not possible in two due to severe airway problems in one and pulmonary arteries anatomy in one. They died at 11 and 16 months, respectively. Two patients (33%) underwent cavopulmonary shunt with 1 (17%) being alive at 18 months – 12 months after cavopulmonary shunt. The second patient proceeded to Fontan completion at 19 months but required catheter takedown 3 months later and died 3.5 years later.
Conclusions:Univentricular hearts with common arterial trunk carry extremely poor short- to medium-term outcomes. This should inform antenatal and postnatal counselling and decision-making.
Abnormal infant neurobehavior and later neurodevelopmental delays in children with critical CHD
- Kathleen Campbell, Lauren Malik, Courtney Jones, Zhining Ou, Angela Presson, Thomas A. Miller, Sarah Winter, Kristi Glotzbach
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- 14 July 2022, pp. 1102-1111
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Infants with critical CHD have abnormal neurobehavior assessed by the Neonatal ICU Network Neurobehavioral Scales. This retrospective cohort study hypothesized associations between abnormal infant neurobehavior in the first month of life and later neurodevelopmental outcomes at 1−2 years of age. Associations between abnormal infant attention (orienting to and tracking stimuli) on the Neonatal ICU Network Neurobehavioral Scales and later motor, cognitive, and language neurodevelopmental outcomes on the Bayley Scales of Infant Development-III at follow-up were examined with descriptive statistics and univariable and multivariable regression. Multiple imputation was used to account for missing outcome data. 189 infants with critical CHD were included, and 69% had abnormal neurobehavioral attention scores. 58 (31%) returned as toddlers for neurodevelopmental follow-up, of which 23% had motor delay. Abnormal infant attention had high sensitivity (92%, 95% CI 60−100%) but low specificity (36%, 95% CI 23−52%) for later motor delay. Higher infant attention scores were associated with higher later motor scores in univariable analysis (coefficient 3.49, 95% CI 0.52,6.46, p = 0.025), but not in multivariable analyses. Neither cognitive nor language scores were associated with infant attention scores. Lower birth weight and male sex were significantly associated with lower motor scores in multivariable analysis (p = 0.048, 0.007). Although impaired infant attention is interdependent with other clinical and demographic risk factors, it may be a sensitive clinical marker of risk for later motor delay. In children with critical CHD, impaired infant attention may be capturing early signs of abnormal visual-motor neurodevelopment.
Association of steroid administration with larger coronary artery abnormalities in patients with Kawasaki disease
- Takanori Suzuki, Satoru Kawai, Eiji Morihana, Shinji Kawabe, Naomi Iwata, Kazuyoshi Saito, Tetsushi Yoshikawa, Kazushi Yasuda
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- 14 July 2022, pp. 1112-1116
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We sought to elucidate the risk profiles of patients with Kawasaki disease who developed coronary artery abnormalities through a retrospective analysis with special reference to steroid treatment. Demographics of the patients were obtained from medical records, and characteristics of the coronary artery abnormalities were evaluated by echocardiography and coronary angiography, which included number, location, size, and length of coronary artery abnormalities (we evaluated by cardiac catheterisation with the American Heart Association classification with segments). We divided the patients into two groups based on steroid use and compared their characteristics and the complications of coronary artery abnormalities and cardiac events. A total of 29 patients were diagnosed with coronary artery abnormalities by echocardiography and coronary angiography during the study period (24 male; median age, 24 months [range: 2–84 months]). Eighteen patients were treated with aspirin and intravenous immunoglobulin (63%, non-steroid group), whereas 11 received aspirin and intravenous immunoglobulin plus steroids (37%, steroid group). No significant differences were found in the number and location of coronary artery abnormalities between the steroid and non-steroid groups. However, the size and number of segments for coronary artery abnormalities were significantly larger and shorter, respectively, in the steroid group (z-score: non-steroid group 6.3 versus steroid group 8.7; p < 0.01). The coronary artery abnormality segments under steroid use were also shorter (non-steroid group versus steroid group, two segments versus one segment; p = 0.02). Coronary artery abnormality size was larger in patients who used steroids than that of non-steroids. This study showed that steroid use significantly affected coronary artery abnormality size in patients with Kawasaki disease. However, cardiac complications from coronary artery abnormalities and cardiac events were comparable between the steroid and non-steroid groups. Further prospective, multicentre studies are needed to confirm these findings.
Cardiac involvement in multisystem inflammatory syndrome in children: single-centre experience
- Vildan Atasayan, Özge P. Akbay, Şengül Çağlayan, Betül Sözeri, Canan Hasbal Akkuş, Alican Vuran, Taliha Öner, Mehmet Karacan
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- 15 July 2022, pp. 1117-1123
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Background:
This study aimed to evaluate electrocardiographic and echocardiographic findings, Holter recordings of the multisystem inflammatory syndrome in children, and to identify prognostic factors for cardiac involvement.
Methods:We retrospectively reviewed demographic characteristics, medical data, laboratory findings, electrocardiogram and echocardiographic findings, 24-hour Holter recordings, need for an ICU, and extracorporeal membrane oxygenation in multisystem inflammatory syndrome in children. Acute left ventricular systolic dysfunction was defined as left ventricular ejection fraction (EF) ≤%55 on echocardiography.
Results:Sixty-seven children were included in the study. 24-hour Holters were recorded in 61.2% of the patients and 49.2% were normal. On echocardiographic examination, 14.9% of the patients had systolic dysfunction (EF ≤ 55%). While 32.8% of patients had mild mitral regurgitation, 3% had moderate mitral regurgitation, and 6% had mild aortic regurgitation. There was no statistically significant difference in EF values between the group with arrhythmia in Holter and the group with normal Holter results (p ≥ 0.05). B-type natriuretic peptide was positively correlated with C-reactive protein, ferritin, and fibrinogen. Significant effectivity of the B-type natriuretic peptide value was observed in the differentiation of those with EF ≤ and > 55%. Extracorporeal membrane oxygenation support was needed for three (4.5%) patients. One patient who died had systemic juvenile idiopathic arthritis.
Conclusions:Neutrophil/lymphocyte ratio, C-reactive protein, D-dimer, ferritin, troponin, and B-type natriuretic peptide were found to be significantly higher in patients with systolic dysfunction. Also, the cut-off value of 1700 pg/ml for B-type natriuretic peptide was significantly effective. These parameters may indicate the severity of the disease but should be supported by prospective studies.
Utilizing technology to expand home monitoring to high-risk infants with CHD
- Dana Hartman, Eric Ebenroth, Anne Farrell
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- 15 July 2022, pp. 1124-1128
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Infants born with single ventricle physiology that require an aorto-pulmonary shunt are at high risk for sudden cardiac death, particularly during the interstage period between the first-stage palliation and the second-stage palliation. Home monitoring programs have decreased interstage mortality in the hypoplastic left heart syndrome population prompting programs to expand the home monitoring program to other high-risk populations. At our mid-sized program, we implemented the Locus Health home monitoring platform first in the hypoplastic left heart syndrome population, then expanding to the single ventricle shunt population. Interstage mortality for the hypoplastic left heart syndrome population after initiation of the home monitoring program went from 18% prior to 2009 to 7% as of the end of 2020 (n = 99), with 2.8% mortality from 2013 to 2020 and 0% mortality since initiation of the Locus program in 2017. Caregiver surveys done prior to discharge and then 3 weeks later were used to document caregiver experience using the digital home monitoring program. Caregivers reported overall positive experience with the digital application, with 91.8% stating that they felt confident taking care of their baby at home. Transitioning the home monitoring program from a traditional binder to an iPad with the Locus Health application allowed us to expand the program, utilize the electronic medical record, bill for the service, and demonstrate positive experiences for caregivers. Overall engagement and adherence with the program by caregivers were 50.94 and 45.45%, with a total of 112 patient episodes. Reimbursement from private insurance providers was 22% of the billed amount for 2020.
A comparison of ECG-based home monitoring devices in adults with CHD
- Lindsay K.D. Pengel, Daniëlle Robbers-Visser, Maarten Groenink, Michiel M. Winter, Mark J. Schuuring, Berto J. Bouma, Jouke P. Bokma
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- Published online by Cambridge University Press:
- 18 July 2022, pp. 1129-1135
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Background:
Various electrocardiogram (ECG)-based devices are available for home monitoring, but the reliability in adults with CHD is unknown. Therefore, we determined the accuracy of different ECG-based devices compared to the standard 12-lead ECG in adult CHD.
Methods and results:This is a single-centre, prospective, cross-sectional study in 176 consecutive adults with CHD (54% male, age 40 ± 16.6 years, 24% severe CHD, 84% previous surgery, 3% atrial fibrillation (AF), 24% right bundle branch block). Diagnostic accuracy of the Withings Scanwatch (lead I), Eko DUO (precordial lead), and Kardia 6L (six leads) was determined in comparison to the standard 12-lead ECG on several tasks: 1) AF classification (percentage correct), 2) QRS-morphology classification (percentage correct), and 3) ECG intervals calculation (QTc time ≤ 40 ms difference). Both tested AF algorithms had high accuracy (Withings: 100%, Kardia 6L: 97%) in ECGs that were classified. However, the Withings algorithm classified fewer ECGs as inconclusive (5%) compared to 31% of Kardia (p < 0.001). Physician evaluation of Kardia correctly classified QRS morphology more frequently (90% accuracy) compared to Eko DUO (84% accuracy) (p = 0.03). QTc was underestimated on all ECG-based devices (p < 0.01). QTc duration accuracy was acceptable in only 51% of Withings versus 70% Eko and 74% Kardia (p < 0.001 for both comparisons).
Conclusions:Although all devices demonstrated high accuracy in AF detection, the Withings automatic algorithm had fewest uninterpretable results. Kardia 6L was most accurate in overall evaluation such as QRS morphology and QTc duration. These findings can inform both patients and caregivers for optimal choice of home monitoring.
Single ventricular strain measures correlate with peak oxygen consumption in children and adolescents with Fontan circulation
- Suma Goudar, Daniel Forsha, David A. White, Ashley Sherman, Girish Shirali
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- 22 July 2022, pp. 1136-1142
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Introduction:
Children with a single ventricle post-Fontan palliation are at increased risk of poor outcomes with peak oxygen consumption acting as a surrogate outcome marker. The purpose of this study is to evaluate the relationship between peak oxygen consumption and echocardiographic measures of ventricular function and deformation, including ventricular global longitudinal strain and dyssynchrony, in children and adolescents following Fontan palliation.
Methods:Patients (age 8–21 years) with single ventricle post-Fontan palliation were prospectively recruited and participated in an echocardiogram, including views optimised for two-dimensional speckle tracking, and a cardiopulmonary exercise test on a cycle ergometer to maximal volitional fatigue.
Results:Thirty-eight patients (mean age 13.7 ± 2.3 years) post-Fontan palliation had either a single left ventricular (n = 20), single right ventricular (n = 14), or biventricular (n = 4) morphology. Peak oxygen consumption (24.9 ± 5.6 ml/kg/minute) was correlated with global longitudinal strain (r = −0.435, p = 0.007), a strain discoordination time to peak index (r = −0.48, p = 0.003), and the presence of an electro-mechanical dyssynchrony strain pattern (p = 0.008). On multivariate regression modelling, these three variables were associated with peak oxygen consumption independently of age and sex. The single right ventricular group had evidence of possible diastolic dysfunction by E/e’ compared to the single left ventricular and biventricular groups (p = 0.001).
Conclusions:Strain analysis measures are correlated with peak oxygen consumption in this cohort of children, adolescents, and young adults following Fontan palliation, suggesting that ventricular mechanics may influence the efficiency of the Fontan circulation.
Lessons learned from linking two complementary databases: the Society of Thoracic Surgeons Congenital Heart Surgery Database and The Vermont Oxford Network Expanded Database
- Jeremy M. Archer, Connie S. Nixon, Livia Sura, Dan Neal, Jeffrey P. Jacobs
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- 27 July 2022, pp. 1143-1149
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The Society of Thoracic Surgeons Congenital Heart Surgery Database and the Vermont Oxford Network Expanded Database are both large, international, well-established quality and outcomes databases with high penetration in their respective fields of congenital heart surgery and neonatology. Previous studies have shown the value of combining large databases for research purposes. Our aim was to examine the feasibility and value of combining these databases on a local level.
We included patients from both databases, cared for at our centre and born from 2015–2020, who had cardiac surgery as neonates or during the birth hospitalisation. We examined the number of patients from each database and overlap between the two. We compared cardiac diagnoses, surgeries performed, pre-operative factors, mortality, and length of stay between databases.
Of the 255 patients meeting criteria, 209 (81.9%) had records in both databases. The most common diagnoses in both were hypoplastic left heart syndrome, coarctation, and transposition of the great arteries. Surgical data were incompletely recorded in Vermont Oxford. Gestational age, birth weight, multiple gestation, mortality, and length of stay did not differ significantly between the databases, while the percentage of patients with an extracardiac malformation or genetic syndrome recorded was higher in the Society for Thoracic Surgeons group.
Larger-scale matching and comparison studies using these databases are feasible and desirable; for some variables, a record with data from both databases may be more complete. Specific attention should be given to inclusion criteria, reconciling different schema of diagnoses, and formulating questions relying on each database’s relative strengths.
Incidence and recovery of post-surgical heart block in children following cardiac surgery
- Rohit Madani, Elizabeth Aronoff, Jessica Posey, Mohua Basu, Tawanda Zinyandu, Paul Chai, Robert Whitehill, Kevin O. Maher, Asaad G. Beshish
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- 29 July 2022, pp. 1150-1156
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Background:
A subset of patients who develop post-surgical heart block have recovery of atrioventricular node function. Factors predicting recovery are not understood. We investigated our centre’s incidence of post-surgical heart block and examine factors associated with recovery of atrioventricular node function.
Methods:We conducted a single-centre retrospective study of patients 0 – 21 years who underwent cardiac surgery between January 2010 and December 2019 and experienced post-operative heart block. Data including patient and clinical characteristics and operative variables were collected and analysed.
Results:Of 6333 surgical hospitalisations, 128 (2%) patients developed post-operative heart block. Of the 128 patients, 90 (70%) had return of atrioventricular node function, and 38 (30%) had pacemaker placement. Of the 38 patients who underwent pacemaker placement, 6 (15.8%) had recovery of atrioventricular node function noted on long-term follow-up. Median time from onset of heart block to late atrioventricular node recovery was 13 days (Interquartile range: 5 – 117). Patients with single-ventricle physiology (p = 0.04), greater weight (p = 0.03), and shorter cardiopulmonary bypass time (p = 0.015) were more likely to have recovery. The use of post-operative steroids was similar between all groups (p = 0.445). Infectious or wound complications were similar between pacemaker groups (p = 1).
Conclusions:Two per cent of patients who underwent congenital cardiac surgery developed post-operative heart block, and 0.6% underwent pacemaker placement. Early recovery of atrioventricular node was associated with greater weight at the time of surgery, single-ventricle physiology, and shorter cardiopulmonary bypass time. Late recovery of atrioventricular node conduction following pacemaker placement occurred in 15.8% of patients.
Evaluation of subclinical atherosclerosis and cardiac functions in children of mothers with gestational diabetes and maternal obesity
- Melda Ekici Avci, Öykü Tosun
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- 29 July 2022, pp. 1157-1164
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Background and aims:
We aimed to evaluate the effects of maternal obesity or gestational diabetes on body composition, lipid, and glucose metabolism, arterial morphology, and functions in children, and to investigate these effects in terms of cardiometabolic diseases.
Methods:The study group was composed of 48 children who had a history of gestational diabetes or maternal obesity, and the control group was composed of 33 children. Echocardiographic assessments were performed. Socio-economic status and education level of mothers were obtained.
Results:In the study group, carotid intima-media thickness, epicardial adipose tissue thickness, and arterial stiffness values were found to be significantly higher compared to the control group (p < 0.001, p < 0.001, p = 0.003, respectively), while arterial distensibility and arterial strain values were found to be significantly lower (p = 0.003, p = 0.008, respectively). Among the children who had similar body mass index in both groups, children in the study group had higher carotid intima-media thickness and epicardial adipose tissue thickness values. Arterial stiffness values were significantly reduced (p = 0.028) and arterial distensibility and strain values were significantly increased (p = 0.039, p = 0.033, respectively) in the children whose mothers had gestational diabetes and high socio-economic status. Left ventricular mass and left ventricular end-diastolic internal thickness were found to be significantly increased in the children who had obese and unemployed mothers (p = 0.04, p = 0.03, respectively).
Conclusion:Low socio-economic status was found to be associated with increased maternal obesity and gestational diabetes. Poor socio-economic status, poor glycaemic control and being overweight during pregnancy indicate negative cardiometabolic outcomes for children in the long term.
Rescue high-frequency oscillatory ventilation combined with intermittent mandatory ventilation for infants with acute respiratory distress syndrome after congenital heart surgery
- Yi-Rong Zheng, Shi-Hao Lin, Yu-Kun Chen, Hua Cao, Qiang Chen
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- Published online by Cambridge University Press:
- 01 August 2022, pp. 1165-1171
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Background:
This study aimed to evaluate the efficacy and safety of high-frequency oscillation ventilation combined with intermittent mandatory ventilation in infants with acute respiratory distress syndrome after congenital heart surgery.
Methods:We retrospectively analysed the clinical data of 32 infants who were ventilated due to acute respiratory distress syndrome after congenital heart surgery between January, 2020 and January, 2022. We adopted high-frequency oscillation ventilation combined with intermittent mandatory ventilation as the rescue ventilation mode for infants who were failing conventional mechanical ventilation.
Results:After rescue high-frequency oscillation ventilation combined with intermittent mandatory ventilation, the dynamic compliance (Cdyn), PaO2 and PaO2/FiO2 ratio of the infants improved compared with conventional mechanical ventilation (p < 0.05). Moreover, high-frequency oscillation ventilation combined with intermittent mandatory ventilation resulted in a significant decrease in arterial-alveolar oxygen difference (AaDO2), FiO2, and oxygenation index (p < 0.05). No significant effect on haemodynamic parameters was observed. Moreover, no serious complications occurred in the two groups.
Conclusion:Rescue high-frequency oscillation ventilation combined with intermittent mandatory ventilation significantly improved oxygenation in infants who failed conventional mechanical ventilation for acute respiratory distress syndrome after congenital heart surgery. Thus, this strategy is considered safe and feasible. However, further studies must be conducted to confirm the efficacy and safety of high-frequency oscillation ventilation combined with intermittent mandatory ventilation as a rescue perioperative respiratory support strategy for CHD.
Impact of interdisciplinary counselling for parental decision-making in cases of pregnancies with prenatally diagnosed CHD
- Katja Schneider, Dafni Bousi, Rüdiger Stressig
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- Published online by Cambridge University Press:
- 02 August 2022, pp. 1172-1176
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Introduction:
Parental counselling after antenatal diagnosis of a congenital heart defect requires a high degree of professional and emotional competence and ultimately a sense of responsibility on the part of the consulting physicians. However, little is known about parents` perceptions and evaluation of these consultations.
Material/Methods:Survey on 425 women who received interdisciplinary counselling after antenatal diagnosis of a complex fetal congenital heart defect in a specialized prenatal outpatient clinic with an affiliated paediatric heart centre. Two questionnaires were used to retrospectively evaluate the parental perception and impact on counselling, particularly on decision-making. Questionnaires differed between women conceiving the child and women who terminated the pregnancy.
Results:400 women continued, 25 women terminated the pregnancy after diagnosis and counselling. Good quality of life was reported for 68% of the children, 15% died postnatally in the further course. 95% rated the counselling as good or very good regardless of the child´s outcome. 73% described the counselling as highly important for their subsequent decision. The possibility of termination of pregnancy was discussed with 37% of the respondents, 22% of them found it outrageous or very distressing. Of all respondents, one woman would have made a different decision regarding continuation of the pregnancy in retrospect.
Conclusion:Retrospectively, parents rate prenatal counselling extremely positively, irrespective of the severity of the child´s heart defect. It can be assumed that the consistent joint interdisciplinary consultation and the high reputation of the cooperating heart centre have had an influence on the below-average rate of termination of pregnancy.
Segmental and global longitudinal strain differences between Kawasaki disease and multi-system inflammatory syndrome in children
- Enrico Piccinelli, Carles Bautista-Rodriguez, Jethro Herberg, Heechan Kang, Sylvia Krupickova, Ivan B Altamar, Sara Moscatelli, Jolanda Sabatino, Manjit Josen, Josefa Paredes, Elisabeth Whittaker, Yogen Singh, Alain Fraisse, Giovanni Di Salvo
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- Published online by Cambridge University Press:
- 03 August 2022, pp. 1177-1183
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Background:
Multi-system inflammatory syndrome in children and Kawasaki disease have overlapping clinical features but comparative echocardiographic studies are lacking.
Methods:We reviewed echocardiography findings of all multi-system inflammatory syndrome cases between 1st April and 31st July, 2020 and typical Kawasaki disease patients with coronary arteries abnormalities consecutively followed between 1st October, 2016 and June 30th, 2019.
Results:We included 40 multi-system inflammatory syndrome children (25 males, 62.5%) and 45 Kawasaki disease patients (31 males, 68.9%) at a mean age of 6.4 years old and 8 years old, respectively. Four out of 40 multi-system inflammatory syndrome children had coronary arteries abnormalities. Left ventricle ejection fraction was normal in both groups. Global longitudinal strain was normal although Kawasaki disease group had significantly lower values (–20.0 versus –21.7%; p = 0.02). Basal segments were the most affected in Kawasaki disease patients with significant differences in the basal anterior, anterolateral, and anteroseptal strain: –18.2 versus –23.0% (p = 0.002), –16.7 versus –22.0% (p < 0.001), –16.7 versus –19.5% (p = 0.034), respectively. The basal anterolateral and anteroseptal segments in Kawasaki disease patients were the only ones with an absolute reduction of longitudinal strain (–16.7% both) consistent with the greater left main coronary involvement in this cohort.
Conclusions:Our findings are consistent with the transient cardiac involvement in multi-system inflammatory syndrome, as opposed to the subtle and chronic myocardial involvement in Kawasaki disease children with coronary arteries abnormalities. We speculate that the mechanism of cardiac impairment in the few multi-system inflammatory syndrome children with reduced global longitudinal strain is not related to coronary arteries abnormalities.