PROGRESS IN PEDIATRIC CARDIOLOGY最新文献

{"title":"Improving COVID-19 disease severity surveillance measures: The case for implementing accessible screening to protect high-risk populations","authors":"Daniel F. Maywalt ,&nbsp;Secil Ertorer ,&nbsp;Steven E. Lipshultz","doi":"10.1016/j.ppedcard.2024.101734","DOIUrl":"https://doi.org/10.1016/j.ppedcard.2024.101734","url":null,"abstract":"<div><h3>Background</h3><p>The COVID-19 pandemic disproportionately affected minority groups and people of low socioeconomic status, necessitating early identification of high-risk populations.</p></div><div><h3>Objectives</h3><p>We sought to investigate the varying effects of COVID-19 on low-income and high-diversity areas of Buffalo, NY. Our goal was to demonstrate the feasibility of real-time surveillance in identifying COVID-19-infected high-risk populations during an active pandemic, to indicate how this approach may be used to reduce morbidity among all high-risk populations, including children with heart disease.</p></div><div><h3>Methods</h3><p>We assessed rates of poverty and diversity for the 17 ZIP Codes in Buffalo, NY, and characterized each as high- or low-ethnic diversity and high- or low-income. Independent sample <em>t</em>-tests were conducted to compare COVID-19 positive tests and hospitalizations for high- and low-diversity and high- and low-income categories.</p></div><div><h3>Results</h3><p>The percentage of positive COVID-19 tests did not differ significantly between the high- and low-diversity ZIP Codes (4.1 % and 2.8 % respectively; difference, 466 positive tests; <em>P</em> = 0.22) or between the high- and low-income ZIP Codes (4.3 % and 2.8 % respectively; difference, 539 tests; <em>P</em> = 0.14). Among the 3161 patients hospitalized for COVID-19 between May 2nd and November 19th, 2020, the percentage differed significantly between the high- and low-diversity ZIP Codes (1.4 % and 0.9 % respectively; difference, 154 patients; <em>P</em> = 0.04) and between the high- and low-income ZIP Codes (1.5 % and 0.9 % respectively, difference, 1633 patients; <em>P</em> = 0.01).</p></div><div><h3>Conclusion</h3><p>Despite similar rates of positive COVID-19 tests, residents of low-income neighborhoods were at higher risk (67 % higher) for COVID-19 hospitalizations than were residents of high-income neighborhoods. Likewise, residents of high diversity neighborhoods were at a 56 % greater risk of COVID-19 hospitalization when compared with residents of low diversity neighborhoods. This study demonstrates that real-time data collection and analyses are available for identifying high-risk populations for COVID-19 hospitalizations. These findings could aid in optimizing early and intensive COVID-19 testing for high-risk groups, including pediatric cardiology patients, during both the current pandemic and future infectious disease outbreaks with the aim of reducing morbidity.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"74 ","pages":"Article 101734"},"PeriodicalIF":0.9,"publicationDate":"2024-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141289765","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Exercise capacity after COVID-related multisystem inflammatory syndrome in children-A cross-sectional study","authors":"Gulbadin Mufti ,&nbsp;Santhosh Shivabasappa ,&nbsp;Prabavathy Gopalakrishnan ,&nbsp;Jaikumar G. Ramamoorthy ,&nbsp;Sandhiya Selvarajan ,&nbsp;Raja J. Selvaraj ,&nbsp;Santhosh Satheesh ,&nbsp;Sridhar Balaguru ,&nbsp;Bhagwati Prasad Pant ,&nbsp;Avinash Anantharaj","doi":"10.1016/j.ppedcard.2024.101733","DOIUrl":"10.1016/j.ppedcard.2024.101733","url":null,"abstract":"<div><h3>Background</h3><p>After recovery from COVID-19 associated multisystem inflammatory syndrome in children (MISC), exercise restrictions were advised for 6 months. Studies done to assess exercise capacity at 3–6 months post recovery from MISC, prior to withdrawal of exercise restrictions, yielded varying information. Whether their exercise capacity was on par with their peers at/beyond 1-year post-recovery needed to be assessed.</p></div><div><h3>Objectives</h3><p>Primary: To compare the exercise capacity between children with a history of MISC and unaffected children</p><p>Secondary: To compare the exercise capacity between a) children with a history of MISC and children with a history of COVID but not MISC b) children with a history of COVID but not MISC and unaffected children.</p></div><div><h3>Methods</h3><p>Children (aged ≥8 years) who had recovered from MISC &gt;1-year ago (<em>n</em> = 21) were compared with unaffected children (<em>n</em> = 42) and children who had COVID but not MISC (n = 21) respectively using cardiopulmonary exercise testing (CPET). Comparisons were made between 1. Post-MISC vs unaffected 2. Post-MISC vs post-COVID 3. Post-COVID vs unaffected.</p></div><div><h3>Results</h3><p>Compared with unaffected children, post-MISC and post-COVID groups had similar peak oxygen uptake (VO₂ max) but significantly lesser exercise duration. Children who were very sick during their hospitalization for MISC had an exercise capacity comparable to others in the post-MISC group. Overweight/obese children in post-MISC group had a significantly lesser exercise capacity. But, the numbers of overweight/obese children and very sick children were not adequate to run a separate sub-group analysis.</p></div><div><h3>Conclusions</h3><p>At/beyond 1 year post-recovery from MISC, exercise capacity was comparable to healthy children and children who had COVID but not MISC, but exercise duration was significantly less. Overweight/obese children need to be followed up longer with emphasis on regular exercises. Children who were very sick during MISC hospitalization had recovered their exercise capacity.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"74 ","pages":"Article 101733"},"PeriodicalIF":0.9,"publicationDate":"2024-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141145445","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Use of a mechanical crimper for mounting covered stents in aortic coarctation","authors":"Catalina Vargas-Acevedo ,&nbsp;Ernesto Mejia ,&nbsp;Yoshiyuki Kagiyama ,&nbsp;Natalie Soszyn ,&nbsp;Jenny E. Zablah ,&nbsp;Gareth J. Morgan","doi":"10.1016/j.ppedcard.2024.101730","DOIUrl":"10.1016/j.ppedcard.2024.101730","url":null,"abstract":"<div><h3>Background</h3><p>Despite the continued evolution in use of covered stents in congenital cardiology, no standard technique is available for crimping stents onto balloons.</p></div><div><h3>Objectives</h3><p>We aimed to describe the use of a mechanical crimper to mount covered stents and its effects on stent integrity, uniformity, and sheath size for intervention.</p></div><div><h3>Methods</h3><p>Single-center retrospective review of patients with coarctation of the aorta (CoA) and variants of arch obstruction undergoing stent angioplasty with covered stents between January 2019 and December 2022. Sheath size used for intervention was recorded and compared to the manufacturers' IFU. A bench testing model was then performed to confirm the decrease in sheath size by mechanically crimping six covered stents. Percent reduction in size after crimping, stent uniformity, and PTFE integrity after balloon inflation were recorded.</p></div><div><h3>Results</h3><p>Twenty-five events were identified for review. Median age at procedure was 18 years, and the most common diagnosis was isolated CoA (80 %). 76 % (n = 19) of stents were mechanically crimped, and 24 % (n = 6) were manually crimped. The median sheath size for mechanically crimped stents was −2 Fr compared with a median of 0 Fr for manually crimped stents (<em>p</em> = 0.007). Bench testing revealed a median 12.8 % reduction in stent diameter after mechanical crimping. All mechanically crimped stents were successfully introduced through sheaths 2 Fr smaller than the IFU.</p></div><div><h3>Conclusion</h3><p>The use of mechanical crimpers for mounting covered endovascular stents allows the delivery of these devices through smaller profile sheaths, facilitating intervention in smaller patients and reducing the risk of vascular access related injuries without affecting stent performance.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"74 ","pages":"Article 101730"},"PeriodicalIF":0.9,"publicationDate":"2024-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141145057","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pediatric cardiomyopathy illustrates the importance of reinterpreting the significance of genetic variants","authors":"Teresa M. Lee ,&nbsp;Erin Miller ,&nbsp;Arthi Sridhar ,&nbsp;Xiao Fan ,&nbsp;Phillip J. Dexheimer ,&nbsp;Neha Bansal ,&nbsp;Justin Godown ,&nbsp;Daphne T. Hsu ,&nbsp;Paul Kantor ,&nbsp;Sonya Kirmani ,&nbsp;Ashwin K. Lal ,&nbsp;Joseph W. Rossano ,&nbsp;Jeffrey A. Towbin ,&nbsp;Steven A. Webber ,&nbsp;Ling Shi ,&nbsp;Taye H. Hamza ,&nbsp;Bruce J. Aronow ,&nbsp;Surbhi Bhatnaghar ,&nbsp;Lisa J. Martin ,&nbsp;Jeffrey Schubert ,&nbsp;Steven E. Lipshultz","doi":"10.1016/j.ppedcard.2024.101732","DOIUrl":"10.1016/j.ppedcard.2024.101732","url":null,"abstract":"<div><h3>Background</h3><p>Clinical genetic testing is increasingly being utilized to establish a molecular diagnosis to help manage children with cardiomyopathy and to assess the risk of cardiomyopathy among family members. However, as evidence and guidelines evolve, variant classification can change with the potential to impact counseling and family screening.</p></div><div><h3>Objectives</h3><p>The main purpose of this study was to investigate whether variants in cardiomyopathy genes previously interpreted by clinical genetic testing laboratories would be reclassified under current guidelines for the interpretation of sequence variants.</p></div><div><h3>Methods</h3><p>In 211 children enrolled in the Pediatric Cardiomyopathy Registry, we compared the results of previous clinical genetic testing with the results of research testing in 37 cardiomyopathy genes.</p></div><div><h3>Results</h3><p>The mean time difference between initial testing and reinterpretation was 7 years. Using the 2015 American College of Medical Genetics and Genomics guidelines for the interpretation of sequence variants, we found that 18 % of the tested population had a change in variant classification. Ninety-two percent of the initial classifications were performed before the publication of the guidelines, with 82 % of reclassifications resulting in a variant downgrade. Most of these were changes from the pathogenic or likely pathogenic category to a variant of uncertain significance. Reclassification frequency was similar across types of cardiomyopathy.</p></div><div><h3>Conclusion</h3><p>Our results highlight that a portion of variants get downgraded, and periodic reinterpretation of genetic testing results is necessary for all types of cardiomyopathy—particularly for variant interpretations prior to 2015. Importantly, variant reclassification has potential impact on the clinical management of at-risk patients.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"74 ","pages":"Article 101732"},"PeriodicalIF":0.9,"publicationDate":"2024-05-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141143639","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Long-term impact of coronary artery transfer techniques during the arterial switch operation on neo-aortic root dilation","authors":"Rafael Ayala ,&nbsp;Maria Dudakova ,&nbsp;Ulrike M.M. Bauer ,&nbsp;Brigitte Stiller ,&nbsp;Rouven Kubicki ,&nbsp;Fabian A. Kari ,&nbsp;National Register for Congenital Heart Defect Investigators","doi":"10.1016/j.ppedcard.2024.101731","DOIUrl":"10.1016/j.ppedcard.2024.101731","url":null,"abstract":"<div><h3>Background</h3><p>Aortic dilation (AD) remains one of the most common complications needing re-operation after ASO (arterial switch operation) in simple transposition of the great arteries (TGA).</p></div><div><h3>Objectives</h3><p>We sought to clarify which coronary artery transfer techniques help to minimize postoperative neo-aortic root dilation after ASO for TGA.</p></div><div><h3>Methods</h3><p>117 patients were identified in the German National Register for Congenital Heart Defects presenting transposition of the great arteries with intact ventricular septum (TGA-IVS) with minimum 5-year follow-up who underwent ASO between 1986 and 2015 at 16 centers. Our study population was divided into two groups depending on the sinus tissue gain during coronary re-implantation. Group one (<em>n</em> = 72) included the tissue-gaining techniques slit, trap-door, J-/V-/U-incision, and pericardial augmentation. Group two (<em>n</em> = 45) included ‘punch’ as the no-tissue gaining technique. Transthoracic echocardiography data were analyzed retrospectively for development of neoaortic dilation defined by <em>Z</em>-score ≥ 2.0. As secondary endpoint was analyzed, the need for reintervention due to aortic pathology.</p></div><div><h3>Results</h3><p>Median follow-up was 11 (5–29) years; cumulative follow-up was 853 patient-years for group one and 19 (5–34) years; cumulative follow-up was 853 patient-years for group two, comprising 1706 patient-years, <em>p</em> &lt; 0.0001. We observed statistically significant higher <em>Z</em>-scores at the aortic valve annulus level in group one (<em>p</em> = 0.03). There was no statistically significant difference between both groups regarding re-operation.</p></div><div><h3>Conclusion</h3><p>Neo-aortic roots of patients who underwent sinus gain tissue coronary artery transfer techniques show higher <em>Z</em>-scores than the punch technique in long-term postoperative follow-up. Lifelong surveillance of patients after ASO seems necessary.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"74 ","pages":"Article 101731"},"PeriodicalIF":0.9,"publicationDate":"2024-05-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141048719","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Does black widow spider bite the heart?","authors":"Behzad Alizadeh ,&nbsp;Pegah Bahrami Taghanaki ,&nbsp;Shirin Sadat Ghiasi ,&nbsp;Elham Nikrouh ,&nbsp;Ghazaleh Pourali ,&nbsp;Anahita Alizadeh Ghamsari ,&nbsp;Feisal Rahimpour","doi":"10.1016/j.ppedcard.2024.101728","DOIUrl":"https://doi.org/10.1016/j.ppedcard.2024.101728","url":null,"abstract":"<div><h3>Background</h3><p>Black widow spider (BWS) bites account for 0.5 % of global poisonings and 21 % of poisonings in North-East Iran. In addition to local reactions, systemic cardiovascular manifestations are likely to occur.</p></div><div><h3>Objective</h3><p>This study aims to present cases of patients who experienced cardiac symptoms after being envenomed by BWS.</p></div><div><h3>Methods</h3><p>A descriptive retrospective cross-sectional evaluation was conducted on all patients who were bitten by a BWS in 2020 and presented with cardiac manifestations. They were referred to our tertiary pediatric cardiology center in Mashhad, Iran for evaluation. All accessible variables including history, physical examination, laboratory tests prioritizing cardiology, as well as electrocardiography and echocardiography findings, were evaluated.</p></div><div><h3>Result</h3><p>Six patients, ranging in age from 5.5 to 13 years old, were bitten by BWS. None of the patients had a past or drug history and were admitted due to pain complaints at various bite sites that radiated in different directions. Four patients showed signs of tachycardia, and two showed hypertension, but no signs of infection were observed. Half of the patients experienced systemic complications. Upon arrival at the hospital, four patients showed elevated CKMB levels, which decreased during their hospitalization. Troponin I and N-Terminal Pro B-type Natriuretic Peptide levels were high in all patients. One patient with unstable hemodynamics showed pro-BNP over 35,000. T wave inversion was observed in six patients, ST segment depression in three patients, and ST-segment elevation in one patient, with overlap between them.</p></div><div><h3>Conclusion</h3><p>Taking notice of cardiovascular presentations after a BWS bite is considerable.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"74 ","pages":"Article 101728"},"PeriodicalIF":0.9,"publicationDate":"2024-04-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141084309","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cardiac effects of levalbuterol vs. albuterol in pediatric asthma attack patients: A systematic review and meta-analysis","authors":"Baraa Shebli ,&nbsp;Moamen Mostafa Asla ,&nbsp;Mike Ghabally ,&nbsp;Muhammad Besher Shabouk ,&nbsp;Rand Batal ,&nbsp;Mahmoud Malhis","doi":"10.1016/j.ppedcard.2024.101725","DOIUrl":"10.1016/j.ppedcard.2024.101725","url":null,"abstract":"<div><h3>Background</h3><p>Beta-2 agonists are the standard of care for asthmatic patients. Racemic albuterol and levalbuterol are two of the most commonly used bronchodilators in this category. Although their efficacy has been tested excessively, their effects on heart rate remain debatable in the medical literature.</p></div><div><h3>Aim of review</h3><p>This review aims to summarize all available data in the literature concerning the effects of Racemic Albuterol versus Levalbuterol on heart rate in asthmatic children.</p></div><div><h3>Key scientific concepts of review</h3><p>Our search covered five different databases: PubMed, SCOPUS, Wiley Online Library, Web of Science, and Cochrane Library. We included clinical trials investigating heart rate in asthmatic pediatric patients, either as a primary or secondary outcome. The primary outcome was heart rate changes. Secondary outcomes were respiratory rate, FEV1 peak percent changes, potassium serum levels, SpO2 peak changes, asthma score, and adverse effects. Eight clinical trials were included; seven of them were eligible for meta-analysis. In a dosing ratio of levalbuterol: albuterol =1:4, levalbuterol showed better outcomes on heart rate changes when compared with racemic albuterol (mean difference = −5.97, <em>p</em> = 0.02). However, this difference was dose-dependent as it vanished with equivalent dosing of levalbuterol: albuterol = 1:2. Levalbuterol also had a better effect on FEV1 changes (mean difference = 3.72, <em>p</em> = 0.003). However, there was no statistically significant difference between the two drugs regarding changes in respiratory rate, SpO2, asthma score, or adverse effects. In conclusion, levalbuterol and racemic albuterol have almost the same effect on heart rate in asthmatic children when they are used in equivalent dosing.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"74 ","pages":"Article 101725"},"PeriodicalIF":0.9,"publicationDate":"2024-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140765001","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effectiveness of intrapericardial fibrinolysis in an infant with purulent pericarditis","authors":"Ana Calabuig Adobes ,&nbsp;Marcos Clavero Adell ,&nbsp;José María Errea Albiol ,&nbsp;Daniel Palanca Arias","doi":"10.1016/j.ppedcard.2024.101727","DOIUrl":"https://doi.org/10.1016/j.ppedcard.2024.101727","url":null,"abstract":"<div><p>A 15-month-old infant was diagnosed with massive pericardial effusion with hemodynamic compromise. A percutaneous pericardiocentesis was performed to evacuate the fluid, revealing purulent content. Pericardial fluid cultures showed <em>Streptococcus pneumoniae,</em> so she was treated with intravenous Cefotaxime during 4 weeks. The patient's hemodynamic situation improved following the evacuation, but 72 h later, there was a deterioration in the cardiac function related to the reappearance of the effusion and the presence of fibrin tracts. Intrapericardial fibrinolysis with rt-PA was performed using the initial pericardial drain, resulting in the evacuation of purulent content and, subsequently, serosanguineous fluid. The patient remained stable after fibrinolysis without recurrence of the effusion, and surgical pericardiectomy was not necessary.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"73 ","pages":"Article 101727"},"PeriodicalIF":0.9,"publicationDate":"2024-04-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140555650","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A multiple variable index and extracardiac-Fontan associated hepatic fibrosis","authors":"William N. Evans ,&nbsp;Ruben J. Acherman ,&nbsp;Alvaro Galindo ,&nbsp;Abraham Rothman ,&nbsp;Michael L. Ciccolo ,&nbsp;Humberto Restrepo","doi":"10.1016/j.ppedcard.2024.101726","DOIUrl":"https://doi.org/10.1016/j.ppedcard.2024.101726","url":null,"abstract":"<div><h3>Background</h3><p>The serial follow-up of Fontan-associated liver disease is challenging, as laboratory values are frequently normal, especially with mild to moderate liver disease.</p></div><div><h3>Objective</h3><p>We investigated a composite index's correlation with hepatic biopsy total fibrosis scores in extracardiac Fontan patients.</p></div><div><h3>Methods</h3><p>We identified extracardiac Fontan patients undergoing cardiac catheterization and transvenous hepatic biopsies between June 2013 and September 2023 and liver shear wave elastography between January 2017 and July 2023. We developed a composite index from the following: 1) elastography values, 2) sex, 3) history of a neonatal aortopulmonary shunt for pulmonary flow obstruction, 4) pacemaker, 5) Fontan duration, 6) bilirubin values, 7) univentricular dysfunction, 8) atrioventricular valvar regurgitation, 9) mean Fontan pressures, and 10) venovenous collaterals presence. We correlated the index with hepatic total fibrosis scores (0–8), the sum of pathology grading (0 to 4) performed for sinusoidal and portal fibrosis. We defined a hepatic total fibrosis score of 0–3 as none to mild and 4–8 as moderate to severe fibrosis.</p></div><div><h3>Results</h3><p>We identified 62 patients who underwent 92 transvenous liver biopsies, with 30 patients undergoing 2 biopsies. The average age at biopsy was 15 ± 2 years. We found a strong correlation (rho = 0.8, <em>p</em> = .00001) between liver total fibrosis scores and composite index values. A receiver operating characteristic analysis demonstrated that an index cut-off value of ≥26 predicted a total fibrosis score of ≥4 with a sensitivity of 71 % and a specificity of 75 % (AUC = 0.73, 95 % CI 0.63, 0.83, <em>p</em> = .0001).</p></div><div><h3>Conclusions</h3><p>We developed a composite index with a moderate predictive ability to discriminate none to mild from moderate to severe hepatic fibrosis. Nevertheless, additional data is needed to assist further validation and determine its clinical utility in the serial follow-up of Fontan associated liver disease.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"73 ","pages":"Article 101726"},"PeriodicalIF":0.9,"publicationDate":"2024-04-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140543443","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Executive function and maladaptive coping in a young adult cohort of individuals with congenital heart disease","authors":"Sarah A. Mathena ,&nbsp;Vanessa F. Eller ,&nbsp;Scott E. Klewer ,&nbsp;Jennifer G. Andrews","doi":"10.1016/j.ppedcard.2024.101723","DOIUrl":"10.1016/j.ppedcard.2024.101723","url":null,"abstract":"<div><h3>Background</h3><p>Young adults with congenital heart disease (CHD) are more likely to exhibit executive dysfunction and maladaptive coping yet are less likely to get diagnosed than the general population for psychiatric disorders. The etiology of psychiatric diagnosis is inconclusive amongst CHD populations, and more research is needed to evaluate risk factors contributing to psychiatric disorders, including executive function, coping mechanisms, anxiety, depression, fatigue and sleep disorders, parental style/involvement, and sense of coherence.</p></div><div><h3>Objectives</h3><p>The study proposed to demonstrate the presence of risk for psychiatric diagnoses amongst young adults with CHD and evaluate factors contributing to mental health and executive dysfunction comorbidities.</p></div><div><h3>Methods</h3><p>We used a mixed methods study of quantitative scales and a guided interview on a cohort of 11 young adults (18–24) with CHD who are considered in the American Heart Association high-risk group for developmental disorders or disabilities. We assessed for risk of anxiety and depression, maladaptive and disengagement coping, executive dysfunction, and other factors associated with mental health using the Reponses to Stress (RSQ), Patient Reported Outcomes Measurement Information System (PROMIS), and the Amsterdam Executive Function Inventory (AEFI) scales. Qualitative interviews focused on experiences living with CHD, social and family support, and executive functioning skills.</p></div><div><h3>Results</h3><p>Our mean participant age was 20 years, and 36 % had some college education. PROMIS scoring showed increased risk for poor physical function (mean <em>r</em> = 31.8), anxiety (mean <em>r</em> = 64.9), depression (mean <em>r</em> = 66.8), and pain interference (mean <em>r</em> = 66.6). AEFI results also showed poor executive functioning skills, particularly in Self-Control/Self-Monitoring (mean 18.1) and Planning and Initiative (mean 5.3). 27 % of participants had medical record documentation of depression. Interviews showed participants overinflated sense of coherence and poor executive functioning, leading to two main themes: 1) perceptions of their disability, including denial of their CHD diagnosis and disengagement with mental health needs, and 2) challenges in transition care.</p></div><div><h3>Conclusions</h3><p>Our study indicates a need for larger scale interventions for psychological young adults with CHD as part of the transition process to adult care.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"73 ","pages":"Article 101723"},"PeriodicalIF":0.9,"publicationDate":"2024-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140280254","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}