PROGRESS IN PEDIATRIC CARDIOLOGY最新文献

{"title":"It is a fine line with flecainide: A case of flecainide toxicity in a pediatric patient","authors":"Aura Daniella Santi-Turchi ,&nbsp;Keerthana Banala ,&nbsp;Michelle Jadotte ,&nbsp;Sherrie Joy Baysa ,&nbsp;Steven Fishberger","doi":"10.1016/j.ppedcard.2024.101761","DOIUrl":"10.1016/j.ppedcard.2024.101761","url":null,"abstract":"<div><div>Flecainide is a class IC antiarrhythmic used in the treatment of supraventricular and ventricular tachyarrhythmias in the pediatric population. It has a narrow therapeutic index that requires careful dosing and monitoring of levels, as well as education on preparation and administration. Many different factors alter the bioavailability of this medication, and special consideration must be taken among pediatric patients. Clinical signs of toxicity include lethargy, bradycardia, altered mental status, and ventricular arrhythmias. Management of flecainide toxicity involves administering sodium bicarbonate as well as lipid emulsion therapy. We present the case of a child found to have flecainide toxicity and discuss management strategies.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2024-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142553178","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":"Clinical insights: Resolving the specter of cardiac sequelae in multisystem inflammatory syndrome in children (MIS-C) – A 24-month follow-up case series","authors":"Carolina A.B. Capuruco ,&nbsp;Maicon Rodrigues Albuquerque ,&nbsp;Daniela Valadão Rosa ,&nbsp;Fernanda da Silva Barbosa ,&nbsp;Caio Augusto Domingos ,&nbsp;Cleonice Carvalho de Coelho Mota ,&nbsp;Débora Marques de Miranda","doi":"10.1016/j.ppedcard.2024.101759","DOIUrl":"10.1016/j.ppedcard.2024.101759","url":null,"abstract":"<div><h3>Background</h3><div>During the COVID-19 pandemic, multisystem inflammatory syndrome (MIS-C) emerged as a novel and severe complication.</div></div><div><h3>Objectives</h3><div>The present prospective study followed patients in Brazil from February 2020 to December 2022, and evaluated children discharged from reference centers with an MIS-C diagnosis. The aim of the study is to describe long-term cardiac findings.</div></div><div><h3>Methods</h3><div>Following a clinical and imaging procedure, children with MIS-C were followed up.</div></div><div><h3>Results</h3><div>Thirty-six children during hospitalization coursed with fever; 61.1 % presented with gastrointestinal symptoms, 77.7 % with cardiocirculatory manifestations, and 28 % with respiratory issues. Shock was observed in 37 % of patients, and the average hospital stay was 9 ± 5 days, with 54 % exhibiting severe symptoms requiring intensive care. Elevated inflammatory and cardiac markers were common; 42 % of patients presented elevated troponin and D-dimer levels. During intensive care, echocardiographic abnormalities were found in 56 % of the patients, including coronary dilation and pericardial effusion. All patients were hospitalized and treated with intravenous immunoglobulin, oral steroids, and acetylsalicylic acid, resulting in 86 % recovery. However, 14 % of the patients had persistent (mild cardiac) alterations at discharge. Sex and previous chronic conditions did not affect the persistence of cardiac findings, whereas marginal age differences indicated that older children tend to have more severe symptoms. BMI was identified as a risk factor but must be considered carefully. Six months post-discharge, all patients successfully recovered from cardiac alterations.</div></div><div><h3>Conclusion</h3><div>Only a few case series have reported persistent MIS-C findings, and this case series elicits a positive post-discharge prognosis for MIS-C over time.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142533603","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":"Guideline-directed medical therapy for dilated cardiomyopathy in the era of pediatric ventricular assist devices: Improving transplant-free survival","authors":"Benjamin A. Olsen ,&nbsp;Melanie D. Everitt","doi":"10.1016/j.ppedcard.2024.101755","DOIUrl":"10.1016/j.ppedcard.2024.101755","url":null,"abstract":"<div><h3>Background</h3><div>Cardiomyopathy remains the leading cause of transplantation in children greater than one year of age, with low five-year transplant free survival. Despite these poor outcomes, the potential for myocardial recovery in pediatric dilated cardiomyopathy (DCM) has long been reported. In the current era of guideline directed medical therapy (GDMT), however, achievement of myocardial recovery has not been rigorously studied. Ventricular assist devices (VADs) can also be employed as a bridge to recovery, but VAD explants for recovery are rare.</div></div><div><h3>Aims of review</h3><div>A focus of the Sixth International Conference on Childhood Cardiomyopathy was achievement of myocardial recovery in the era of GDMT and VADs. Key questions included: (1) what is the potential for myocardial recovery; (2) what role does GDMT play in achieving myocardial recovery; (3) can durable left ventricular assist devices (LVADs) be a reasonable means to achieving recovery; (4) how do rates of recovery compare between GDMT and LVADs; and (5) what are the next steps in improving rates of myocardial recovery? This review aims to answer these questions by providing a summary of the existing literature.</div></div><div><h3>Key scientific concepts of review</h3><div>The future directions for pediatric DCM are to use GDMT and VADs as complementary therapies to improve not only survival to transplant, but to assess for recovery parameters so that more can be learned about the potential for transplant-free survival in patients supported by VAD and GDMT.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2024-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142421104","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":"Decreased ventricular repolarization variability in one-year-olds of gestational diabetes mothers","authors":"Martin Schmidt ,&nbsp;Alexander Hammer ,&nbsp;Hagen Malberg ,&nbsp;Silvia M. Lobmaier ,&nbsp;Peter Ewert ,&nbsp;Renate Oberhoffer-Fritz ,&nbsp;Annette Wacker-Gussmann","doi":"10.1016/j.ppedcard.2024.101756","DOIUrl":"10.1016/j.ppedcard.2024.101756","url":null,"abstract":"<div><h3>Background</h3><div>Gestational diabetes mellitus (GDM) is currently the most common medical complication in pregnancy, affecting approximately 13 % of all pregnant women. Although long-term effects on the offspring are still unclear, previous studies indicate ventricular myocardial changes.</div></div><div><h3>Objectives</h3><div>The aim of our study was to investigate whether early biomarkers such as QT interval variability (QTV) are sensitive enough to predict these structural changes in children of GDM mothers.</div></div><div><h3>Methods</h3><div>We focused on children of GDM mothers. Healthy children of mothers without GDM served as controls. All of them were examined at the German Heart Center, Munich, Germany. Heart rate variability (HRV) and QTV measures were extracted from 10 to 15 min Einthoven II electrocardiograms, split into 5 min windows, to characterize the effects of possible autonomic nervous system alterations and cellular ventricular mutations.</div></div><div><h3>Results</h3><div>75 children were included in this prospective observational case-control study; 42 of them were children of GDM mothers. The median age at the examination was 12 months (11–13 months). We found decreased QTV as a measure of ventricular repolarization variability in one-year-olds of GDM mothers compared to healthy controls (<em>p</em> &lt; 0.05).</div></div><div><h3>Conclusion</h3><div>We have found increased very low frequency HRV in females and decreased QTV in male children of GDM mothers, which suggests diverse responses and could reflect increased sympathetic tone and altered ventricular myocardium at a cellular level, respectively. Further work is required to understand the long-term significance of these findings in terms of providing an easy-to-use and cost-effective technology for early diagnosis of myocardial damage.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2024-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142421103","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Health related quality of life in children following Fontan surgery: A Malaysian perspective","authors":"Ming Chern Leong ,&nbsp;Mohamed Ajmal Haj Mohamed ,&nbsp;Nurul Fazira Basran ,&nbsp;Nor Athirah Azeman ,&nbsp;Putri Yubbu","doi":"10.1016/j.ppedcard.2024.101757","DOIUrl":"10.1016/j.ppedcard.2024.101757","url":null,"abstract":"<div><h3>Background</h3><div>Children with Fontan circulation suffer from morbidities and physical limitations that affect their quality of life. The study examined the health-related quality of life (HRQoL) of children in Malaysia following the Fontan procedure.</div></div><div><h3>Methods</h3><div>All patients who are 8 years and older and who had undergone Fontan palliation for at least 1 year were assessed using electronically distributed, aged-based Pediatric Quality of Life Inventory™ (PedsQL) Generic and Cardiac modules. The scores from the patients were compared with those of aged-match students in the Klang Valley and patient's parents.</div></div><div><h3>Results</h3><div>A total of 133 patients [time after Fontan: 6.24 years (IQR: 2.54, 8.54); SpO₂: 92 % (IQR: 88, 95)] and parents and 130 normal subjects participated in the study. Fontan patients have reduced HRQOL scores compared to their healthy peers in almost all domains. Patients with NYHA class I demonstrated higher PedsQL scores in almost all domains. Patients with SpO2 &lt; 95 % and taking more medication had lower PedsQL scores in total and physical score. Similarly, those with many complications show significantly low PedsQL scores in all domains. There was poor agreement between patients' and parents' perceived HRQoL in most of the domains in the generic module, but the difference was not as explicit in the cardiac module.</div></div><div><h3>Conclusions</h3><div>Fontan patients had a lower perceived quality of life compared to healthy controls. Physical ability and Fontan complications influenced the perceived quality of life, especially in younger children. Parents generally underestimate the impact of Fontan towards their children's quality of life.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2024-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142433758","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":"Immediate and short-term outcomes of percutaneous balloon mitral commissurotomy for severe mitral stenosis at a Resource Limited Setting, Addis Ababa, Ethiopia","authors":"Mersha Mamo ,&nbsp;Esubalew Woldeyes ,&nbsp;Samson Zegeye ,&nbsp;Seife Feleke ,&nbsp;Ermiyas Endewunet Melaku","doi":"10.1016/j.ppedcard.2024.101754","DOIUrl":"10.1016/j.ppedcard.2024.101754","url":null,"abstract":"<div><h3>Background</h3><p>Rheumatic heart disease is a common cause of mitral stenosis (MS) in developing nations. Percutaneous mitral balloon commissurotomy (PMBC) is the treatment of choice for patients with MS with favorable anatomy.</p></div><div><h3>Objective</h3><p>This study aimed to assess immediate and short-term outcomes of percutaneous balloon mitral commissurotomy for symptomatic patients with rheumatic severe mitral stenosis.</p></div><div><h3>Methods</h3><p>The clinical records of patients who underwent Percutaneous Mitral Balloon Commissurotomy (PBMC) at Saint Paul Hospital Millennium Medical College, Cardiac Center were reviewed. A retrospective observational study was conducted from February 01, 2018, to August 30, 2021. All eligible patients who underwent Percutaneous Mitral Balloon Commissurotomy during the study period at the cardiac center were included in this study. Ninety-one patients were enrolled in the study after excluding repeat procedures, those with incomplete data, and lost medical records. Data were collected through chart reviews using a structured questionnaire by trained physicians, with daily supervision by the investigators. Data analysis was performed using SPSS version 25. Associations were checked using a paired <em>t</em>-test and Fisher's Exact Test, with statistical significance set at a <em>p</em>-value of &lt;0.05.</p></div><div><h3>Result</h3><p>Percutaneous mitral balloon commissurotomy was successful in 91.2 % of patients. Following the procedure, there was a significant increase in the mean mitral valve area from 0.799 cm² to 1.674 cm² and a reduction in the trans-mitral mean pressure gradient from 20 mmHg to 7.71 mmHg (<em>P</em> &lt; 0.001). Atrial fibrillation and a high Wilkins score were identified as correlates of non-successful PMBC. A Wilkin's score of ≤8 and a post-procedure mitral valve area &gt; 1.5 cm² were associated with New York Heart Association functional class improvement three months after the procedure.</p></div><div><h3>Conclusion</h3><p>In this study, percutaneous mitral balloon Commissurotomy (PMBC) demonstrated an excellent success rate. The majority of patients exhibited significant improvement in the New York Heart Association (NYHA) functional class.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2024-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142272564","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":"Double-chambered left ventricle in a patient with Wolff–Parkinson–White syndrome: A case report","authors":"Marco Busco ,&nbsp;Francesca Augusta Gabrielli ,&nbsp;Gianluigi Bencardino ,&nbsp;Pasquale Notarstefano ,&nbsp;Gemma Pelargonio","doi":"10.1016/j.ppedcard.2024.101753","DOIUrl":"10.1016/j.ppedcard.2024.101753","url":null,"abstract":"<div><p>Congenital heart diseases like non-compaction cardiomyopathy, congenital left ventricular diverticulum, and aneurysm are potential triggers of arrhythmias. Any condition affecting the correct embryological development of the atrioventricular annuli might potentially be the basis for accessory pathways and WPW Syndrome; we are describing a first reported association with a rare congenital heart disease, a double-chambered left ventricle. Although typically diagnosed in pediatric age, it can remain asymptomatic and incidentally found in adulthood. The embryological alteration in the myocardial compaction might explain both the formation of an accessory chamber and the persistence of an anomalous electrical connection between atria and ventricles.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2024-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142147677","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 de novo FBN1 missense variant associated with a severe phenotype of early onset Marfan syndrome","authors":"Sara Markholt ,&nbsp;Jens Skaerbaek ,&nbsp;Kim Munk ,&nbsp;Brian N. Andersen ,&nbsp;Dorte L. Lilballe ,&nbsp;Jenny Blechingberg ,&nbsp;Jesper P. Petersen ,&nbsp;Jesper V. Bjerre ,&nbsp;Pernille A. Gregersen ,&nbsp;Kasper J. Kyng","doi":"10.1016/j.ppedcard.2024.101751","DOIUrl":"10.1016/j.ppedcard.2024.101751","url":null,"abstract":"<div><p>Early-onset Marfan syndrome is a rare subtype of Marfan Syndrome (MFS), manifesting early in life. Affected individuals typically present with a severe phenotype, with critical signs and symptoms as early as the neonatal or infantile period, most often due to a <em>de novo</em> pathogenic <em>FBN1</em> variant. We describe a patient with a rare <em>de novo</em> missense variant c.3284G&gt;C (p.(Cys1095Ser)) in exon 27 in <em>FBN1</em> leading to early onset MFS with severe cardiovascular involvement including heart enlargement with regurgitation of both atrioventricular valves, aortic dilatation and regurgitation manifest from birth, with a fatal outcome.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1058981324000493/pdfft?md5=e26303f111c9d5b310bd45292b186d98&pid=1-s2.0-S1058981324000493-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142147675","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Closure of an intrahepatic porto-systemic shunt in a newborn with hypoglycemia, hyperammonemia, and hyperbilirubinemia","authors":"Kencie Ely ,&nbsp;Alvaro Galindo ,&nbsp;Humberto Restrepo ,&nbsp;Abraham Rothman","doi":"10.1016/j.ppedcard.2024.101752","DOIUrl":"10.1016/j.ppedcard.2024.101752","url":null,"abstract":"<div><p>A newborn with hypoglycemia was transferred to the neonatal intensive care unit. An elevated bilirubin level prompted an abdominal ultrasound, which revealed a large portosystemic shunt. Due to persistent hypoglycemia, hyperammonemia, and hyperbilirubinemia for 3 weeks, the patient was taken to cardiac catheterization, where the portosystemic shunt was closed with an Amplatzer Vascular Plug IV. The patient's metabolic abnormalities abated. He remained asymptomatic 12 months after the procedure.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2024-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142147676","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":"ECG findings are poor predictors for adverse events and cardiac death in Barth syndrome","authors":"Alexander Hutchinson,&nbsp;Carolyn L. Taylor,&nbsp;Shahryar M. Chowdhury,&nbsp;Lanier Jackson","doi":"10.1016/j.ppedcard.2024.101750","DOIUrl":"10.1016/j.ppedcard.2024.101750","url":null,"abstract":"<div><h3>Background</h3><p>Patients with Barth syndrome (BTHS) can present with cardiomyopathy. BTHS subjects are at risk for cardiac adverse outcomes throughout life, including malignant arrhythmias and death. Electrocardiogram (ECG) parameters have never been assessed as a tool to predict adverse outcomes in individuals with BTHS.</p></div><div><h3>Objectives</h3><p>The purpose of this study was to identify any ECG parameters, including QRS fragmentation, presence of arrhythmia, or abnormal intervals, that could predict adverse outcomes and cardiac death among the BTHS population.</p></div><div><h3>Methods</h3><p>We performed a retrospective case referent study on subjects with BTHS (<em>n</em> = 43) and compared them with our reference group, subjects with idiopathic dilated cardiomyopathy (DCM) from a single institution (<em>n</em> = 53) from 2007 to 2021. BTHS data was obtained from subjects attending the biennial Barth Syndrome Foundation International Scientific, Medical, and Family Conferences (BSFISMFC) from 2002 to 2018. ECG data from the first and last available ECGs prior to an adverse event or cardiac death was analyzed. Then, multivariable regression was performed to determine odd ratios between ECG characteristics and adverse events/cardiac death.</p></div><div><h3>Results</h3><p>No ECG variables were statistically significant predictors of adverse events or cardiac death in the BTHS group. Last ECG QRS fragmentation trended to statistically significance (OR 13.3, <em>p</em> = 0.12) in predicting adverse events in the DCM group.</p></div><div><h3>Conclusion</h3><p>No ECG parameters, including QRS fragmentation, presence of arrhythmia, or abnormal interval values, predict adverse events or cardiac death among BTHS patients. QRS fragmentation may be a predictor of adverse events in the DCM population.</p></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2024-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142147674","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}