Pub Date : 2024-11-09DOI: 10.1016/j.ppedcard.2024.101767
Vaishnavi Divya Nagarajan , Laura A. Miller-Smith , Yoshi O. Otaki , Ashok Muralidaran , Grant H. Burch , Laurie B. Armsby , Becky J. Riggs
16-month-old with single ventricle Glenn physiology underwent early placement of an extra-cardiac non-fenestrated Fontan. Post-operative course complicated by a nearly occlusive inferior vena cava thrombus and an intra-Fontan thrombus causing multiorgan system failure. Both thrombi were emergently removed with fluoroscopy, ultrasound, and computer guided clot-aspirator in the pediatric catheterization lab.
{"title":"A novel approach to thrombectomy and catheter directed tissue-type plasminogen activator in a toddler post-fontan","authors":"Vaishnavi Divya Nagarajan , Laura A. Miller-Smith , Yoshi O. Otaki , Ashok Muralidaran , Grant H. Burch , Laurie B. Armsby , Becky J. Riggs","doi":"10.1016/j.ppedcard.2024.101767","DOIUrl":"10.1016/j.ppedcard.2024.101767","url":null,"abstract":"<div><div>16-month-old with single ventricle Glenn physiology underwent early placement of an extra-cardiac non-fenestrated Fontan. Post-operative course complicated by a nearly occlusive inferior vena cava thrombus and an intra-Fontan thrombus causing multiorgan system failure. Both thrombi were emergently removed with fluoroscopy, ultrasound, and computer guided clot-aspirator in the pediatric catheterization lab.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"75 ","pages":"Article 101767"},"PeriodicalIF":0.6,"publicationDate":"2024-11-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142704807","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}
Pub Date : 2024-10-24DOI: 10.1016/j.ppedcard.2024.101763
Theodore J. Millette , James J. Gangemi , Shelby C. White , Michael J. Shorofsky
Left ventricular aneurysms and pseudoaneurysms are rare in the pediatric population. We report a case of a preterm neonate with critical aortic valve stenosis who developed a left ventricular pseudoaneurysm and perforation following transcatheter balloon aortic valvuloplasty on day four of life. The possible etiologies of myocardial injury and the unique surgical repair technique are discussed.
{"title":"Left ventricular pseudoaneurysm following balloon aortic valvuloplasty in a preterm neonate: Case report","authors":"Theodore J. Millette , James J. Gangemi , Shelby C. White , Michael J. Shorofsky","doi":"10.1016/j.ppedcard.2024.101763","DOIUrl":"10.1016/j.ppedcard.2024.101763","url":null,"abstract":"<div><div>Left ventricular aneurysms and pseudoaneurysms are rare in the pediatric population. We report a case of a preterm neonate with critical aortic valve stenosis who developed a left ventricular pseudoaneurysm and perforation following transcatheter balloon aortic valvuloplasty on day four of life. The possible etiologies of myocardial injury and the unique surgical repair technique are discussed.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"75 ","pages":"Article 101763"},"PeriodicalIF":0.6,"publicationDate":"2024-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142650615","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}
Pub Date : 2024-10-24DOI: 10.1016/j.ppedcard.2024.101762
Anis Munirah Mohd Kori , Nursyahirah Anum Mohd Radzi , Ammar Mohamad Ziyadi , Intan Juliana Abd Hamid , Nor Rosidah Ibrahim , Noraida Ramli , Mohd Rizal Mohd Zain
Primary cardiac tumors in fetuses are rare and mainly represent rhabdomyoma. The tumors can be clinically silent or cause hemodynamically significant obstructions. We present a case of giant neonatal cardiac rhabdomyoma with right inflow obstruction complicated by fetal supraventricular tachycardia. Fetal echocardiography revealed the presence of supraventricular tachycardia with multiple cardiac masses, one of which caused an obstruction of the right inflow of the heart. As the baby developed hemodynamic instability due to the mass effect, oral sirolimus was initiated postnatally. Despite the limitation of evidence on the usage of sirolimus in neonatal population, this case report revealed the benefits of postnatal sirolimus in a decrease in the size of the mass without any adverse medication effects. Sirolimus has demonstrated efficacy in neonatal cardiac rhabdomyoma, however, a large prospective study is needed to demonstrate the efficacy.
{"title":"Fetal supraventricular tachycardia with giant cardiac rhabdomyoma: Role of post natal sirolimus in a developing country","authors":"Anis Munirah Mohd Kori , Nursyahirah Anum Mohd Radzi , Ammar Mohamad Ziyadi , Intan Juliana Abd Hamid , Nor Rosidah Ibrahim , Noraida Ramli , Mohd Rizal Mohd Zain","doi":"10.1016/j.ppedcard.2024.101762","DOIUrl":"10.1016/j.ppedcard.2024.101762","url":null,"abstract":"<div><div>Primary cardiac tumors in fetuses are rare and mainly represent rhabdomyoma. The tumors can be clinically silent or cause hemodynamically significant obstructions. We present a case of giant neonatal cardiac rhabdomyoma with right inflow obstruction complicated by fetal supraventricular tachycardia. Fetal echocardiography revealed the presence of supraventricular tachycardia with multiple cardiac masses, one of which caused an obstruction of the right inflow of the heart. As the baby developed hemodynamic instability due to the mass effect, oral sirolimus was initiated postnatally. Despite the limitation of evidence on the usage of sirolimus in neonatal population, this case report revealed the benefits of postnatal sirolimus in a decrease in the size of the mass without any adverse medication effects. Sirolimus has demonstrated efficacy in neonatal cardiac rhabdomyoma, however, a large prospective study is needed to demonstrate the efficacy.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"75 ","pages":"Article 101762"},"PeriodicalIF":0.6,"publicationDate":"2024-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142658469","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}
Pub Date : 2024-10-18DOI: 10.1016/j.ppedcard.2024.101761
Aura Daniella Santi-Turchi , Keerthana Banala , Michelle Jadotte , Sherrie Joy Baysa , Steven Fishberger
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.
氟卡尼是一种 IC 类抗心律失常药物，用于治疗儿童室上性和室性快速性心律失常。它的治疗指数较窄，需要谨慎给药和监测药物浓度，并进行配药和用药方面的教育。许多不同的因素会改变这种药物的生物利用度，因此必须特别考虑到儿科患者。中毒的临床表现包括嗜睡、心动过缓、精神状态改变和室性心律失常。处理非卡尼中毒的方法包括使用碳酸氢钠和脂质乳剂治疗。我们介绍了一例发现福卡尼中毒的患儿，并讨论了处理策略。
{"title":"It is a fine line with flecainide: A case of flecainide toxicity in a pediatric patient","authors":"Aura Daniella Santi-Turchi , Keerthana Banala , Michelle Jadotte , Sherrie Joy Baysa , 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":"75 ","pages":"Article 101761"},"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}
Implantation of an epicardial pacemaker in young children is carried out in a pocket above the rectus muscle. In extremely rare cases, pediatric patients may experience migration of the pacemaker into the abdominal cavity. Symptoms can range from mild abdominal discomfort, diarrhea, vomiting to potentially dangerous intestinal obstruction due to perforation of the large intestine. We present a case of a 3-year-old child with migration of the pacemaker into the sigmoid colon with the formation of a colonic fistula. Early diagnosis helps to avoid serious complications in the case of pacemaker migration. Any change in the position of the pacemaker will indicate the need for pacemaker reimplantation to prevent life-threatening symptoms.
{"title":"Migration of a pacemaker into the sigmoid colon in a three-year-old child: A case report","authors":"I.A. Soynov, A.N. Arkhipov, S.N. Manukian, D.A. Elesin, T.S. Khapaev, A.B. Romanov","doi":"10.1016/j.ppedcard.2024.101760","DOIUrl":"10.1016/j.ppedcard.2024.101760","url":null,"abstract":"<div><div>Implantation of an epicardial pacemaker in young children is carried out in a pocket above the rectus muscle. In extremely rare cases, pediatric patients may experience migration of the pacemaker into the abdominal cavity. Symptoms can range from mild abdominal discomfort, diarrhea, vomiting to potentially dangerous intestinal obstruction due to perforation of the large intestine. We present a case of a 3-year-old child with migration of the pacemaker into the sigmoid colon with the formation of a colonic fistula. Early diagnosis helps to avoid serious complications in the case of pacemaker migration. Any change in the position of the pacemaker will indicate the need for pacemaker reimplantation to prevent life-threatening symptoms.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"75 ","pages":"Article 101760"},"PeriodicalIF":0.6,"publicationDate":"2024-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142658474","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}
Pub Date : 2024-10-10DOI: 10.1016/j.ppedcard.2024.101759
Carolina A.B. Capuruco , Maicon Rodrigues Albuquerque , Daniela Valadão Rosa , Fernanda da Silva Barbosa , Caio Augusto Domingos , Cleonice Carvalho de Coelho Mota , Débora Marques de Miranda
Background
During the COVID-19 pandemic, multisystem inflammatory syndrome (MIS-C) emerged as a novel and severe complication.
Objectives
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.
Methods
Following a clinical and imaging procedure, children with MIS-C were followed up.
Results
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.
Conclusion
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.
{"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 , Maicon Rodrigues Albuquerque , Daniela Valadão Rosa , Fernanda da Silva Barbosa , Caio Augusto Domingos , Cleonice Carvalho de Coelho Mota , 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":"75 ","pages":"Article 101759"},"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}
Pub Date : 2024-10-06DOI: 10.1016/j.ppedcard.2024.101758
Nichelle L. Duncan , Rebecca Harrell , Scarlett Anthony , Cierra Buckman , Lauren Sarno , Samuel F. Sears
Background
Examination of sleep in pediatric cardiology patients has typically relied on self-reports with unknown reliability to objective assessments. Wearable technologies provide an objective data source for evaluating sleep. Improved understanding and treatment of sleep in pediatric heart patients may be an untapped aspect that could improve health-related quality of life.
Objectives
The purpose of this study was to identify patterns of duration and quality of sleep in pediatric cardiology patients using both subjective and objective assessment.
Methods
Patients were recruited in a pediatric cardiology clinic and completed a set of sleep and quality of life questionnaires, including the Pittsburg Sleep Quality Index (PSQI), the Pediatric Cardiac Quality of Life Inventory (PCQLI), and the Cardiac Anxiety Questionnaire (CAQ). Patients also agreed to wear an actigraphy watch for 7 days, and caregivers were also recruited as proxy raters.
Results
The sample included 31 patients with a mean age of 15.58 (SD = 1.89). Patients self-reported an average sleep duration of 7.61 h (SD = 1.67, range = 3–11). In contrast, objective sleep data indicated that patients obtained an average of 5.98 h of sleep nightly (SD = 1.01, range = 3.23–7.46) with a sleep efficiency score of 73.2 (SD = 6.23) and a sleep latency period of 21.04 min (SD = 19.16, range = 1–63). Parent-reported sleep duration on the PSQI had a mean of 8.10 h (SD = 1.34, range = 6–11). Thirty four percent of patients met PSQI cut off score for poor sleep based on self-report and 30 % based on parental-report.
Conclusions
Pediatric cardiac patients report better sleep duration than objective assessments. These data suggest that sleep complaints from pediatric cardiac patients may benefit from objective assessment and may need enhanced clinical attention since complaints may overestimate the duration of sleep.
{"title":"Are you asleep? Comparing sleep metrics in pediatric cardiac patients with objective and subjective indicators","authors":"Nichelle L. Duncan , Rebecca Harrell , Scarlett Anthony , Cierra Buckman , Lauren Sarno , Samuel F. Sears","doi":"10.1016/j.ppedcard.2024.101758","DOIUrl":"10.1016/j.ppedcard.2024.101758","url":null,"abstract":"<div><h3>Background</h3><div>Examination of sleep in pediatric cardiology patients has typically relied on self-reports with unknown reliability to objective assessments. Wearable technologies provide an objective data source for evaluating sleep. Improved understanding and treatment of sleep in pediatric heart patients may be an untapped aspect that could improve health-related quality of life.</div></div><div><h3>Objectives</h3><div>The purpose of this study was to identify patterns of duration and quality of sleep in pediatric cardiology patients using both subjective and objective assessment.</div></div><div><h3>Methods</h3><div>Patients were recruited in a pediatric cardiology clinic and completed a set of sleep and quality of life questionnaires, including the Pittsburg Sleep Quality Index (PSQI), the Pediatric Cardiac Quality of Life Inventory (PCQLI), and the Cardiac Anxiety Questionnaire (CAQ). Patients also agreed to wear an actigraphy watch for 7 days, and caregivers were also recruited as proxy raters.</div></div><div><h3>Results</h3><div>The sample included 31 patients with a mean age of 15.58 (SD = 1.89). Patients self-reported an average sleep duration of 7.61 h (SD = 1.67, range = 3–11). In contrast, objective sleep data indicated that patients obtained an average of 5.98 h of sleep nightly (SD = 1.01, range = 3.23–7.46) with a sleep efficiency score of 73.2 (SD = 6.23) and a sleep latency period of 21.04 min (SD = 19.16, range = 1–63). Parent-reported sleep duration on the PSQI had a mean of 8.10 h (SD = 1.34, range = 6–11). Thirty four percent of patients met PSQI cut off score for poor sleep based on self-report and 30 % based on parental-report.</div></div><div><h3>Conclusions</h3><div>Pediatric cardiac patients report better sleep duration than objective assessments. These data suggest that sleep complaints from pediatric cardiac patients may benefit from objective assessment and may need enhanced clinical attention since complaints may overestimate the duration of sleep.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101758"},"PeriodicalIF":0.6,"publicationDate":"2024-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143519231","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}
Pub Date : 2024-09-29DOI: 10.1016/j.ppedcard.2024.101755
Benjamin A. Olsen , Melanie D. Everitt
Background
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.
Aims of review
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.
Key scientific concepts of review
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.
背景心肌病仍然是一岁以上儿童接受移植的主要原因，其五年无移植存活率很低。尽管治疗效果不佳，但小儿扩张型心肌病（DCM）的心肌恢复潜力却早有报道。然而，在目前指导性医疗疗法（GDMT）的时代，心肌恢复的实现还没有得到严格的研究。第六届儿童心肌病国际会议的重点是在 GDMT 和 VAD 时代实现心肌恢复。关键问题包括(1) 心肌恢复的潜力有多大；(2) GDMT 在实现心肌恢复中扮演什么角色；(3) 耐用的左心室辅助装置 (LVAD) 能否成为实现恢复的合理手段；(4) GDMT 和 LVAD 的恢复率如何比较；以及 (5) 提高心肌恢复率的下一步是什么？本综述旨在通过对现有文献进行总结来回答这些问题。综述的主要科学概念小儿 DCM 的未来发展方向是将 GDMT 和 VAD 作为辅助疗法，不仅提高移植存活率，而且评估恢复参数，以便更多地了解 VAD 和 GDMT 支持下的患者无移植存活的潜力。
{"title":"Guideline-directed medical therapy for dilated cardiomyopathy in the era of pediatric ventricular assist devices: Improving transplant-free survival","authors":"Benjamin A. Olsen , 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":"75 ","pages":"Article 101755"},"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}
Pub Date : 2024-09-29DOI: 10.1016/j.ppedcard.2024.101756
Martin Schmidt , Alexander Hammer , Hagen Malberg , Silvia M. Lobmaier , Peter Ewert , Renate Oberhoffer-Fritz , Annette Wacker-Gussmann
Background
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.
Objectives
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.
Methods
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.
Results
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 (p < 0.05).
Conclusion
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.
{"title":"Decreased ventricular repolarization variability in one-year-olds of gestational diabetes mothers","authors":"Martin Schmidt , Alexander Hammer , Hagen Malberg , Silvia M. Lobmaier , Peter Ewert , Renate Oberhoffer-Fritz , 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> < 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":"75 ","pages":"Article 101756"},"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}
Pub Date : 2024-09-29DOI: 10.1016/j.ppedcard.2024.101757
Ming Chern Leong , Mohamed Ajmal Haj Mohamed , Nurul Fazira Basran , Nor Athirah Azeman , Putri Yubbu
Background
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.
Methods
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.
Results
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 < 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.
Conclusions
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.
{"title":"Health related quality of life in children following Fontan surgery: A Malaysian perspective","authors":"Ming Chern Leong , Mohamed Ajmal Haj Mohamed , Nurul Fazira Basran , Nor Athirah Azeman , 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); SpO<sub>2</sub>: 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 < 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":"75 ","pages":"Article 101757"},"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}
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