PROGRESS IN PEDIATRIC CARDIOLOGY最新文献

{"title":"Corrigendum to “Aortic arch atresia in Williams syndrome” [Prog Pediatr Cardiol 65 (2022) 101510]","authors":"Stefanie M. Miller , Peace C. Madueme , Peter D. Wearden , Jennifer S. Nelson","doi":"10.1016/j.ppedcard.2024.101794","DOIUrl":"10.1016/j.ppedcard.2024.101794","url":null,"abstract":"","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"75 ","pages":"Article 101794"},"PeriodicalIF":0.6,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143084206","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":"Corrigendum to “Progress in pediatric cardiology chest pain for the pediatric primary care provider” [Prog. Pediatr. Cardiol. 65 (2022) 101515]","authors":"Gursharon Sanghera , Vanessa Hormaza , Gul H. Dadlani","doi":"10.1016/j.ppedcard.2024.101791","DOIUrl":"10.1016/j.ppedcard.2024.101791","url":null,"abstract":"","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"75 ","pages":"Article 101791"},"PeriodicalIF":0.6,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143095124","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":"Cardiovascular disease associated with genetic defects","authors":"Elliot G. Griffith ,&nbsp;Keegan Musaalo ,&nbsp;Sarah Hunter Jackson ,&nbsp;Emily R. Ribeiro","doi":"10.1016/j.ppedcard.2024.101765","DOIUrl":"10.1016/j.ppedcard.2024.101765","url":null,"abstract":"<div><h3>Background</h3><div>Numerous genes affect cardiac morphogenesis and are implicated in congenital heart diseases. Some genetic mutations are associated with problems involving the respiratory tract, kidneys, brain, and immune system, which may affect morbidity and mortality. Therefore, proper identification of genetic disease is important.</div></div><div><h3>Aim of review</h3><div>The purpose of this article is to review cardiac embryology, common forms of congenital heart disease that may occur from disruption of embryologic development and common genetic defects that may cause malformations and clinical syndromes.</div></div><div><h3>Key scientific concepts of review</h3><div>During embryologic development, a heart tube is formed early in gestation and folds on itself. Proper heart looping and lateralization rely on specific patterning pathways. A defect in the right-left patterning pathways may cause heterotaxy. Heart septation subsequently partitions the heart into the four cardiac chambers. Disorders of heart septation include atrioventricular septal defects, which are associated with Down syndrome. During conotruncal development, the outflow tract separates into the pulmonary trunk and aorta. Failure of normal conotruncal development may cause truncus arteriosus, tetralogy of Fallot, coarctation of the aorta, or interrupted aortic arch. Truncus arteriosus may be associated with DiGeorge syndrome, CHARGE syndrome, or VACTERL association. Tetralogy of Fallot is associated with DiGeorge syndrome and mutations in <em>NOTCH1</em>. Coarctation of the aorta is associated with Turner syndrome, and interrupted aortic arch is associated with DiGeorge syndrome. Genetic testing is important in patients who have CHD and may help predict extracardiac disease and prognosis. Geneticists and genetic counselors are important in this process.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"75 ","pages":"Article 101765"},"PeriodicalIF":0.6,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143094911","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":"Procedural success and complications of transthoracic echocardiography guided atrial septal defect device closure in children at a tertiary cardiac center in Nepal","authors":"Subhash Chandra Shah ,&nbsp;Manish Shrestha ,&nbsp;Shilpa Aryal ,&nbsp;Amshu Shakya Bajracharya ,&nbsp;K.C. Vidhata ,&nbsp;Kul Ratna Thapa ,&nbsp;Sumit Kumar Shah ,&nbsp;Urmila Shakya","doi":"10.1016/j.ppedcard.2024.101773","DOIUrl":"10.1016/j.ppedcard.2024.101773","url":null,"abstract":"<div><h3>Background</h3><div>Although transthoracic echocardiography (TTE) is easy and more widely available with excellent acquisitions in children, there is limited evidence regarding its use in guiding transcatheter closure of ASD.</div></div><div><h3>Objectives</h3><div>We aimed to evaluate the safety, feasibility, and outcome of transcatheter closure of ASD in children guided by TTE in combination with fluoroscopy.</div></div><div><h3>Methods</h3><div>All children aged 4–15 years who were considered for and underwent transcatheter ASD closure of ASD Secundum type under TTE and fluoroscopy guidance at the National Heart Centre in Kathmandu, Nepal, from August 2018 through May 2021, were retrospectively reviewed.</div></div><div><h3>Results</h3><div>Of the 94 children, transcatheter closure was attempted in only 89 patients, and implantation of the device was successful in all of them (100 %). The procedure was done under total intravenous anesthesia in 79.8 % of children and local anesthesia in the rest. The ASD size varied between 7 and 32 (15.2 ± 5.8) mm. ASDs were closed using the device size ranged between 10 and 38(19.2 ± 1) mm. The mean of device/patient weight and device/ASD size ratio were 82 ± 0.33 and 1.28 ± 0.25, respectively. Similarly, mean duration of the procedure and fluoroscopy was 31.2 ± 8.6 and 6.5 ± 2.8 min, respectively. Five children (5.6 %) had tiny residual shunt, which closed spontaneously, as documented by TTE within 6 months after the procedure. A new onset atrial tachycardia was detected in one child during follow up. Otherwise, we observed no major early postprocedural or late complications during follow up period of 18.4 ± 8.5 months.</div></div><div><h3>Conclusion</h3><div>TTE is a safe and feasible guiding tool in children with adequate acoustic windows for the deployment of the ASD device under fluoroscopy.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"76 ","pages":"Article 101773"},"PeriodicalIF":0.6,"publicationDate":"2024-11-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143171125","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":"Developmental screening and assessment in congenital heart disease","authors":"Kahlea Haladwala,&nbsp;Edwin Boyer,&nbsp;Ginger Llivina,&nbsp;Stephanie Anderson,&nbsp;Induja Gajendran,&nbsp;Sara Shank","doi":"10.1016/j.ppedcard.2024.101772","DOIUrl":"10.1016/j.ppedcard.2024.101772","url":null,"abstract":"<div><h3>Background</h3><div>Congenital heart disease is a wide category of structural and functional heart abnormalities present at birth, including defects in the cardiac muscle, septa, valves, arteries, or veins. Patients with congenital heart disease are at an increased risk of developing adverse neurodevelopmental outcomes.</div></div><div><h3>Aim of review</h3><div>The purpose of this article is to review developmental screening and evaluation along with neurodevelopmental outcomes associated with congenital heart disease, including risk factors and genetic syndromes.</div></div><div><h3>Key scientific concepts of review</h3><div>Congenital heart disease is associated with adverse neurodevelopmental outcomes in various domains, including motor, language, cognitive, and academic abilities. Risk factors for neurodevelopmental delay may include abnormal fetal oxygen delivery, prematurity, low birth weight, cyanosis, surgical intervention, and genetic factors. Genetic syndromes associated with congenital heart disease and neurodevelopmental disabilities include Down (trisomy 21), Turner, 22q11.2 deletion, Williams, CHARGE, Noonan, Alagille, and Kabuki syndromes. Developmental screening with a standardized tool may identify the risk of abnormal development. All children receive general developmental screening at the 9-, 18-, and 30-month visits and autism-specific screening at 18- and 24-month visits. General developmental screening tests address multiple developmental domains, whereas other screening tests may focus on specific conditions such as autism or developmental domains, including speech and language. Developmental evaluation with standardized testing and rating scales by a qualified professional is recommended for children with congenital heart disease who are at high risk of neurodevelopmental sequelae. Clinical practice in cardiac developmental centers is highly varied, with most resources used for evaluation of children between birth and age 5 years. The need for early therapeutic intervention for high-risk pediatric patients with congenital heart disease supports early referral for evaluation and treatment. In high-risk school-aged patients, developmental evaluation may improve access to academic services, an individualized education plan, small group academic instruction, and instructional supports.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"76 ","pages":"Article 101772"},"PeriodicalIF":0.6,"publicationDate":"2024-11-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143171150","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":"Supraventricular tachycardia in children","authors":"Zoha Nizami ,&nbsp;Phoebe Garcia ,&nbsp;Paras Ahuja ,&nbsp;Aaron James Nipper ,&nbsp;Sachi Patel ,&nbsp;Hridhay Sheth ,&nbsp;Induja Gajendran ,&nbsp;Reshvinder Dhillon","doi":"10.1016/j.ppedcard.2024.101771","DOIUrl":"10.1016/j.ppedcard.2024.101771","url":null,"abstract":"<div><h3>Background</h3><div>Supraventricular tachycardia (SVT) affects 1 in 500 children and is characterized by rapid heart rate originating from the atrial tissue above the atrioventricular node and interventricular septum.</div></div><div><h3>Aim of review</h3><div>The purpose of this article is to review the etiology, pathophysiology, types, clinical presentation, diagnosis, and treatment of SVT in children.</div></div><div><h3>Key scientific concepts of review</h3><div>SVT results from reentry circuits, abnormal automaticity, or triggered activity. Contributing factors include congenital heart defects, electrolyte imbalances, and genetic predisposition. The types of SVT include atrioventricular nodal reentrant tachycardia, atrioventricular reentrant tachycardia, atrial tachycardia, and junctional ectopic tachycardia. Infants with SVT may present with poor feeding, vomiting, irritability, increased sleepiness, syncope, or diaphoresis. Toddlers and school-aged children may experience palpitations, chest pain, dizziness, shortness of breath, or syncope. Diagnostic tests include the electrocardiogram, Holter monitor, exercise stress test, and electrophysiologic study. Acute treatment options include vagal maneuvers, pharmacologic cardioversion, and electrical cardioversion. Long-term treatment options include antiarrhythmic drugs, catheter ablation, and surgical treatment. Complications of SVT include hemodynamic instability, thromboembolic events, congestive heart failure, exercise limitation, and decreased quality of life. Special considerations include missed diagnosis in neonates and infants, the association of SVT with congenital heart disease, and transition of care from pediatric to adult cardiology. Future directions and research may include advancements in genetic and molecular biomarkers and ablation methods. It is important to provide education and counseling to patients and their families, including information about the condition, treatment options, potential complications, and psychological support.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"76 ","pages":"Article 101771"},"PeriodicalIF":0.6,"publicationDate":"2024-11-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143170222","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":"Indications for cardiac anesthesia in children","authors":"Karen S. Bender ,&nbsp;Ryan Ford ,&nbsp;Noel Godang ,&nbsp;Connor Posey ,&nbsp;Chase Smith ,&nbsp;Gul Dadlani","doi":"10.1016/j.ppedcard.2024.101764","DOIUrl":"10.1016/j.ppedcard.2024.101764","url":null,"abstract":"<div><h3>Background</h3><div>Children with congenital and acquired heart disease are at high risk for developing anesthesia-related cardiac arrest. Children with single ventricle physiology, left ventricular outflow tract obstruction, including Williams syndrome, cardiomyopathy, and pulmonary hypertension are at the highest risk for developing anesthesia-related cardiac arrest.</div></div><div><h3>Aim of review</h3><div>The purpose of this article is to review anesthesia in children with cardiovascular diseases, factors associated with anesthesia-related cardiac arrest, and treatment to decrease anesthesia-related mortality.</div></div><div><h3>Key scientific concepts of review</h3><div>Children with congenital heart disease have fewer complications and lower mortality when the anesthesiologist has specialized training and experience in pediatric cardiac anesthesia. Comprehensive evaluation before anesthesia includes a review of the patient, planned procedure, risks, and interventions for risk reduction. Admission for initiation of intravenous fluids at the start of fasting may be advised, potentially preventing risks associated with decreased preload from fasting. The anesthetic plan includes selection of agents and monitoring for induction, maintenance, emergence, and postanesthesia care. Patients with single ventricle physiology may require adjustments of pulmonary and systemic vascular resistance to optimize pulmonary and systemic blood flow. Left ventricular outflow tract obstruction may be subvalvular, valvular, or supravalvular, static or dynamic, and associated with an increased risk of perioperative cardiac events, including arrhythmias, myocardial ischemia, and heart failure. Patients with Williams syndrome may have supravalvular aortic stenosis, pulmonary artery stenosis, biventricular outflow tract disease, or coronary artery abnormalities; anesthesia typically includes intravenous induction and strategies to minimize blood pressure variation and tachycardia. In patients with pulmonary hypertension crisis under anesthesia, prompt treatment includes mild hyperventilation with 100 % oxygen and initiation of nitric oxide. Multidisciplinary collaboration between specialists, including anesthesiologists, cardiologists, surgeons, radiologists, and interventional specialists, may facilitate the development of the safest possible anesthetic plans.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"76 ","pages":"Article 101764"},"PeriodicalIF":0.6,"publicationDate":"2024-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143170223","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":"Treatment of patent foramen ovale in children and adolescents","authors":"Eva Nunlist ,&nbsp;Bijay Shrestha ,&nbsp;Eru Sujakhu","doi":"10.1016/j.ppedcard.2024.101770","DOIUrl":"10.1016/j.ppedcard.2024.101770","url":null,"abstract":"<div><h3>Background</h3><div>Patent foramen ovale is a fetal communication between the atria that is caused by incompetence of the valve of the oval fossa. It is a common incidental finding on echocardiography in about 25 % of adults.</div></div><div><h3>Aim of review</h3><div>The purpose of this article is to review patent foramen ovale in children and adolescents, associated clinical conditions, and treatment options.</div></div><div><h3>Key scientific concepts of review</h3><div>Patent foramen ovale can be associated with a range of morbidities, including migraine headaches, cryptogenic ischemic stroke, transient ischemic attack, paradoxical embolus, syncope, decompression sickness, and platypnea-orthodeoxia syndrome (a rare condition leading to dyspnea and hypoxemia when standing or sitting upright). Notably, a patent foramen ovale is more prevalent in patients having migraines with aura (50 %) than those without aura (27 %). Closure of patent foramen ovale has been observed to reduce the median number of migraine days, although it does not impact overall headache frequency. Patent foramen ovale, as a cardiac anomaly, is found in a significant proportion of pediatric stroke cases due to its potential for paradoxical emboli shunting to the brain. In the pediatric population, percutaneous patent foramen ovale closure is safe and, when combined with antiplatelet therapy, effectively reduces the risk of new brain infarcts and stroke recurrence. Young patients with sickle cell disease and patent foramen ovale have an increased risk of bleeding while on anticoagulation therapy; hence, transcatheter patent foramen ovale closure is preferable to prevent neurological sequelae. Additionally, there is evidence showing a four-fold higher frequency of syncope in patients with patent foramen ovale, suggesting a significant association. The 2020 American Academy of Neurology Practice Advisory recommends patent foramen ovale closure for secondary stroke prevention in patients under 60 with embolic strokes of unknown etiology. When clinically indicated, patent foramen ovale closure is a feasible and safe intervention in children and adolescents, promising to reduce stroke-related morbidity.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"76 ","pages":"Article 101770"},"PeriodicalIF":0.6,"publicationDate":"2024-11-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143170224","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":"Infantile scimitar syndrome with severe pulmonary hypertension with novel 3p26 microdeletion/12q23–24 microduplication: Case report and literature review","authors":"Junpei Kawamura ,&nbsp;Yoshihiro Takahashi ,&nbsp;Koji Nakae ,&nbsp;Kentaro Ueno ,&nbsp;Yukiko Tazaki ,&nbsp;Ikeda Toshiro ,&nbsp;Yasuhiro Okamoto","doi":"10.1016/j.ppedcard.2024.101768","DOIUrl":"10.1016/j.ppedcard.2024.101768","url":null,"abstract":"<div><div>Infantile scimitar syndrome is a rare type of partial pulmonary venous return that is sometimes complicated by severe pulmonary hypertension, which has a poor prognosis. As the genetic background of Scimitar syndrome remains unclear, we report a case of an infant with scimitar syndrome that was analyzed using a chromosomal microarray. Fetal echocardiography revealed an abnormal return of the entire right pulmonary vein to the inferior vena cava. The patient presented with abnormal physical features and a history of atrial septal defects, multiple muscular ventricular septal defects, chronic pleural chylothorax, and hypertrophic pyloric stenosis. The patient died at the age of 3 months despite receiving multidisciplinary treatment with nitric oxide and a pulmonary vasodilator for severe pulmonary hypertension. Chromosomal microarray analysis revealed a copy number loss of 3p.26.1–26.3 (6.5 Mb), a copy number gain of 12q23.2–24.3 (30.8 Mb), and a determined karyotype of 46, XY, der (3) t (3;12) (p26.1;q23.2), arr [grch37] 3p26.1p26.3(62,199_6,541,934) x1,12q23.2q24.3(102,869,918_133,747,247) x3. We report a case of multiple malformations, including Scimitar syndrome and severe pulmonary hypertension with a novel unbalanced translocation involving a 3p26.1–26.3 microdeletion and a 12q23.2–24.3 microduplication. The relationship between unbalanced translocations and the phenotype and prognosis of scimitar syndrome requires further investigation.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"76 ","pages":"Article 101768"},"PeriodicalIF":0.6,"publicationDate":"2024-11-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143171116","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 novel approach to thrombectomy and catheter directed tissue-type plasminogen activator in a toddler post-fontan","authors":"Vaishnavi Divya Nagarajan ,&nbsp;Laura A. Miller-Smith ,&nbsp;Yoshi O. Otaki ,&nbsp;Ashok Muralidaran ,&nbsp;Grant H. Burch ,&nbsp;Laurie B. Armsby ,&nbsp;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}