Lisette M Harteveld, Nico A Blom, J Gert van Dijk, Robert H A M Reijntjes, Paul J van Someren, Fabian I Kerkhof, Irene M Kuipers, Lukas A J Rammeloo, Arend D J Ten Harkel
{"title":"Fluid responsiveness of ambulatory paediatric patients with a Fontan circulation by passive leg raising.","authors":"Lisette M Harteveld, Nico A Blom, J Gert van Dijk, Robert H A M Reijntjes, Paul J van Someren, Fabian I Kerkhof, Irene M Kuipers, Lukas A J Rammeloo, Arend D J Ten Harkel","doi":"10.1017/S1047951125001209","DOIUrl":"https://doi.org/10.1017/S1047951125001209","url":null,"abstract":"<p><strong>Background: </strong>Passive leg raising is used to predict who will benefit from fluid therapy in critically ill patients, including children. Patients with a Fontan circulation may have a different haemodynamic response to a fluid challenge by passive leg raising.</p><p><strong>Methods: </strong>The haemodynamic response of 31 paediatric patients with a Fontan circulation from the outpatient clinic (median age 14.0 years) and 35 healthy controls (median age 12.8 years) to passive leg raising was evaluated non-invasively by echocardiography for the assessment of, e.g., velocity time integral across the (neo)aortic valve, blood pressure measurements, and respiration. Participants were considered responders when the velocity time integral increased ≥ 10.0%.</p><p><strong>Results: </strong>Overall, patients and controls did not differ in the haemodynamic response. Twelve patients (38.7%) and 8 controls (22.9%) were responders, which was not statistically different (<i>P</i> = 0.22). Responders in the patient and control group also had a similar echo-estimated velocity time integral increase of + 18.9% and + 15.2%, respectively (<i>P</i> = 0.91). There was no difference in echo-estimated velocity time integral change between patient and control non-responders with a decrease of -1.4% and -6.4%, respectively (<i>P</i> = 0.70) and no difference in the amount of patients who were negatively affected by passive leg raising, ith a decrease of ≤-10.0% in 7 patients (22.6%) and 9 controls (25.7%)(<i>P</i> = 0.77).</p><p><strong>Conclusion: </strong>The haemodynamic response of ambulatory paediatric patients with a Fontan circulation to passive leg raising is like that of healthy controls. Patients who did not respond were similarly affected as healthy controls. Whether the haemodynamic response is different in critically ill patients warrants further investigation.</p><p><strong>Trail registration: </strong>The Netherlands National Trial Register (NTR), Trial: NL6415; date of registration 20-07-2017; Trial information: https://www.trialregister.nl/trial/6415.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-6"},"PeriodicalIF":0.9,"publicationDate":"2025-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143647359","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Patent ductus arteriosus rupture during percutaneous device closure: a case report.","authors":"Hyun Ho Kim, Jihye You","doi":"10.1017/S1047951125001313","DOIUrl":"https://doi.org/10.1017/S1047951125001313","url":null,"abstract":"<p><p>Patent ductus arteriosus is a common condition in preterm neonates, often necessitating medical or surgical intervention. This report presents a case of a preterm neonate born at 27 gestational weeks and who experienced patent ductus arteriosus rupture during a device closure procedure. Patent ductus arteriosus rupturing during device closure is rare and life threatening but can be successfully managed with prompt recognition and intervention.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.9,"publicationDate":"2025-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143647369","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Pulmonary vasodilator-induced haemodynamic decline and recovery post-discontinuation in two single-ventricle cases.","authors":"Yuichi Ishikawa, Yuki Iwaya, Ayako Kuraoka","doi":"10.1017/S1047951125000538","DOIUrl":"https://doi.org/10.1017/S1047951125000538","url":null,"abstract":"<p><p>Pulmonary vasodilators increased the systemic-to-pulmonary collateral flow in two single-ventricle cases, causing haemodynamic decline. Discontinuation reversed the collateral flow and improved heart failure, highlighting the need for careful monitoring. Serial cardiac MRI, as a non-invasive tool, may be essential to detect changes in collateral flow and guide vasodilator use for optimal management in this population.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.9,"publicationDate":"2025-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143584763","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Musa Öztürk, Merve Tanrısever Türk, Pelin Özlem Şimşek Kiper, Hayrettin Hakan Aykan
{"title":"Extremely rare association: Desbuquois dysplasia type 1 with coronary-cameral fistula.","authors":"Musa Öztürk, Merve Tanrısever Türk, Pelin Özlem Şimşek Kiper, Hayrettin Hakan Aykan","doi":"10.1017/S1047951125000551","DOIUrl":"https://doi.org/10.1017/S1047951125000551","url":null,"abstract":"<p><strong>Purpose: </strong>Desbuquois dysplasia type 1 is a rare autosomal recessive chondrodysplasia characterised by distinct skeletal abnormalities and multisystem involvement, including pulmonary, renal, and ocular abnormalities, has also been reported. Cardiac complications, although infrequently discussed in the literature, include aortopathy and atrioventricular valve prolapse, potentially due to defective proteoglycan production.</p><p><strong>Case report: </strong>This case report details a 7-year-old male diagnosed with Desbuquois dysplasia type 1 and a coronary-cameral fistula, both of which are exceedingly rare conditions. Genetic analysis revealed a previously reported homozygous pathogenic variant in the calcium-activated nucleotidase 1 gene, ENST00000c.898C>T; p.Arg300Cys. Echocardiographic findings indicated significant cardiac enlargement, mitral valve prolapse, coronary-cameral fistula, pulmonary hypertension, advanced aortic root enlargement and aneurysmatic ascending aorta, and atrial septal defect, necessitating careful clinical management.</p><p><strong>Conclusion: </strong>This case underscores the complexity of Desbuquois dysplasia and its associated cardiac anomalies, highlighting the need for further research into the systemic implications of this disorder. To the best of our knowledge, this case has importance as it is the first of its kind in the literature.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.9,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143482262","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Successful embolisation of coronary artery fistulas with the Azur® CX peripheral coil system in three paediatric patients.","authors":"Emine Gulsah Torun, Utku Pamuk, Velihan Çayhan, Hazım Alper Gürsu","doi":"10.1017/S1047951125000447","DOIUrl":"https://doi.org/10.1017/S1047951125000447","url":null,"abstract":"<p><p>Coronary artery fistulas are rare cardiac anomalies, often asymptomatic and frequently discovered incidentally during routine cardiac imaging. Transcatheter closure with coil embolisation is increasingly recognised as a preferable alternative to surgical intervention due to its lower complication rates. In this report, we present three paediatric cases in which coronary artery fistulas were successfully embolised using the Azur® CX Peripheral Coil System. This system demonstrates low-profile suitability for occluding highly tortuous vessels in children, enabling controlled coil delivery via a microcatheter. Its hydrogel-coated coils allow for expansion between gaps, making it highly effective as a minimally invasive treatment.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-4"},"PeriodicalIF":0.9,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143482275","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mehtap Küçük, İlkay Erdoğan, Birgül Varan, N Kürşad Tokel
{"title":"Use of ivabradine in tachycardia due to hyperthyroidism in a child: a case report and literature review.","authors":"Mehtap Küçük, İlkay Erdoğan, Birgül Varan, N Kürşad Tokel","doi":"10.1017/S1047951125000502","DOIUrl":"https://doi.org/10.1017/S1047951125000502","url":null,"abstract":"<p><p>Sinus tachycardia due to hyperthyroidism is generally treated with beta-blockers. But some patients do not respond to beta-blockers or may have non-tolerable side effects or have contraindications. We presented a case with persistent sinus tachycardia secondary to hyperthyroidism refractory to maximal doses of propranolol. After ivabradine treatment, her heart rate was < 100 bpm within 24 hours. There were no electrocardiogram changes or side effects. The use of ivabradine is promising and can be considered in cases where tachycardia cannot be controlled in children with hyperthyroidism.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-4"},"PeriodicalIF":0.9,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143482278","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Late presentation of distal aortopulmonary window with anomalous origin of right coronary artery from main pulmonary artery with isolated left subclavian artery.","authors":"Anish Gupta, Abisho R Starlet, Yash Shrivastava","doi":"10.1017/S1047951125000563","DOIUrl":"https://doi.org/10.1017/S1047951125000563","url":null,"abstract":"<p><p>We present the case of a 9-year-old girl with a distal aortopulmonary window (APW) with anomalous origin of right coronary artery from the main pulmonary artery and an isolated left subclavian artery arising from the main pulmonary artery. This report highlights the unusual combination of the three anomalies and late presentation of APW.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-4"},"PeriodicalIF":0.9,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143482270","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Muhammad I Prayudijanto, Suprohaita R Talib, Syarif Rohimi, Winda Azwani, Pandu C Lestari, Citra Raditha, Anna Tjandrajani, Rizky Adriansyah, Najib Advani
{"title":"Incomplete Kawasaki disease with peripheral facial nerve palsy and giant coronary artery aneurysm: rare case report.","authors":"Muhammad I Prayudijanto, Suprohaita R Talib, Syarif Rohimi, Winda Azwani, Pandu C Lestari, Citra Raditha, Anna Tjandrajani, Rizky Adriansyah, Najib Advani","doi":"10.1017/S1047951124036667","DOIUrl":"https://doi.org/10.1017/S1047951124036667","url":null,"abstract":"<p><p>Kawasaki disease is an idiopathic vasculitis that involves the participation of various physiological systems and the inflammation of small-to medium-sized arteries, resulting in the formation of aneurysms. It most commonly affects children under 5 years. The development of coronary artery aneurysms is a significant concern in the context of Kawasaki disease, rendering it the prevailing acquired cardiac condition among children. It has been observed that 1-30% of Kawasaki illness patients experience neurological complications. However, facial nerve palsy is uncommon, with a prevalence of only 0.9%. Infants with Kawasaki disease and facial nerve palsy tend to be under 6 months of age and had a higher chance to develop coronary artery aneurysms compared to those without facial nerve palsy whose condition was never treated.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-4"},"PeriodicalIF":0.9,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143448245","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Bilgehan B Biçer, Hayrettin Hakan Aykan, Şafak Alpat, Mustafa Yılmaz
{"title":"Multiple papillary fibroelastoma presenting with mitral, tricuspid, and pulmonary valve involvement and surgical treatment: case report.","authors":"Bilgehan B Biçer, Hayrettin Hakan Aykan, Şafak Alpat, Mustafa Yılmaz","doi":"10.1017/S1047951124036503","DOIUrl":"https://doi.org/10.1017/S1047951124036503","url":null,"abstract":"<p><p>Approximately 90% of primary paediatric cardiac tumours are benign lesions. Depending on their location and size, benign cardiac tumours may cause inflow and outflow obstructions, cyanosis, valvular insufficiencies, myocardial ischaemia, associated dysfunction, systemic embolisation, arrhythmias, and even sudden death. Decision-making and timing for surgery can be challenging in children. Herein, we present an asymptomatic 11-year-old girl with papillary fibroelastoma in the mitral, tricuspid, and pulmonary valves, discussing the decision-making process and successful surgical management.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-4"},"PeriodicalIF":0.9,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143448292","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Kawasaki disease: the most common cause of acquired heart disease among children globally.","authors":"Rakesh Kumar Pilania, Adriana H Tremoulet, Shankar Prinja, Nagib Dahdah, Surjit Singh","doi":"10.1017/S1047951125000459","DOIUrl":"https://doi.org/10.1017/S1047951125000459","url":null,"abstract":"<p><p>Kawasaki disease is a childhood vasculitic disorder that has a special predilection for coronary arteries. Kawasaki disease has been reported from all regions of the world, with an increasing incidence in several countries. Kawasaki disease is now the most common cause of acquired heart disease in children all over the world. However, it is concerning that the estimated vast majority of Kawasaki disease cases in low- and middle-income countries are not getting diagnosed and treated. The World Health Organization acknowledges cardiovascular disease in their priority of actions. The World Health Organization is invited to acknowledge the reality of Kawasaki disease in its list of cardiovascular diseases and take steps to facilitate the diagnosis and treatment of Kawasaki disease, especially in low- and middle-income countries. It is a disease of public health importance and needs urgent prioritisation by the World Health Organization.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.9,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143448289","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}