{"title":"Investigating the possible connection between cystic hygroma, nuchal translucency, and the genetic link underlying cardiac lymphatic abnormalities and congenital heart disease: is there an association?","authors":"Marios Loukas, Ishank Gupta, Brenna Wilson, Shamfa Joseph, Rachel Levene, Michael Montalbano","doi":"10.1017/S1047951125001404","DOIUrl":"https://doi.org/10.1017/S1047951125001404","url":null,"abstract":"<p><p>Although a direct link between cardiac lymphatics and congenital heart disease (CHD) has not been established, research indicates that abnormalities in the cardiac lymphatic system are involved in several congenital disorders that present with cardiac defects. However, this contribution remains poorly understood and is still an emerging area of study. One theory proposes that abnormal lymphatic development, which can present as cystic hygroma or increased nuchal translucency, may give rise to heart defects such as coarctation of the aorta, hypoplastic left heart syndrome, or bicuspid aortic valves. The genetic pathways for the development of the cardiac lymphatic system and the heart's major vessels may overlap; therefore, mutations in these genes could result in simultaneous defects in both systems. The close anatomical proximity between cardiac lymphatics and the great vessels of the heart suggests a \"cause and effect\" relationship, where an abnormality in one could affect the other and lead to congenital defects. Given that congenital heart disease is the most common birth defect in the United States, this systematic review, following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, seeks to explore the potential link between cardiac lymphatics and CHD. Understanding this connection could have significant clinical implications by paving the way for new diagnostic approaches and therapeutic strategies for CHDs.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-7"},"PeriodicalIF":0.9,"publicationDate":"2025-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143794608","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}
Martina Larsson, Mats Synnergren, Thushara Rodrigo
{"title":"Case report: morphological challenges to surgical repair in complete atrioventricular septal defect with isolated ventricular component.","authors":"Martina Larsson, Mats Synnergren, Thushara Rodrigo","doi":"10.1017/S1047951125001441","DOIUrl":"https://doi.org/10.1017/S1047951125001441","url":null,"abstract":"<p><p>We describe a trisomy 21 patient with postnatal diagnosis of atrioventricular septal defect with isolated ventricular component who had a complicated post-surgical course following complete repair. Clinical outcomes included moderate-severe residual atrioventricular valve regurgitation needing re-operation, complete heart block leading to pacemaker insertion, seizures secondary to subdural haemorrhages, and chylothorax. We describe the surgical considerations specific to this cardiac morphology.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.9,"publicationDate":"2025-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143762899","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}
Vincent Bui, Silvana Molossi, Lindsay F Eilers, Shagun Sachdeva, Tam T Doan
{"title":"Mitral annular disjunction versus myocardial Ischemia in coronary artery anomalies.","authors":"Vincent Bui, Silvana Molossi, Lindsay F Eilers, Shagun Sachdeva, Tam T Doan","doi":"10.1017/S1047951125001465","DOIUrl":"https://doi.org/10.1017/S1047951125001465","url":null,"abstract":"<p><p>Both mitral annular disjunction and coronary artery anomalies are associated with ventricular arrhythmia, though data on their co-occurrence are limited. We herein present two patients with anomalous aortic origin of a coronary artery and mitral annular disjunction who had ventricular arrhythmia. Mitral annular disjunction should be considered as a potential cause of ventricular arrhythmia when stress tests rule out ischaemia.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1-3"},"PeriodicalIF":0.9,"publicationDate":"2025-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143762901","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}
Cardiology in the YoungPub Date : 2025-04-01Epub Date: 2025-02-27DOI: 10.1017/S104795112500054X
Furkan Donbaloğlu, Vehbi Doğan, Serpil Kaya Çelebi, Meryem Beyazal, İlker U Sayıcı, Zeynep Donbaloğlu
{"title":"Aetiological distribution and clinical features in children with large pericardial effusion who underwent pericardiocentesis.","authors":"Furkan Donbaloğlu, Vehbi Doğan, Serpil Kaya Çelebi, Meryem Beyazal, İlker U Sayıcı, Zeynep Donbaloğlu","doi":"10.1017/S104795112500054X","DOIUrl":"10.1017/S104795112500054X","url":null,"abstract":"<p><strong>Background: </strong>We aimed to evaluate the clinical and laboratory characteristics and aetiological factors of patients who underwent pericardiocentesis for moderate to large pericardial effusion.</p><p><strong>Method: </strong>A total of 38 patients who underwent pericardiocentesis due to moderate-severe pericardial effusion and not related to cardiac surgery were included in the study.</p><p><strong>Results: </strong>The male-to-female ratio was 2.16, and found to be 7.5 in patients over 3 years of age. Mean age and body weight of the patients were 69.4 ± 74.9 months and 22.5 ± 22.4 kg. Dyspnoea (51.7%) was the most common complaint, followed by chest pain (37.9%). Tamponade was present in 23.7% of the patients. The largest diameter of effusion was 24.4 ± 10.4 mm. The amount of fluid drained was 279.24 ± 279 ml. Macroscopic appearance was serous in 12 (34.3%), and haemorrhagic in 18 (51.4%). No complication related to procedure was seen. Aetiology for efusion was infectious in 26%, idiopathic in 18%, iatrogenic in 11%, rheumatological in 11%, malignancy in 8%, cardiomyopathy in 8%, and other factors related in 18%. Of the 38 patients, 16 received nonsteroidal anti-inflammatory drugs (NSAID), and colchicine and corticostreoid were added in nine and two patients, respectively. A total of eight (21%) patients died during follow-up.</p><p><strong>Conclusion: </strong>In conclusion, percutaneous pericardiocentesis can be applied safely and the underlying aetiology is decisive in the prognosis of the patient. Although pericardial effusion in children is often due to inflammation of the pericardium, it can develop as a finding of many local or systemic diseases that should be kept in mind.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"784-790"},"PeriodicalIF":0.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143514811","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}
Cardiology in the YoungPub Date : 2025-04-01Epub Date: 2025-02-27DOI: 10.1017/S1047951125001271
Amy L Kiskaddon, Nhue L Do, Ernest K Amankwah, Daniel M Witt, Vera Ignjatovic, Therese M Giglia, Gary M Woods, Hilary B Whitworth, Arabela C Stock, Neil A Goldenberg
{"title":"Arterial thromboembolism, antithrombotic therapy, and risk of recurrent thromboembolism in children with CHD undergoing cardiac surgery.","authors":"Amy L Kiskaddon, Nhue L Do, Ernest K Amankwah, Daniel M Witt, Vera Ignjatovic, Therese M Giglia, Gary M Woods, Hilary B Whitworth, Arabela C Stock, Neil A Goldenberg","doi":"10.1017/S1047951125001271","DOIUrl":"10.1017/S1047951125001271","url":null,"abstract":"<p><strong>Introduction: </strong>Data on arterial thromboembolism in children undergoing cardiac surgery are limited. We sought to characterise, and estimate rates of, incident and recurrent arterial thromboembolism, and describe antithrombotic therapies for treatment in a large multinational population of children with CHD undergoing cardiac surgery.</p><p><strong>Methods: </strong>We queried the TriNetX global electronic health record (derived real-world data research platform) from 2017 to 2024 for patients less than 18 years of age and an index arterial thromboembolism within 1 year of congenital cardiac surgery. Data were descriptively analysed.</p><p><strong>Results: </strong>Of 20,102 children who underwent an index cardiac surgery for CHD, 206 (1.1%) developed an index arterial thromboembolism within 1 year of surgery: 111 (53.9%) had only arterial thromboembolism and 95 (46.1%) had concomitant venous thromboembolism. The most common anatomic site for arterial thromboembolism was the lower extremity (<i>n</i> = 141, 68.4%), and the most common surgery was the Glenn procedure (<i>n</i> = 35, 17%). Unfractionated heparin was utilised in 136 (67 %) and aspirin in 91 (44.2%) patients. Recurrent thromboembolism occurred in 36 (17.5%) patients within 1 year of the index thromboembolism.</p><p><strong>Conclusions: </strong>Among children undergoing congenital cardiac surgery, arterial thromboembolism was rare (1% of patients), but the 1-year risk of recurrent thromboembolism was high, at 17.5%. Multicentre prospective cohort studies are warranted to further evaluate risk factors for recurrent thromboembolism, to facilitate future risk-stratified interventional trials designed to reduce the high thromboembolism recurrence risk in these children.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"679-682"},"PeriodicalIF":0.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143514815","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}
Cardiology in the YoungPub Date : 2025-04-01Epub Date: 2025-02-27DOI: 10.1017/S1047951125000460
Andrey Semyashkin, Julia Nesteruk, Dimitra Giannikopouloui, Michael Scheid, Gleb Tarusinov, Aktam Tannous, Marcel Te Vrugt, Lotfi Ben Mime
{"title":"Pulmonary artery banding for dilated and depressed left ventricle: dilated cardiomyopathy versus left ventricular non-compaction cardiomyopathy.","authors":"Andrey Semyashkin, Julia Nesteruk, Dimitra Giannikopouloui, Michael Scheid, Gleb Tarusinov, Aktam Tannous, Marcel Te Vrugt, Lotfi Ben Mime","doi":"10.1017/S1047951125000460","DOIUrl":"10.1017/S1047951125000460","url":null,"abstract":"<p><strong>Objectives: </strong>To retrospectively assess the suitability of pulmonary artery banding as a treatment strategy for dilated cardiomyopathy and left ventricular non-compaction cardiomyopathy with depressed left ventricular ejection fraction.</p><p><strong>Methods: </strong>The study was retrospective and included consecutive patients who met the inclusion criteria: diagnosed with dilated cardiomyopathy or left ventricular non-compaction cardiomyopathy and left ventricular ejection fraction less than 35%. Cardiac indices were documented, and clinical outcomes were followed for 5 years.</p><p><strong>Results: </strong>This study included 21 patients with depressed left ventricular ejection fraction due to dilated cardiomyopathy (n = 11) or left ventricular non-compaction cardiomyopathy (n = 10), treated either with anti-congestion medication alone or in combination with pulmonary artery banding. The groups treated with pulmonary artery banding showed significant improvement in left ventricular ejection fraction compared to controls (ANOVA, <i>p</i> = 0.0002), with no major adverse events. In the subgroup with left ventricular non-compaction, pulmonary artery banding led to significant improvement of the left ventricular ejection fraction (<i>p</i> = 0.00002) and significant reductions in the Z scores of left ventricular end-diastolic diameter (<i>p</i> = 0.0002) and of end-diastolic volume (<i>p</i> = 0.004).</p><p><strong>Conclusions: </strong>Pulmonary artery banding appears to be a viable strategy for improving heart function in patients with non-compaction and dilated cardiomyopathy and depressed left ventricular ejection fraction. While pulmonary artery banding demonstrated more pronounced benefits in the subgroup with non-compaction cardiomyopathy, significantly enhancing cardiac restoration indices throughout the follow-up period, warranting further investigation in larger studies.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"717-721"},"PeriodicalIF":0.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143514841","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}
Cardiology in the YoungPub Date : 2025-04-01Epub Date: 2025-03-12DOI: 10.1017/S1047951125000216
Ying Lyu, Hongyan Lyu, Xinxin Lyu
{"title":"A case of protein-losing enteropathy secondary to constrictive pericarditis in a 3-year-old boy: a rare case report.","authors":"Ying Lyu, Hongyan Lyu, Xinxin Lyu","doi":"10.1017/S1047951125000216","DOIUrl":"10.1017/S1047951125000216","url":null,"abstract":"<p><p>This article presents the case of a 3-year-old boy whose primary symptom was hypoproteinemia. After evaluations at multiple hospitals, he was sequentially diagnosed with protein-losing enteropathy and constrictive pericarditis. Following pericardiectomy, his albumin levels returned to normal. Additionally, a review of several similar cases is provided, with the aim of aiding in the diagnosis of related conditions.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"863-865"},"PeriodicalIF":0.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143603855","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}
Cardiology in the YoungPub Date : 2025-04-01Epub Date: 2025-03-10DOI: 10.1017/S1047951125000599
Casey N Bor, Gyasi Moscou-Jackson, Christopher W Mastropietro, Sandra L Staveski
{"title":"Wake-up call: paediatric cardiac intensive care nurse and advanced practice provider well-being and intent to vacate.","authors":"Casey N Bor, Gyasi Moscou-Jackson, Christopher W Mastropietro, Sandra L Staveski","doi":"10.1017/S1047951125000599","DOIUrl":"10.1017/S1047951125000599","url":null,"abstract":"<p><p>This study aimed to assess Well-Being Index scores in paediatric cardiac ICU (PCICU) registered nurses and advanced practice providers. Secondary objectives included identifying factors correlating with at-risk Well-Being Index scores and exploring predictors of these scores, with attention to the impact of the coronavirus disease 2019 pandemic. A multicentre electronic survey was conducted between October 2021 and January 2022. Registered nurses and advanced practice providers working in PCICUs at US centres participating in the Collaborative Research from the Pediatric Cardiac Intensive Care Society were included.The survey included the nine-item Well-Being Index and questions about demographics and factors influencing well-being, such as coronavirus disease 2019. The Well-Being Index is a validated tool to predict workforce distress and well-being. Out of 218 participants (180 registered nurses, 38 advanced practice providers), 137 registered nurses (76%) and 15 advanced practice providers (39%) had at-risk Well-Being Index scores. A total of 61% of nurses and 34% of advanced practice providers reported an intent to leave. Intent to leave was significantly linked to lower well-being for registered nurses (<i>p</i> = 0.002). Leadership support reduced registered nurses' distress risk by 68% compared to no support (<i>p</i> = 0.04). Increased stress since coronavirus disease 2019 raised registered nurses' poor well-being risk by four times (<i>p</i> = 0.001). PCICU registered nurses and advanced practice providers are at risk for poor well-being, exacerbated by the pandemic. Those with poor well-being may be more likely to leave. Leadership support is vital for nurses' well-being. Further research is needed to establish baseline well-being.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"661-667"},"PeriodicalIF":0.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143584766","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}
Cardiology in the YoungPub Date : 2025-04-01Epub Date: 2025-03-03DOI: 10.1017/S1047951125000575
Utku Pamuk, Harun Terin, Hazim Alper Gursu
{"title":"Transcatheter closure of a huge complex pulmonary arteriovenous malformation with four devices: a case report.","authors":"Utku Pamuk, Harun Terin, Hazim Alper Gursu","doi":"10.1017/S1047951125000575","DOIUrl":"10.1017/S1047951125000575","url":null,"abstract":"<p><p>Pulmonary arteriovenous malformations are abnormal vascular connections between pulmonary arteries and veins, often causing right-to-left shunting. In this report, a 4-year-old boy with low oxygen saturation was diagnosed with a large, complex pulmonary arteriovenous malformation involving four feeding arteries. Percutaneous transcatheter closure was performed using four devices to occlude the major feeding arteries, resulting in increased arterial oxygen saturation from 72 to 98%. This report depicts the successful use of multiple devices for percutaneous closure of a complex pulmonary arteriovenous malformation in a child and highlights the minimally invasive and effective nature of this approach.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"860-862"},"PeriodicalIF":0.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143536696","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}
Cardiology in the YoungPub Date : 2025-04-01Epub Date: 2025-03-13DOI: 10.1017/S1047951125001246
Sara F Morehous, Jeffrey B Anderson, Eunice Hahn, Nicholas J Ollberding, Christopher J Statile
{"title":"Performance against standardization recommendations for outpatient care of common forms of congenital heart disease.","authors":"Sara F Morehous, Jeffrey B Anderson, Eunice Hahn, Nicholas J Ollberding, Christopher J Statile","doi":"10.1017/S1047951125001246","DOIUrl":"10.1017/S1047951125001246","url":null,"abstract":"<p><strong>Background: </strong>The American College of Cardiology has published clinical practice algorithms for common congenital heart lesions, including atrial septal defect, patent ductus arteriosus, valvar pulmonary stenosis, aortic coarctation, and ventricular septal defect. The purpose of this study was to define the current practice patterns in the management of these lesions and describe the impact of departure from these recommendations.</p><p><strong>Methods: </strong>This was a retrospective analysis of the most recent 100 outpatient appointments for each lesion at our centre. Electronic medical records were queried to determine whether the scheduling, testing, and follow-up plan for each appointment were consistent with the published algorithms.</p><p><strong>Results: </strong>A total of 500 visits were evaluated (150 new visits; 350 follow-up visits); 32% (<i>n</i> = 162) of encounters did not receive appropriate testing, 37% (<i>n</i> = 186) departed from recommended follow-up plans, and of the 350 follow-up visits, 45% (<i>n</i> = 156) departed from scheduling guidelines. Impact of these departures was quantified in reference to over- or under-expenditure of clinical resources. Of the aberrant testing encounters, 60% (<i>n</i> = 97) saw too few tests. Of the deviant follow-up plans created, 74% (<i>n</i> = 138) brought patients back to clinic too soon.</p><p><strong>Conclusion: </strong>This study explores the deviation between current practice patterns and published clinical care guidelines. There is considerable variation across domain of analysis, diagnosis, and encounter type, resulting in uneven resource utilisation. Standardisation of care in these areas will improve utilisation and can be a starting point for improvement work.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"812-817"},"PeriodicalIF":0.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143623622","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}