Cardiology in the Young最新文献

{"title":"Ventriculocoronary connection with mitral stenosis/aortic stenosis hypoplastic left heart: a case report.","authors":"John Karpuk, Gabriel Solorzano, Jess Randall","doi":"10.1017/S104795112402626X","DOIUrl":"https://doi.org/10.1017/S104795112402626X","url":null,"abstract":"<p><p>Ventriculocoronary connections develop most commonly in children with mitral stenosis/aortic atresia hypoplastic left heart. These connections can lead to myocardial ischaemia and dysfunction. We report a newborn with mitral stenosis/aortic stenosis hypoplastic left heart who endured systemic ventricular injury post-Norwood, secondary to a large ventriculocoronary fistula. He was treated medically and had favourable outcomes following bidirectional Glenn procedure.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342169","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":"Evaluation of growth and feeding tolerance in infants with single-ventricle physiology receiving retrospective standard of care feeding regimens compared with protocolised formula or exclusive human milk feeding regimens.","authors":"Megan Horsley, Amiee Trauth, David S Cooper, Cynthia Blanco, Zhiqian Gao, Lindsey Justice","doi":"10.1017/S1047951124025617","DOIUrl":"https://doi.org/10.1017/S1047951124025617","url":null,"abstract":"<p><strong>Objective: </strong>Determine whether weight gain velocity (g/day) 30 days after initiating feeds following cardiac surgery and other clinical outcomes improve in infants with single-ventricle physiology fed an exclusive human milk (EHM) diet with early fortification compared to non-protocolised \"standard of care.\"</p><p><strong>Methods: </strong>This retrospective cohort study compares term infants with single-ventricle physiology who underwent neonatal surgical palliation. The retrospective control group (RCG) was fed according to non-protocolised standard of care at a single centre and was compared with infants in a previous protocolised multi-site randomised controlled trial assigned to either an EHM group or a control group (TCG). The primary outcome measure is weight gain velocity. Secondary outcomes include change in weight z-score, and incidence of feeding intolerance and necrotising enterocolitis.</p><p><strong>Results: </strong>We evaluated 45 surgically palliated neonates with single-ventricle physiology compared to the prior trial patients (EHM = 55, TCG = 52). Baseline demographics were similar between groups, except the RCG had fewer patients with hypoplastic left heart syndrome (51% vs. 77% vs. 84%, p = 0.0009). The RCG grew similarly to the TCG (7.5 g/day vs. 8.2 g/day), and both groups had significantly lower growth than the EHM group (12 g/day). Necrotising enterocolitis/suspected necrotising enterocolitis were similar in the RCG versus TCG but significantly higher in the RCG compared to the EHM group (20.5% vs. 3.6%, p = 0.033). Incidences of other morbidities were similar.</p><p><strong>Conclusions: </strong>Neonates with single-ventricle physiology have improved short-term growth and decreased risk of necrotising enterocolitis or suspected necrotising enterocolitis when receiving an EHM diet after surgical palliation compared to non-protocolised feeding with bovine formula.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342077","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":"Transcatheter occlusion of venovenous collaterals in the total cavopulmonary Fontan circulation.","authors":"David W Baker, Gajon Uthayakumaran, Ngaire Polwart, Melissa G Y Lee, Will Wilson, Julian Ayer, David Tanous, Yves D'Udekem, David S Celermajer, Clare O'Donnell, Rachael Cordina","doi":"10.1017/S1047951124025708","DOIUrl":"https://doi.org/10.1017/S1047951124025708","url":null,"abstract":"<p><strong>Background: </strong>Venovenous collaterals are abnormal connections between the systemic and pulmonary venous systems. They are commonly seen in the Fontan circulation and may lead to significant hypoxaemia. Transcatheter closure of venovenous collaterals is a potential but controversial treatment as the long-term benefits and outcomes are not well understood.</p><p><strong>Methods: </strong>This retrospective cohort study utilised data from the Australian and New Zealand Fontan Registry. Patients who underwent transcatheter venovenous collateral occlusion for hypoxemia from the year 2000 onwards were included. Atriopulmonary and Kawashima-type Fontan circulations were excluded to reflect a more contemporary Fontan cohort.</p><p><strong>Results: </strong>Nineteen patients (age 19.3 ± 7.8 years, 53% female) underwent transcatheter venovenous collateral occlusion. Compared to baseline, mean oxygen saturation was improved at latest follow-up (90.5% vs 87.0%; p = 0.003). Nine patients achieved a clinically significant response (defined as an increase of at least 5% to 90% or greater), and this was associated with lower baseline Fontan pressures (12.9 v 15.6 mmHg; p = 0.02). No heart failure hospitalisations, arrhythmia, transplant referrals, or mortality were observed during the median follow-up period of 4 years. Two patients experienced thromboembolic events and five patients underwent re-intervention.</p><p><strong>Conclusion: </strong>Transcatheter occlusion of venovenous collaterals in Fontan patients with chronic hypoxaemia resulted in a modest increase in oxygenation over a median follow-up of 4 years and longer-term prognosis did not appear to be adversely affected. Lower Fontan pressures at baseline were associated with a greater improvement in oxygenation.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342082","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":"Exploring relationships between parental stress, coping, and psychological outcomes for parents of infants with CHD.","authors":"Tríona Casey, Catherine Matthews, Marie Lavelle, Damien Kenny, David Hevey","doi":"10.1017/S104795112402568X","DOIUrl":"https://doi.org/10.1017/S104795112402568X","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to explore relationships between parental stress, coping, and outcomes for parents of infants with CHD, via observational approach reflecting domains of the Parental Stress and Resilience in CHD (PSRCHD) model.</p><p><strong>Methods: </strong>Fifty-five parents of 45 infants with CHD completed questionnaires with measures of parental stress, Problem-Focused Coping (PFC), Emotion-Focused Coping (EFC), Avoidant Coping (AC), mental health (symptoms of anxiety and symptoms of depression), post-traumatic growth (PTG) and quality of life (QoL). Demographic and infant clinical data were obtained.</p><p><strong>Results: </strong>Parental stress showed significant small to medium positive correlations with MH and PTG, but no significant correlations with QoL. EFC and AC showed significant small to medium positive correlations with MH, and medium negative correlations with parental QoL. EFC and PFC had significant small to medium correlations with PTG. PFC and AC had significant small to medium correlations with infant QoL. Hierarchical multiple regression analyses indicated that parental symptoms of anxiety, PTG, parental QoL, infant QoL were significantly predicted by models comprising of parental stress, coping styles, and clinical controls (adjusted <i>R</i>² = 13.0-47.9%, <i>p</i> range < 0.001-.048), with results for parental symptoms of depression falling marginally above significance (adjusted <i>R</i>² = 12.3%, <i>p</i> = .056).</p><p><strong>Conclusions: </strong>Parental stress, coping styles, and length of hospital stay are related to psychological outcomes in parents of infants with CHD. Future research may use the PSRCHD framework to assess mechanisms underlying CHD parents' stress and coping experiences and investigate longitudinal relationships between parental factors and parent and child outcomes.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342078","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":"Emergent veno-arterial extra-corporeal membrane oxygenator support for refractory acute myocarditis in paediatric patients.","authors":"Mimoza Maldi, Guido M Olivieri, Simone Ghiselli, Andrea Busti, Stefano M Marianeschi","doi":"10.1017/S1047951124025447","DOIUrl":"https://doi.org/10.1017/S1047951124025447","url":null,"abstract":"<p><p>Acute myocarditis leading to severe heart failure in paediatric patients is an uncommon but potentially life-threatening condition. The prompt implant of mechanical circulatory devices such as veno-arterial extra-corporeal membrane oxygenation remains the best treatment option to restore an adequate perfusion and improve patient survival in case of refractory cardiogenic shock cases. While few reports describe the in-hospital course of this dramatic disease, with an in-hospital mortality under veno-arterial extra-corporeal membrane oxygenation support around 30%, our study aims to analyse both short- and long-term outcomes after extra-corporeal membrane oxygenation implant.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342076","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":"The Kids Heart BioBank: supporting 20 years of patient care and research into CHD.","authors":"Desiree C K Hilton, Bridget R O'Malley, Andrew D Cole, Richard P Harvey, Sally L Dunwoodie, Gary F Sholler, David S Winlaw, Gillian M Blue","doi":"10.1017/S1047951124025654","DOIUrl":"https://doi.org/10.1017/S1047951124025654","url":null,"abstract":"<p><strong>Introduction: </strong>The cause of most CHD is unknown and considered complex, implicating genetic and environmental factors in disease causation. The Kids Heart BioBank was established in 2003 to accelerate genetic investigations into CHD.</p><p><strong>Methods: </strong>Recruitment includes patients undergoing interventions for CHD at The Children's Hospital at Westmead. Informed consent is obtained from parents/guardians, and blood is collected at the time of cardiac intervention from which DNA is extracted and stored. Associated detailed clinical information and a family history are stored in the purpose-designed database.</p><p><strong>Results: </strong>To date, the Kids Heart BioBank contains biospecimens and associated clinical information from over 4,900 patients with CHD and their families. Two-thirds (64.1%) of probands have been included in research studies with 28.9% of participants who underwent genomic sequencing receiving a molecular diagnosis with direct clinical utility. The value of this resource to patients and families is highlighted by the high consent rate (94.6%) and the low withdrawal of consent rate (0.4%). The Kids Heart BioBank has supported many large national and international collaborations and contributed significantly to CHD research.</p><p><strong>Conclusions: </strong>The Kids Heart BioBank is an invaluable resource and, together with other similar resources, the resulting research has paved the way for clinical genetic testing options for CHD patients, previously not possible. With research in the field moving away from diagnosing monogenic disease, the Kids Heart BioBank is ideally placed to support the next chapter of research efforts into complex disease mechanisms, requiring large patient cohorts with detailed phenotypic information.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342081","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":"Efficacy and speed of effect after the first dose of aspirin in children with congenital heart disease.","authors":"Irene E Regan, Sean T Kelleher, Dermot Cox, Colin J McMahon","doi":"10.1017/S1047951124000581","DOIUrl":"https://doi.org/10.1017/S1047951124000581","url":null,"abstract":"<p><strong>Background: </strong>Many paediatric studies report that patients must be established on aspirin therapy for a minimum of 5 days to achieve adequate response. This is not always practical especially in critical settings. Prospective identification of patients that are unresponsive to aspirin sooner could potentially prevent thrombotic events.</p><p><strong>Aims: </strong>The aim of this study was to investigate prospectively if the first dose of aspirin is effective in decreasing platelet aggregation, and thromboxane formation and if this can be measured after 2 hours in paediatric cardiology patients. A secondary aim was to identify a cut-off for a novel marker of aspirin responsiveness the maximum amplitude with arachidonic acid, which could potentially dramatically reduce the blood volume required. Third, we aimed to prospectively identify potentially non-responsive patients by spiking a sample of their blood <i>ex vivo</i> with aspirin.</p><p><strong>Results: </strong>The majority (92.3%) of patients were responsive, when measured 2 hours post first dose of aspirin. Non-response or inadequate response (7.7%) can also be identified at 2 hours after taking the first dose of aspirin. Additionally, we have shown a novel way to reduce blood sample volume requirements by measurement of the maximum amplitude with arachidonic acid as a marker of response, particularly for monitoring.</p><p><strong>Conclusions: </strong>These findings of rapid efficacy in the majority of patients offer assurance in a sound, practical way to attending clinicians, patients, and families.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342075","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":"Utilisation of a post-Fontan management pathway reduces chest tube drainage and hospital readmission rates.","authors":"Adam M Skaff, Sandra D Kikano, Jeffrey G Weiner, Genevieve E Staudt, Patrick O Maynord, David P Bichell, David A Parra","doi":"10.1017/S1047951124025861","DOIUrl":"https://doi.org/10.1017/S1047951124025861","url":null,"abstract":"<p><strong>Background: </strong>Complications following the Fontan procedure include prolonged pleural drainage and readmission for effusions. To address these complications, a post-Fontan management pathway was implemented with primary goals of reducing chest tube duration/reinsertion rates and decreasing hospital length of stay and readmissions.</p><p><strong>Methods: </strong>Fontan patients were identified by retrospective chart review (2017-2019) to obtain baseline data for chest tube duration/reinsertion rates, hospital length of stay, and readmission rates for effusion. A post-Fontan management pathway was implemented (2020-2021) utilising post-operative vasopressin, nasal cannula oxygen until chest tube removal, and discharge regimen of three times daily diuretics, sildenafil, and afterload reducing medications. Patients were followed to evaluate primary outcomes.</p><p><strong>Results: </strong>The pre- and post-pathway groups were similar in single ventricle morphology, demographics, and pre-operative haemodynamics. Forty-three and 36 patients were included in the pre- and post-pathway cohorts, respectively. There were statistically significant reductions in chest tube duration (8 vs. 5 days, <i>p</i> ≤ 0.001), chest tube output on post-operative day 4 (20.4 vs. 9.9 mL/kg/day, <i>p</i> = 0.003), and hospital readmission rates for effusion (13[30%] vs. 3[8%], <i>p</i> = 0.02) compared to baseline. There was an absolute reduction in hospital length of stay (11 vs. 9.5 days, <i>p</i> = 0.052). When combining average cost savings for the Fontan hospitalisations, readmissions for effusion, and cardiac catheterisations within 6 months of Fontan completion, there was a $325,144 total cost savings for 36 patients following pathway implementation.</p><p><strong>Conclusion: </strong>Implementation of a post-Fontan management pathway resulted in significant reductions in chest tube duration and output, and readmission rates for effusion in the perioperative period.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342168","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":"A case of a tetralogy of Fallot patient treated only with classic Blalock-Taussig-Thomas shunt in modern life.","authors":"Thuy Thuc Minh Pham, Thuy Thi Minh Huynh, Phuc Nang Vu","doi":"10.1017/S1047951124025666","DOIUrl":"https://doi.org/10.1017/S1047951124025666","url":null,"abstract":"<p><p>We showed images of classic Blalock-Taussig-Thomas shunt in a 35-year-old male patient with tetralogy of Fallot who underwent palliative surgery in 1992. It is a rare image echocardiography in our modern life.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342166","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":"Aberrant right subclavian artery stenting and embolisation for button battery-induced recurrent arterio-oesophageal fistula.","authors":"Saïd Bichali, Alix Maltezeanu, Ali Houeijeh","doi":"10.1017/S1047951124025691","DOIUrl":"https://doi.org/10.1017/S1047951124025691","url":null,"abstract":"<p><p>A young child, who had a previously unsuspected aberrant right subclavian retro-oesophageal artery, swallowed a button battery complicated with recurrent life-threatening bleeding, and survived after repeated percutaneous treatment as a bridge to surgery.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2024-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342167","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}