Cardiology in the Young最新文献

{"title":"Surgical resection is an effective adjunct to the management of an advanced metastatic osteosarcoma.","authors":"Hani K Najm, Samuel M Hoenig, John P Costello","doi":"10.1017/S1047951125001192","DOIUrl":"10.1017/S1047951125001192","url":null,"abstract":"<p><p>Cardiac involvement of metastatic osteosarcoma is exceedingly rare and carries a dismal prognosis. Documentation of the surgical management of cardiac metastatic osteosarcoma is limited. We describe the successful resection of a ∼ 3.9 x 2.2 cm mass involving the right atrioventricular groove and right ventricular inferolateral wall, granting our patient 12 months of excellent cardiac function in the face of advanced metastatic disease.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"853-855"},"PeriodicalIF":0.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143623625","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 isolated right subclavian artery and right patent ductus arteriosus successfully treated with catheter intervention in early infancy.","authors":"Makoto Yoshida, Tao Fujioka, Akio Inage","doi":"10.1017/S104795112500040X","DOIUrl":"10.1017/S104795112500040X","url":null,"abstract":"<p><p>In isolated subclavian artery, abnormal aortic arch development causes a loss of continuity with the aorta. Patent ductus arteriosus is a known cause of congestive heart failure. Herein, we present a rare case of congestive heart failure caused by isolated right subclavian artery and right patent ductus arteriosus associated with left-sided aortic arch treated by early closure.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"842-844"},"PeriodicalIF":0.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143555774","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":"Sodium-glucose cotransporter-2 inhibitor use in patients with a Fontan circulation.","authors":"Stephanie S Gaydos, Kimberly E McHugh, Frances K Woodard, Rochelle N Judd, Thomas J Brenzel, Heather T Henderson, Andrew J Savage, Andrew M Atz, David Gregg","doi":"10.1017/S1047951125000514","DOIUrl":"10.1017/S1047951125000514","url":null,"abstract":"<p><strong>Background: </strong>Sodium-glucose cotransporter-2 inhibitors reduce cardiovascular outcomes in patients with congestive heart failure and a biventricular circulation. Congestive heart failure in Fontan univentricular circulation is distinctly different. Experience with sodium-glucose cotransporter-2 inhibitors in this group has not yet been well described.</p><p><strong>Objectives: </strong>This work describes safety and tolerability of sodium-glucose cotransporter-2 inhibitors in patients with Fontan circulation.</p><p><strong>Methods: </strong>Single-centre review of patients with Fontan circulation prescribed a sodium-glucose cotransporter-2 inhibitors for congestive heart failure. Primary outcome was tolerability or need for discontinuation. Secondary outcomes were changes in New York Heart Association class, congestive heart failure hospitalisation, ventricular function, exercise performance, and laboratory values.</p><p><strong>Results: </strong>We identified 25 patients with Fontan circulation prescribed an sodium-glucose cotransporter-2 inhibitors, most with a systemic right ventricle. Over a third of subjects had at least moderately reduced baseline ventricular function. Baseline catheterisation showed a mean Fontan pressure of 17.1 ± 3.7 mmHg and pulmonary capillary wedge pressure 11.7 ± 3.2 mmHg at rest; 59% had occult diastolic dysfunction with abnormal pulmonary capillary wedge pressure elevation following volume expansion. Most were on congestive heart failure medications and/or a pulmonary vasodilator prior to sodium-glucose cotransporter-2 inhibitors addition, and three had a congestive heart failure hospitalisation within the previous year. All reported good medication tolerance except one patient was nonadherent to medications and two discontinued sodium-glucose cotransporter-2 inhibitors for perceived side effects. There were no significant differences in secondary outcomes. There was, however, a downward trend of serum brain natriuretic peptide (<i>n</i> = 13) and improved peak VO2 (<i>n</i> = 6), though neither statistically significant (<i>p</i> > 0.05).</p><p><strong>Conclusion: </strong>This series, the largest published to date, suggests that sodium-glucose cotransporter-2 inhibitors are safe and tolerable congestive heart failure therapy in Fontan circulation. Further research is warranted to explore therapy in this unique population.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"745-747"},"PeriodicalIF":0.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143499310","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":"Markers of growth and nutrition in children with acquired chylothorax post CHD surgery.","authors":"Kevin N Marzotto, Karin R Videlefsky, Meghan P Howell, Thomas R Kimball, Frank A Pigula, Kurt D Piggott","doi":"10.1017/S1047951125001222","DOIUrl":"10.1017/S1047951125001222","url":null,"abstract":"<p><strong>Background: </strong>Acquired chylothorax is an established complication of CHD surgery, affecting 2-9% of patients. CHD places a child at risk for failure to thrive, with subsequent chylothorax imposing additional risk.</p><p><strong>Objective: </strong>We conducted a retrospective chart review to ascertain quantitative markers of nutrition and growth in children affected by chylothorax following CHD surgery between 2018 and 2022 compared to controls.</p><p><strong>Methods: </strong>We utilised electronic medical record system, EPIC, at Children's Hospital, New Orleans, targeting subjects < 18 years old who underwent CHD surgery between 2018 and 2022 and developed a subsequent chylothorax. Study subjects were identified using the 10th revision of the International Classification of Diseases codes (ICD-10 codes: J94.0, I89.8, and J90.0). Each chylothorax case (<i>n</i> = 20) was matched by procedure type and age to a control with no chylothorax (<i>n</i> = 20). Data were recorded in REDCap and analysed using SPSS.</p><p><strong>Results: </strong>After removal of outliers, we analysed 19 total matched pairs. There was no statistical difference in growth velocity (<i>p</i> = 0.12), weight change (operation to discharge) (<i>p</i> = 0.95), weight change (admission to discharge) (<i>p</i> = 0.35), Z-score change (operation to discharge) (<i>p</i> = 0.90), Z-score change (admission to discharge) (<i>p</i> = 0.21), serum protein (<i>p</i> = 0.88), or serum albumin (<i>p</i> = 0.82). Among cases, linear regression demonstrated no significant association between maximum chylous output and growth velocity (<i>p</i> = 0.91), weight change (operation to discharge) (<i>p</i> = 0.15), or weight change (admission to discharge) (<i>p</i> = 0.98).</p><p><strong>Conclusions: </strong>We did not observe statistically significant markers of growth or nutrition in children with chylothorax post-CHD surgery compared to those without chylothorax. Multisite data collection and analysis is required to better ascertain clinical impact and guide clinical practice.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"791-797"},"PeriodicalIF":0.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143536689","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":"Kabuki patients with CHDs: outcomes after cardiac surgery.","authors":"Regina W Lam, Victoria Haro, Sarah Tabbutt, Hiroo Kinami, V Mohan Reddy, Minso Kim","doi":"10.1017/S1047951125000435","DOIUrl":"10.1017/S1047951125000435","url":null,"abstract":"<p><strong>Objective: </strong>This study aims to examine the surgical outcome of Kabuki syndrome patients after neonatal congenital heart surgery.</p><p><strong>Methods: </strong>This was a single-centre retrospective study of Kabuki syndrome patients undergoing neonatal congenital heart surgery from 2018 to 2023. Primary outcome was survival to discharge after index surgery. Secondary outcomes were morbidities and complications. Survival and hospital length of stay were compared to neonates with non-Kabuki genetic anomalies undergoing congenital heart surgery in the same time period.</p><p><strong>Results: </strong>A total of seven patients were reviewed. All Kabuki syndrome patients had left-sided lesions including three with hypoplastic left heart syndrome, three with aortic stenosis and/or aortic arch hypoplasia, and one with an isolated coarctation of aorta. Hospital survival was 5/7 (71% compared to 88% for neonates with non-Kabuki genetic anomalies). To date, four remain alive, including one with hypoplastic left heart syndrome. A higher percentage of Kabuki syndrome patients had unplanned interventions (43% vs 15% in non-Kabuki), abnormal brain imaging (29% vs 5%), and bacteremia (29% vs 9%). Median total ventilator days for Kabuki patients were also longer (16 days vs 6 days in non-Kabuki) as was hospital length of stay (66 days vs 41 days).</p><p><strong>Conclusions: </strong>Despite survival to discharge after index operation, Kabuki syndrome patients with single ventricle physiology remain at high risk of mortality and morbidity after cardiac surgery. However, they may be discharged without ventilator dependency and survive to toddler years.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"722-725"},"PeriodicalIF":0.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143499307","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":"Percutaneous closure of anomalous left coronary artery from pulmonary artery rare procedure in desperate situation: case report.","authors":"Mirza Mohd Kamran, Arun Gopi, Saurabhi Das, Zeeshan Lakhani, Amira Sheikh, Janeel Musthafa, G Raghuram, Musthafa P P Mohammad","doi":"10.1017/S1047951125000472","DOIUrl":"10.1017/S1047951125000472","url":null,"abstract":"<p><strong>Background: </strong>When anomalous left coronary artery from the pulmonary artery is diagnosed in infancy, it is associated with high mortality, but collaterals permit rare adult survival despite coronary steal. We report normalisation of perfusion abnormality after transcatheter proximal left coronary occlusion in a symptomatic adult with inducible ischaemia.</p><p><strong>Methods: </strong>A forty-eight-year-old female presented with exertional chest pain; EKG showed sinus rhythm with left axis deviation with intraventricular conduction defect. Echocardiogram diagnosed an anomalous left coronary artery arising from the pulmonary artery with normal ventricular function which was further confirmed by CT. The case was discussed with the surgical team, and it was decided to surgically ligate. The patient did not give consent for the surgical procedure; the patient was taken up for the procedure.</p><p><strong>Results: </strong>Device was released safely after confirming the position on echocardiogram after the procedure showed improvement in intraventricular conduction defect and QTc duration, and also no significant ischaemic changes were noted. Myocardial perfusion scintigraphy was done after 6 months of procedure which showed improvement in contractile function and perfusion of left ventricle.</p><p><strong>Conclusions: </strong>Device closure in long-standing anomalous left coronary artery from pulmonary artery case is safe, and long-term follow-up is needed.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"866-871"},"PeriodicalIF":0.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143522592","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":"Navigating challenges in paediatric warfarin therapy: a call for standardised guidelines and innovations.","authors":"Niveditha Krishnappa, J Kumaravel, Rupesh Kumar, Amol N Patil","doi":"10.1017/S1047951125001180","DOIUrl":"10.1017/S1047951125001180","url":null,"abstract":"","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"876-877"},"PeriodicalIF":0.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143584757","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":"Are all rings created equal? A single centre experience of fetal and paediatric vascular rings.","authors":"Elizabeth E LaSalle, Bruke A Tedla, Fraser Golding, Zaineb Boulil, Heather Y Sun","doi":"10.1017/S1047951125000356","DOIUrl":"10.1017/S1047951125000356","url":null,"abstract":"<p><strong>Background: </strong>Vascular rings cause highly variable clinical presentations. This study assesses the impact of prenatal versus postnatal diagnosis on clinical outcomes.</p><p><strong>Methods: </strong>We conducted a single centre retrospective review of isolated vascular ring patients (without significant CHD) from 2011 to 2022 and compared clinical and operative data between patients with prenatal and postnatal diagnoses.</p><p><strong>Results: </strong>Of 177 patients, 45% (N = 80) had prenatal diagnosis. Between 2018 and 2022, 78% had prenatal diagnosis compared to 41% from 2013 to 2018 and 4% before 2013 (p < 0.001). 76.3% (N = 135) had a right aortic arch with left ligamentum arteriosum, 22.6% (N = 40) had a double aortic arch, and 1.1% (N = 2) had a left aortic arch with right ligamentum arteriosum. Postnatal diagnosis patients were more likely to have preoperative respiratory symptoms (55.7%), medications (34.0%), or admissions (24.7%) (versus 32.5%, 10.0%, and 11.3% of the prenatal diagnosis patients, <i>p</i> < 0.05) and require surgical repair (68.0% versus 38.8% of prenatal diagnosis patients, <i>p</i> < 0.0001). 54.8% of patients had surgical repair; prenatal diagnosis patients were younger at surgery, 7.5 (3-11) months compared to 16.0 (5-18) months in the postnatal diagnosis patients (p = .0014). Double aortic arch patients were more likely to require surgical repair (90.0%, compared to 44.5% with right aortic arch, <i>p</i> < 1e^-4). Postnatal diagnosis patients had more residual postoperative symptoms (40.9% versus 16.1% in prenatal diagnosis patients, <i>p</i> = 0.01).</p><p><strong>Conclusion: </strong>Prenatal diagnosis of vascular rings improves clinical surveillance, resulting in earlier surgical repair in symptomatic patients and diminished morbidity. Higher risk double aortic arch patients should have a tailored evaluation pathway.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"702-710"},"PeriodicalIF":0.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143514813","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":"Vitamin D levels correlate with exercise capacity in adults with CHD.","authors":"Inne Vanreusel, Wendy Hens, Emeline M Van Craenenbroeck, Bernard P Paelinck, Vincent F M Segers, An Van Berendoncks","doi":"10.1017/S1047951125000526","DOIUrl":"10.1017/S1047951125000526","url":null,"abstract":"<p><strong>Introduction: </strong>Vitamin D is crucial for normal organ function, vascular health and exercise performance, yet its deficiency is widespread. Patients with CHD often exhibit reduced exercise capacity. Limited research exists on vitamin D in CHD.</p><p><strong>Methods: </strong>This study investigates serum 25-hydroxy vitamin D levels in 55 adult CHD patients (median age 31 years) compared to 55 age- and gender-matched controls without cardiac disease and examines associations with exercise capacity, peripheral microvascular function, muscle strength and biventricular function in CHD. Therefore, patients underwent fingertip arterial tonometry, transthoracic echocardiography, muscle strength measurements and cardiopulmonary exercise testing.</p><p><strong>Results: </strong>Results indicated that 93% of CHD patients and 91% of controls had 25-hydroxy vitamin D levels <30 ng/ml, with both groups showing varying values depending on the season in which the studies were conducted. No significant difference in 25-hydroxy vitamin D levels was found between patients and controls. While vitamin D levels in CHD patients did not significantly correlate with age, body mass index, blood pressure, peripheral microvascular function, high-sensitivity C-reactive protein, cholesterol levels, N-terminal-pro hormone B-type natriuretic peptide, ventricular function or muscle strength, a significant correlation was found with percent-predicted peak oxygen consumption (<i>ρ</i>=0.41, <i>p</i> = 0.005 and <i>ρ</i>=0.34, <i>p</i> = 0.02 for reference values following Wasserman and the LowLands registry, respectively), even after adjusting for season (<i>p</i> = 0.03 and 0.05, respectively).</p><p><strong>Conclusions: </strong>In conclusion, vitamin D levels were similar between CHD patients and controls, but vitamin D insufficiency is common and linked to reduced exercise capacity in CHD. Further research is needed to determine whether vitamin D supplementation combined with exercise could be beneficial in CHD with vitamin D insufficiency.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"748-755"},"PeriodicalIF":0.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143596383","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":"Thickened feeds for infants with critical CHD: a survey of current practices.","authors":"Courtney E Jones, Hema Desai, Susan Willette, Karli A Negrin, Kristi Glotzbach, Samantha C Butler","doi":"10.1017/S1047951125001179","DOIUrl":"10.1017/S1047951125001179","url":null,"abstract":"<p><strong>Introduction: </strong>Dysphagia is common in infants born with critical CHD. Thickened liquids are often used to treat dysphagia, but associated risks limit widespread use among feeding specialists. This survey aims to assess dysphagia treatment patterns and thickened liquid use across paediatric cardiac surgical centres.</p><p><strong>Methods: </strong>A 24-question, cross-sectional survey. Convenience and snowball sampling methods were used to engage 52 paediatric cardiac surgical centres affiliated with the Cardiac Newborn Neuroprotective Network. Descriptive statistics were used to analyse and compare responses.</p><p><strong>Results: </strong>Twenty-six individual respondents represented 21 unique paediatric cardiac surgical centres. Most responses were from experienced, speech-language pathologists (78%) at medium size centres (88%). Ninety-three percent of responding centres used thickened liquids to treat dysphagia and 81% only after formal instrumental assessment of swallowing. Thickened oral feeding was used for single-ventricle patients by 85% versus 69% for two-ventricle patients. Barriers to recommending thickened oral feedings included the cost of thickening agents, parental non-adherence, and gastrointestinal concerns.</p><p><strong>Conclusions: </strong>This is the first survey to report multi-institutional dysphagia treatment practice variation at United States congenital cardiac surgical centres. Thickened oral feedings are frequently used across centres in high-risk critical CHD patients but treatment benefit remains unclear. This survey highlights a broad scientific community poised to direct dysphagia research in critical CHD to address practice variation, short- and long-term impact of thickened oral feeding on feeding outcomes, and barriers to use and access of thickening agents.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"711-716"},"PeriodicalIF":0.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143623742","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}