Pediatric Cardiology最新文献

{"title":"Cytokeratin-18 is Elevated Prior to Conventional Measures of Liver Disease in Fontan-Associated Liver Disease.","authors":"Joshua D Kurtz, Shahryar M Chowdhury, Allison K Black, Andrea N Lambert, Ashley E Neal, Theresa Kluthe, Joshua D Sparks","doi":"10.1007/s00246-024-03637-z","DOIUrl":"https://doi.org/10.1007/s00246-024-03637-z","url":null,"abstract":"<p><p>The Fontan procedure is used to palliate complex forms of congenital heart disease. This results in adverse hepatic sequelae now known as Fontan-associated liver disease (FALD). Conventional laboratory measures of liver disease do not correlate well with FALD severity. Cytokeratin-18 (CK-18) is a measure of cell death and is sensitive in detecting other causes of liver disease. Our aim was to assess the use of a novel measure of liver disease, CK-18, in Fontan patients. This is a single-center, prospective, cross-sectional study of Fontan patients aged 8-21 years old. We performed ultrasound elastography, echocardiography, magnetic resonance imaging, and serum laboratory testing. Novel laboratory test CK-18 levels in Fontan subjects were compared to healthy age-matched controls. Thirteen Fontan patients were evaluated with a median age 15 years (10, 14), 4 Hypoplastic left heart syndrome, 11 were male, and 5 were symptomatic. Fontan patients had normal AST/ALT, but a significantly elevated liver stiffness by elastography (median 13.4 kPa). Hepatic stiffness by elastography was associated with diastolic-indexed (rho = 0.58, p = 0.04) ventricular volumes. Compared to 10 aged-matched controls, CK-18 was higher in the Fontan group-cleaved CK-18 protein (p < 0.01) and full CK-18 protein, (p = 0.02). CK-18 was positively associated with AST and ALT. Elevated CK-18 levels were found in Fontan patients compared to controls suggesting hepatic cell death even in these relatively healthy Fontan patients. CK-18 was elevated prior to changes in traditional testing. CK-18 may be a useful sensitive marker of liver disease in FALD.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142140753","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":"Impact of Drug Elution on Infectious Outcomes after Ductal Stenting in Newborns.","authors":"Keyhan Sayadpour Zanjani, Aliakbar Zeinaloo, Elaheh Malakan Rad, Sara Norouzi, Kayvan Mirnia, Mojtaba Gorji","doi":"10.1007/s00246-024-03625-3","DOIUrl":"https://doi.org/10.1007/s00246-024-03625-3","url":null,"abstract":"<p><p>Ductal stenting (DS) is an interesting palliation for neonates with duct-dependent pulmonary circulation. With the introduction of drug-eluting stents (DES), which have a lower rate of restenosis and longer patency, these stents have replaced bare metal stents (BMS) in the global market. DES release Rapamycin group drugs, which have anti-proliferative and immunosuppressive effects. While the released drug amount is negligible in adult patients, it can lead to high blood levels in neonates, potentially increasing their risk of infections. We conducted a retrospective observational study on infants (under 2 months of age) with duct-dependent pulmonary circulation who underwent successful DS procedures between September 2013 and September 2023. Infants who received at least one DES were categorized into the DES group, while those receiving only BMS were categorized into the BMS group. We compared the prevalence of sepsis (both clinical and proven) and mortality between the two groups. We identified 53 infants (58.4% males), comprising 30 (56.6%) in the DES group and 23 (43.4%) in the BMS group. In the DES group, there were 11 cases (36.7%) of sepsis, including 8 clinical and 3 culture-positive cases. In contrast, the BMS group had 4 cases (13%) of sepsis, all of which were clinical (p = 0.053). Three patients (5.6%) died due to sepsis: two from the DES group and one from the BMS group. Six patients were suspected of having immunodeficiency (22q11.2 deletion or asplenia), with three in each group. Among these, only one patient in the DES group developed clinical sepsis. Our findings indicate that drug elution did not significantly increase the risk of infection following DS in newborns.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142133422","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":"Heterotaxy Syndromes and Transposition: Common Genetic Basis or a Serendipitous Association?","authors":"Thazhathu Veettil Sreelal, Saurabh Kumar Gupta, Pujitha Vidiyala, Niraj Nirmal Pandey","doi":"10.1007/s00246-024-03638-y","DOIUrl":"https://doi.org/10.1007/s00246-024-03638-y","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142126335","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":"Abstract","authors":"","doi":"10.1007/s00246-024-03609-3","DOIUrl":"https://doi.org/10.1007/s00246-024-03609-3","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2024-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142209899","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":"Integrating Contrastive Learning and Cycle Generative Adversarial Networks for Non-invasive Fetal ECG Extraction.","authors":"Rongrong Qu, Tingqiang Song, Guozheng Wei, Lili Wei, Wenjuan Cao, Jiale Song","doi":"10.1007/s00246-024-03633-3","DOIUrl":"10.1007/s00246-024-03633-3","url":null,"abstract":"<p><p>Fetal electrocardiogram (FECG) contains crucial information about the fetus during pregnancy, making the extraction of FECG signal essential for monitoring fetal health. However, extracting FECG signal from abdominal electrocardiogram (AECG) poses several challenges: (1) FECG signal is often contaminated by noise, and (2) FECG signal is frequently overshadowed by high-amplitude maternal electrocardiogram (MECG). To address these issues and enhance the accuracy of signal extraction, this paper proposes an improved Cycle Generative Adversarial Networks (CycleGAN) with integrated contrastive learning for FECG signal extraction. The model introduces a dual-attention mechanism in the generator of the generative adversarial network, incorporating a multi-head self-attention (MSA) module and a channel-wise self-attention (CSA) module to enhance the quality of generated signals. Additionally, a contrastive triplet loss is integrated into the CycleGAN loss function, optimizing training to increase the similarity between the extracted FECG signal and the scalp fetal electrocardiogram. The proposed method is evaluated using the ADFECG dataset and the PCDB dataset both from the Physionet. In terms of signal extraction quality, Mean Squared Error is reduced to 0.036, Mean Absolute Error (MAE) to 0.009, and Pearson Correlation Coefficient reaches 0.924. When validating the model performance, Structural Similarity Index achieves 95.54%, Peak Signal-to-Noise Ratio (PSNR) reaches 38.87 dB, and R-squared (R²) attains 95.12%. Furthermore, the positive predictive value (PPV), sensitivity (SEN) and F1-score for QRS wave cluster detection on the ADFECG dataset also reached 99.56%, 99.43% and 99.50%, respectively. On the PCDB dataset, the positive predictive value (PPV), sensitivity (SEN) and F1-score for QRS wave cluster detection also reached 98.24%, 98.60% and 98.42%, respectively. All of them are higher than other methods. Therefore, the proposed model has important applications in effective monitoring of fetal health during pregnancy.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142120411","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":"Impact of Age and of the Patent Ductus Arteriosus on Pulmonary Hemodynamics in Children with Complete Atrioventricular Septal Defect.","authors":"Lucas Oliveira Rocha, Nelson Itiro Miyague, Leo Agostinho Solarewicz, Miguel Morita Fernandes-Silva","doi":"10.1007/s00246-024-03636-0","DOIUrl":"https://doi.org/10.1007/s00246-024-03636-0","url":null,"abstract":"<p><p>Complete atrioventricular septal defect (CAVSD) can lead to the development of pulmonary obstructive vascular disease due to high pulmonary blood flow and pressures. This study aimed to evaluate the changes in pulmonary hemodynamics with aging and with patent ductus arteriosus (PDA) in children with CAVSD. We retrospectively evaluated 137 children (94% with trisomy 21, median age of 195 (25-2963) days, 58.4% female) with CASVD referred to cardiac catheterization from January 2000 to December 2020. Those with associated congenital heart disease, except PDA, had been excluded. They were divided into three age terciles (T1, T2, and T3). Aging was directly associated with higher mean (T1: 34.2 ± 9.1; T2: 37.1 ± 5.8; T3: 42 ± 10.6 mmHg, p < 0.001) and diastolic (T1: 19.4 ± 5.3; T2 21.6 ± 5.0; T3: 26.0 ± 9.5 mmHg, P < 0.001) pulmonary arterial pressures, and with higher pulmonary vascular resistance (T1: 3.24 ± 1.69, T2: 3.47 ± 1.19; T3: 4.49 ± 3.91 Wu.m², p = 0.023). This resulted in a loss of eligibility for anatomical correction, which became evident only after 300 days of age. PDA was associated with a higher mean (37.2 [35.9; 38.5] vs. 41.3 [37.5; 45.0] mmHg, p = 0.049) and diastolic (21.7 [20.7; 22.6] vs. 26.4 [24.1; 29.0] mmHg, p = 0.001) pulmonary pressure, and resistor-compliance time (0.28 [0.26; 0.29] vs. 0.36 [0.31; 0.40], p = 0.001) after adjusting for age and sex. In children with CAVSD, aging was associated with worsening of pulmonary vascular hemodynamics, particularly when PDA was associated, resulting in loss of eligibility for anatomical correction after 10 months of age as the first surgical option.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142120410","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":"Improving Reproducibility of Volumetric Evaluation Using Computed Tomography in Pediatric Patients with Congenital Heart Disease.","authors":"Hyun-Hae Cho, So Mi Lee, Sun Kyoung You","doi":"10.1007/s00246-024-03630-6","DOIUrl":"https://doi.org/10.1007/s00246-024-03630-6","url":null,"abstract":"<p><p>The volumetric data obtained from the cardiac CT scan of congenital heart disease patients is important for defining patient's status and making decision for proper management. The objective of this study is to evaluate the intra-observer, inter-observer, and interstudy reproducibility of left ventricular (LV) and right ventricular (RV) or functional single-ventricle (FSV) volume. And compared those between manual and using semi-automated segmentation tool. Total of 127 patients (56 female, 71 male; mean age 82.1 months) underwent pediatric protocol cardiac CT from January 2020 to December 2022. The volumetric data including both end-systolic and -diastolic volume and calculated EF were derived from both conventional semiautomatic region growing algorithms (CM, TeraRecon, TeraRecon, Inc., San Mateo, CA, USA) and deep learning-based annotation program (DLS, Medilabel, Ingradient, Inc., Seoul, Republic of Korea) by three readers, who have different background knowledge or experience of radiology or image extraction before. The reproducibility was compared using intra- and inter-observer agreements. And the usability was measured using time for reconstruction and number of tests that were reconfigured before the reconfiguration time was reduced to less than 5 min. Inter- and intra-observer agreements showed better agreements degrees in DLS than CM in all analyzers. The time used for reconstruction showed significantly shorter in DLS compared with CM. And significantly small numbers of tests before the reconfiguration is needed in DLS than CM. Deep learning-based annotation program can be more accurate way for measurement of volumetric data for congenital heart disease patients with better reproducibility than conventional method.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142110707","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":"Impact of a Multidisciplinary Service Line and Program for Transcatheter Device Closure of the Neonatal Ductus Arteriosus.","authors":"R Allen Ligon, Shazia Bhombal, Marissa Adamson, Sarah Hash, Dennis W Kim, Ashley S LeFevre, Justin B Long, Sarah McLaughlin, Marcos Mills, Katie Roddy, Lucas Tucker, Cyrus Samai, Anthony Piazza, Shannon E Hamrick","doi":"10.1007/s00246-024-03629-z","DOIUrl":"https://doi.org/10.1007/s00246-024-03629-z","url":null,"abstract":"<p><p>Outline a quality initiative establishing an institutional service line for neonatal transcatheter device closure of the patent ductus arteriosus (TDC-PDA). A retrospective descriptive observational study surrounds programmatic approach to TDC-PDA in premature neonates with process measure spanning education, implementation, referral, and post-procedural care. Metrics tracked pre- and post-program creation with statistical analyses performed. Neonatal TDC-PDA referrals increased exponentially since program inception (n = 13 in year prior; n = 42 year 1; n = 74 year 2), especially in patients weighing less than 1.3 kg (12.5%; 55%; 50%), and were associated with an increased procedural success rate (81%; 95%; 99%). Procedural checklist creation decreased procedural \"out of isolette\" time (median 93 min; 59; 52), and procedural-related complication or clinical sequelae (19%; 12%; 4%). A multidisciplinary service line and program dedicated to neonatal TDC-PDA can result in a significant increase in referrals as well as procedural efficacy and safety for this medically fragile population.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142081180","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":"Nutrition Provision in Children with Heart Disease on Extracorporeal Membrane Oxygenation (ECMO).","authors":"Jason S Kerstein, Caroline R Pane, Lynn A Sleeper, Emily Finnan, Ravi R Thiagarajan, Nilesh M Mehta, Kimberly I Mills","doi":"10.1007/s00246-024-03628-0","DOIUrl":"https://doi.org/10.1007/s00246-024-03628-0","url":null,"abstract":"<p><p>Nutrition provision for children with heart disease supported with extracorporeal membrane oxygenation (ECMO) involves nuanced decision making. We examined nutrition provision while on ECMO in the CICU and the relationship between energy and protein adequacy and end organ function as assessed by pediatric sequential organ failure assessment (pSOFA) scores in children with heart disease supported with ECMO. Children (≤ 21 years-old) with congenital or acquired heart disease who received ECMO in the cardiac intensive care unit were included. There were 259 ECMO runs in 252 patients over an 8-year study period (2013-2020). Median energy delivery and adequacy were 26.1 [8.4, 45.9] kcal/kg/day and 58.3 [19.8, 94.6]%, respectively. Median protein delivery and adequacy were 0.98 [0.36, 1.64] g/kg/day and 35.7 [13.4, 60.3]%, respectively. pSOFA increased by a median of four points during the ECMO run. Change in pSOFA score was not associated with energy or protein adequacy (p = 0.46 and p = 0.72, respectively). Higher energy and protein adequacy-from parenteral nutrition-correlated with increased hospital-acquired infections (HAIs, p = 0.031 and p = 0.003, respectively). Achieving nutritional adequacy was dependent on the use of parenteral nutrition. Similar clinical outcomes with regard to end organ function but with an increased incidence of HAIs suggests the need to explore the role of optimal enteral nutrition delivery on ECMO.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142056249","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":"Short and Mid-term Outcomes of Ductal Graft with Bilateral Pulmonary Banding in Hypoplastic Left Heart Syndrome and Variants.","authors":"Ayla Oktay, Ahmet Arnaz, Canan Ayabakan, Tayyar Sarioglu, Yusuf Kenan Yalcinbas","doi":"10.1007/s00246-024-03624-4","DOIUrl":"10.1007/s00246-024-03624-4","url":null,"abstract":"<p><p>This study evaluates a novel surgical technique inspired by the hybrid Norwood procedure, which involves ductal graft placement and bilateral pulmonary artery banding. This technique is designed to avoid the high-risk classical Norwood stage 1 operation and to avoid the need for first stage invasive catheter interventions and interstage catheter interventions when ductal stent placement is not feasible in developing countries with limited resources. Between June 2015 and December 2022, 14 patients with hypoplastic left heart syndrome and variants were treated using this procedure at our center. Polytetrafluoroethylene graft was used for ductal graft procedure and polytetrafluoroethylene bands were used for bilateral pulmonary banding. Atrial septectomy was done selectively. Patients with adequate left heart growth underwent biventricular repair and pulmonary debanding. Patients without left heart growth underwent cavopulmonary anastomosis or necessary intermediate surgical revisions. Seven patients out of 14 reached the second stage. Two received biventricular repair, three underwent bidirectional cavopulmonary anastomosis, and two required repeat palliations other than bidirectional cavopulmonary anastomosis. Modifications of the Norwood procedure is an ongoing process due to the complex nature of hypoplastic left heart syndrome and variants. As experience grows, the mortality associated with ductal graft and bilateral pulmonary banding procedure may decrease, making this technique an alternative for centers where classical Norwood operation or hybrid palliation is not possible, especially in developing countries with limited resources. We might predict promising outcomes, especially for patients with hypoplastic left heart complex where biventricular repair is expected.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142018291","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}