Pediatric Cardiology最新文献

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Role of Cardiac CTA to Evaluate Branch Pulmonary Artery Stenosis and Ductal Insertion Pattern in Right-Sided Congenital Heart Defects. 心脏 CTA 在评估右侧先天性心脏病肺动脉分支狭窄和导管插入模式中的作用
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2024-10-01 Epub Date: 2023-07-21 DOI: 10.1007/s00246-023-03234-6
Ana L Vasquez Choy, Rafael Zonana Amkie, Dilachew A Adebo
{"title":"Role of Cardiac CTA to Evaluate Branch Pulmonary Artery Stenosis and Ductal Insertion Pattern in Right-Sided Congenital Heart Defects.","authors":"Ana L Vasquez Choy, Rafael Zonana Amkie, Dilachew A Adebo","doi":"10.1007/s00246-023-03234-6","DOIUrl":"10.1007/s00246-023-03234-6","url":null,"abstract":"<p><p>There is limited experience in evaluating abnormal ductus arteriosus (DA) insertion pattern by contrast-enhanced cardiac computed tomography (cardiac CT) in patients with right-sided obstructive cardiac defects. Retrospective review of 38 infants with right-sided obstructive cardiac defects who underwent a preoperative cardiac CT between 2016 and 2021. We reviewed the types of cardiac lesions, patterns of ductal insertion, frequency of pulmonary artery (PA) stenosis requiring intervention, total dose length product (DLP), and effective radiation dose. Of 38 infants, 45% were female, the median gestational age and weight were 37 (range 34-40) weeks and 2.95 (range 2-4) kg. The most common pathologies were pulmonary atresia with ventricular septal defect (24%) and tetralogy of Fallot (24%). The abnormal ductal insertion patterns were DA inserting into the left PA in 39%, DA bifurcating into branch PA in 32%, and DA inserting into the right PA in 13%. Of the 38 infants, 76% developed branch PA stenosis requiring intervention. Among patients with abnormal DA insertion, 44% required branch PA arterioplasty during their index surgery compared to 17% without abnormal DA insertion. Regardless of the type of abnormal DA insertion, 67% developed bilateral branch PA stenosis over time. The mean DLP was 8 mGy-cm and the mean calculated effective radiation dose was 0.312 mSv. The utilization of contrast-enhanced cardiac CT in infants with right-sided obstructive heart defects can offer crucial insights into abnormal ductus arteriosus insertion patterns. This information is valuable for effective procedure planning and for monitoring the development of branch pulmonary artery stenosis.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10223218","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}
引用次数: 0
Unusual Vascular Ring in the Fetus. 胎儿体内的异常血管环
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2024-10-01 Epub Date: 2023-08-11 DOI: 10.1007/s00246-023-03261-3
A Savis, C Oakley, M P M Van Poppel, D F A Lloyd, K Pushparajah, T V Vigneswaran, V Zidere
{"title":"Unusual Vascular Ring in the Fetus.","authors":"A Savis, C Oakley, M P M Van Poppel, D F A Lloyd, K Pushparajah, T V Vigneswaran, V Zidere","doi":"10.1007/s00246-023-03261-3","DOIUrl":"10.1007/s00246-023-03261-3","url":null,"abstract":"<p><p>We present the clinical course and echocardiographic and genetic findings of two fetuses with an unusual vascular ring, created by a left aortic arch with a right arterial duct and an aberrant right subclavian artery. One fetus was diagnosed with 22q11.2 microdeletion and the other became symptomatic in infancy. It is important to consider the position of the arterial ductal ligament in patients who present with tracheoesophageal compressive symptoms in the presence of a left aortic arch. These cases also highlight that a vascular ring formed from a left arch may have similar associations to a vascular ring formed by a right aortic arch.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10346812","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}
引用次数: 0
Acute Kidney Injury and Mid-term Outcomes After Extra-Cardiac Fontan Conversion. 心外丰坦转换术后的急性肾损伤和中期预后。
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2024-10-01 Epub Date: 2023-06-24 DOI: 10.1007/s00246-023-03220-y
Tatsuya Kunigo, Risa Oikawa, Minoru Nomura
{"title":"Acute Kidney Injury and Mid-term Outcomes After Extra-Cardiac Fontan Conversion.","authors":"Tatsuya Kunigo, Risa Oikawa, Minoru Nomura","doi":"10.1007/s00246-023-03220-y","DOIUrl":"10.1007/s00246-023-03220-y","url":null,"abstract":"<p><p>The aim of this study was to investigate the association of postoperative acute kidney injury and unplanned re-admission rate due to heart failure at 2 years follow-up in patients who had extra-cardiac Fontan conversion. This was a retrospective single-center study of patients who underwent conversion from classic Fontan to extra-cardiac Fontan between January 2014 and December 2021. Acute kidney injury was defined using the Kidney Disease Improving Global Outcomes criteria. A total of 47 patients underwent Fontan conversion. Acute kidney injury occurred in 22 patients (46.8%) and 5 patients with acute kidney injury needed renal replacement therapy. Unplanned re-admission rate at 2-year follow-up was significantly higher in patients with acute kidney injury even when renal function returned to baseline (8 [36.4%] vs. 3 [12.0%], p = 0.026 by the log-rank test). In conclusion, postoperative acute kidney injury after extra-cardiac Fontan conversion was associated with unplanned re-admission due to heart failure at 2-year follow-up even though renal function was recovered.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9671809","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}
引用次数: 0
Adaptive Growth of the Ductus Arteriosus and Aortic Isthmus in Various Ductus-Dependent Complex Congenital Heart Diseases. 各种导管依赖性复杂先天性心脏病中动脉导管和主动脉峡部的适应性生长。
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2024-10-01 Epub Date: 2023-07-21 DOI: 10.1007/s00246-023-03236-4
Liza Hashim, Daniel Vari, Abdul M Bhat, Takeshi Tsuda
{"title":"Adaptive Growth of the Ductus Arteriosus and Aortic Isthmus in Various Ductus-Dependent Complex Congenital Heart Diseases.","authors":"Liza Hashim, Daniel Vari, Abdul M Bhat, Takeshi Tsuda","doi":"10.1007/s00246-023-03236-4","DOIUrl":"10.1007/s00246-023-03236-4","url":null,"abstract":"<p><strong>Background: </strong>The ductus arteriosus (DA) is critical in maintaining postnatal circulation in neonates with obstructed systemic circulation (OSC) and pulmonary circulation (OPC). We hypothesized that the size of the DA and aortic isthmus (AoI) undergoes adaptive growth in utero to counteract the hemodynamic challenges in these congenital heart diseases (CHD).</p><p><strong>Methods: </strong>Postnatal echocardiograms of neonates diagnosed prenatally with ductal-dependent CHD who were started on prostaglandins within 24 h of birth were reviewed. We assessed the cross-sectional area of the aortic valve opening, pulmonary valve opening, AoI, and DA by calculating (diameter)<sup>2</sup>/body surface area. Neonates were classified into OSC or OPC then subgrouped depending upon the patency of semilunar valves: OSC with and without aortic atresia (OSC-AA and OSC-nAA, respectively) and OPC with and without pulmonary atresia (OPC-PA and OPC-nPA, respectively).</p><p><strong>Results: </strong>Ninety-four cases were studied. The DA in OSC was significantly larger than OPC, and the DA in OSC-AA was significantly larger than OSC-nAA. The size of the AoI was significantly larger in OPC than OSC and larger in OSC-AA than OSC-nAA. Within the OSC-nAA group, there was no significant difference in the size of the DA, AoI, or pulmonary valve opening between those with retrograde flow (RF) at the AoI and without (nRF) except the aortic valve opening was significantly larger in nRF. All groups had comparable cross-sectional areas of systemic output.</p><p><strong>Conclusions: </strong>Our findings suggest that DA and AoI show compensatory growth to maintain critical blood flow to vital organs against primary anatomical abnormalities in ductus-dependent CHD. (249 words).</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10204577","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}
引用次数: 0
Isosorbide DiNitrate Effect on Hemodynamic Profile, Liver Stiffness, and Exercise Tolerance in Fontan Circulation (The NEET Clinical Trial). 硝酸异山梨酯对丰坦循环血流动力学特征、肝脏硬度和运动耐受性的影响(NEET 临床试验)。
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2024-10-01 Epub Date: 2023-04-21 DOI: 10.1007/s00246-023-03156-3
Amee M Bigelow, Kyle W Riggs, David L S Morales, Alexander R Opotowsky, Adam M Lubert, Jonathan R Dillman, Gruschen R Veldtman, Haleh C Heydarian, Andrew T Trout, David S Cooper, Stuart L Goldstein, Clifford Chin, Joseph J Palermo, Nicholas J Ollberding, Wayne A Mays, Tarek Alsaied
{"title":"Isosorbide DiNitrate Effect on Hemodynamic Profile, Liver Stiffness, and Exercise Tolerance in Fontan Circulation (The NEET Clinical Trial).","authors":"Amee M Bigelow, Kyle W Riggs, David L S Morales, Alexander R Opotowsky, Adam M Lubert, Jonathan R Dillman, Gruschen R Veldtman, Haleh C Heydarian, Andrew T Trout, David S Cooper, Stuart L Goldstein, Clifford Chin, Joseph J Palermo, Nicholas J Ollberding, Wayne A Mays, Tarek Alsaied","doi":"10.1007/s00246-023-03156-3","DOIUrl":"10.1007/s00246-023-03156-3","url":null,"abstract":"<p><p>After Fontan operation, decreased venous capacitance and venoconstriction are adaptive mechanisms to maintain venous return and cardiac output. The consequent higher venous pressure may adversely impact end-organ function, exercise capacity and result in worse clinical outcomes. This pilot study evaluated the safety and effect of isosorbide dinitrate (ISDN), a venodilator, on exercise capacity, peripheral venous pressure (PVP), and liver stiffness in patients with Fontan circulation. In this prospective single-arm trial, 15 individuals with Fontan circulation were evaluated at baseline and after 4 weeks of therapeutic treatment with ISDN. Primary aims were to assess the safety of ISDN and the effect on maximal exercise. We also aimed to evaluate the effect of ISDN on ultrasound-assessed liver stiffness, markers of submaximal exercise, and PVP at rest and peak exercise. Repeated measures t-tests were used to assess change in variables of interest in response to ISDN. Mean age was 23.5 ± 9.2 years (range 11.2-39.0 years), and 10/15 (67%) were male. There was no statistically significant change in peak VO<sub>2</sub> (1401 ± 428 to 1428 ± 436 mL/min, p = 0.128), but VO<sub>2</sub> at the anaerobic threshold increased (1087 ± 313 to 1115 ± 302 mL/min, p = 0.03). ISDN was also associated with a lower peak exercise PVP (22.5 ± 4.5 to 20.6 ± 3.0 mmHg, p = 0.015). Liver stiffness was lower with ISDN, though the difference was not statistically significant (2.3 ± 0.4 to 2.1 ± 0.5 m/s, p = 0.079). Of the patients completing the trial, mild headache was common (67%), but there were no major adverse events. Treatment with ISDN for 4 weeks is well-tolerated in patients with a Fontan circulation. ISDN is associated with an increase in VO<sub>2</sub> at anaerobic threshold, lower peak PVP, and a trend toward lower liver stiffness. Larger, longer duration studies will be necessary to define the impact of ISDN on clinical outcomes in the Fontan circulation.Clinical Trial Registration: URL: https://clinicaltrials.gov . Unique identifier: NCT04297241.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10119822/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9427355","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Improvement in Palivizumab Administration Prior to Discharge for Hospitalized Infants with Hemodynamically Significant Congenital Heart Disease: A Quality Improvement Initiative. 改善患有血流动力学显著的先天性心脏病的住院婴儿出院前的帕利珠单抗给药:一项质量改善倡议
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2024-10-01 Epub Date: 2023-05-05 DOI: 10.1007/s00246-023-03163-4
Andrea L Jones, Matthew J Campbell, Brittany Abernathy, Stephanie Neubert, Alyssa Hager, Hailey Collier, Evan Zachary Ramsey, Anna Simon, Susan Schachtner, Shobha Natarajan
{"title":"Improvement in Palivizumab Administration Prior to Discharge for Hospitalized Infants with Hemodynamically Significant Congenital Heart Disease: A Quality Improvement Initiative.","authors":"Andrea L Jones, Matthew J Campbell, Brittany Abernathy, Stephanie Neubert, Alyssa Hager, Hailey Collier, Evan Zachary Ramsey, Anna Simon, Susan Schachtner, Shobha Natarajan","doi":"10.1007/s00246-023-03163-4","DOIUrl":"10.1007/s00246-023-03163-4","url":null,"abstract":"<p><p>In this quality improvement initiative, we aimed to increase provider adherence with palivizumab administration guidelines for hospitalized infants with hemodynamically significant congenital heart disease. We included 470 infants over four respiratory syncytial virus (RSV) seasons from 11/2017 to 03/2021 (baseline season: 11/2017-03/2018). Interventions included the following: education, including palivizumab in the sign-out template, identifying a pharmacy expert, and a text alert (seasons 1 and 2: 11/2018-03/2020) that was replaced by an electronic health record (EHR) best practice alert (BPA) in season 3 (11/2020-03/2021). The text alert and BPA prompted providers to add \"Need for RSV immunoprophylaxis\" to the EHR problem list. The outcome metric was the percentage of eligible patients administered palivizumab prior to discharge. The process metric was the percentage of eligible patients with \"Need for RSV immunoprophylaxis\" on the EHR problem list. The balancing metric was the percentage of palivizumab doses administered to ineligible patients. A statistical process control P-chart was used to analyze the outcome metric. The mean percentage of eligible patients who received palivizumab prior to hospital discharge increased significantly from 70.1% (82/117) to 90.0% (86/96) in season 1 and to 97.9% (140/143) in season 3. Palivizumab guideline adherence was as high or higher for those with \"Need for RSV immunoprophylaxis\" on the problem list than for those without it in most time periods. The percentage of inappropriate palivizumab doses decreased from 5.7% (n = 5) at baseline to 4.4% (n = 4) in season 1 and 0.0% (n = 0) in season 3. Through this initiative, we improved adherence with palivizumab administration guidelines for eligible infants prior to hospital discharge.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10625646/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9763571","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Measured Oxygen Consumption During Pediatric Cardiac Catheterization is More Accurate than Assumed Oxygen Consumption. 小儿心脏导管术中的实测耗氧量比假定耗氧量更准确。
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2024-10-01 Epub Date: 2023-05-27 DOI: 10.1007/s00246-023-03186-x
Bradford H Ralston, Andrew T Waberski, Joshua P Kanter, Jacob W Schick, Tacy E Downing
{"title":"Measured Oxygen Consumption During Pediatric Cardiac Catheterization is More Accurate than Assumed Oxygen Consumption.","authors":"Bradford H Ralston, Andrew T Waberski, Joshua P Kanter, Jacob W Schick, Tacy E Downing","doi":"10.1007/s00246-023-03186-x","DOIUrl":"10.1007/s00246-023-03186-x","url":null,"abstract":"<p><p>When calculating cardiac index (C.I.) by the Fick method, oxygen consumption (VO<sub>2</sub>) is often unknown, so assumed values are typically used. This practice introduces a known source of inaccuracy into the calculation. Using a measured VO<sub>2</sub> (mVO<sub>2</sub>) from the CARESCAPE E-sCAiOVX module provides an alternative that may improve accuracy of C.I. calculations. Our aim is to validate this measurement in a general pediatric catheterization population and compare its accuracy with assumed VO<sub>2</sub> (aVO<sub>2</sub>). mVO<sub>2</sub> was recorded for all patients undergoing cardiac catheterization with general anesthesia and controlled ventilation during the study period. mVO<sub>2</sub> was compared to the reference VO<sub>2</sub> (refVO<sub>2</sub>) determined by the reverse Fick method using cardiac MRI (cMRI) or thermodilution (TD) as a reference standard for measurement of C.I. when available. 193 VO<sub>2</sub> measurements were obtained, including 71 with a corresponding cMRI or TD measure of cardiac index for validation. mVO<sub>2</sub> demonstrated satisfactory concordance and correlation with the TD- or cMRI-derived refVO<sub>2</sub> (ρ<sub>c</sub> = 0.73, r<sup>2</sup> = 0.63) with a mean bias of - 3.2% (SD ± 17.3%). Assumed VO<sub>2</sub> demonstrated much weaker concordance and correlation with refVO<sub>2</sub> (ρ<sub>c</sub> = 0.28, r<sup>2</sup> = 0.31) with a mean bias of + 27.5% (SD ± 30.0%). Subgroup analysis of patients < 36 months of age demonstrated that error in mVO<sub>2</sub> was not significantly different from that observed in older patients. Many previously reported prediction models for assuming VO<sub>2</sub> performed poorly in this younger age range. Measured oxygen consumption using the E-sCAiOVX module is significantly more accurate than assumed VO<sub>2</sub> when compared to TD- or cMRI-derived VO<sub>2</sub> in a pediatric catheterization lab.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9882141","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}
引用次数: 0
Comparative Effectiveness of Surgical Ligation and Catheter Closure of Patent Ductus Arteriosus in Preterm Infants. 早产儿动脉导管未闭手术结扎和导管闭合的疗效比较
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2024-10-01 Epub Date: 2023-06-14 DOI: 10.1007/s00246-023-03199-6
Carl Tabb, Sanjeev Aggarwal, Monika Bajaj, Girija Natarajan
{"title":"Comparative Effectiveness of Surgical Ligation and Catheter Closure of Patent Ductus Arteriosus in Preterm Infants.","authors":"Carl Tabb, Sanjeev Aggarwal, Monika Bajaj, Girija Natarajan","doi":"10.1007/s00246-023-03199-6","DOIUrl":"10.1007/s00246-023-03199-6","url":null,"abstract":"<p><p>To evaluate the association, if any, between closure modality (surgical ligation SL vs. catheter CC) of a hemodynamically significant patent ductus arteriosus (PDA), after failure of or contraindication to medical therapy, and immediate procedural complications, and post-procedure physiologic status in preterm (gestational age < 32 weeks) infants. In this single-center retrospective cohort study, data were accessed on infants < 32 weeks gestation, who underwent SL or CC of PDA, born from 2019-2021. The choice of modality was determined by parents, after they were provided information on both procedures. Our cohort (n = 112) included 36 (32.1%) infants who underwent SL while 76 (67.9%) underwent CC. The SL group of infants were significantly more immature at birth, younger on admission to the level IV NICU and received more mean (SD) surfactant doses than the CC group. Higher proportions of infants in the SL group had 5-min Apgar scores ≤ 5, seizures, severe intracranial hemorrhage and had received medical therapy for PDA. Both procedures were highly efficacious, with 1 unsuccessful device placement attempt and had low associated adverse events. Two (2.6%) infants had device migration 24 h after CC. SL was associated with a higher rate of immediate postoperative hypothermia whereas, in the CC group, mean airway pressure was significantly lower 48 h after, compared to before the procedure. SL and CC are comparable in short-term efficacy and safety for PDA closure. Long-term outcomes data are needed following both procedures.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9632309","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}
引用次数: 0
Does High-Intensity Exercise Cause Acute Liver Injury in Patients with Fontan Circulation? A Prospective Pilot Study. 高强度运动会导致丰唐循环患者急性肝损伤吗?一项前瞻性试点研究。
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2024-10-01 Epub Date: 2023-05-23 DOI: 10.1007/s00246-023-03193-y
Alexis Gumm, Salil Ginde, George Hoffman, Melodee Liegl, Cara Mack, Pippa Simpson, Nghia Vo, Grzegorz Telega, Bernadette Vitola, Ankur Chugh
{"title":"Does High-Intensity Exercise Cause Acute Liver Injury in Patients with Fontan Circulation? A Prospective Pilot Study.","authors":"Alexis Gumm, Salil Ginde, George Hoffman, Melodee Liegl, Cara Mack, Pippa Simpson, Nghia Vo, Grzegorz Telega, Bernadette Vitola, Ankur Chugh","doi":"10.1007/s00246-023-03193-y","DOIUrl":"10.1007/s00246-023-03193-y","url":null,"abstract":"<p><p>The Fontan procedure results in chronic hepatic congestion and Fontan-associated liver disease (FALD) characterized by progressive liver fibrosis and cirrhosis. Exercise is recommended in this population, but may accelerate the progression of FALD from abrupt elevations in central venous pressure. The aim of this study was to assess if acute liver injury occurs after high-intensity exercise in patients with Fontan physiology. Ten patients were enrolled. Nine had normal systolic ventricular function and one had an ejection fraction < 40%. During cardiopulmonary exercise testing, patients had near-infrared spectroscopy (NIRS) to measure oxygen saturation of multiple organs, including the liver, and underwent pre- and post-exercise testing with liver elastography, laboratory markers, and cytokines to assess liver injury. The hepatic and renal NIRS showed a statistically significant decrease in oxygenation during exercise, and the hepatic NIRS had the slowest recovery compared to renal, cerebral, and peripheral muscle NIRS. A clinically significant increase in shear wave velocity occurred after exercise testing only in the one patient with systolic dysfunction. There was a statistically significant, albeit trivial, increase in ALT and GGT after exercise. Fibrogenic cytokines traditionally associated with FALD did not increase significantly in our cohort; however, pro-inflammatory cytokines that predispose to fibrogenesis did significantly rise during exercise. Although patients with Fontan circulation demonstrated a significant reduction in hepatic tissue oxygenation based on NIRS saturations during exercise, there was no clinical evidence of acute increase in liver congestion or acute liver injury following high-intensity exercise.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9507575","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}
引用次数: 0
Contemporary Outcomes in Fetuses Diagnosed with Vascular Rings. 确诊患有血管环的胎儿的当代结局
IF 1.5 4区 医学
Pediatric Cardiology Pub Date : 2024-10-01 Epub Date: 2023-06-24 DOI: 10.1007/s00246-023-03219-5
Ian Milligan, William Border, Ritu Sachdeva, Erik Michelfelder
{"title":"Contemporary Outcomes in Fetuses Diagnosed with Vascular Rings.","authors":"Ian Milligan, William Border, Ritu Sachdeva, Erik Michelfelder","doi":"10.1007/s00246-023-03219-5","DOIUrl":"10.1007/s00246-023-03219-5","url":null,"abstract":"<p><p>Vascular rings are increasingly identified on fetal echocardiography. The purpose of this study is to analyze clinical outcomes and patterns of diagnostic testing in fetuses with vascular rings diagnosed by echocardiography. A retrospective cohort study was performed of fetuses with postnatally confirmed vascular rings from 2017 to 2022. Clinical outcomes included type and timing of symptoms, and timing of surgical intervention. Freedom from symptoms and/or surgery was assessed by Kaplan-Meier analysis. Frequency of genetic and diagnostic testing (barium esophagogram, CT/MRI angiogram, and bronchoscopy) was also assessed. Overall, 46 patients were evaluated (91% with a right aortic arch/left ductus and 4% with a double aortic arch). A vascular ring was isolated in 59%, associated with structural heart lesions in 33%, and associated with noncardiac anomalies in 8%. Prenatal diagnoses increased over time. Symptoms developed in 24% (11/46); 82% (9/11) had respiratory and 45% (5/11) had gastroesophageal complaints. Surgery was performed in 17% (11/46). Symptoms presented bimodally, prior to 100 or after 400 days of life. There was no difference in the type of symptoms for early (< 100 days) or late (> 400 days) presenters. Symptomatic patients received more diagnostic testing. Genetic testing was obtained in 46% and positive in 33%, with 22q11 deletion and Trisomy 21 being identified. Prenatal diagnoses of vascular rings increased over time, with subjects developing symptoms bimodally in early or late infancy. The frequency of genetic testing was suboptimal given the prevalence of genetic abnormalities seen in this population.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10054528","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}
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