Jamie K Harrington, Michael P DiLorenzo, Monica Bhatia, Nicholas Boscamp, Usha S Krishnan
{"title":"Assessment of Biventricular Systolic and Diastolic Function Using Conventional and Strain Echocardiography in Children with Sickle Cell Disease Surviving 1-year After Hematopoietic Stem Cell Transplant.","authors":"Jamie K Harrington, Michael P DiLorenzo, Monica Bhatia, Nicholas Boscamp, Usha S Krishnan","doi":"10.1007/s00246-024-03646-y","DOIUrl":"https://doi.org/10.1007/s00246-024-03646-y","url":null,"abstract":"<p><p>Hematopoietic stem cell transplant (HSCT) is a potentially curative therapy for children with sickle cell disease (SCD). The effects of HSCT on ventricular function are not well characterized in children with SCD. Echocardiograms from children with SCD who underwent HSCT between 2007 and 2017 were retrospectively analyzed before and 1-year after HSCT. Left ventricular (LV) volumes, mass, and ejection fraction were calculated by the 5/6 area*length method. LV end-diastolic and systolic dimensions, septal, and posterior wall thickness, and fractional shortening were measured by M-mode. Mitral and tricuspid inflow Dopplers (E and A waves) as well as mitral, tricuspid, and septal tissue Dopplers (E', A') were assessed. E/A, E'/A' and E/E' ratios were calculated. Biventricular strain imaging was performed using speckle-tracking echocardiography. Peak global systolic longitudinal and circumferential LV strain, and global longitudinal right ventricular strain, as well as early and late diastolic strain rate, were measured on LV apical 4-chamber, LV short-axis mid-papillary, and RV apical views, respectively. Forty-seven children (9.7 ± 5.5 years, 60% male) met inclusion criteria. Pre-HSCT, subjects had mild LV dilation with normal LV systolic function by conventional measure of ejection fraction and fractional shortening. There was a significant reduction in LV volume, mass, and ejection fraction after HSCT, but measurements remained within normal range. LV longitudinal and circumferential strain were normal pre-HSCT and showed no significant change post-HSCT. RV strain decreased after HSCT, but the absolute change was small, and mean values were normal both pre- and post-HSCT. Conventional measures of diastolic function were all normal pre-HSCT. Post-HSCT there was a reduction in select parameters, but all parameters remained within normal range. Early and late diastolic strain rate parameters showed no significant change from pre- to post-HSCT. At one-year after HSCT in children with SCD conventional measures of systolic and diastolic function are within normal limits. Except for a small decrease in RV systolic strain with values remaining within normal limits, systolic strain and diastolic strain rate values did not significantly change 1-year after HSCT.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142372474","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}
Megan M Wilde, Kurt R Schumacher, Sunkyung Yu, Ray Lowery, Jessica Stoscup, Karen Uzark, Heang M Lim
{"title":"Identifying Frailty and Its Risk Factors in Pediatric Patients with Fontan Physiology.","authors":"Megan M Wilde, Kurt R Schumacher, Sunkyung Yu, Ray Lowery, Jessica Stoscup, Karen Uzark, Heang M Lim","doi":"10.1007/s00246-024-03661-z","DOIUrl":"https://doi.org/10.1007/s00246-024-03661-z","url":null,"abstract":"<p><p>Frailty is a clinical syndrome common in adults with chronic disease with resultant vulnerability to adverse health outcomes. Little is known about frailty in pediatric patients, including those with single-ventricle heart disease. This study aimed to examine the prevalence of frailty and its associated risk factors in patients with Fontan circulation. A single-center, prospective cohort study assessed frailty in patients (10-21 years old) after Fontan palliation. Slowness, weakness, exhaustion, shrinkage, and diminished physical activity were evaluated and scored using a modified Fried frailty assessment comprised of validated pediatric tests. Providers estimated subjects' degree of frailty. Patient-reported quality of life (QOL) was assessed. Of 54 participants (median age 15.3 years, 61% male), 18 (33%) were identified as frail, while 26 (48%) were pre-frail. Patients frequently exhibited frailty in the domains of slowness (93%), weakness (41%), and diminished physical activity (39%). There was poor correlation between frailty scores and provider estimates of frailty (Kappa = 0.11). Frail subjects had lower PedsQL physical functioning scores (mean 62.8 ± SD 18.5 in Frail vs. 75.7 ± 16.0 in No/pre-Frail; p = 0.01). Factors associated with frailty included protein-losing enteropathy (p = 0.03) and at least one hospitalization in the last year (p = 0.047). One-third of pediatric patients after Fontan palliation were frail which was associated with lower physical functioning and higher healthcare utilization. Providers poorly recognized frailty. These findings highlight the need for improved screening and support for an at-risk population where frailty is not easily identified.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142375716","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 Effect of Stenting on Post-Stenotic Dilatation in Coarctation of The Aorta.","authors":"Elchanan Bruckheimer, Nili Schamroth Pravda, Yuval Barak-Corren, Alexander Lowenthal, Gabi Amir, Roee Arnon, Idan Shaul, Tamir Dagan, Einat Birk","doi":"10.1007/s00246-024-03664-w","DOIUrl":"https://doi.org/10.1007/s00246-024-03664-w","url":null,"abstract":"<p><p>The effect of stenting of native aortic coarctation (CoA) on post-stenotic dilatation (PSD) has not been previously described. We hypothesized that CoA stenting may lead to positive remodeling of PSD. Retrospective analysis of patients who underwent stent implantation for native CoA from 1999 to 2021 was performed. Primary outcome was incremental change PSD diameter and the PSD/DescAo (Descending Aorta) following stent implantation and comparison between covered and bare-metal stents. 90 consecutive patients, (26 female, average age at first intervention 12.0 years) were included. 35 patients (38.9%) underwent dilatation with bare-metal stents and 55 patients (61.1%) with covered stents. The covered stent subgroup was older (14.0 vs. 9.2 years old, p < 0.001) and PSD was larger (17.0 vs 14.0 mm, p < 0.001). Over a mean of 3.2 years, mean inter-catheterization growth of the PSD was blunted [- 0.05 mm, 95% CI (- 1.5 to 1.4)]. The covered stent subgroup demonstrated a negative inter-catheterization growth compared to the bare-metal stent subgroup (- 0.7 vs 1.6 mm, p < 0.001). When controlled for somatic growth, the PDS/DescAo decreased more significantly among those with covered stent vs bare metal (- 0.12 vs - 0.058, p = 0.004). Stenting of native CoA blunts the growth of PSD; covered stents were significantly associated with regression of the diameter of the PSD over time compared to bare-metal stents.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142366118","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}
Pediatric CardiologyPub Date : 2024-10-01Epub Date: 2023-05-20DOI: 10.1007/s00246-023-03182-1
Jamie Penk, Will Cagle, Adrian Holloway, Julie Connolly, Alison Miles, Rupal Bhakta, Parag Jain, Sarah Prochaska Davis, Christine Riley, Dantin Jeramy Roddy
{"title":"Multicenter Evaluation of Pre-operative Feeding in Infants with Ductal Dependent Circulation.","authors":"Jamie Penk, Will Cagle, Adrian Holloway, Julie Connolly, Alison Miles, Rupal Bhakta, Parag Jain, Sarah Prochaska Davis, Christine Riley, Dantin Jeramy Roddy","doi":"10.1007/s00246-023-03182-1","DOIUrl":"10.1007/s00246-023-03182-1","url":null,"abstract":"<p><p>Enteral feeds are often withheld from neonates with ductal dependent cardiac lesions who are receiving prostaglandins. This is despite positive benefits of enteral feeding. We describe a multicenter cohort of these neonates who were fed pre-operatively. We also give a granular description of vital sign measurements and other risk factors prior to feeding. A retrospective chart review was performed at seven centers. Inclusion criteria were full-term neonates under one month of age with ductal dependent lesions receiving prostaglandins. These neonates were fed for at least 24 h during the pre-operative period. Premature neonates were excluded. Using the inclusion criteria, 127 neonates were identified. While being fed, 20.5% of the neonates were intubated, 10.2% were on inotropes, and 55.9% had an umbilical arterial catheter in place. Median oxygen saturations in the six hours prior to feeding were 92.5% in patients with cyanotic lesions, median diastolic blood pressure was 38 mmHg and median somatic NIRS were 66.5%. The median peak daily feeding volume reached was 29 ml/kg/day (IQ range 15.5-96.8 ml/kg/day). One patient developed suspected necrotizing enterocolitis (NEC) in this cohort. Only one adverse event occurred, which was an aspiration thought to be related to feeding, but did not result in intubation or cessation of feeds. NEC was rare among neonates with ductal dependent lesions while receiving enteral nutrition pre-operatively. Umbilical arterial catheters were in place in the majority of these patients. Hemodynamic measures demonstrated a high median oxygen saturation prior to initiation of feeds.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1461-1465"},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9496001","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}
Pediatric CardiologyPub Date : 2024-10-01Epub Date: 2023-08-22DOI: 10.1007/s00246-023-03270-2
Ernesto Mejia, Natalie Soszyn, Gareth J Morgan, Ryan A Leahy
{"title":"Radiofrequency Perforation of an Atretic Pulmonary Valve with a Modified Coronary Wire and Electrocautery Pencil.","authors":"Ernesto Mejia, Natalie Soszyn, Gareth J Morgan, Ryan A Leahy","doi":"10.1007/s00246-023-03270-2","DOIUrl":"10.1007/s00246-023-03270-2","url":null,"abstract":"<p><p>Radiofrequency (RF) perforation of an atretic pulmonary valve is commonly performed in patients with pulmonary atresia with intact ventricular septum with specifically designed RF wires. In difficult anatomy or low-resource centers, this may instead be successfully performed with a modified coronary guide wire and an electrocautery surgical pencil.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1607-1609"},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10042606","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}
Pediatric CardiologyPub Date : 2024-10-01Epub Date: 2023-05-27DOI: 10.1007/s00246-023-03179-w
Aditya P Desai, Mark H Hoyer
{"title":"Coarctation Angioplasty in Young Children: Does IMPACT Registry Data Accurately Reflect Clinical Outcomes?","authors":"Aditya P Desai, Mark H Hoyer","doi":"10.1007/s00246-023-03179-w","DOIUrl":"10.1007/s00246-023-03179-w","url":null,"abstract":"<p><p>Balloon angioplasty for recoarctation of the aorta is routinely performed in young children and is considered successful when the systolic gradient is reduced to < 10 mmHg. IMPACT defines acute procedural success solely based on a final gradient of < 10 mmHg and stratifies participating institutions based on these acute outcomes. Between February 2012 and December 2020, IMPACT data was analyzed for 110 coarctation interventions. Electronic medical records were reviewed, and primary endpoints were one of the following: (1) final analysis end date (June 2021), (2) patient death, or (3) most recent transcatheter or surgical reintervention. 64 (58.2%) interventions had a post-procedure CA gradient < 10 mmHg. Comparison of clinical patient outcome for acute success demonstrated no significant relationship using IMPACT (p = 0.70) criteria. There was no statistically significant difference between clinical success and failure for: pre- and post-treatment systolic gradients; absolute or percent change in systolic gradient; and pre-treatment aorta diameter. Clinical outcome and patient age did show a significant difference (p = 0.0093) with better clinical outcomes in older patients. Our analysis did not reveal a statistically significant difference between IMPACT criteria for successful treatment of CA and clinical outcome. These findings underscore a need to identify other clinical metrics that better predict outcome following CA balloon angioplasty treatment.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1455-1460"},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9882139","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}
Pediatric CardiologyPub Date : 2024-10-01Epub Date: 2024-07-22DOI: 10.1007/s00246-024-03593-8
Ulrich Krause
{"title":"Rebuttal to \"Comment on: Fate After the Mustard Procedure for d-Transposition of the Great Arteries: Impact of Age, Complexity, and Atrial Tachyarrhythmias: A Single Center Experience\" by Iman Akram.","authors":"Ulrich Krause","doi":"10.1007/s00246-024-03593-8","DOIUrl":"10.1007/s00246-024-03593-8","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1612"},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11390857/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141734830","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}
Pediatric CardiologyPub Date : 2024-10-01Epub Date: 2023-06-09DOI: 10.1007/s00246-023-03202-0
Elizabeth B Aronoff, Clifford Chin, Alexander R Opotowsky, Wayne A Mays, Sandra K Knecht, Jennah Goessling, Malloree Rice, Justine Shertzer, Samuel G Wittekind, Adam W Powell
{"title":"Facility-Based and Virtual Cardiac Rehabilitation in Young Patients with Heart Disease During the COVID-19 Era.","authors":"Elizabeth B Aronoff, Clifford Chin, Alexander R Opotowsky, Wayne A Mays, Sandra K Knecht, Jennah Goessling, Malloree Rice, Justine Shertzer, Samuel G Wittekind, Adam W Powell","doi":"10.1007/s00246-023-03202-0","DOIUrl":"10.1007/s00246-023-03202-0","url":null,"abstract":"<p><p>Cardiac rehabilitation (CR) is an important tool for improving fitness and quality of life in those with heart disease (HD). Few pediatric centers use CR to care for these patients, and virtual CR is rarely used. In addition, it is unclear how the COVID-19 era has changed CR outcomes. This study assessed fitness improvements in young HD patients participating in both facility-based and virtual CR during the COVID-19 pandemic. This retrospective single-center cohort study included new patients who completed CR from March 2020 through July 2022. CR outcomes included physical, performance, and psychosocial measures. Comparison between serial testing was performed with a paired t test with P < 0.05 was considered significant. Data are reported as mean ± standard deviation. There were 47 patients (19 ± 7.3 years old; 49% male) who completed CR. Improvements were seen in peak oxygen consumption (VO<sub>2</sub>, 62.3 ± 16.1 v 71 ± 18.2% of predicted, p = 0.0007), 6-min walk (6 MW) distance (401 ± 163.8 v 480.7 ± 119.2 m, p = < 0.0001), sit to stand (16.2 ± 4.9 v 22.1 ± 6.6 repetitions; p = < 0.0001), Patient Health Questionnaire-9 (PHQ-9) (5.9 ± 4.3 v 4.4 ± 4.2; p = 0.002), and Physical Component Score (39.9 ± 10.1 v 44.9 ± 8.8; p = 0.002). Facility-based CR enrollees were less likely to complete CR than virtual patients (60%, 33/55 v 80%, 12/15; p = 0.005). Increases in peak VO<sub>2</sub> (60 ± 15.3 v 70.2 ± 17.8% of predicted; p = 0.002) were seen among those that completed facility-based CR; this was not observed in the virtual group. Both groups demonstrated improvement in 6 MW distance, sit-to-stand repetitions, and sit-and-reach distance. Completion of a CR program resulted in fitness improvements during the COVID-19 era regardless of location, although peak VO<sub>2</sub> improved more for the in-person group.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1533-1541"},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10251322/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9618459","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}
Pediatric CardiologyPub Date : 2024-10-01Epub Date: 2023-07-01DOI: 10.1007/s00246-023-03188-9
Conor P O'Halloran, Sandhya Ramlogan, Nazia Husain, Jeremy Fox, Alan W Nugent, Paul Tannous
{"title":"Echocardiogram-Guided Balloon Valvuloplasty of the Aortic Valve in Neonates and Infants Reduces Contrast Exposure with Maintained Efficacy and Less Aortic Regurgitation.","authors":"Conor P O'Halloran, Sandhya Ramlogan, Nazia Husain, Jeremy Fox, Alan W Nugent, Paul Tannous","doi":"10.1007/s00246-023-03188-9","DOIUrl":"10.1007/s00246-023-03188-9","url":null,"abstract":"<p><p>Balloon aortic valvuloplasty (BAV) is performed in children with significant aortic stenosis (AS). Traditionally, contrast angiography measures the annulus and assesses aortic regurgitation (AR) after each dilation. Echocardiographic guidance is hypothesized to reduce contrast and radiation exposure, without compromising efficacy or safety. Patients < 10 kg undergoing BAV from 2013 to 2022 were retrospectively investigated. Agreement between echocardiographic and angiographic annulus measurements was assessed. Echocardiogram-guided (eBAV) and traditional angiogram-guided (tBAV) outcomes were compared controlling for weight, critical AS, and other congenital heart disease (CHD). Twelve eBAV and 19 tBAV procedures were performed. The median age was 33 days, median weight was 4.3 kg, 7 patients (23%) had critical AS, and 9 patients (29%) had other CHD. Annulus measurements by intraprocedural echocardiography and angiography displayed excellent correlation (ICC 0.95, p < 0.001). eBAV patients received less contrast (0.5 vs 3.5 ml/kg, p < 0.01). Five recent eBAV procedures were performed contrast free. Radiation exposure was not statistically different between the eBAV and tBAV groups (155 vs 313 µGy·M<sup>2</sup>, p = 0.12). One eBAV patient (8%) and 3 tBAV patients (16%, p = 0.62) experienced serious adverse events. Technical success (gradient < 35 mmHg and increase in AR by ≤ 1 grade) occurred in 11 eBAV patients (92%) and 16 tBAV patients (84%, p = 0.22). AR increased in 2 eBAV patients (17%) and 8 tBAV patients (44%, p = 0.02). eBAV was associated with similar efficacy and significantly lower contrast exposure and risk of aortic regurgitation. There was strong agreement between aortic valve annulus measurements by intraprocedural echocardiography and angiography, ultimately permitting contrast free BAV.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1485-1492"},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9726891","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}