keyword
https://read.qxmd.com/read/38497452/impaired-relaxation-in-induced-pluripotent-stem-cell-derived-cardiomyocytes-with-pathogenic-tnni3-mutation-of-pediatric-restrictive-cardiomyopathy
#21
JOURNAL ARTICLE
Renjie Wang, Moyu Hasegawa, Hidehiro Suginobe, Chika Yoshihara, Yoichiro Ishii, Atsuko Ueyama, Kazutoshi Ueda, Kazuhisa Hashimoto, Masaki Hirose, Ryo Ishii, Jun Narita, Takuji Watanabe, Takuji Kawamura, Masaki Taira, Takayoshi Ueno, Shigeru Miyagawa, Hidekazu Ishida
BACKGROUND: Restrictive cardiomyopathy (RCM) is characterized by impaired diastolic function with preserved ventricular contraction. Several pathogenic variants in sarcomere genes, including TNNI3 , are reported to cause Ca2+ hypersensitivity in cardiomyocytes in overexpression models; however, the pathophysiology of induced pluripotent stem cell (iPSC)-derived cardiomyocytes specific to a patient with RCM remains unknown. METHODS AND RESULTS: We established an iPSC line from a pediatric patient with RCM and a heterozygous TNNI3 missense variant, c...
March 19, 2024: Journal of the American Heart Association
https://read.qxmd.com/read/38497441/accumulating-sedentary-time-and-physical-activity-from-childhood-to-adolescence-and-cardiac-function-in-adolescence
#22
JOURNAL ARTICLE
Eero A Haapala, Marja H Leppänen, Earric Lee, Kai Savonen, Jari A Laukkanen, Mika Kähönen, Soren Brage, Timo A Lakka
BACKGROUND: Increased physical activity (PA) may mitigate the negative cardiovascular health effects of sedentary behavior in adolescents. However, the relationship of PA and sedentary time from childhood with cardiac function in adolescence remains underexplored. Therefore, we investigated the associations of cumulative sedentary time and PA from childhood to adolescence with cardiac function in adolescence. METHODS AND RESULTS: Participants were 153 adolescents (69 girls) who were aged 6 to 8 years at baseline, 8 to 10 years at 2-year follow-up, and 15 to 17 years at 8-year follow-up...
March 19, 2024: Journal of the American Heart Association
https://read.qxmd.com/read/38497437/predicting-high-risk-fetal-cardiac-disease-anticipated-to-need-immediate-postnatal-stabilization-and-intervention-with-planned-pediatric-cardiac-operating-room-delivery
#23
REVIEW
Amol Moray, Proscovia M Mugaba, Chloe Joynt, Angela McBrien, Luke G Eckersley, Ernest Phillipos, Paula Holinski, Lindsay Ryerson, James Yashu Coe, Sujata Chandra, Billy Wong, Michele Derbyshire, Maria Lefebvre, Mohammed Al Aklabi, Lisa K Hornberger
BACKGROUND: Distances between delivery and cardiac services can make the care of fetuses with cardiac disease at risk of acute cardiorespiratory instability at birth a challenge. In 2013 we implemented a fetal echocardiography-based algorithm targeting fetuses considered high risk for acute cardiorespiratory instability at ≤2 hours of birth for delivery in our pediatric cardiac operating room of our children's hospital, and, herein, examine our experience. METHODS AND RESULTS: We reviewed maternal and postnatal medical records of all fetuses with cardiac disease encountered January 2013 to March 2022 considered high risk for acute cardiorespiratory instability...
March 19, 2024: Journal of the American Heart Association
https://read.qxmd.com/read/38493473/do-children-with-asymptomatic-ventricular-preexcitation-have-similar-quality-of-life-as-healthy-children
#24
JOURNAL ARTICLE
Emilia Szafran, Michał Bartecki, Anna Bukowska-Posadzy, Artur Baszko, Jarosław Walkowiak, Waldemar Bobkowski
BACKGROUND: To our knowledge, no studies have assessed quality of life (QoL) in asymptomatic children with a preexcitation electrocardiogram pattern. AIM: To evaluate the QoL of children with asymptomatic Wolff-Parkinson-White (WPW) syndrome. METHODS: This study involved QoL assessment of 31 children with asymptomatic preexcitation and 82 healthy children using the WHOQOL-BREF and the Pediatric Arrhythmia Related Score (PARS), a specific questionnaire that we have developed, which is related to patients' feelings and observations concerning arrhythmia...
2024: Kardiologia Polska
https://read.qxmd.com/read/38493457/pacemaker-remote-monitoring-challenges-in-pediatric-population-single-center-long-term-experience
#25
JOURNAL ARTICLE
Dagmar Kowal, Artur Baszko, Krzysztof Czyż, Waldemar Bobkowski, Przemyslaw Mitkowski, Błażej Rubiś
No abstract text is available yet for this article.
March 17, 2024: Kardiologia Polska
https://read.qxmd.com/read/38489093/correction-to-safety-and-feasibility-of-skin-to-skin-contact-in-the-delivery-room-for-high-risk-cardiac-neonates
#26
Molly K Ball, R B Seabrook, R Corbitt, C Stiver, K Nardell, A K Medoro, L Beer, A Brown, J Mollica, R Bapat, T Cosgrove, K T Texter
No abstract text is available yet for this article.
March 15, 2024: Pediatric Cardiology
https://read.qxmd.com/read/38489092/computational-fluid-dynamic-assessment-of-patients-with-congenital-heart-disease-from-3d-rotational-angiography
#27
JOURNAL ARTICLE
Jenny E Zablah, Michael J Shorofsky, Kelly Cao, Debanjan Mukherjee
For congenital heart disease patients, multiple imaging modalities are needed to discern anatomy and functional information such as differential blood flow. During cardiac catheterization, 3D rotational angiography (3DRA) can provide CTA-like images, enabling anatomical information and intraprocedural guidance. We seek to establish whether unique aspects of this technique can also generate quantitative functional blood flow information. We propose that systematic integration of 3DRA imaging, catheter hemodynamic information, and computational fluid dynamics (CFD), can provide quantitative information regarding blood flow dynamics and energetics, without additional imaging or procedures...
March 15, 2024: Pediatric Cardiology
https://read.qxmd.com/read/38489091/aspirin-responsiveness-in-a-cohort-of-pediatric-patients-with-right-ventricle-to-pulmonary-artery-conduits-and-transcatheter-valve-replacement-systems
#28
JOURNAL ARTICLE
Sean T Kelleher, Irene E Regan, Dermot Cox, Kathryn Shaw, Orla Franklin, Damien P Kenny, Kevin P Walsh, Colin J McMahon
The aim of this study was to determine the rate of aspirin responsiveness in a cohort of pediatric patients with in situ xenograft valved right ventricle to pulmonary artery (RV-PA) conduits and/or transcatheter valve replacements (TVR). Aspirin is routinely prescribed to these patients. Optimizing anti-platelet therapy could promote valve longevity and reduce the risk of infective endocarditis in this at-risk group. This was a prospective, observational study. Patients were recruited from both ward and outpatient settings...
March 15, 2024: Pediatric Cardiology
https://read.qxmd.com/read/38485760/risk-factors-and-outcomes-associated-with-gaps-in-care-in-children-with-congenital-heart-disease
#29
JOURNAL ARTICLE
Michael B Rosamilia, Jason Williams, Courtney A Bair, Hillary Mulder, Karen E Chiswell, Alfred A D'Ottavio, Robert J Hartman, Charlie J Sang, Karl F Welke, Michael J Walsh, Timothy M Hoffman, Andrew P Landstrom, Jennifer S Li, Lauren A Sarno
Adults with congenital heart disease (CHD) benefit from cardiology follow-up at recommended intervals of ≤ 2 years. However, benefit for children is less clear given limited studies and unclear current guidelines. We hypothesize there are identifiable risks for gaps in cardiology follow-up in children with CHD and that gaps in follow-up are associated with differences in healthcare utilization. Our cohort included children < 10 years old with CHD and a healthcare encounter from 2008 to 2013 at one of four North Carolina (NC) hospitals...
March 14, 2024: Pediatric Cardiology
https://read.qxmd.com/read/38480572/differential-regulation-of-immune-related-genes-in-the-developing-heart
#30
JOURNAL ARTICLE
Mathieu Garand, Susie S Y Huang, Brian Dineen, Ian A Glass, Pirooz Eghtesady
In many congenital heart defects, it can be difficult to ascertain primary pathology from secondary consequences from altered flow through the developing heart. The molecular differences between the growing right and left ventricles (RV and LV, respectively) following the completion of septation and the impact of sex on these mechanisms have not been investigated. We analyzed RNA-seq data derived from twelve RV and LVs, one with Hypoplastic Left Heart Syndrome (HLHS), to compare the transcriptomic landscape between the ventricles during development...
March 13, 2024: Pediatric Cardiology
https://read.qxmd.com/read/38480571/analysis-of-cytokine-profiles-in-pediatric-myocarditis-multicenter-study
#31
JOURNAL ARTICLE
Yoji Nomura, Takanori Suzuki, Katsuyuki Kunida, Hidetoshi Uchida, Ryoichi Ito, Yasunori Oshima, Machiko Kito, Yuki Imai, Satoru Kawai, Kei Kozawa, Kazuyoshi Saito, Tadayoshi Hata, Junichiro Yoshimoto, Tetsushi Yoshikawa, Kazushi Yasuda
Acute myocarditis (AM) is an inflammatory disease of the heart muscle that can progress to fulminant myocarditis (FM), a severe and life-threatening condition. The cytokine profile of myocarditis in children, especially in relation to fulminant myocarditis, is not well understood. This study aims to evaluate the cytokine profiles of acute and fulminant myocarditis in children. Pediatric patients diagnosed with myocarditis were included in the study. Cytokine levels were measured using a multiplexed fluorescent bead-based immunoassay...
March 13, 2024: Pediatric Cardiology
https://read.qxmd.com/read/38480570/cardiac-rhabdomyomas-presenting-with-critical-cardiac-obstruction-in-neonates-and-infants-treatment-strategies-and-outcome-a-single-center-experience
#32
JOURNAL ARTICLE
Li Yen Ng, Jonathan McGuinness, Terence Prendiville, Orla Franklin, Mark Walsh, Damien Kenny, Lars Nolke, Colin J McMahon
Cardiac rhabdomyomas are the most common benign pediatric heart tumor in infancy, which are commonly associated with tuberous sclerosis complex (TSC). Most rhabdomyomas are asymptomatic and spontaneously regress over time. However, some cases especially in neonates or small infants can present with hemodynamic instability. Surgical resection of the tumor, which has been the gold standard in alleviating obstruction, is not always possible and may be associated with significant morbidity and mortality. Recently, mammalian target of rapamycin inhibitors (mTORi) have been shown to be safe and effective in the treatment of TSC...
March 13, 2024: Pediatric Cardiology
https://read.qxmd.com/read/38480569/clinical-outcomes-and-echocardiographic-predictors-of-reintervention-after-interrupted-aortic-arch-repair
#33
JOURNAL ARTICLE
Hannah L McMullen, Jamie K Harrington, David Blitzer, Nikhil Pasumarti, Stéphanie Levasseur, Emile Bacha, David Kalfa
Left ventricular outflow tract obstruction (LVOTO) remains a significant complication after primary repair of interrupted aortic arch with ventricular septal defect (IAA-VSD). Clinical and echocardiographic predictors for LVOTO reoperation are controversial and procedures to prophylactically prevent future LVOTO are not reliable. However, it is important to identify the patients at risk for future LVOTO intervention after repair of IAA-VSD. Patients who underwent single-stage IAA-VSD repair at our center 2006-2021 were retrospectively reviewed, excluding patients with associated cardiac lesions...
March 13, 2024: Pediatric Cardiology
https://read.qxmd.com/read/38478047/morphology-clinical-associations-and-pathologic-follow-up-of-quadricuspid-aortic-valves-in-children
#34
JOURNAL ARTICLE
Omotola Ajayi, Faraz Alizadeh, Sepehr Sekhavat, Kristin Bonello, Rebecca S Beroukhim, Sunil J Ghelani
While quadricuspid morphology is commonly observed in truncal valves, quadricuspid aortic valves (QAV) are rare and their natural history is not well described. This retrospective study of 37 patients describes the diagnostic associations and morphologic variability of QAVs in children (median age at diagnosis 4.3 y IQR 0-12 y). Associated congenital heart diseases were present in 54% (most commonly tetralogy of Fallot (TOF) and valvar pulmonary stenosis). Among patients with isolated QAV, 11 had genetic syndrome and 5 had skeletal anomalies...
March 13, 2024: Pediatric Cardiology
https://read.qxmd.com/read/38472658/socioeconomic-influences-on-outcomes-following-congenital-heart-disease-surgery
#35
JOURNAL ARTICLE
Kristin Schneider, Sarah de Loizaga, Andrew F Beck, David L S Morales, JangDong Seo, Allison Divanovic
Associations between social determinants of health (SDOH) and adverse outcomes for children with congenital heart disease (CHD) are starting to be recognized; however, such links remain understudied. We examined the relationship between community-level material deprivation on mortality, readmission, and length of stay (LOS) for children undergoing surgery for CHD. We performed a retrospective cohort study of patients who underwent cardiac surgery at our institution from 2015 to 2018. A community-level deprivation index (DI), a marker of community material deprivation, was generated to contextualize the lived experience of children with CHD...
March 12, 2024: Pediatric Cardiology
https://read.qxmd.com/read/38467893/variations-in-the-evaluation-and-management-of-vascular-rings-a-survey-of-american-clinicians
#36
JOURNAL ARTICLE
Daniel M Pasternack, Achiau Ludomirsky, Reina B Tan, Cynthia Amirtharaj
Vascular rings are arterial malformations that lead to the compression of the trachea and/or esophagus. While "tight" rings often produce symptoms and require surgery, "loose" rings rarely produce symptoms. Given advances in fetal echocardiography, this diagnosis is now more often made prenatally. This poses a new conundrum in the management of asymptomatic patients, leading to practice variation and creating a target for clinical system improvement. Hence, we conducted this survey aiming to demonstrate the practice variation existing in current evaluation and management of these patients...
March 11, 2024: Pediatric Cardiology
https://read.qxmd.com/read/38465648/cardiovascular-management-of-patients-undergoing-hematopoietic-stem-cell-transplantation-from-pretransplantation-to-survivorship-a-scientific-statement-from-the-american-heart-association
#37
REVIEW
Salim S Hayek, Vlad G Zaha, Carmel Bogle, Anita Deswal, Amelia Langston, Seth Rotz, Alexi Vasbinder, Eric Yang, Tochukwu Okwuosa
Hematopoietic stem cell transplantation can cure various disorders but poses cardiovascular risks, especially for elderly patients and those with cardiovascular diseases. Cardiovascular evaluations are crucial in pretransplantation assessments, but guidelines are lacking. This American Heart Association scientific statement summarizes the data on transplantation-related complications and provides guidance for the cardiovascular management throughout transplantation. Hematopoietic stem cell transplantation consists of 4 phases: pretransplantation workup, conditioning therapy and infusion, immediate posttransplantation period, and long-term survivorship...
March 11, 2024: Circulation
https://read.qxmd.com/read/38456890/obesity-predisposes-anthracycline-treated-survivors-of-childhood-and-adolescent-cancers-to-subclinical-cardiac-dysfunction
#38
JOURNAL ARTICLE
Ian A George, BriAnna Souder, Amy Berkman, David H Noyd, M Jay Campbell, Piers C A Barker, Michael Roth, Michelle A T Hildebrandt, Kevin C Oeffinger, Andrew W McCrary, Andrew P Landstrom
Anthracyclines are effective chemotherapeutics used in approximately 60% of pediatric cancer cases but have a well-documented risk of cardiotoxicity. Existing cardiotoxicity risk calculators do not include cardiovascular risk factors present at the time of diagnosis. The goal of this study is to leverage the advanced sensitivity of strain echocardiography to identify pre-existing risk factors for early subclinical cardiac dysfunction among anthracycline-exposed pediatric patients. We identified 115 pediatric patients with cancer who were treated with an anthracycline between 2013 and 2019...
March 8, 2024: Pediatric Cardiology
https://read.qxmd.com/read/38453700/programmatic-palliative-care-consultations-in-pediatric-heart-transplant-evaluations
#39
JOURNAL ARTICLE
Erika J Mejia, Rui Xiao, Jennifer K Walter, Chris Feudtner, Kimberly Y Lin, Aaron G DeWitt, Meeta Prasad Kerlin
Guidelines advocate for integrating palliative care into the management of heart failure (HF) and of children with life-limiting disease. The potential impact of palliative care integration into pediatric HF on patient-centered outcomes is poorly understood. The present study sought to assess the association of programmatic implementation of palliative care into the heart transplant evaluation process with hospital-free days (HFD) and end of life (EOL) treatment choices. The study included patients less than 19 years of age who underwent a heart transplant evaluation between February 2012 and April 2020 at a single center...
March 8, 2024: Pediatric Cardiology
https://read.qxmd.com/read/38452996/achieving-consensus-severity-graded-definitions-of-fontan-associated-complications-to-characterize-fontan-circulatory-failure
#40
JOURNAL ARTICLE
Kurt R Schumacher, Ari Cedars, Kiona Allen, David Goldberg, Adrianna Batazzi, Garrett Reichle, Frank DiPaola, David Selewski, Melissa Cousino, David N Rosenthal
BACKGROUND: Fontan physiology leads to chronic changes in other organ systems that may affect long-term survival and the success of heart transplantation. Inadequate assessment and treatment of the extra-cardiac effects of Fontan may contribute to poor outcomes. Severity graded/ordinal consensus definitions of Fontan complications are lacking which limits understanding of how Fontan-specific morbidity affects patient outcomes. METHODS AND RESULTS: A panel of Fontan patient and physiology experts, including pediatric, adult congenital, heart failure, and critical care cardiology, and pediatric nephrology, hepatology and psychology, convened to develop definitions of Fontan complications...
March 5, 2024: Journal of Cardiac Failure
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