Article
Obstetrics & Gynecology
Shannon L. Son, Lauren L. Hosek, Miranda C. Stein, Amanda A. Allshouse, Anna B. Catino, Arvind K. Hoskoppal, Daniel A. Cox, Kevin J. Whitehead, Ian M. Lindsay, Sean Esplin, Torri D. Metz
Summary: This retrospective longitudinal cohort study found an association between pregnancy and subsequent long-term adverse cardiovascular outcomes in individuals with congenital heart disease. These findings can provide important information for counseling and guidance of individuals with congenital heart disease who are considering pregnancy.
AMERICAN JOURNAL OF OBSTETRICS AND GYNECOLOGY
(2022)
Article
Cardiac & Cardiovascular Systems
Syed Shahyan Bakhtiyar, Sara Sakowitz, Konmal Ali, Nikhil L. Chervu, Arjun Verma, Ming-Sing Si, David D'Alessandro, Peyman Benharash
Summary: The study evaluates the survival rate after heart transplantation in adults with single and biventricular CHD and compares it to that of non-CHD transplant recipients. Single-ventricle physiology is associated with higher short-term mortality, but 10-year conditional survival is similar for biventricular CHD patients and most single-ventricle CHD patients, and notably better for biventricular CHD patients compared to non-CHD heart transplant recipients.
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY
(2023)
Article
Cardiac & Cardiovascular Systems
Jacob C. Scherba, Ravi Karra, Joseph W. Turek, Nenad Bursac
Summary: Human cardiac organoid systems are of great importance for studying the mechanism of early heart development and gaining a better understanding of congenital cardiac disease.
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2022)
Article
Anesthesiology
Viviane G. Nasr, Steven J. Staffa, David F. Vener, ShengXiang Huang, Morgan L. Brown, Mark Twite, Wanda C. Miller-Hance, James A. DiNardo
Summary: This study analyzes the distribution of pediatric cardiac anesthesiologists in different types of procedures and found that the majority of practicing doctors in this field have not completed fellowship training. With increasing patient survival and the retirement of workforce, there will be a continued need for subspecialty training.
ANESTHESIA AND ANALGESIA
(2022)
Article
Cardiac & Cardiovascular Systems
Mohamed F. Elsisy, Joseph A. Dearani, Juan A. Crestanello, Elena A. Ashikhmina, Charlotte S. Van Dorn, Elizabeth H. Stephens
Summary: This study found that delayed sternal closure (DSC) yielded better outcomes for hemodynamic instability and severe coagulopathy after complex congenital heart surgery compared to perioperative sternal reopening.
ANNALS OF THORACIC SURGERY
(2022)
Article
Biology
Fei Liang, Bo Wang, Juan Geng, Guoling You, Jingjing Fa, Min Zhang, Hunying Sun, Huiwen Chen, Qihua Fu, Xiaoqing Zhang, Zhen Zhang
Summary: Chromosome 4q deletion is common in congenital heart disease patients, and the SORBS2 gene within this interval plays a role in SHF development, with its variants contributing to CHD pathogenesis. Knockdown of SORBS2 in human embryonic stem cells causes cardiomyocyte differentiation defects, which can be rescued by exogenous SHH, and Sorbs2(-/-) mouse mutants exhibit atrial septal anomalies associated with paradoxical thromboembolism.
Article
Obstetrics & Gynecology
Luis D. Pacheco, George Saade, Vineet Shrivastava, Raj Shree, Uri Elkayam
Summary: Pregnancy in individuals with a mechanical heart valve is classified as very high risk, requiring special anticoagulation management to prevent complications and mortality. Adjustments to anticoagulation regimens may be made based on valve type, trimester, individual risk tolerance, and timing of delivery.
AMERICAN JOURNAL OF OBSTETRICS AND GYNECOLOGY
(2022)
Article
Cell Biology
Jose M. Inacio, Mafalda M. Nunes, Micael Almeida, Fernando Cristo, Rui Anjos, Jose A. Belo
Summary: Using hiPSCs as a disease model, this study investigated the effects of DAND5 on human CM differentiation and proliferation. The results showed that the absence of DAND5 promotes hiPSC-CM proliferation and alters their electrophysiological maturation properties. This research helps to elucidate the molecular mechanism underlying congenital heart disease and may lead to new therapies for adult CHD.
Article
Pediatrics
Julie Molin, Maria Munk Paerregaard, Christian Pihl, Caroline Boye Thygesen, Adrian Pietersen, Sofie Dannesbo, Jakob Boesgaard Norsk, Anna Axelsson Raja, Ruth Ottilia B. Vogg, Anne-Sophie Sillesen, Kasper Karmark Iversen, Henning Bundgaard, Alex Horby Christensen
Summary: This study aimed to evaluate the cardiac findings in newborn twins from the general population and investigate whether systematic evaluation of cardiac parameters is necessary for newborn twins. The results showed that compared to singletons, twins had a higher prevalence of non-severe structural heart disease, such as ventricular septal defects, and some differences in cardiac parameters. However, after considering potential confounding factors, the effect of twinning on cardiac parameters was relatively small.
Article
Cardiac & Cardiovascular Systems
Chetna K. Pande, Lisa Noll, Natasha Afonso, Faridis Serrano, Sonia Monteiro, Danielle Guffey, Kriti Puri, Barbara-Jo Achuff, Ayse Akcan-Arikan, Lara Shekerdemian
Summary: This study explores the association between cardiac surgery-associated acute kidney injury (CS-AKI) and neurodevelopmental delay in infants undergoing surgery for congenital heart disease. Infants with recurrent CS-AKI in the first year of life are more likely to experience delays in neurodevelopment and have lower scores in cognitive, language, and motor domains.
ANNALS OF THORACIC SURGERY
(2022)
Review
Pediatrics
Francesca Sperotto, Addison Gearhart, Aparna Hoskote, Peta M. A. Alexander, Jessica A. Barreto, Victoria Habet, Eleonore Valencia, Ravi R. Thiagarajan
Summary: Children with cardiac disease are at a higher risk of cardiac arrest, with limited knowledge on resuscitation strategies based on physiology. This review aims to summarize the current recommendations, highlight knowledge gaps, and emphasize the need for further research to improve care and outcomes for this high-risk population. Factors associated with cardiac arrest and mortality include younger age, lower weight, genetic syndrome, and factors related to resources and institutional characteristics.
EUROPEAN JOURNAL OF PEDIATRICS
(2023)
Article
Cardiac & Cardiovascular Systems
Catherine G. Williamson, Shayan Ebrahimian, Nameer Ascandar, Yas Sanaiha, Sara Sakowitz, Reshma M. Biniwale, Peyman Benharash
Summary: This study assessed the impact of congenital heart disease (CHD) on resource utilization and clinical outcomes in patients undergoing major elective non-cardiac operations. The results showed that patients with CHD had higher rates of in-hospital mortality, longer hospitalization durations, higher costs, and increased risks of complications and readmission.
Article
Food Science & Technology
Lu Zhang, Yanyan Yang, Lin Zhang, Jianmin Ma, Ruicong Sun, Yu Tian, Xiaoli Yuan, Bingyu Liu, Tao Yu, Zhirong Jiang
Summary: Formaldehyde exposure during pregnancy can cause fetal congenital heart disease (CHD). High-throughput sequencing was used to identify the expression profiles of long non-coding RNAs (lncRNAs) in heart tissues of normal and formaldehyde-exposed newborn rats. A total of 763 differentially expressed lncRNAs were identified, and the Ras and hedgehog signaling pathways were identified as important regulatory pathways in CHD caused by formaldehyde exposure. Several upregulated lncRNAs were validated and may provide a reference for diagnosing and treating CHD.
FOOD AND CHEMICAL TOXICOLOGY
(2023)
Article
Public, Environmental & Occupational Health
Mazin Barry, Syed Abdul Bari, Muhammad Yasin Akhtar, Faizah Al Nahdi, Richilda Erlandez, Abdullah Al Khushail, Yahya Al Hebaishi
Summary: This study investigated the clinical and microbiological characteristics of infective endocarditis in a cardiac center in Saudi Arabia over a 5-year period, revealing common symptoms of fever and murmurs, and the most prevalent pathogens being Staphylococcus aureus and Streptococcus viridans. Surgical intervention was performed in 26% of cases, with an overall in-hospital mortality rate of 6.76%.
JOURNAL OF EPIDEMIOLOGY AND GLOBAL HEALTH
(2021)
Review
Cardiac & Cardiovascular Systems
Nazia Hilal, Zi Chen, Ming Hui Chen, Sangita Choudhury
Summary: RAS proteins act as binary switches in signaling, but can be permanently activated in diseases like cancer and RASopathies due to mutations. The exact mechanisms of RAS protein regulation are still unclear. RAS/MAPK syndromes are developmental disorders caused by genetic mutations in genes associated with the RAS/mitogen-activated protein kinase pathway, affecting 1 in 1,000-2,500 children. These syndromes have overlapping features and an association with cardiomyopathy and congenital heart disease. Despite progress in understanding RASopathies, there are still unidentified causal genes in patients diagnosed with RASopathies.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2023)
Article
Clinical Neurology
Celine Thibault, Maryam Y. Naim, Nicholas S. Abend, Daniel J. Licht, J. William Gaynor, Rui Xiao, Shavonne L. Massey
Article
Pediatrics
Martin J. Elliott, Derek Roebuck, Nagarajan Muthialu, Richard Hewitt, Colin Wallis, Paolo DeCoppi, Denise Macintyre, Clare Ann McLaren
FRONTIERS IN PEDIATRICS
(2021)
Article
Cardiac & Cardiovascular Systems
Jeffrey P. Jacobs, Rodney C. G. Franklin, Marie J. Beland, Diane E. Spicer, Steven D. Colan, Henry L. Walters, Frederique Bailliard, Lucile Houyel, James D. St Louis, Leo Lopez, Vera D. Aiello, J. William Gaynor, Otto N. Krogmann, Hiromi Kurosawa, Bohdan J. Maruszewski, Giovanni Stellin, Paul Morris Weinberg, Marshall Lewis Jacobs, Jeffrey R. Boris, Meryl S. Cohen, Allen D. Everett, Jorge M. Giroud, Kristine J. Guleserian, Marina L. Hughes, Amy L. Juraszek, Stephen P. Seslar, Charles W. Shepard, Shubhika Srivastava, Andrew C. Cook, Adrian Crucean, Lazaro E. Hernandez, Rohit S. Loomba, Lindsay S. Rogers, Stephen P. Sanders, Jill J. Savla, Elif Seda Selamet Tierney, Justin T. Tretter, Lianyi Wang, Martin J. Elliott, Constantine Mavroudis, Christo Tchervenkov
Summary: Significant progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care, with the development of the IPCCC ICD-11 Nomenclature by ISNPCHD in collaboration with WHO. This unified approach harmonizes clinical and administrative terminology for better global understanding and communication within the field.
CARDIOLOGY IN THE YOUNG
(2021)
Review
Cardiac & Cardiovascular Systems
Jeffrey P. Jacobs, Rodney C. G. Franklin, Marie J. Beland, Diane E. Spicer, Steven D. Colan, Henry L. Walters III, Frederique Bailliard, Lucile Houyel, James D. St. Louis, Leo Lopez, Vera D. Aiello, J. William Gaynor, Otto N. Krogmann, Hiromi Kurosawa, Bohdan J. Maruszewski, Giovanni Stellin, Paul Morris Weinberg, Marshall Lewis Jacobs, Jeffrey R. Boris, Meryl S. Cohen, Allen D. Everett, Jorge M. Giroud, Kristine J. Guleserian, Marina L. Hughes, Amy L. Juraszek, Stephen P. Seslar, Charles W. Shepard, Shubhika Srivastava, Andrew C. Cook, Adrian Crucean, Lazaro E. Hernandez, Rohit S. Loomba, Lindsay S. Rogers, Stephen P. Sanders, Jill J. Savla, Elif Seda Selamet Tierney, Justin T. Tretter, Lianyi Wang, Martin J. Elliott, Constantine Mavroudis, Christo I. Tchervenkov
Summary: Substantial progress has been made in standardizing the nomenclature for paediatric and congenital cardiac care, with the International Paediatric and Congenital Cardiac Code (IPCCC) now being a component of the Eleventh Revision of the International Classification of Diseases (ICD-11) worldwide. The collaboration between ISNPCHD and WHO has resulted in the harmonization of clinical and administrative nomenclature in the field, with a total of 367 paediatric and congenital cardiac terms now included in ICD-11.
WORLD JOURNAL FOR PEDIATRIC AND CONGENITAL HEART SURGERY
(2021)
Article
Pediatrics
Whitney L. Quong, Neil Bulstrode, Arun Beeman, Madhavan Ramaswamy, Brannavan Sivakumar, Colin Wallis, Martin J. Elliott, Nagarajan Muthialu
Summary: This study retrospectively analyzed the clinical recovery and functional benefits of children who underwent placement of intrathoracic prosthesis. The results showed that intrathoracic prosthesis placement improved respiratory conditions and quality of life in children with congenital single lung or postpneumonectomy PPS.
JOURNAL OF PEDIATRIC SURGERY
(2022)
Review
Cardiac & Cardiovascular Systems
Joanna Catherine Ee-Sien Lim, Martin John Elliott, John Wallwork, Bruce Keogh
Summary: The success of cardiac surgery has greatly improved the survival rate of children with congenital heart disease. This article reviews the significant contributions made by British pioneers in this field and discusses the challenges for the future, particularly in managing and reducing the adverse sequelae of congenital cardiac surgery through safe innovation.
Article
Cardiac & Cardiovascular Systems
Priscilla Alderson, Marc Cohen, Ben Davies, Martin J. Elliott, Mae Johnson, Alessandra Lotteria, Rosa Mendizabal, Emma Stockton, Michael Stylianou, Katy Sutcliffe, Hugo Wellesley
Summary: The study aimed to summarize the main themes in the literature about pediatric anesthesia and compare them with actual practice. The findings showed varying views in medical, ethics, and English legal literature regarding children's consent and refusal, while healthcare professionals in the hospitals tended to inform young children and respect their choices.
JOURNAL OF CARDIOTHORACIC SURGERY
(2022)
Biographical-Item
Cardiac & Cardiovascular Systems
Martin J. Elliott, John E. Deanfield
Summary: This manuscript is a personal tribute to Professor Marc de Leval from two of his colleagues, highlighting his personal qualities and career history. Marc was not only a cardiologist's surgeon, but also a surgeon's cardiologist, bridging the two disciplines and prioritizing the well-being of his patients. He aimed to minimize the need for surgery and embraced interventional cardiology as a beneficial approach.
CARDIOLOGY IN THE YOUNG
(2022)
Biographical-Item
Cardiac & Cardiovascular Systems
Martin J. Elliott, John E. Deanfield
WORLD JOURNAL FOR PEDIATRIC AND CONGENITAL HEART SURGERY
(2022)
Article
Multidisciplinary Sciences
J. William Gaynor, Nancy B. Burnham, Richard F. Ittenbach, Marsha Gerdes, Judy C. Bernbaum, Elaine Zackai, Daniel J. Licht, William W. Russell, Erin E. Zullo, Thomas Miller, Hakon Hakonarson, Kayan A. Clarke, Gail P. Jarvik, Antonia M. Calafat, Asa Bradman, David C. Bellinger, Frederick M. Henretig, Eric S. Coker
Summary: This study investigated the impact of environmental chemical exposures on neurodevelopmental outcomes in toddlers with congenital heart defects after cardiac surgery. The findings suggest that infants with congenital heart defects, like other children, are exposed to complex mixtures of environmental chemicals in daily life. Higher exposure biomarker concentrations were associated with significantly worse performance for language and motor skills in this population.
Article
Surgery
Jonathan B. Edelson, Xuemei Zhang, Andrew B. Goldstone, Joseph W. Rossano, Matthew J. O'Connor, J. William Gaynor, Jonathan J. Edwards, Carol Wittlieb-Weber, Katsuhide Maeda
Summary: Rejection remains a primary cause of graft loss after heart transplant. Combining heart transplant with kidney or liver transplant reduces the risk of rejection compared to isolated heart transplant.
CLINICAL TRANSPLANTATION
(2023)
Article
Medicine, Research & Experimental
Richard F. Ittenbach, J. William Gaynor, Jenny M. Dorich, Nancy B. Burnham, Guixia Huang, Madisen T. Harvey, Jeremy J. Corsmo
Summary: The purpose of this study was to establish a new measure of understanding of informed consent for use in clinical research. A total of 109 teens/young adults completed the consenting process of a hypothetical biobanking study. The final uConsent scale consisted of 19 items that mapped directly onto the Basic Elements of Informed Consent. The scale showed good reliability and validity.
CTS-CLINICAL AND TRANSLATIONAL SCIENCE
(2023)
Article
Genetics & Heredity
Helen M. Stanley, Brian R. White, Christopher J. LaRosa, Mark W. Cocalis, J. William Gaynor, Alanna Strong, Balram Gangaram
Summary: Pathogenic variants in PRDM6 have been found to be etiologically associated with non-syndromic PDA. We present three patients with PDA and CoA found to harbor PRDM6 variants, including a novel, likely-pathogenic variant.
AMERICAN JOURNAL OF MEDICAL GENETICS PART A
(2023)
Article
Biochemistry & Molecular Biology
Emilie J. Benson, Danielle I. Aronowitz, Rodrigo M. Forti, Alec Lafontant, Nicolina R. Ranieri, Jonathan P. Starr, Richard W. Melchior, Alistair Lewis, Jharna Jahnavi, Jake Breimann, Bohyun Yun, Gerard H. Laurent, Jennifer M. Lynch, Brian R. White, J. William Gaynor, Daniel J. Licht, Arjun G. Yodh, Todd J. Kilbaugh, Constantine D. Mavroudis, Wesley B. Baker, Tiffany S. Ko
Summary: The effects of cardiopulmonary bypass on cerebral oxygen supply and metabolic demands during neonatal congenital heart surgery are generally unknown. However, a study using swine models found that following the initiation of cardiopulmonary bypass, oxygen extraction fraction increased while cerebral blood flow and oxygen metabolism decreased, and this persisted for up to 8 hours after cardiopulmonary bypass.
Article
Genetics & Heredity
Alyssa L. Rippert, Sarah Trackman, Danielle Burstein, J. William Gaynor, Heather Griffis, Christine Seymour, Rebecca Ahrens-Nicklas
Summary: This study aimed to assess the association between CB scores and clinical outcomes in pediatric CM patients. The results showed no significant association between CB scores and clinical outcomes in pediatric CM patients. The study highlights the deficits in variant interpretation for pediatric CM and cautions the use of this tool for stratifying clinical outcomes in the pediatric population.