Article
Cardiac & Cardiovascular Systems
Carsten Rickers, Philip Wegner, Michael Silberbach, Erin Madriago, Dominik Daniel Gabbert, Arash Kheradvar, Inga Voges, Jens Scheewe, Tim Attmann, Michael Jerosch-Herold, Hans-Heiner Kramer
Summary: The coronary microcirculation of the systemic ventricle in young HLHS patients shows significant differences compared with controls. Independent risk factors for reduced hyperemic MBF included HLHS subtype, late gadolinium enhancement, right ventricular diastolic dysfunction, and increasing age at total cavopulmonary connection. The coronary conductance was negatively correlated with systemic blood oxygen saturation.
CIRCULATION-CARDIOVASCULAR IMAGING
(2021)
Article
Cardiac & Cardiovascular Systems
Markus Krane, Martina Dressen, Gianluca Santamaria, Ilaria My, Christine M. Schneider, Tatjana Dorn, Svenja Laue, Elisa Mastantuono, Riccardo Berutti, Hilansi Rawat, Ralf Gilsbach, Pedro Schneider, Harald Lahm, Sascha Schwarz, Stefanie A. Doppler, Sharon Paige, Nazan Puluca, Sophia Doll, Irina Neb, Thomas Brade, Zhong Zhang, Claudia Abou-Ajram, Bernd Northoff, Lesca M. Holdt, Stefanie Sudhop, Makoto Sahara, Alexander Goedel, Andreas Dendorfer, Fleur V. Y. Tjong, Maria E. Rijlaarsdam, Julie Cleuziou, Nora Lang, Christian Kupatt, Connie Bezzina, Rudiger Lange, Neil E. Bowles, Matthias Mann, Bruce D. Gelb, Lia Crotti, Lutz Hein, Thomas Meitinger, Sean Wu, Daniel Sinnecker, Peter J. Gruber, Karl-Ludwig Laugwitz, Alessandra Moretti
Summary: The study revealed disruptions in gene programs and cellular processes in patients with hypoplastic left heart syndrome (HLHS), particularly in key aspects of fetal ventricular cardiogenesis such as the cell cycle and cardiomyocyte maturation. These abnormalities result in defects in early differentiation of cardiac progenitors, leading to left ventricular hypoplasia.
Article
Physiology
Huseyin Enes Salman, Reema Yousef Kamal, Ziyad M. Hijazi, Huseyin Cagatay Yalcin
Summary: Congenital heart defects (CHDs) impact a wide range of societies with an incidence rate of 1.0-1.2%. Genetic factors and mechanobiological forces may both contribute to the initiation and progression of CHDs. Hypoplastic left heart syndrome (HLHS) is a critical CHD, accounting for 25-40% of all prenatal cardiac deaths. Comparing healthy and HLHS hearts helps understand the main hemodynamic differences associated with HLHS.
FRONTIERS IN PHYSIOLOGY
(2022)
Article
Cardiac & Cardiovascular Systems
Jeanne L. Theis, Jessie J. Hu, Rhianna S. Sundsbak, Jared M. Evans, William R. Bamlet, M. Yasir Qureshi, Patrick W. O'Leary, Timothy M. Olson
Summary: This study identified defects in cardiomyopathy-associated genes in patients with HLHS through whole genome sequencing, indicating potential impact on the functional reserve of the single-ventricle circulation.
CIRCULATION-GENOMIC AND PRECISION MEDICINE
(2021)
Article
Developmental Biology
Rebecca L. Wilson, Weston Troja, Jennifer Courtney, Alyssa Williams, Helen N. Jones
Summary: Congenital heart defects are the most common birth defect worldwide and are associated with fetal growth abnormalities. This study reveals that in a mouse model, the genotype Scup130(m/)+Pcdha9(m/+) impairs placental development and function, particularly in males.
Article
Cardiac & Cardiovascular Systems
Angela N. Baybayon-Grandgeorge, Ashley E. Pietra, Shelley D. Miyamoto, Anastacia M. Garcia
Summary: Cardiac dysfunction and heart failure are the leading causes of death in infants and children with hypoplastic left heart syndrome (HLHS) and other congenital heart defects. Current therapies for these conditions are relatively ineffective, necessitating the search for novel treatment strategies.
JOURNAL OF CARDIOVASCULAR DEVELOPMENT AND DISEASE
(2022)
Review
Pediatrics
Walter Knirsch, Alexandra De Silvestro, Michael von Rhein
Summary: Background: Patients with hypoplastic left heart syndrome (HLHS) undergoing staged palliation until Fontan procedure are at risk for impaired neurodevelopmental outcome. The Hybrid procedure with bilateral pulmonary artery banding, ductal stenting, and balloon atrioseptostomy may offer a less invasive stage I procedure compared to the Norwood stage I procedure. This review analyzed neurodevelopmental and functional outcomes after Hybrid procedure as stage I procedure.
FRONTIERS IN PEDIATRICS
(2023)
Article
Cardiac & Cardiovascular Systems
Caitlin Milligan, Kimberly I. Mills, Shirley Ge, Anna Michalowski, Nancy Braudis, Laura Mansfield, Meena Nathan, Lynn A. Sleeper, Sarah A. Teele
Summary: This study aimed to develop a novel clinical risk prediction score for feeding tube utilization (FTU) in infants with hypoplastic left heart syndrome (HLHS) following stage 1 palliation (S1P). The study found that abnormal head imaging, duration of ventilation, and presence of vocal cord dysfunction were associated with FTU. The risk score developed based on these factors could potentially improve nutritional morbidity and hospital length of stay in this population.
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2023)
Article
Cardiac & Cardiovascular Systems
Jeanne L. Theis, Talha Niaz, Rhianna S. Sundsbak, Zachary C. Fogarty, William R. Bamlet, Donald J. Hagler, Timothy M. Olson
Summary: By performing whole-genome sequencing and filtering for rare, predicted-damaging variants, the study identified the significant role of CELSR1 gene in familial BAV and HLHS, further implicating the importance of planar cell polarity pathway perturbation in congenital heart disease.
CIRCULATION-GENOMIC AND PRECISION MEDICINE
(2022)
Article
Cardiac & Cardiovascular Systems
Jeanne L. Theis, Timothy M. Olson
Summary: Hypoplastic left heart syndrome is a genetically complex disorder, and whole genome sequencing is a useful tool for identifying genes associated with the disease. This review discusses family and cohort-based strategies for analyzing whole genome sequencing datasets and identifies potential candidate genes for HLHS.
JOURNAL OF CARDIOVASCULAR DEVELOPMENT AND DISEASE
(2022)
Review
Pediatrics
Tara Bharucha, Nicola Viola
Summary: Tricuspid regurgitation (TR) is frequently observed in surgically palliated patients with hypoplastic left heart syndrome and is associated with increased morbidity and mortality. The dysfunction of the tricuspid valve is the result of both inherent structural malformations and unique physiological circumstances caused by right ventricular pressure and volume overload. Traditional echocardiographic imaging can detect anatomical defects and dysfunction causing varying degrees of regurgitation, while advanced techniques such as 3D echocardiography, strain imaging, and transesophageal 3D echocardiography may provide better understanding of the complex interactions between abnormal valve anatomy, ventricular function, mechanical synchrony, and TR. Recognizing the specific mechanisms of TR can improve patient-specific care by guiding precise surgical interventions and determining the optimal timing for intervention on the valve.
FRONTIERS IN PEDIATRICS
(2023)
Article
Medicine, General & Internal
Iwona Strzelecka, Malgorzata Biedrzycka, Filip Franciszek Karuga, Bartosz Szmyd, Katarzyna Batarowicz, Maria Respondek-Liberska
Summary: The study observed a higher incidence of HLHS in specific months and years, as well as a higher incidence of SV in the fall/winter season. The research suggests that pollution levels and seasons play a significant role in the development of these cardiac conditions, highlighting the importance of continuing research on the effects of PM10 and benzo(a)pyrene exposure on maternal and fetal health.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Acoustics
K. Haberer, D. Fruitman, A. Power, L. K. Hornberger, L. Eckersley
Summary: The study found that features such as right-to-left foramen ovale flow and higher mitral valve annulus Z-scores are important predictors of biventricular circulation in fetal HLHC patients. Additionally, certain characteristics such as FO flow direction and specific Z-scores can effectively predict the failure of BV circulation.
ULTRASOUND IN OBSTETRICS & GYNECOLOGY
(2021)
Article
Cardiac & Cardiovascular Systems
Rachel J. J. Shustak, Jennifer A. A. Faerber, Alyson Stagg, David A. A. Hehir, Shobha S. S. Natarajan, Tamar J. J. Preminger, Anita Szwast, Jonathan J. J. Rome, Therese M. M. Giglia, Chitra Ravishankar, Laura Mercer-Rosa, Monique M. M. Gardner
Summary: This study is the first to examine the impact of home monitoring on unanticipated interstage readmissions in infants with hypoplastic left heart syndrome. The study found that our institution's Infant Single Ventricle Management and Monitoring Program (ISVMP) is associated with increased frequency of readmissions but comparable readmission days and fewer severe unanticipated readmissions. These findings suggest that home monitoring can reduce interstage morbidity without increasing readmission days.
JOURNAL OF THE AMERICAN HEART ASSOCIATION
(2023)
Article
Cell Biology
Anum Rahman, Taylor DeYoung, Lindsay S. Cahill, Yohan Yee, Sarah K. Debebe, Owen Botelho, Mike Seed, Rajiv R. Chaturvedi, John G. Sled
Summary: This study reports the successful development of a surgically induced mouse model that overcomes previous limitations, providing a fully penetrant cardiac phenotype with retrograde aortic arch flow, non-apex-forming left ventricles, and hypoplastic ascending aortas, allowing mice to survive to term.
DISEASE MODELS & MECHANISMS
(2021)
Editorial Material
Cardiac & Cardiovascular Systems
Nicholas D. Andersen, Mohammed K. Alsarraj, Joseph W. Turek
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2020)
Editorial Material
Cardiac & Cardiovascular Systems
Douglas M. Overbey, Joseph W. Turek, Nicholas D. Andersen
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2021)
Editorial Material
Cardiac & Cardiovascular Systems
Jennifer I. Sherwin, Veerajalandhar Allareddy, Nicholas D. Andersen
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2020)
Article
Cardiac & Cardiovascular Systems
Ming-Sing Si, John Conte, Jennifer C. Romano, Matthew A. Romano, Nicholas D. Andersen, Marc W. Gerdisch, John P. Kupferschmid, Andrew C. Fiore, Mamdouh Bakhos, Juan J. Bonilla, J. Ryan Burke, J. Scott Rankin, Lawrence M. Wei, Vinay Badhwar, Joseph W. Turek
Summary: This study presents the methods and results of valve repair in 20 patients with unicuspid aortic valves. The findings suggest that aortic ring annuloplasty effectively reduces annular diameter, allowing more leaflet to midline coaptation, and converting the repair procedure into a simple and reproducible one.
ANNALS OF THORACIC SURGERY
(2021)
Editorial Material
Cardiac & Cardiovascular Systems
Nicholas D. Andersen, Joseph W. Turek
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2021)
Editorial Material
Cardiac & Cardiovascular Systems
James M. Meza, Neel Prabhu, Reid C. Chamberlain, Joseph W. Turek, Nicholas D. Andersen
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2021)
Editorial Material
Cardiac & Cardiovascular Systems
Joseph R. Nellis, Nicholas D. Andersen, Joseph W. Turek
SEMINARS IN THORACIC AND CARDIOVASCULAR SURGERY
(2021)
Editorial Material
Cardiac & Cardiovascular Systems
Andrew M. Vekstein, Joseph W. Turek, Nicholas D. Andersen
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2021)
Editorial Material
Cardiac & Cardiovascular Systems
Douglas M. Overbey, Joseph W. Turek, Nicholas D. Andersen
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2021)
Article
Cardiac & Cardiovascular Systems
Neel K. Prabhu, Joseph W. Turek, Nicholas D. Andersen
Summary: This case report showcases the treatment of a newborn with complex congenital heart disease using a novel technique to minimize ischemic injury.
Editorial Material
Cardiac & Cardiovascular Systems
Douglas M. Overbey, Joseph W. Turek, Nicholas D. Andersen
SEMINARS IN THORACIC AND CARDIOVASCULAR SURGERY
(2021)
Editorial Material
Cardiac & Cardiovascular Systems
Nicholas D. Andersen, Joseph W. Turek
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2021)
Article
Cardiac & Cardiovascular Systems
Kyle W. Riggs, Farhan Zafar, Marshall L. Jacobs, Jeffrey P. Jacobs, Dylan Thibault, Kristine J. Guleserian, Karen Chiswell, Nick Andersen, Kevin D. Hill, David L. S. Morales, Roosevelt Bryant, James S. Tweddell
Summary: There is an association between airway anomalies and cardiac surgery outcomes, with patients with airway anomalies having higher risks of complications and mortality during surgery. Tracheal surgery during the same hospitalization increases the likelihood of operative mortality, major morbidity, and tracheostomy.
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2021)
Editorial Material
Cardiac & Cardiovascular Systems
Neel K. Prabhu, Nicholas D. Andersen, Joseph W. Turek
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2021)
Editorial Material
Cardiac & Cardiovascular Systems
Nicholas D. Andersen
ANNALS OF THORACIC SURGERY
(2023)
Article
Cardiac & Cardiovascular Systems
Andrew B. Harvey, Renelyn A. Woltes, Raymond N. Deepe, Hannah G. Tarolli, Jenna R. Drummond, Allison Trouten, Auva Zandi, Jeremy L. Barth, Rupak Mukherjee, Martin J. Romeo, Silvia G. Vaena, Ge Tao, Robin Muise-Helmericks, Paula S. Ramos, Russell A. Norris, Andy Wessels
Summary: This study highlights the importance of SOX9 in the regulation of epicardial cell invasion and emphasizes the role of EPDCs in regulating atrioventricular valve development and homeostasis. It also reports a novel expression profile of Cd109, a gene with previously unknown relevance in heart development.
JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY
(2024)
Review
Cardiac & Cardiovascular Systems
MariaSanta C. Mangione, Jinhua Wen, Dian J. Cao
Summary: mTOR, a mechanistic target of rapamycin, is an evolutionarily conserved pathway that plays a fundamental role in nutrient sensing, growth, metabolism, lifespan, and aging. Recent studies have highlighted the regulatory role of mTOR in innate immune responses and its involvement in the pathogenesis of cardiovascular diseases, especially in acute inflammation and atherosclerotic cardiovascular disease. This review also discusses mTOR's role in trained immunity, immune senescence, and clonal hematopoiesis, as well as its architecture and regulatory complexes.
JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY
(2024)
Article
Cardiac & Cardiovascular Systems
Junlin Li, Yajun Gong, Yiren Wang, Huihui Huang, Huan Du, Lianying Cheng, Cui Ma, Yongxiang Cai, Hukui Han, Jianhong Tao, Gang Li, Panke Cheng
Summary: Myocardial ischemia-reperfusion injury is closely related to the final infarct size in acute myocardial infarction. Regulatory T cells play an important role in the inflammatory response after AMI, but different subtypes of Tregs have different effects on the injury.
JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY
(2024)
Article
Cardiac & Cardiovascular Systems
Yuxin Chu, Yutao Hua, Lihao He, Jin He, Yunxi Chen, Jing Yang, Ismail Mahmoud, Fanfang Zeng, Xiaochang Zeng, Gloria A. Benavides, Victor M. Darley-Usmar, Martin E. Young, Scott W. Ballinger, Sumanth D. Prabhu, Cheng Zhang, Min Xie
Summary: This study demonstrates that administering beta-hydroxybutyrate (beta-OHB) at the time of reperfusion can reduce infarct size and preserve cardiac function by activating autophagy and preserving mitochondrial homeostasis, potentially through mTOR inhibition.
JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY
(2024)