Article
Engineering, Biomedical
Hong Shen Wong, Hadi Wiputra, Andreas Tulzer, Gerald Tulzer, Choon Hwai Yap
Summary: In cases of fetal aortic stenosis and evolving Hypoplastic Left Heart Syndrome (feHLHS), specific abnormalities and altered fluid dynamics are observed in the left ventricles (LV). The feHLHS LV shows narrowed and fast diastole mitral inflow, elevated energy losses, intraventricular pressure gradients, and vortex formation numbers, and poor blood turnover, indicating energy inefficiency of flow and additional burden on the LV.
ANNALS OF BIOMEDICAL ENGINEERING
(2022)
Article
Pediatrics
Lilia Oreto, Giuseppe Mandraffino, Rita Emanuela Calaciura, Daniela Poli, Placido Gitto, Michele Benedetto Saitta, Ermanno Bellanti, Scipione Carerj, Concetta Zito, Fiore Salvatore Iorio, Paolo Guccione, Salvatore Agati
Summary: Treatment options for hypoplastic borderline left ventricle (LV) depend on the LV's development, including univentricular palliation or biventricular repair, while hybrid palliation can defer major surgery. This study evaluates the anatomical modifications of borderline LV after hybrid palliation. Echocardiography monitoring plays a significant role in assessing LV growth.
Article
Medicine, General & Internal
Jing Ma, Yaping Yuan, Li Zhang, Shizhen Chen, Haiyan Cao, Liu Hong, Juanjuan Liu, Xiaoyan Song, Jiawei Shi, Yi Zhang, Li Cui, Xin Zhou, Mingxing Xie
Summary: Right ventricular function is a critical factor affecting the prognosis of fetuses with hypoplastic left heart syndrome (HLHS). This study found that myocardial deformation parameters of the right ventricle were significantly lower in HLHS fetuses, and there were morphological and functional changes in the cardiac microstructure that were related to cardiac function.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Acoustics
A. Venardos, J. Colquitt, S. A. Morris
Summary: This study compares the rates of left-sided cardiac structure growth and Z-score change in fetuses with severe left-heart hypoplasia (LHH) and severe aortic stenosis (AS). The results show that the left-sided structures grow faster in fetuses with LHH and the Z-scores decrease at a slower rate compared to fetuses with AS. Furthermore, the majority of infants with LHH do not require single-ventricle palliation.
ULTRASOUND IN OBSTETRICS & GYNECOLOGY
(2022)
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
Cardiac & Cardiovascular Systems
Meaghan J. Beattie, Lynn A. Sleeper, Minmin Lu, Sarah A. Teele, Roger E. Breitbart, Jesse J. Esch, Joshua W. Salvin, Urvi Kapoor, Olubunmi Oladunjoye, Sitaram M. Emani, Puja Banka
Summary: This study aimed to investigate the long-term morbidity and mortality of patients with borderline hypoplastic left heart undergoing single to biventricular conversion. The results showed that the presence of endocardial fibroelastosis and smaller left ventricular stroke volume/body surface area were independent factors associated with adverse outcomes. Normal preoperative left ventricular end-diastolic pressure was not sufficient to reassure against diastolic dysfunction.
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2023)
Article
Cardiac & Cardiovascular Systems
Hannah H. Nam, Maura Flynn, Andras Lasso, Christian Herz, Patricia Sabin, Yan Wang, Alana Cianciulli, Chad Vigil, Jing Huang, Jared Vicory, Beatriz Paniagua, David Allemang, David J. Goldberg, Mohammed Nuri, Meryl S. Cohen, Gabor Fichtinger, Matthew A. Jolley
Summary: Using echocardiography and software modeling, this study found that the tricuspid valve structure differs in patients with hypoplastic left heart syndrome and a Fontan circulation who have moderate or greater tricuspid regurgitation. Additionally, the right ventricle volume is associated with tricuspid valve structure and dysfunction. Therefore, an image-informed patient-specific approach to surgical planning may be necessary for optimal outcomes in this vulnerable population.
CIRCULATION-CARDIOVASCULAR IMAGING
(2023)
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)
Review
Cardiac & Cardiovascular Systems
Diego Ramonfaur, Xiaoya Zhang, Abraham P. Garza, Jose Fernando Garcia-Pons, Sylvia C. Britton-Robles
Summary: Hypoplastic left heart syndrome is a rare congenital disorder that causes early childhood death if left untreated. The complex treatment process involves multiple surgical procedures at different stages, including the Norwood, bidirectional Glenn, and Fontan procedures. The prognosis has improved in recent decades, but is still influenced by various factors. Novel alternatives have been proposed to improve survival and neurodevelopment, but are not widely used. Attention has also been given to improving the neurodevelopmental outcomes and quality of life for these patients.
CARDIOLOGY IN REVIEW
(2023)
Article
Obstetrics & Gynecology
Christian Enzensberger, Oliver Graupner, Stefanie Fischer, Markus Meister, Maleen Reitz, Malena Goette, Vera Mueller, Aline Wolter, Johannes Herrmann, Roland Axt-Fliedner
Summary: This study used 2D STE to confirm the prenatal remodeling of right ventricular function in HLH patients and explore the influencing factors of myocardial restructuring. The results showed that HLH fetuses had slightly lower right ventricular systolic function compared to healthy controls, and the presence of left ventricular endocardial fibroelastosis and restrictive foramen ovale further compromised right ventricular function.
ARCHIVES OF GYNECOLOGY AND OBSTETRICS
(2023)
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
Katrin Fricke, Mats Mellander, Katarina Hanseus, Phan-Kiet Tran, Mats Synnergren, Jens Johansson Ramgren, Annika Rydberg, Jan Sunnegardh, Magnus Dalen, Gunnar Sjoberg, Constance G. Weismann, Petru Liuba
Summary: The study found that left ventricular morphology and choice of shunt have a significant impact on adverse outcomes in patients with hypoplastic left heart syndrome, especially during stage 1 palliation. Globular left ventricle and aortic atresia-mitral stenosis are independent morphological risk factors that affect short- and long-term outcomes, particularly when a Blalock-Taussig shunt is used during stage 1 palliation.
JOURNAL OF THE AMERICAN HEART ASSOCIATION
(2022)
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
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.
Editorial Material
Medicine, General & Internal
Pasquale Giugliano, Paola Ciamarra, Mariavictoria De Simone, Alessandro Feola, Pierluca Zangani, Carlo Pietro Campobasso, Gelsomina Mansueto
Summary: The article presents a case of HLHS in a newborn that was not diagnosed prenatally. Autopsy results revealed hypoplasia of the left and right cardiac cavities. The study emphasizes the importance of early diagnosis for successful surgical intervention.