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)
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.
Review
Cardiac & Cardiovascular Systems
Hannah Bellsham-Revell
Summary: Hypoplastic left heart syndrome is a spectrum of left heart underdevelopment that requires staged palliation to the Fontan circulation for management, with potential long-term intervention needed for the branch pulmonary arteries. Echocardiography is the mainstay for assessment, but MRI and CT are increasingly used for more challenging extracardiac structures. Both MRI and CT require specific conditions and training, but can provide excellent imaging guidance for interventions.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2021)
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
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)
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
Federica Ferrigno, Alessio Franceschini, Richard Kirk, Antonio Amodeo
Summary: This article describes a patient with Jacobsen syndrome and hypoplastic left heart syndrome who underwent cardiac transplantation. The patient exhibited recognized morphological features as well as immunological and hematological issues. Despite post-transplant complications, the patient's overall health has improved over the past five years.
JOURNAL OF CARDIOVASCULAR DEVELOPMENT AND DISEASE
(2023)
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.
Review
Pediatrics
Lilia Oreto, Paolo Guccione, Placido Gitto, Letteria Bruno, Rosanna Zanai, Nadia Grasso, Enrico Iannace, Concetta Zito, Scipione Carerj, Salvatore Agati
Summary: Hypoplastic left heart syndrome is a complex congenital cardiac defect. Univentricular palliation is the preferred treatment, although biventricular repair is an option in some cases. Hybrid palliation, which involves surgical banding of the pulmonary arteries and transcatheter stenting of the ductus arteriosus, may be a suitable alternative to traditional Norwood operation in certain high-risk or borderline left ventricle cases. Echocardiography plays a crucial role in diagnosing and managing patients with hypoplastic left heart undergoing hybrid palliation.
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)
Article
Biophysics
Sheldon Ho, Wei Xuan Chan, Choon Hwai Yap
Summary: The left atrial ligation (LAL) chick embryonic heart model demonstrates morphological and hemodynamic features similar to human hypoplastic left heart syndrome (HLHS), with compromised atrial function and reduced velocities in the ventricle. Changes in ventricular shape and atrioventricular junction position result in more sluggish flow near the ventricular wall and apex, leading to lower wall shear stresses and higher oscillatory indices, which may contribute to the HLHS morphology in the LAL model.
BIOMECHANICS AND MODELING IN MECHANOBIOLOGY
(2021)
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
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
Engineering, Biomedical
S. Samaneh Lashkarinia, Wei Xuan Chan, Efthymios Motakis, Sheldon Ho, Hummaira Banu Siddiqui, Mervenur Coban, Bortecine Sevgin, Kerem Pekkan, Choon Hwai Yap
Summary: Left atrial ligation is a mechanical model of hypoplastic left heart syndrome, providing insight into the biomechanical origins of the syndrome. Using finite element modeling and single-cell RNA sequencing, this study investigated the myocardial mechanics and gene expressions associated with left atrial ligation.
ANNALS OF BIOMEDICAL ENGINEERING
(2023)
Review
Cardiac & Cardiovascular Systems
Hisato Yagi, Cecilia W. Lo
Summary: Hypoplastic left heart syndrome (HLHS) is a complex congenital heart disease characterized by underdevelopment of the left-sided heart structures. The developmental cause for the left-sided heart defects in HLHS is still unknown. However, there is evidence suggesting a potential disturbance in laterality, as observed through the co-occurrence of rare organ situs defects and pathogenic variants in genes regulating left-right patterning in HLHS patients. Further studies in mutant mice also support the role of laterality disturbance in the development of HLHS.
JOURNAL OF CARDIOVASCULAR DEVELOPMENT AND DISEASE
(2023)
Article
Pediatrics
Heidi Maenpaa, Arja Hakkinen, Anne Sarajuuri
PEDIATRIC PHYSICAL THERAPY
(2016)
Article
Pediatrics
A. Sarajuuri, T. Lonnqvist, F. Schmitt, F. Almqvist, E. Jokinen
Article
Pediatrics
Anne Sarajuuri, Eero Jokinen, Riina Puosi, Leena Mildh, Ilkka Mattila, Aulikki Lano, Tuula Lonnqvist
JOURNAL OF PEDIATRICS
(2010)
Article
Clinical Neurology
Riina Puosi, Marit Korkman, Anne Sarajuuri, Eero Jokinen, Leena Mildh, Ilkka Mattila, Tuula Lonnqvist
JOURNAL OF THE INTERNATIONAL NEUROPSYCHOLOGICAL SOCIETY
(2011)
Article
Cardiac & Cardiovascular Systems
Anne Sarajuuri, Tuula Lonnqvist, Leena Mildh, Irmeli Rajantie, Marianne Eronen, Ilkka Mattila, Eero Jokinen
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2009)
Article
Pediatrics
Anne Sarajuuri, Eero Jokinen, Leena Mildh, Anna-Mari Tujulin, Ilkka Mattila, Leena Valanne, Tuula Lonnqvist
Article
Pediatrics
Laura Mirjami Jolma, Mikko Koivu-Jolma, Anne Sarajuuri, Paulus Torkki, Ilona Autti-Ramo, Heli Satila
Summary: This study conducted a retrospective chart review of 55 individuals diagnosed with fetal alcohol spectrum disorder (FASD) in a central hospital in Finland. The aim was to identify common problems and interventions during different periods of childhood and estimate associated costs. The findings showed that the healthcare costs were highest during the early years and varied across different age groups of FASD patients.
Article
Cardiac & Cardiovascular Systems
Anne Sarajuuri, Eero Jokinen, Riina Puosi, Marianne Eronen, Leena Mildh, Ilkka Mattila, Leena Valanne, Tuula Lonnqvist
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2007)
