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Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome
 
 
Review

Flow-Mediated Factors in the Pathogenesis of Hypoplastic Left Heart Syndrome

by 1,2,3,*, 4 and 1,2,3
1
Mouse Imaging Centre, The Hospital for Sick Children, Toronto, ON M5T 3H7, Canada
2
Translational Medicine, The Hospital for Sick Children, Toronto, ON M5G 1X8, Canada
3
Department of Medical Biophysics, University of Toronto, Toronto, ON M5G 1L7, Canada
4
Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, Toronto, ON M5G 1X8, Canada
*
Author to whom correspondence should be addressed.
Academic Editor: Paul Grossfeld
J. Cardiovasc. Dev. Dis. 2022, 9(5), 154; https://doi.org/10.3390/jcdd9050154
Received: 30 March 2022 / Revised: 1 May 2022 / Accepted: 6 May 2022 / Published: 12 May 2022
Hypoplastic left heart syndrome (HLHS) is a life-threatening congenital heart disease that is characterized by severe underdevelopment of left heart structures. Currently, there is no cure, and affected individuals require surgical palliation or cardiac transplantation to survive. Despite these resource-intensive measures, only about half of individuals reach adulthood, often with significant comorbidities such as liver disease and neurodevelopmental disorders. A major barrier in developing effective treatments is that the etiology of HLHS is largely unknown. Here, we discuss how intracardiac blood flow disturbances are an important causal factor in the pathogenesis of impaired left heart growth. Specifically, we highlight results from a recently developed mouse model in which surgically reducing blood flow through the mitral valve after cardiogenesis led to the development of HLHS. In addition, we discuss the role of interventional procedures that are based on improving blood flow through the left heart, such as fetal aortic valvuloplasty. Lastly, using the surgically-induced mouse model, we suggest investigations that can be undertaken to identify the currently unknown biological pathways in left heart growth failure and their associated therapeutic targets. View Full-Text
Keywords: hypoplastic left heart syndrome; mouse; fetus; blood flow; mitral valve; aorta; stenosis; left ventricle; fetal aortic valvuloplasty; hemodynamics hypoplastic left heart syndrome; mouse; fetus; blood flow; mitral valve; aorta; stenosis; left ventricle; fetal aortic valvuloplasty; hemodynamics
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MDPI and ACS Style

Rahman, A.; Chaturvedi, R.R.; Sled, J.G. Flow-Mediated Factors in the Pathogenesis of Hypoplastic Left Heart Syndrome. J. Cardiovasc. Dev. Dis. 2022, 9, 154. https://doi.org/10.3390/jcdd9050154

AMA Style

Rahman A, Chaturvedi RR, Sled JG. Flow-Mediated Factors in the Pathogenesis of Hypoplastic Left Heart Syndrome. Journal of Cardiovascular Development and Disease. 2022; 9(5):154. https://doi.org/10.3390/jcdd9050154

Chicago/Turabian Style

Rahman, Anum, Rajiv R. Chaturvedi, and John G. Sled. 2022. "Flow-Mediated Factors in the Pathogenesis of Hypoplastic Left Heart Syndrome" Journal of Cardiovascular Development and Disease 9, no. 5: 154. https://doi.org/10.3390/jcdd9050154

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