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Transl Pediatr. 2018 Apr;7(2):176-187. doi: 10.21037/tp.2018.04.01.

Regenerative medicine therapy for single ventricle congenital heart disease.

Translational pediatrics

Chetan Ambastha, Gregory J Bittle, David Morales, Nathaniel Parchment, Progyaparamita Saha, Rachana Mishra, Sudhish Sharma, Alexander Vasilenko, Muthukumar Gunasekaran, Manal T Al-Suqi, Deqiang Li, Peixin Yang, Sunjay Kaushal

Affiliations

  1. Division of Cardiac Surgery, University of Maryland School of Medicine, Baltimore, MD, USA.

PMID: 29770299 PMCID: PMC5938254 DOI: 10.21037/tp.2018.04.01

Abstract

One of the most complex forms of congenital heart disease (CHD) involving single ventricle physiology is hypoplastic left heart syndrome (HLHS), characterized by underdevelopment of the left ventricle (LV), mitral and aortic valves, and narrowing of the ascending aorta. The underdeveloped LV is incapable of providing long-term systemic flow, and if left untreated, the condition is fatal. Current treatment for this condition consists of three consecutive staged palliative operations: the first is conducted within the first few weeks of birth, the second between 4 to 6 months, and the third and final surgery within the first 4 years. At the conclusion of the third surgery, systemic perfusion is provided by the right ventricle (RV), and deoxygenated blood flows passively to the pulmonary vasculature. Despite these palliative interventions, the RV, which is ill suited to provide long-term systemic perfusion, is prone to eventual failure. In the absence of satisfying curative treatments, stem cell therapy may represent one innovative approach to the management of RV dysfunction in HLHS patients. Several stem cell populations from different tissues (cardiac and non-cardiac), different age groups (adult-

Keywords: Heart defects; congenital; heart failure; hypoplastic left heart syndrome (HLHS); regenerative medicine; stem cells

Conflict of interest statement

Conflicts of Interest: The authors have no conflicts of interest to declare.

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