Clinical Progress in Cell Therapy for Single Ventricle Congenital Heart Disease
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- cell transplantation
- congenital heart disease
- hypoplastic left heart syndrome
- single ventricle
- stem cell
One of the fundamental goals of congenital cardiac surgery is to correct the circulatory and structural physiology of congenital heart disease (CHD) by skillfully reconstructing the cardiac anatomy. In the majority of CHD patients, complete correction is achieved with low mortality and reasonable durability, a tribute to surgical strides during the last 2 decades. However, the most challenging CHD patients present with a developmentally hypoplastic heart chamber, which necessitates heroic palliative operations to establish a single ventricle that delivers oxygenated blood to the body, leaving deoxygenated blood to be delivered passively to the pulmonary circulation.
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This anatomy is crafted in a staged fashion. Typically, the stage I palliative operation (Norwood operation) is performed in the first weeks of life and commits the single ventricle to pumping to both the systemic and pulmonary circulations. The stage II operation (bidirectional cavo-pulmonary anastomosis), at ≈6 months, redirects superior vena caval blood to the pulmonary circulation by transecting the proximal superior vena caval and performing a direct anastomosis to the right pulmonary artery. The stage III operation (Fontan), at ≈3 years, directs the remaining inferior vena caval blood flow to the pulmonary artery. Though outcomes have been improving over the past few decades, these procedures are not curative, and overall life expectancy with these conditions remains markedly diminished.1 Much of this mortality is because of dysfunction of the single ventricle, which is usually the morphological right ventricle (RV). The RV is not intended to pump against systemic pressures long term and in the best circumstances fails by the second or third decade of life.2 The only viable option for the failing ventricle is heart transplantation; however, survival after heart transplantation is poor after single ventricle staged palliation.3 Along with increased mortality because of inevitable failure, there is …