Hypoplastic left heart syndrome is a congenital heart defect that occurs in 20 per 100,000 live births. Patients are born with severe underdevelopment of the left side of the heart which, if left untreated, is uniformly fatal. A series of operations is performed, including a cavopulmonary (Glenn) shunt and total cavopulmonary connection (Fontan procedure), which connect the superior (SVC) and inferior vena cavae (IVC) respectively in an end-to-side fashion to the left (LPA) and right pulmonary arteries (RPA), resulting in a T-shaped junction. This bypasses the heart on the venous side as blood flows from the IVC and SVC directly into the pulmonary arteries. Early survival rates following the Fontan are as high as 90%. However, these figures drop to 60% survival after 10 years , and most patients exhibit diminished exercise capacity.
Evaluation of Hemodynamic Efficiency in a New “Y-Graft” Design for the Fontan Operation
Bernstein, AJ, Marsden, AL, Spilker, RL, Reddy, VM, Taylor, CA, & Feinstein, JA. "Evaluation of Hemodynamic Efficiency in a New “Y-Graft” Design for the Fontan Operation." Proceedings of the ASME 2007 Summer Bioengineering Conference. ASME 2007 Summer Bioengineering Conference. Keystone, Colorado, USA. June 20–24, 2007. pp. 473-474. ASME. https://doi.org/10.1115/SBC2007-176279
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