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Definition
By Mayo Clinic staffHypoplastic left heart syndrome is a complex and rare heart defect present at birth (congenital). In hypoplastic left heart syndrome, the left side of the heart is critically underdeveloped.
If your baby is born with hypoplastic left heart syndrome, the left side of the heart can't effectively pump blood to the body, so the right side of the heart must pump blood both to the lungs and to the rest of the body.
Medication to prevent closure of the connection (ductus arteriosus) between the right and left sides, followed by either surgery or a heart transplant, is necessary to treat hypoplastic left heart syndrome. With advances in care, the outlook for babies born with hypoplastic left heart syndrome is better now than in the past.
- Fraser CD, et al. Congenital heart disease. In: Townsend CM, et al. Sabiston Textbook of Surgery. 18th ed. Philadelphia, Pa.: Saunders Elsevier; 2007. http://www.mdconsult.com/das/book/body/186905321-10/962096861/1565/579.html#4-u1.0-B978-1-4160-3675-3..50064-2--cesec58_3551. Accessed March 3, 2010.
- Bernstein D. Cyanotic congenital heart disease: Lesions associated with increased pulmonary blood flow. In: Kliegman RM, et al. Nelson Textbook of Pediatrics. 18th ed. Saunders Elsevier; 2007. http://www.mdconsult.com/das/book/body/186905321-10/962096861/1608/1010.html#4-u1.0-B978-1-4160-2450-7..50433-3--cesec35_8312. Accessed March 3, 2010.
- Fontan procedure-requiring lesions. In: Libby P, et al., eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 8th ed. Philadelphia, Pa.: Saunders Elsevier; 2007. http://www.mdconsult.com/book/player/book.do?method=display&type=bookPage&decorator=header&eid=4-u1.0-B978-1-4160-4106-1..50064-9--cesec370&displayedEid=4-u1.0-B978-1-4160-4106-1..50064-9--cesec382&uniq=186905321&isbn=978-1-4160-4106-1&sid=962096861#lpState=open&lpTab=contentsTab&content=4-u1.0-B978-1-4160-4106-1..50064-9--cesec381%3Bfrom%3Dtoc%3Btype%3DbookPage%3Bisbn%3D978-1-4160-4106-1. Accessed March 3, 2010.
- Hypoplastic left heart syndrome. American Heart Association. http://www.americanheart.org/presenter.jhtml?identifier=1353. Accessed March 3, 2010.
- Grossfield P, et al. Hypoplastic left heart syndrome: New genetic insights. Journal of the American College of Cardiology. 2009;53:1072.
- If your child has a congenital heart defect - Our guide for parents. American Heart Association. http://www.americanheart.org/presenter.jhtml?identifier=3007586. Accessed March 3, 2010.
- Waltzman M. Initial evaluation of shock in children. http://www.uptodate.com/home/index.html. Accessed March 3, 2010.
- Gordon BM, et al. Decreasing number of deaths of infants with hypoplastic left heart syndrome. The Journal of Pediatrics. 2008;153:354.
- Grogan M (expert opinion). Mayo Clinic, Rochester, Minn. March 16, 2010.
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