Hypoplastic left heart syndrome

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Tests and diagnosis

By Mayo Clinic staff

Before birth
It's possible for a baby to be diagnosed with hypoplastic left heart syndrome while it's still in the womb. Doctors often can identify the condition on a routine ultrasound exam during pregnancy, sometimes as early as during the first trimester.

After birth
After your baby is born, his or her doctor may suspect a heart defect, such as hypoplastic left heart syndrome, if your baby has gray-blue skin or has trouble breathing. Your baby's doctor may also suspect a heart defect if he or she hears a heart murmur — an abnormal whooshing sound caused by turbulent blood flow.

Doctors typically use an echocardiogram to diagnose hypoplastic left heart syndrome. This test uses high-pitched sound waves that bounce off your baby's heart to produce moving images that can be viewed on a video screen. In a baby with hypoplastic left heart syndrome, the echocardiogram reveals a smaller than normal left ventricle and aorta. Because this test can track blood flow, it also shows blood moving from the right ventricle into the aorta (retrograde flow). In addition, an echocardiogram can identify associated heart defects, such as an atrial septal defect.

References
  1. 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.
  2. 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.
  3. 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.
  4. Hypoplastic left heart syndrome. American Heart Association. http://www.americanheart.org/presenter.jhtml?identifier=1353. Accessed March 3, 2010.
  5. Grossfield P, et al. Hypoplastic left heart syndrome: New genetic insights. Journal of the American College of Cardiology. 2009;53:1072.
  6. 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.
  7. Waltzman M. Initial evaluation of shock in children. http://www.uptodate.com/home/index.html. Accessed March 3, 2010.
  8. Gordon BM, et al. Decreasing number of deaths of infants with hypoplastic left heart syndrome. The Journal of Pediatrics. 2008;153:354.
  9. Grogan M (expert opinion). Mayo Clinic, Rochester, Minn. March 16, 2010.
DS00744 May 27, 2010

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