Hypoplastic Left Heart Syndrome (HLHS)

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Hypoplastic Left Heart Syndrome (HLHS):  the left side of the heart is underdeveloped–including the left ventricle, the aorta (which carries oxygen-rich blood from the heart to the body), and the valves controling the bloodflow of the left ventrical (mitral valve, aortic valve). Excess pressure in the heart leads to an atrial septal defect and a patent ductus arteriosus.

  • hypoplastic Aorta (AO) [hypo=under, plasia=formation or development] the main vessel carrying oxygen-rich blood from the heart to the body is underdeveloped and too narrow.
  • Aortic Valve  Stenosis [aortic=the main artery leaving the heart, stenosis=narrowing of a passage ]–a narrowing of the aortic artery (which carries blood from the heart to every organ except the lungs) at or near the aortic valve.
  • Atrial Septal Defect (ASD) [septum=wall between the chambers of the heart, atriums=top chambers of the heart] – holes in the inner walls of the heart allowing extra blood flow between the two upper chambers of the heart (atriums).
  • Hypoplastic Left Ventricle [hypo=under, plasia=formation or development, ventricles=lower chambers of the heart] The left ventricle is underdeveloped.  It is too small. This means the heart will have to work harder and won’t last as long. Sometimes the valves entering or leaving the left ventricle are also too small. (Aortic Valve, Mitral Valve).
  • Mitral Valve Stenosis (MS) the valve that controls blood flow between the right atrium and the left ventricle in this heart.  (In a normal heart, the mitral valve is located between the left artery and the left ventricle.)
  • large Patent Ductus Arteriosus (PDA) [patent=open, ductus=duct, arteriosus=artery] – an extra passageway between the pulmonary artery (carrying oxygen-poor blood from the heart to the lungs) and the aorta (carrying oxygen-rich blood from the heart to the body).  The ductus arteriosus is open in a fetus.  This allows extra bloodflow in the forming baby while it’s getting oxygen from Mom instead of its own lungs.  The PDA normally closes around 10 days after birth.
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2 Thoughts to “Hypoplastic Left Heart Syndrome (HLHS)”

  1. Dian_coleman

    I have a daughter who was born with HLHS and don’t know to the degree she has this condition. How would I be able to find this out? Thanks

    1. Your cardiologist is the only one who can answer this question for you. 
      You can call his office and speak with his nurse.  She’ll have access
      to your daughter’s medical files and can help explain them to you OR
      send you information specific to your daughter. You can even ask for a copy of her medical records – but they may be difficult to decipher yourself.

      How old is your daughter?  If she is still young (and you are going to
      cardiology appointments regularly), you can write down your questions
      and make sure your cardiologist answers them at your next appointment.

      Even after he explains, if you don’t feel like you could explain it to
      your best friend, tell him that you’re not quite getting it and ask him
      to explain it again.

      There are LOTS of HLHS support groups.  Here’s one on facebook that seems active: http://www.facebook.com/pages/HLHS/155906247758907

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