Hypoplastic Right Heart Syndrome (HRHS) ii

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Hypoplastic Right Heart Syndrome (HRHS):  the right side of the heart is underdeveloped–including the right ventricle, the pulmonary artery (which carries oxygen-poor blood from the heart to the lungs), and the valves controling the bloodflow of the right ventrical (tricuspid valve, pulmonary valve). These valves may be narrowed (stenosis) or missing (atresia). Excess pressure in the heart may result in atrial and ventricular septal defects (ASD & VSD).

  • Atrial Septal Defect (ASD) [septum=wall between the chambers of the heart, atria=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 (atria).
  • Hypoplastic Right Ventricle (RV) [hypo=under, plasia=formation or development, ventricles=lower chambers of the heart] The right 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 right ventricle are also too small or fused shut.  (Tricuspid Valve,  Pulmonary Valve).
  • hypoplastic Pulmonary Artery (PA) [hypo=under, plasia=formation or development] the main vessel carrying oxygen-poor blood from the heart to the lungs is underdeveloped and too narrow.
  • Pulmonary Valve Atresia (a) [pulmonary=having to do with the lungs, atresia=without openings] a complete blockage of the pulmonary artery (which carries blood from the heart to the lungs) caused by a missing or fused-shut pulmonary valve.
  • (overriding) Tricuspid Valve Stenosis (t) [overriding=reaching too far, stenosis=narrowing of a passage ]– The valve between the right atrium and the right ventricle is too narrow (stenosis).  It also pushes the blood passed the right ventricle and into the left ventricle (overriding) because the right ventricle is too small.
  • Ventricular Septal Defect (VSD) [septum=wall between the chambers of the heart, ventricles=lower chambers of the heart] – holes in the inner walls of the heart allowing extra blood flow between the two lower chambers of the heart (ventricles). This causes the oxygen-rich and oxygen-poor blood to mix before leaving the heart.
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