Januari 06, 2017
hypoplastic left heart syndrome (hlhs). the term "hypoplastic" refers to underdevelopmentof a tissue or organ. in hlhs, most of the left-sided heart structuresare hypoplastic. this is a view from inside the right atrium. an atrial septal defect, or asd, is a holebetween the right and left atria. this is a view from inside the left atrium. the mitral valve is also hypoplastic and iseither severely narrowed or not formed at all. the abnormal mitral valve prevents blood fromentering the left ventricle. blood flow is
then diverted across the asd and into theright atrium. blood return from both the lungs and body flows into the right ventricle. theright ventricle pumps blood to both the lungs and the body. this is why hlhs is referredto as a "single ventricle" defect. in hlhs, a pda (patent ductus arteriosus)exists to provide blood flow from the pulmonary artery to the body. babies with hlhs havea bluish appearance, known as cyanosis, as a result of mixing oxygen-rich and oxygen-poorblood. if the pda closes, the baby will become critically ill. a continuous iv medicationcalled prostaglandin is needed to keep the pda open after birth. surgery is typically performed within thefirst few weeks of life.
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