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Hypoplastic Left Heart Syndrome (HLHS)
Single Ventricle DefectHLHS is grouped into the category of single ventricle defects. In HLHS, the left side of the heart is severely underdeveloped, and the mitral and aortic valves are either entirely atretic (closed), or hypoplastic (small). As seen in the diagram below, the L) ventricle is tiny, and in HLHS, the first part of the aorta is very small, often only a few millimeters around.
Due to this, the L) side of the heart cannot provide circulation to the body.
The right side of the heart develops normally, and pumps blood to the lungs. However because the left side is so small, blood returning from the lungs into the L) atrium must pass through an atrial septal defect (see ASD chapter for more) to shunt into the right side of the heart.
The R) side must then pump blood to the lungs and out to the body, and a patent ductus arteriosus (see PDA chapter for more) is the only way blood can move through the heart to reach the body.
Once this begins to close however, circulation to the body will decrease, leading to very low blood flow to vital organs, that can lead in turn to shock.
Signs & Symptoms
Due to the above, we can expect to see:☞ Reduced oxygen saturations, from blood mixing in the heart.
☞ Cyanosis
☞ Respiratory distress, as the lungs will be receiving a large amount of blood.
☞ Weak peripheral pulses depending on ductus arteriosus.
☞ Sleepiness, poor feeding, (and respiratory distress may worsen as the ductus closes.)
☞ Faint or no present heart murmur in newborns.Signs of shock and progression include:
☞ Seizures
☞ Kidney failure,
☞ Liver failure
☞ Worsening cardiac function
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