Newborn cute infant baby with hand holding clip on umbilical cord at a hospital nursery.

Congenital heart disease: hypoplastic left heart syndrome

By Cristiano Antonino On Mar 31, 2023

Hypoplastic left heart syndrome is among the most complex heart defects occurring in the newborn, and is one of the most frequently diagnosed heart defects during the fetal echocardiogram; this syndrome is the set of malformations in which the left sections of the heart muscle are less developed

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Hypoplastic left heart syndrome, an overview

In a normal heart, the left atrium and left ventricle have the function of pumping oxygenated blood into the aorta and the rest of the body.

In children with hypoplastic left heart syndrome, the mitral valve, which separates the atrium from the left ventricle, will either be excessively small or completely closed; the aortic valve, which separates the left ventricle and aorta, will also be excessively small or completely closed; the left ventricle will see incomplete development of a tissue or organ, resulting from an insufficient or sub-standard number of cells.

This will make the left ventricle unable to support the proper circulatory supply that will be needed to provide adequate blood flow to all organs; the right side of the heart, on the other hand, which pumps blood to the lungs, will be its normal size.

At birth, the ductus arteriosus will be the one that allows blood to circulate throughout the body reaching the organs; ductus arteriosus that connects the pulmonary artery and the aorta,

In the first few days of life, the ductus arteriosus will begin to close causing a decrease in blood flow to the rest of the body and consequently the organs will receive less blood causing a state of shock. If left untreated, hypoplastic heart syndrome will be fatal.

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Prenatal diagnosis of hypoplastic left heart syndrome will see expectant mothers give birth in stabilised centres, designed to intervene promptly should the newborn need to be stabilised in order to prevent the worsening of the unborn baby’s clinical condition.

Heart disease may be diagnosed even a few days or a few hours after birth, so it will be necessary to intervene promptly to stabilise the newborn’s clinical condition.

The newborn may need: gastric tube to feed him if he has difficulty; infusion of prostaglandins to allow the ductus arteriosus to remain open; intubation and mechanical ventilation; atrio-sectostomy to separate the atria allowing better communication.

The following will then be performed: echocardiogram and electrocardiogram to assess the anatomy and electrical activity of the heart; chest X-ray to assess cardiac dimensions; cardiac catheterisation; cardiac MRI to better analyse cardiac structures and interaction with surrounding organs.

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Hypoplastic left heart syndrome will be fatal if early intervention is not taken, but today there are several treatments for this heart disease

The surgical treatment will be aimed at separating the two circulations; the right side will have to do the work of the left ventricle by pumping oxygen-rich blood through the surgically reconstructed aorta; the deoxygenated blood will flow from the venous system into the pulmonary circulation without passing through the heart.

With Norwood’s operation, a rigid conduit will be interposed between the ventricle and the pulmonary artery trunk in the first few days of life; Glenn’s operation, on the other hand, will be performed at around 5 months of life and will be aimed at creating a connection between the systemic venous circulation and the pulmonary circulation.

Depending on the case, it may be necessary to resort to a treatment that will subject the pulmonary arteries to a kind of surgical bandage, with the placement of a prosthesis that will keep the blood vessel open in the ductus arteriosus, to allow blood to circulate throughout the body.

The life prospects for children with hypoplastic heart syndrome have improved compared to the past; however, they will still need to take medication to improve the pumping function of the heart, and they will need to undergo regular check-ups to monitor the onset of any complications.

In children who have undergone surgical reconstruction, new surgeries may be necessary over the years, or if the therapies are not effective, cardiac transplantation may be required.

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