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About The Treatment

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Hypoplastic Left Heart Syndrome (HLHS) Treatment

Hypoplastic left heart disorder (HLHS) is a congenital heart deformity in which the left half of the heart is severely underdeveloped. It might influence the left ventricle, aorta, aortic valve, or mitral valve.

Why HLHS Treatment is Performed?

The mechanism of the defect needs to be explained to understand the essentiality of this procedure. Since the left half of the heart is underdeveloped, oxygenated blood coming into the left heart from the lungs is redirected into the right side of the heart through the atrial septal defect, where it blends with deoxygenated blood coming back to the heart from the body.

This deoxygenated blood leaves the right side of the heart to the lungs and through the ductus arteriosus to the body. The ductus arteriosus is a vein interfacing the two conduits leaving the heart, the pulmonary artery and the aorta. In the initial period of life (in the womb), bloodstream to the body is uninterrupted because the ductus arteriosus stays open. Once the ductus closes, the blood flow is restricted, and the child may die.

What are the Signs and Symptoms of HLHS?

  • Paleness
  • Rapid, difficult breathing
  • A lack of appetite
  • Low body temperature
  • Weak pulse
  • Being drowsy or inactive
  • Greyish-blue skin colour (cyanosis)
  • Inactivity
  • Dilated pupils
  • A vacant stare
  • Persistent sweating
  • Mottled skin
  • Heavy breathing

How is HLHS is Diagnosed?

  • Chest X-ray This noninvasive imaging test enables your specialist to discount different types of chest discomfort, for example, pneumonia. Imaging with x-rays includes presenting the chest to little measurements of radiation to create photos of the chest and heart.
  • Echocardiogram During this test, a transducer that produces high-recurrence sound waves is used to create moving pictures of the heart. The movement of the dividers of the heart is assessed. If there is diminished movement inside a portion of the heart, this could show reduced blood spill out of narrowing of the coronary course. A differentiate material, which is a liquid with minor air pockets, might be infused in a vein of your arm to check whether there is diminished bloodstream to any territory of your heart.
  • Magnetic Resonance (MR) imaging/angiography The primary role of this exam is to decide if there is ample bloodstream directed to the heart muscle. If there are regions with diminished bloodstream, this could demonstrate plaque with vein narrowing. This bloodstream assessment might be done twice amid the exam. The coronary veins (that supply blood and oxygen to the heart) can likewise be imaged with CT angiography to search for any plaque development, which may bring about the narrowing of the veins. X-ray machines utilise a strong magnetic field, radio waves and a PC to deliver high definition pictures.

What Treatments are Available for HLHS?

The following procedures are used to treat HLHS.

 

  • Modified Norwood Procedure (Stage One) Here, the specialist interfaces the major veins of the heart to allow blood to be directed into the body. A tube is then connected from aorta to the lung's vein (pulmonary artery).
  • Modified Norwood Procedure (Norwood with a Sano Shunt) The Sano shunt procedure helps in controlling the flow of blood between the lungs and coronary arteries. A 5-mm graft is chosen for average sized infants. Distally, the graft is linked with the primary pulmonary artery. The proximal end of the shunt is connected to a constrained infundibular cut in the right ventricle.
  • Cavo-Pulmonary Connection (Stage Two) for all single ventricle conditions The point of this task is to divert the stream of blue blood (deoxygenated blood) to the lungs by connecting the upper body vein (superior vena cava) directly to the lung artery (pneumonic course). The Gore-Tex shunt or band is taken away after the procedure.
  • The Fontan Procedure (Stage Three) The Fontan surgery is used to treat inherent or congenital heart defects in which only one side of the heart is functioning. Specialists utilise the technique to redirect venous (deoxygenated) blood to the pulmonary arteries without a pumping chamber. Venous weight dictates the thrusting power through the pulmonary flow. The single functional ventricle functions as a pump to supply blood to the rest of the body.
  • The Hybrid Procedure The Hybrid approach saves the infant from open heart medical procedure until the point that the child is stronger to endure surgery. This methodology includes stenting of the patent ductus arteriosus (PDA) and banding the veins to the lungs (aspiratory corridors). The infant returns at 4 to a half year of age for the open-heart medical procedure.
  • The Damus Procedure The Damus– Kaye– Stansel (DKS) method is a cardiovascular surgery used to repair congenital heart defects like HLHS. In this procedure, the pulmonary artery and the aorta are joined in case the systemic circulation is hindered.

Postoperative Follow-up

After a medical procedure or a transplant, your infant will require long-lasting care. The cardiologist will track the patient's progress during follow-ups. Different complications might arise after some time and may require treatment or drugs.

The cardiologist will also recommend antibiotics in case the chances of infection is present. The child's movements must be restricted to facilitate the healing.

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Hospitals

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