Left ventricular hypoplasia syndrome

Left ventricular hypoplasia syndrome . It is an abnormality in the structure of the heart present at birth (congenital). This is a group of related defects that, taken together, means that the left ventricle is smaller than usual. Normally, oxygen- poor blood is pumped from the right ventricle of the heart to the lungs, where it is oxygenated and returns to the left ventricle. The left ventricle then pumps the oxygenated blood to the rest of the body. All babies are born with two connections or leads between the two ventricles of the heart; but after a few days, these connections are closed.

Table of Contents

Summary

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1 Introduction
- 1 Tests and Exams
- 2 Treatment
- 3 The left ventricular hypoplasia syndrome can be prevented
2 Sources

Introduction

In people with LVHS, the left ventricle that did not grow to its normal size cannot pump enough blood to the body. Normal shunts present at birth help direct blood circulation to the body; But when these connections are closed, the supply of oxygenated blood decreases. Therefore, after a few days of birth, babies with LVHS will present symptoms of this condition.

Initially, a newborn with a hypoplastic left heart may appear normal. Symptoms can appear in the first hours of life, although they can take up to a few days to appear. These symptoms can be:

Purple skin ( cyanosis ) or a bad color
Cold hands and feet (limbs)
Lethargy
Weak pulse
Poor lactation and feeding
Throbbing heart strong
Rapid breathing
Breathing difficulty

Tests and Exams

A physical exam may show signs of heart failure :

Faster than normal heart rate
Lethargy
Hepatomegaly
Rapid breathing

Also, the pulse may be very weak at various locations (wrist, groin, and other places). Upon auscultation of the chest , there are usually (although not always) abnormal heart sounds .

Exams may include:

Cardiac catheterization
ECG
Echocardiography
Chest x – ray

What problems do people with left ventricular hypoplasia syndrome have?

Children with SCIH who do not receive treatment die. While this abnormality cannot be corrected, postnatal surgery can create the necessary connections, or shunts, to facilitate the pumping of blood to the body. The right ventricle of the heart , which usually only pumps blood to the lungs, will be responsible for pumping blood to both the lungs and the body. This double work will eventually weaken the heart . Children with LVHS need regular follow-up from a cardiologist throughout their lives and may face health problems as they get older.

What causes hypoplastic left heart syndrome? The causes of hypoplastic left heart syndrome (SCIH) are unknown. This abnormality appears before birth, when the left ventricle of the heart does not grow enough.

Treatment

Once the diagnosis of hypoplastic left heart is made , the baby will be taken to the Neonatal Intensive Care Unit (NICU) and a ventilator (ventilator) may be needed to help him breathe. Also, a medicine called prostaglandin E1 is used to maintain blood circulation to the body , keeping the arterial duct open.

These measures do not solve the problem, since the condition always requires surgery . The first surgery , called the Norwood procedure, is performed in the baby’s first days of life. This procedure consists of building a new aorta :

Using the valve and pulmonary artery .
Connecting the old hypoplastic aorta and the coronary arteries to the new aorta .
Removing the wall between the atria ( atrial septum ).
Making an artificial connection from either the right ventricle or a body artery to the pulmonary artery to maintain circulation to the lungs (called a shunt).

After this, the baby usually goes home. The child will need to take daily medications and a pediatric cardiologist will need to closely monitor him to determine when the second stage of surgery should be performed.

The second stage of the surgery is called a Glenn shunt or Hemifontan procedure. This procedure connects the largest vein that carries blue blood from the upper half of the body (superior vena cava) directly to the blood vessels that go to the lungs (pulmonary arteries) to obtain oxygen. This surgery is usually done when the child is between 4 and 6 months old.

During stages I and II, the child may still appear somewhat purple (cyanotic). Stage III, the final step, is called the Fontan procedure. The rest of the veins that carry blue blood from the body (inferior vena cava) connect directly to the blood vessels that go to the lungs. The right ventricle now serves only as the pumping chamber for the body (no longer the lungs and the body). This surgery is usually carried out when the baby is between 18 months and 4 years old. After this last step, the baby will no longer become cyanotic and will have a normal level of oxygen in the blood .

Some patients may need more surgeries when they reach their 20s or 30s if they have difficult to control arrhythmias or other complications from the Fontan procedure. Some doctors consider a heart transplant as an alternative to 3-step surgery , but there are few donated hearts available for young children.