Overview

What is Hypoplastic left heart syndrome?

It is a rare, at-present-congenital heart disorder. In this disease, the development of the left side of the heart is inferior. A child with hypoplastic left heart syndrome does not have good pumping function on the left side of the heart at birth. On the other hand, it should pump blood to different parts and lungs of the body. Medication treatment is necessary to keep the ductus arteriosus, the passageway that connects both the left and right parts of the heart, from closing up. Likewise, a heart transplant or surgery is in order. Because of advances in medical care, the outlook for babies born with this disease has gotten better, symptoms of Hypoplastic left heart syndrome.

What are hypoplastic left heart syndrome symptoms?

Hypoplastic Left Cardiac Syndrome is a severe heart condition in which the left side of the heart does not develop properly. Symptoms that always occur shortly after birth:

Cyanosis: A lack of oxygen resulting in the blue or gray tint of the skin, lips, and nails.
Breathing trouble
Feeding problem: Baby may become tired quickly while being fed.
Lethargy: The child seems remarkably tired or uninterested.

What causes Hypoplastic left heart syndrome?

In the vast majority of cases, a definite cause for HLHS is unidentified. The likely fact is that it is an interaction between certain genes and environmental factors. Some implicit contribution factors are listed below:

Inheritable factors: Mutations in specific genes, such as GJA1 or NKX2-5, have been associated with increased risk for HLHS.
Chromosomal disorders: HLHS may be linked with other disorders such as the Turner pattern or trisomy 18(a redundant dupe of chromosome 18).
Environmental causes: Though some environmental causes are still under study, they are not well-established, but there may be some.

What is the diagnosis and test for Hypoplastic left heart syndrome?

HLHS is always associated with problems before or shortly after birth. Common tests are as follows-

Diagnosis in mother's womb- Fetal echocardiogram-

It can diagnose HLHS and other heart defects when the future child is in the mother's womb. Diagnosis after birth-

This is in an echocardiogram form
EKG is short for electrocardiogram, or in other words, a recording of the electrical activities of all the heart's functions.
A chest X-ray can show the size and location of the heart and lungs.
Pulse oximetry measures oxygen in the blood.

Sometimes, cardiac catheterization can provide more information about heart structure and function in addition to the clinical assessment. Such tests are generally reserved for older children or when less invasive studies are inconclusive.

What is the management of Hypoplastic left heart syndrome?

HLHS is a condition requiring very complex and technical care. Normally, treatment includes medication along with surgery.

Medication-

Prostaglandins- This particular group of medicines helps maintain the open ductus arteriosus (a blood vessel connecting the pulmonary artery and the aorta), which allows blood to flow into the lungs.

Surgery-

HLHS sufferers are often treated with offered reconstruction, a phrase used to describe a series of complex procedures. This includes:

The Norwood Procedure, the first procedure that often occurs within the first week of life. It is the process of opening a new passageway for blood to go into the lungs and body.
Glenn Procedure: The procedure is usually done when the child is about 3-6 months old. It has been described as the diversion of blood from the head to the lungs.
Fontan Procedure: This is the last stage, usually done when the child is 2 to 4 years old. The procedure involves turning all blood from the body to the lungs without passing through the heart.
A heart transplant may be an option in some patients, including if the suggested surgery fails or the patient's status worsens.

Is hypoplastic left heart syndrome well treated with a heart transplant?

The three-stage surgical series can sometimes be replaced with the recommendation by your baby's surgeon for a heart transplant. Babies who receive heart transplants need to stay on lifelong medication.

Is there anything that could reduce my risk of HLHS?

There is never a proven cause in HLHS. Doctors, however, always recommend healthy practices during your pregnancy, as well as:

Avoid smoking and alcohol.
Manage any illness, including diabetes.
Having a healthy diet.
consuming a prenatal vitamin daily with 400 micrograms (mcg) or more of folic acid.
Meet with an expert in genetics before pregnancy if hypoplastic left heart syndrome seems to be hereditary in your family or the family of the other potential parent.

How many children living with hypoplastic left heart syndrome survive?

During the first year of life, 20 to 60 percent of infants with hypoplastic left heart syndrome survive. Since then, about 40 percent of patients have survived the last five, ten, and fifteen years. Normal-weight babies feed better than underweight babies. Some studies indicate that nearly all premature babies survive to age 18.

Living With

How do I manage?

Management requires the care of a cardiologist experienced in adult congenital heart disease. Advances in surgical management mean that children with hypoplastic left heart syndrome can survive to adulthood. Since this is relatively new, it is not yet known what complications an adult with the condition may experience. Adults continue to have regular follow-up care to monitor any changes in their health status.

Why Tender Palm Super-Speciality Hospital for Hypoplastic Left Heart Syndrome?

Tender Palm Hospital has the most experienced team of Cardiologists, Cardiac Surgeons, and diagnostics with the latest and International standard infection control measures in Lucknow, India. The Cardiac Science Centre team has decades of experience in successfully treating Hypoplastic Left Heart Syndrome.