Hypoplastic Left Heart Syndrome - Causes, Symptoms, Diagnosis, and Treatment

Introduction

Hypoplastic left heart syndrome, or HLHS, is a cardiovascular defect present during birth. This defect hampers the regular functioning of the heart. As the fetus develops, the left portion of the heart does not grow correctly. Hypoplastic left heart syndrome can be understood as a congenital heart defect. This congenital heart defect is considered to be a highly critical condition because the newborn needs to have surgery immediately after birth.

Hypoplastic left heart syndrome comes under the category of a critical congenital heart defect. When the fetus develops inside the mother’s womb, the right side of the heart pumps deoxygenated blood from the heart to the lungs, and the left side of the heart transfers oxygenated blood to the rest of the body. There are two openings present in the heart of the fetus that gets closed immediately after birth- patent foramen ovale and patent ductus arteriosus. In babies with hypoplastic left heart syndrome, the oxygenated blood fails to reach the body since the left side of the heart is not well developed. The blood supply to the rest of the body thus becomes extremely difficult.

What Are the Structures That Get Affected Due to Hypoplastic Left Heart Syndrome?

The heart is divided into four chambers, the upper left and right atrium and the lower left and right ventricles. Hypoplastic left heart syndrome hampers the structure and function of several structures on the left portion of the heart. This leads to these particle areas not developing completely. Mentioned below are the structures that get affected due to hypoplastic left heart syndrome.

• The left ventricle is too small.

• The mitral valves are not formed.

• The aortic valve is very small.

• The ascending portion of the aorta is not developed.

• Atrial septal defect.

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What Are the Causes of Hypoplastic Left Heart Syndrome?

Hypoplastic left heart syndrome does not have any established cause for its prevalence. Nevertheless, there are some factors that have been studied to pose the risk of developing hypoplastic left heart syndrome, such as those mentioned below. It should be noted that hypoplastic left heart syndrome is diagnosed as soon as the baby is born. It is also possible to diagnose the defect during pregnancy.

• Genetic variation and defects.

• Chromosome alterations.

• Allergic food is taken during pregnancy.

• Presence of another congenital heart defect.

What Are the Signs and Symptoms of Hypoplastic Left Heart Syndrome?

Generally, babies with hypoplastic left heart syndrome undergo correction and rehabilitation immediately after birth. During this, there are certain clinical manifestations that confirm the diagnosis of hypoplastic left heart syndrome.

Mentioned below are some signs and symptoms of hypoplastic left heart syndrome.

• Difficulty in breathing.

• Pounding heartbeat.

• Feeble pulse.

• Bluish discoloration of the skin.

• Whooshing murmurs in the heart.

• Low levels of oxygen.

• Electrocardiograph showing an unusual pattern.

• Poor feeding.

• Clammy and cool skin.

How Is Hypoplastic Left Heart Syndrome Diagnosed?

Hypoplastic left heart syndrome can be diagnosed during pregnancy or even after birth. Mentioned below are a few of the diagnostic tools that help in the diagnosis of hypoplastic left heart syndrome.

• Pulse oximetry screening.

• Echocardiography.

• Chest x-rays.

• Prenatal testing.

• Presence of family defects.

How to Treat Hypoplastic Left Heart Syndrome?

Hypoplastic left heart syndrome has several treatment modalities. The best treatment option is chosen by the healthcare provider according to the clinical manifestations of the patient. Mentioned below are a few of the treatment and management varieties present for hypoplastic left heart syndrome.

• Medications to increase the strength of the cardiac muscles.

• Medications that aid in the removal of excess fluid from the body.

• High-calorie customized nutrition plan.

• Nutrition given through a feeding tube.

• Norwood surgical procedure is done during the initial two weeks by creating a new aorta and by joining it to the right ventricle. Additionally, a tube is placed that supplies blood to the lungs via the right ventricle or the aorta.

• The Bi-directional Glenn Shunt procedure is done when the patient is approximately six months old by creating a strong and direct link between the superior vena cava and the pulmonary artery.

• Fontan procedure can be performed even up to three years of age where a connection is made between the pulmonary artery and the inferior vena cava that transfers back deoxygenated blood from the ventricles.

• It should be noted that children who undergo surgical correction can not be permanently cured. They may have lifelong complications and may also require regular follow-ups with the cardiologist in order to assess their progress.

Conclusion

Hypoplastic left heart syndrome is a congenital cardiovascular defect that hampers the heart and its structure and thus does not allow it to function to its best efficiency. The left ventricle is the section of the heart that gets affected the most. This leads to the heart not being able to differentiate between oxygen-rich and deoxygenated blood. Patients require immediate treatment. There are several surgical interventions, but none of them can promise a full recovery. Patients need to be examined and evaluated throughout their life in case they have been diagnosed with hypoplastic left heart syndrome.