Congenital Heart and Vascular Malformations: Causes, Symptoms, Diagnosis, and Treatment

Introduction

Congenital heart diseases affect the structure of a baby's heart. It affects the blood flow and how it flows through the heart and the rest of the body. Congenital heart disease can be classified as severe or mild. Mild is like a small hole in the heart or severe such as missing or poorly formed parts of the heart. About one percent of live birth are born with CHDs. Babies with CHD require surgery or other procedures in the initial year of life. Congenital heart diseases are diseases in the heart and great vessels. Approximately every one percent of the child in hundred life births has congenital heart disease. In premature babies and stillborns, this incidence is more.

What Are the Right and Left Shunts?

Shunt means blood from the right or left side of the heart is mixing pathologically. Under certain circumstances, we can see left through right shunt congenital heart disease. Babies born with the atrial septal defect have left to right shunt and slightly more pressure in the left atrium. The blood from the left ventricle is shunted to the right ventricle. In both cases, the left oxygenated blood moves toward the right side of the heart. Usually, the blood coming from the venous side, the hemoglobin, is 75 percent saturated. On the arterial side, hemoglobin saturation of oxygen keeps shifting from the left to the right side, which produces a step-up in oxygen saturation on the right side. The hemoglobin oxygen saturation is more on the right side of the heart.

An atrial septal defect is a pathological hole in the atrium. A ventricular septal defect is a pathological hole in the ventricle. Usually, in babies, there is a small blood vessel connecting the pulmonary artery and aorta called ductus arteriosis.

During intrafoetal life, the pulmonary vessels remain spastic, and the lungs do not work full-fledged. When the baby is in the uterus lungs are not working. The lungs have no oxygen supply, so the pulmonary arterioles remain spastic. The pulmonary arterioles in the lungs during fetal life are constricted. They are not working. Oxygen is the dilator for the pulmonary artery, and hypoxia is a constricting factor for pulmonary arterioles. It means there is no longer oxygen. Oxygen produces dilation of the pulmonary vasculature. In intrauterine life, the oxygen going to the lungs is restricted. The following are the types of congenital heart disease:

• Pulmonary Atresia.

• Single ventricle.

• Teratology of Fallot.

• Tricuspid atresia.

• Ventricluar septal defect.

• Hypoplastic left heart syndrome.

• Ebstein anomaly.

What Are the Clinical Features of Congenital Heart Disease?

• Cyanosis (bluish color to the lips, fingernails, and skin). It occurs when there is not enough oxygen in the blood.

• Fatigue.

• Poor blood flow.

• Breathlessness.

• Heart murmurs.

What Are the Two Main Groups of Congenital Heart Disease?

There are two main groups of CHD. They are:

1. Cyanotic Congenital Heart Disease - Cyanotic CHD includes heart defects that decrease the oxygen delivered to other body parts. It is called a critical congenital heart defect. Babies with cyanotic congenital heart disease have low oxygen levels and will require surgery. Examples include-

• Left Heart Obstructive Lesions - In this, blood flow between the heart and the body parts decreases.

• Right Heart Obstructive Lesions - Reduce blood flow between the heart and lungs.

• Mixing Lesions - The body mixes systemic and pulmonary blood flow with this defect.

2. Acyanotic Congenital Heart Disease - Acyanotic congenital heart disease is a defect where the blood throughout the body is abnormal. For example:

• Hole in the Heart - The heart valve has an abnormal opening called an atrial septal defect, atrioventricular canal, or patent ductus arteriosis.

• Problem With the Aorta - Aorta is the main artery of the body. It carries blood from the heart to other parts of the body.

• Pulmonary Artery Problem - Pulmonary stenosis is when the pulmonary artery carrying blood from the right heart to the lungs gets too narrow. It supplies oxygen to the body.

What Are the Causes of Congenital Heart Disease?

The causes of CHDs are unknown. CHDs also caused by a combination of genes and other factors, such as environment, the mother's diet, health conditions, or medication used during pregnancy. Smoking during pregnancy and taking certain medications have also been linked to heart defects. The common types of congenital heart defects are:

• Septal Defect - Hole in the septum (hole) between the left and right side of the heart.

• Heart Valve Defects - Defects in the valves that control blood flow through the heart.

• Defects in the Large Blood Vessels - It carries blood in and out of the heart.

What Are the Signs and Symptoms of Congenital Heart Disease?

The followings are the signs and symptoms of congenital heart disease:

• Blue-tinted nails or lips.

• Fast or troubled breathing.

• Tiredness when feeding.

• Sleepiness.

For example, a fetal echocardiogram uses a sound wave to create fetal heart images. Cyanotic congestive heart disease is detected by pulse oximetry.

What Are the Tests Done to Diagnose Congenital Heart Disease?

The following tests are done to diagnose congenital heart disease in children, and adults:

• Physical Exam - A healthcare provider will listen to the heart for abnormal sounds during an exam.

• Chest X-ray - A chest X-ray takes images of the inner side of the chest to reveal any structural abnormalities.

• Electrocardiogram - It measures the electrical activity of your heart.

• Echocardiogram - It uses ultrasound to create images of the heart's valves and chambers.

• Magnetic Resonance Imaging(MRI) - MRI can create detailed pictures of the heart.

What Is the Treatment for Congenital Heart Disease?

The treatment for congenital heart disease includes:

• The catheter is placed as a plug into the defect.

• Medications are used to help the heart work more efficiently.

• Oxygen therapy provides higher levels of oxygen than normal room air.

• Prostaglandin E1 relaxes smooth heart muscle and keeps open the ductus arteriosus (a blood vessel normally closed after birth), helping provide needed circulation.

• Surgery is done to repair a defect and open the blocked area to restore blood flow. In severe cases, a heart transplant is done.

Does Adult Congenital Heart Disease Require Special Treatment?

Adults with congenital heart disease can develop specific health problems. They can limit the ability to perform everyday tasks and shorten your life span. Examples include:

• Arrhythmia - It can occur when there is a problem with the heart's structure.

• Endocarditis - It is an infection of the heart. Antibiotics before dental and surgical procedures can help prevent heart infections if indicated for a specific defect.

• Heart Failure - The heart cannot pump adequate blood to fulfill the body's requirements. It is sometimes called congestive heart failure.

• Hypertension - Too much pressure to pump the blood to make it flow through the blood vessel.

• Pregnancy Complications - Women with adult congestive heart disease have a risk of pregnancy complications, including arrhythmia, heart failure, and stroke.

• Pulmonary Hypertension - It is high blood pressure in the blood vessels of the lungs. It can lead to heart failure.

• Stroke - A stroke can occur when a blood vessel in the brain gets blocked. This blockage cuts off the blood supply to the brain.

• Sudden Cardiac Death - It is caused by sudden cardiac arrest. This life-threatening condition occurs when the heart's electrical system malfunctions, most commonly causing a fast heartbeat.

• Valve Dysfunction - If the heart's valves are not working correctly, blood gets restricted into the heart chambers, which causes overload or the heart to work harder than it should.

Conclusion

To prevent congenital heart disease, the following things should be followed: recreational drugs should be avoided, all recommended screening tests during pregnancy should be done to detect problems as early as possible, diabetes and phenylketonuria should be managed, avoid smoking, and avoid alcohol.