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Acyanotic congenital heart disease (ACHD) refers to heart disorders that develop before birth or during birth but do not typically interfere with the amount of oxygen or blood that reaches the body's tissues. Congenital heart disease affects 8 to 9 per 1,000 live births. Approximately 75% of congenital heart disease cases are acyanotic. Acyanotic congenital heart disease can be caused by genetic disorders such as trisomies, maternal infections like rubella, maternal alcohol consumption, and certain medications during pregnancy.  ACHDs are typically diagnosed in infancy or childhood because they cause respiratory, eating, and development issues. Some children with ACHD may not exhibit any symptoms, and their disease is detected during a regular physical examination.

Acyanotic heart disorders do not typically result in cyanosis, which is a bluish tinge to the skin, lips, and nail beds resulting from restricted oxygen supply. If cyanosis develops in a person with an acyanotic cardiac condition, it usually indicates that the heart is unable to pump enough blood and may require additional oxygen. 

What Is Acyanotic Congenital Heart Disease (ACHD)?

Acyanotic heart disease is a type of congenital heart defect which causes abnormal blood flow. These defects do not interfere with the amount of oxygen or blood given to the rest of the body. It may range from mild to severe. Babies with the condition might seem out of breath and fatigued. 

What Are The Symptoms of Acyanotic Congenital Heart Disease?

Acyanotic congenital heart disease involves heart defects that cause abnormal blood flow, but oxygen levels in the blood remain typical. 

Babies born with this disorder do not exhibit any immediate symptoms, but health problems can develop over time.  Abnormal blood flow can lead to increased blood pressure, putting strain on the heart. This can weaken the heart and raise the risk of heart failure.
Another potential risk is abnormally high blood pressure in the lungs, known as pulmonary hypertension. It can  cause symptoms such as:

What Are The Causes of Acyanotic Congenital Heart Diseases?

The following are the risk factors for developing acyanotic congenital heart diseases:

What Are The Different Types of Acyanotic Congenital Heart Diseases?

There are several types of acyanotic congenital heart diseases such as:

  • Aortic stenosis: The aorta is the major artery that carries blood away from your heart to the rest of the body. The aortic valve is located between the heart and the aorta. It opens and closes with each heart cycle to allow blood out of the heart and keep blood from leaking back into the heart. When the aortic valve becomes narrowed and does not open well, it is called aortic stenosis.
  • Atrial septal defect: An atrial septal defect (ASD) is a hole in the wall which separates the heart’s two upper chambers, called the atria. Most ASDs don't close on their own. Tiny ASDs can usually be left alone, but larger ASDs may require a procedure or heart surgery to close them.
  • Atrioventricular septal defect: AnAtrioventricular septal defect is a hole in the center of the heart's wall that separates the upper and lower chambers. The majority of persons who have an atrioventricular septal defect also have abnormalities in one or more of their heart valves. The defect was originally known as an atrioventricular canal defect or endocardial cushion defect.
  • Bicuspid aortic valve: The aortic valve connects the heart to the aorta in just one direction. The valve usually consists of three flaps that open and close to regulate the flow of blood. A bicuspid aortic valve has just two flaps, which might cause it to function improperly.
  • Coarctation of the aorta: With aortic coarctation, the aorta is pinched or narrowed, decreasing the blood flow. 
  • Patent ductus arteriosus: A baby's blood does not need to pass through the lungs to obtain oxygen. Your baby receives oxygen from you instead. The ductus arteriosus is a tiny blood artery that permits fetal blood to bypass the lungs. With patent ductus arteriosus (PDA), the hole fails to close as it should. Extra blood is pumped from the aorta into the pulmonary arteries. PDA is more common in prematurely born newborns.
  • Pulmonary stenosis: The pulmonary artery transports oxygen-poor blood from the right side of the heart to the lungs for oxygenation. The pulmonary valve is located between the right side of the heart and the pulmonary artery. Pulmonary stenosis occurs when the pulmonary valve narrows and fails to open fully.
  • Ventricular septal defect: A ventricular septal defect (VSD) is similar to an atrial septal defect, but it is a hole in the wall that separates the heart’s two lower chambers (the ventricles). VSD is the most common congenital heart defect. Many of these holes may close on their own, but some may require a procedure or surgery to close them.

How To Diagnose Acyanotic Congenital Heart Diseases?

The following are the diagnostic tests that help to diagnose acyanotic congenital heart diseases:

  • Chest X-ray: It helps doctors to visualize the structures inside the chest to reveal structural abnormalities
  • Electrocardiogram: It helps to detect the electrical activity of the heart
  • Echocardiogram: It uses ultrasound waves to create images of the heart’s valves and chambers
  • Right heart catheterization can determine how well your heart is pumping. A thin tube is inserted into a vein and then advanced to the heart. The test can determine how much blood the heart pumps per minute and how much oxygen is in the blood in each heart chamber. This test is also known as pulmonary artery catheterization.
  • Left heart catheterization: This test is similar to right heart catheterization but is carried out on the left side of the heart. Dye is injected into the bloodstream. The dye highlights how blood travels through the arteries, as well as any blockages or irregular pathways. It is also known as coronary angiography.

How To Manage  Acyanotic Congenital Heart Diseases?

Acyanotic heart disease can sometimes resolve itself during childhood. Other times, the heart defect persists until adulthood but does not require treatment. However, if the defect eventually causes symptoms, you may require treatment including:

  • Catheter procedure to place a plug into the defect.
  • Medications to help the heart work more efficiently or to control blood pressure.
  • Surgery to close the defect.


Acyanotic congenital heart disease is a congenital cardiac abnormality that disrupts the normal flow of blood. Sometimes the condition resolves itself during childhood. However, some cardiac problems persist and might eventually need treatment. If you have acyanotic congenital heart disease, it is important to see an adult congenital cardiologist on a regular schedule to check your status.

Frequently Asked Questions(FAQs)

Q: Is aortic stenosis an acyanotic heart disease?

A: Congenital aortic stenosis accounts for around 5% of all heart abnormalities diagnosed during childhood. It belongs to the category of acyanotic congenital heart disease.

Q: Can someone live a long life with a congenital heart defect? 

A: The heart forms by eight weeks into a pregnancy, and the majority of congenital cardiac abnormalities arise during these early stages of development."Many people born with congenital cardiac abnormalities nowadays live well into adulthood and have productive, satisfying lives.

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