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Bland-White-Garland Syndrome - Causes, Symptoms, and Treatment

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An anomalous left coronary artery from the pulmonary artery is a heart disease that occurs in people from birth.

Medically reviewed by

Dr. Muhammad Zohaib Siddiq

Published At November 27, 2023
Reviewed AtNovember 27, 2023

Introduction:

An anomalous left coronary artery from the right pulmonary artery (ALCAPA) is a rare defect that affects the heart. The disease may affect the child during birth which is called congenital ALCAPA. Normally, the coronary artery in the heart comes out from the aorta. But in ALCAPA, the coronary arteries develop from the pulmonary artery. The coronary arteries are responsible for regular blood supply and oxygen to the heart. When these arteries develop from the pulmonary artery, the blood and oxygen supply to the heart gets affected, leading to several heart complications. The complications can be cardiomyopathy (defective blood supply to the heart), heart failure, and sudden cardiac death. If the condition is diagnosed and managed early, it can improve the patient's heart health and save their life. The other name of ALCAPA is Bland-White-Garland syndrome. The article discusses ALCAPA, its causes, signs and symptoms, diagnosis, and treatment.

What Are the Causes of an Anomalous Left Coronary Artery From the Pulmonary Artery?

  • ALCAPA is a rare cardiovascular anomaly affecting infants from birth. The problem occurs when the baby's heart starts developing early during pregnancy. During heart development, there is no proper attachment of the developing blood vessels to the muscles of the heart. The left coronary artery usually comes from the aorta in a normal or healthy heart. The left coronary artery supplies blood and oxygen to the left part of the heart, such as to the left ventricle, left atrium, and mitral valve (present between the left atrium and ventricle). The largest blood vessel, the aorta, pulls oxygen and blood from the heart and provides all other body areas.

  • In the case of ALCAPA, the left coronary artery comes out from the pulmonary artery. The pulmonary artery blood lacks oxygen from the heart and goes to the lungs to take oxygen. During this heart defect, the blood lacking in oxygen is provided to the heart muscles on the left part of the heart. The heart muscle on the left side of the heart does not get proper oxygen leading to the death of heart tissue. This can lead to the generation of a condition called a heart attack.

What Are the Risk Factors for an Anomalous Left Coronary Artery From the Pulmonary Artery?

Certain factors that can increase the risk of this congenital heart defect occurring are as follows:

  • Genetic Factors: Individuals with a family history of congenital heart defects are more prone to developing ALCAPA.

  • Environmental Factors: If a mother during pregnancy is exposed to certain toxins, infections, or medications could contribute to developing the condition.

  • Chromosomal Abnormalities: Some chromosomal abnormalities, such as Down syndrome, in which trisomy 21 are associated with the increased risk of getting congenital heart defects, including ALCAPA.

  • Maternal Health: Poor maternal health or if the mother is suffering from medical conditions such as diabetes or infections during pregnancy which can lead to an increased chance of congenital heart defects.

  • Advanced Maternal Age: Some research studies suggest a link between advanced maternal age and an increased risk of congenital heart defects.

What Are the Signs and Symptoms of an Anomalous Left Coronary Artery From the Pulmonary Artery?

The signs and symptoms of ALCAPA vary in different people. The rare congenital heart defect symptoms become apparent during infancy or childhood. The signs and symptoms of ALCAPA are as follows:

  • Heart Failure Symptoms: Due to the lack of blood and oxygen to the heart muscle, an individual may experience heart failure symptoms such as poor weight gain, rapid breathing, fatigue, and excessive sweating.

  • Irritability: Infants with heart defects may appear irritable and fussy. The baby's sleep also gets affected, and faces difficulty in sleeping.

  • Breathing Difficulties: During feeding to an infant, there can be rapid breathing.

  • Skin Color: The skin color of an infant can be pale or grayish. Due to poor oxygenation, there can be a bluish tint around the lips and fingertips.

  • Chest Pain: Older children may experience chest pain during any physical activity.

  • Arrhythmias: An individual may develop irregular heart rhythms, leading to fainting, dizziness, and palpitations.

  • Heart Attack: In case of coronary steal, there can be backflow of the blood or oxygen toward the pulmonary artery instead of moving toward the heart muscle causing the problem called a heart attack. Coronary steal can only develop in babies if ALCAPA is not treated over time.

How Can an Anomalous Left Coronary Artery From the Pulmonary Artery Be Diagnosed?

The rare congenital heart defect is not diagnosed during the pregnancy period. It is detected only when the parent notices symptoms in their child. In some cases, the condition is diagnosed when the child gets older. The diagnostic procedures are as follows:

  • Chest X-Ray: The enlargement of the heart in the X-ray indicates the diagnosis of the condition.

  • Echocardiogram: In this, the sound waves are used to create an image. This will provide information about the heart structures and blood flow of the heart.

  • Electrocardiogram (ECG): The reports help diagnose a heart attack by measuring the heart's electrical activity.

  • Cardiac Catheterization: The cardiac procedure in which a healthcare professional places a thin tube in the heart to visualize the blood and oxygen levels.

How Is an Anomalous Left Coronary Artery From a Pulmonary Artery Treated?

The treatment measures for an anomalous left coronary artery from a pulmonary artery are as follows:

  • Surgical Treatment: The healthcare provider will advise surgery to treat the heart condition. However, the baby's health condition and blood size will tell about whether surgery can be done. The left coronary artery is removed from the pulmonary artery and attached to the aorta. The connection between the pulmonary artery and the left coronary artery is blocked. The new left coronary artery is formed by taking a vein from the leg and connecting the left coronary artery and the subclavian vein. A heart transplant is recommended in cases where the heart is severely damaged due to limited oxygen and blood supply.

  • Medications: The healthcare provider may prescribe diuretics, inotropic drugs, beta-blockers, and ACE inhibitors. These drugs decrease the heart workload.

Conclusion:

ALCAPA is a rare congenital disability that affects the heart of many babies. The heart defect in which the coronary artery is attached to the pulmonary artery. Normally, it is attached to the aorta. Babies with the condition may feel rapid heart rate, palpitations, fainting, irritability, breathing difficulties, heart attack, and sudden cardiac death. The only treatment to manage the heart condition is surgery. Early detection of ALCAPA prevents further complications such as heart failure and heart attack.

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Dr. Muhammad Zohaib Siddiq
Dr. Muhammad Zohaib Siddiq

Cardiology

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