×

Please Remove Adblock
Adverts are the main source of Revenue for DoveMed. Please remove adblock to help us create the best medical content found on the Internet.

Anomalous Left Coronary Artery from the Pulmonary Artery

Last updated May 1, 2018

Approved by: Krish Tangella MD, MBA, FCAP

Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA) is a rare birth defect affecting the blood vessels of the heart.


What are the other Names for this Condition? (Also known as/Synonyms)

  • ALCAPA (Anomalous Left Coronary Artery from the Pulmonary Artery)
  • Anomalous Origin of the Left Coronary Artery arising from the Pulmonary Artery
  • Bland-White-Garland Syndrome

What is Anomalous Left Coronary Artery from the Pulmonary Artery? (Definition/Background Information)

  • Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA) is a rare birth defect affecting the blood vessels of the heart
  • Normally, 2 coronary arteries (right and left) present on the surface of the heart, carry oxygen-rich blood to the heart muscles from the aorta (which is the largest blood vessel that carries oxygen-rich blood from heart to the whole body). Adequate coronary artery circulation is required to supply blood to the heart muscles so that it can effectively pump blood
  • In ALCAPA, the left coronary artery arises from the pulmonary artery. Normally, pulmonary artery carries oxygen-poor blood from the right side of the heart to the lungs, to get it oxygenated
  • Due to this birth defect, the heart gets ‘lesser’ oxygenated blood, which results in weakening or death of the heart muscle cells, thus causing heart attack, heart failure, and eventually death
  • If this condition progresses beyond childhood, numerous connections (anastomoses) develop between the right coronary artery and the abnormally arising left coronary artery
  • When this occurs, the oxygenated blood from the right coronary empties into the left coronary artery, which in turn empties into the pulmonary artery going to the lungs. This ultimately results in even more decreased blood supply to the left side of the heart (coronary steal phenomenon) resulting in chest pain
  • Infants, who undergo corrective surgery for this defect, are expected to lead a normal life

Who gets Anomalous Left Coronary Artery from the Pulmonary Artery? (Age and Sex Distribution)

  • Anomalous Left Coronary Artery from the Pulmonary Artery is a congenital defect that is usually seen among the newborn infants. If the child survives infancy, the condition may even be present into childhood or even into adulthood
  • ALCAPA is equally distributed among both the male and female population
  • It is not known to particularly affect any specific region, race, or ethnic group

What are the Risk Factors for Anomalous Left Coronary Artery from the Pulmonary Artery? (Predisposing Factors)

Currently there are no known risk factors associated with Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA).

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Anomalous Left Coronary Artery from the Pulmonary Artery? (Etiology)

Currently, the causative factor of Anomalous Left Coronary Artery from the Pulmonary Artery is unknown.

  • Although it is a birth defect, scientists currently believe it is not due to family inheritance, genetic factors, or chromosomal defects
  • Also, studies have shown that teratogens (toxins) are not involved in the development of this condition
  • ALCAPA usually occurs as an isolated defect. Rarely, it may be associated with other heart defects

What are the Signs and Symptoms of Anomalous Left Coronary Artery from the Pulmonary Artery?

This defect causes a poor flow of blood to the heart muscle resulting in weakening or death of these muscles. When the heart muscles die (heart failure), the heart cannot pump blood to different parts of the body. Heart failure leads to abnormal dilation of the heart, which in turn may cause incompetence in in the mitral valve of the heart.

The signs and symptoms observed in infants with Anomalous Left Coronary Artery from the Pulmonary Artery are mainly due to heart failure and its complications. These include:

  • Difficulty in breathing
  • Difficulty in feeding, abnormal crying or sweating during feeding
  • Failure to thrive; the child may appear small for their age
  • Crying and/or distress that can be misinterpreted for a colic
  • Pale skin due to poor oxygenation of blood
  • Irregular or fast heartbeats, heart murmur (abnormal sounds other than the normal heart beats)

If the infant survives beyond infancy, they may present with chest pain (heart attack) and irregular heartbeats (abnormal rhythm of the heart); in some cases, it may even result in sudden death.

How is Anomalous Left Coronary Artery from the Pulmonary Artery Diagnosed?

The following procedures may be used to diagnose Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA):

  • Thorough evaluation of the individual’s medical history and a thorough physical examination including of the heart, lungs, skin, etc.
  • During history taking the physician may want to know the following:
    • When the symptoms began and whether they are becoming worse
    • About one’s personal and family history of congenital heart defects, or other organ defects (if any)
    • The physician will notice signs, such as pale skin, breathing difficulties or feeding difficulties, which may indicate that there is a problem with blood flow from the heart         
  • Uncommonly, ALCAPA may be diagnosed very late, in childhood or adulthood
  • Consultation with pediatric cardiologists is often necessary, as they are the experts in dealing with heart disease in a child
  • Chest x-ray may show an enlarged heart
  • An echocardiogram (ultrasound of the heart) may indicate the abnormally arising left coronary artery, enlarged heart, mitral valve incompetence, and other abnormalities
  • Color doppler (special ultrasound) may show the defect more clearly than an echocardiogram
  • An ECG (electrocardiogram) may show abnormal electrical activity in the heart

The following tests can help in precisely locating the defect:

  • MRI scan of the heart
  • Cardiac catheterization: A thin hollow tube is inserted into a blood vessel away from the heart and is directed into the heart
  • Arteriography: A dye is injected into the blood vessel and later x-ray pictures are taken, showing the abnormal anatomy

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Anomalous Left Coronary Artery from the Pulmonary Artery?

The complications of Anomalous Left Coronary Artery from the Pulmonary Artery could include:

  • Heart rhythm abnormalities may arise due to dilation of the heart, abnormal pouching of the heart (aneurysm), or death of heart musculature. Such conditions may cause an irreversible damage to the heart
  • Individuals with the condition have the possibility of having a heart attack or developing heart failure
  • Individuals with the condition may develop complications following the surgery, such as obstruction to blood flow through the pulmonary valve

ALCAPA can cause one of the most feared complications in adolescence and adulthood, which is sudden death.

How is Anomalous Left Coronary Artery from the Pulmonary Artery Treated?

Anomalous Left Coronary Artery from the Pulmonary Artery is best treated by performing a heart surgery. The surgery will focus on re-connecting the coronary artery that is going to the pulmonary artery, back to the aorta.

  • Mitral valve may have to be replaced, if it is affected (incompetence). A heart valve is responsible for moving blood from one area of the heart to the other. If this valve is incompetent, it will cause further damage to the heart
  • If the heart is very badly damaged, a heart transplant may be the best option available

Other treatment options, if surgery is not immediately possible, may include:

  • Use of diuretics (water pills to remove excess water in the body)
  • Ionotropes (medications to improve heart pumping)
  • Medications to decrease the workload of the heart, such as ACE inhibitors

The child should be able to function normally after surgery; however, regular check up with the cardiologist is suggested.

How can Anomalous Left Coronary Artery from the Pulmonary Artery be Prevented?

  • Anomalous Left Coronary Artery from the Pulmonary Artery is a birth defect that cannot be prevented
  • However, the complications that arise due to this birth defect can be prevented, if the condition is treated promptly and if precautions are taken
  • Adolescents and young adults are advised to keep away from physical activities and exercising, which may prevent certain heart complications, or even sudden death

What is the Prognosis of Anomalous Left Coronary Artery from the Pulmonary Artery? (Outcomes/Resolutions)

  • The outlook for Anomalous Left Coronary Artery from the Pulmonary Artery is typically very poor without surgery or a heart transplant. Infants may die in the first year itself
  • Proper treatment through a corrective surgery may result in better outcomes for infants. Nevertheless, routine visits to the cardiologist are necessary to ensure that the heart is functioning properly
  • Infants, who do overcome the heart defect without undergoing any surgeries early in their life, are prone to complications, such as heart attacks, abnormal heart rhythms, heart failure, which may lead to sudden death

Additional and Relevant Useful Information for Anomalous Left Coronary Artery from the Pulmonary Artery:

  • Heart transplantation is a procedure that surgically removes a damaged or diseased heart and replaces it with a healthy heart from a donor

The following article link will help you understand Heart transplantation surgical procedure:

http://www.dovemed.com/common-procedures/procedures-surgical/heart-transplantation/

What are some Useful Resources for Additional Information?


References and Information Sources used for the Article:


Helpful Peer-Reviewed Medical Articles:


Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: May 31, 2015
Last updated: May 1, 2018