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Anomalous left coronary artery from the pulmonary artery

Anomalous origin of the left coronary artery arising from the pulmonary artery; ALCAPA; ALCAPA syndrome; Bland-White-Garland syndrome

Last reviewed: May 14, 2014.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a heart defect. The left coronary artery, which carries blood to the heart muscle, is connected to the pulmonary artery instead of to the aorta.

ALCAPA is present at birth (congenital).

Causes

An anomalous left coronary artery from the pulmonary artery is a problem that occurs when the baby's heart is developing early in the pregnancy. The developing blood vessels in the heart do not connect correctly.

In the normal heart, the left coronary artery starts in the aorta. The aorta is the major blood vessel that takes oxygen-rich blood from the heart to the rest of the body.

In children with ALCAPA, the left coronary artery starts at the pulmonary artery. The pulmonary artery is the major blood vessel that takes oxygen-poor blood from the heart to the lungs.

Anomalous left coronary artery

When this defect occurs, blood that is lacking in oxygen is carried to the left side of the heart. Therefore, the heart does not get enough oxygen. When the heart muscle is deprived of oxygen, the tissue begins to die. This condition leads to a heart attack in the baby.

A condition known as "coronary steal" further damages the heart in babies with ALCAPA. The low blood pressure in the pulmonary artery causes blood from the abnormal left coronary artery to flow toward the pulmonary artery instead of toward the heart. This results in less blood and oxygen to the heart. This problem will also lead to a heart attack in a baby. Coronary steal develops over time in babies with ALCAPA if the condition is not treated early.

Symptoms

Symptoms of anomalous left coronary artery in an infant include:

  • Crying or sweating during feeding
  • Pale skin
  • Poor feeding
  • Rapid breathing
  • Sweating
  • Symptoms of pain or distress in the baby (often mistaken for colic)

Symptoms can appear within the first 2 months of the baby's life.

Exams and Tests

ALCAPA can be diagnosed in an infant. However, this defect may not be diagnosed until someone is a child or adult.

Signs of ALCAPA include:

Tests for anomalous left coronary artery include:

Treatment

Surgery is needed to correct ALCAPA. Only one surgery is needed in most cases. However, the surgery will depend on the baby's condition and the size of the involved blood vessels.

If the heart muscle supporting the mitral valve is seriously damaged from decreased oxygen, the baby may also need surgery to repair or replace the valve. The mitral valve controls blood flow between the chambers on the left side of the heart.

If the baby's heart is already severely damaged from lack of oxygen, a heart transplant may be an option.

Medicines used include:

  • "Water pills" (diuretics )
  • Drugs that make the heart muscle pump harder (inotropic agents)
  • Drugs that lower the workload on the heart (beta-blockers, ACE inhibitors)

Outlook (Prognosis)

Without treatment, most babies do not survive their first year. Children that do survive without treatment may have serious cardiovascular problems. Babies with this problem who aren't treated could die suddenly during the following years.

With prompt treatment such as surgery, most babies do well and can expect a normal life. However, they need routine follow-ups with a heart specialist (cardiologist).

Possible Complications

Complications of ALCAPA include:

When to Contact a Medical Professional

Call your health care provider if your baby:

  • Is breathing rapidly
  • Looks very pale
  • Seems distressed and cries often

References

  1. Scholz TD, Reinking BE. Congenital heart disease. In: Gleason CA, Devaskar S, eds. Avery's Diseases of the Newborn. 9th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 55.

Review Date: 5/14/2014.

Reviewed by: Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only — they do not constitute endorsementscof those other sites. © 1997–2011 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

Copyright © 2013, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is an independent audit to verify that A.D.A.M. follows rigorous standards of quality and accountability. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial policy, editorial process and privacy policy. A.D.A.M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only — they do not constitute endorsementscof those other sites. © 1997–2011 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

Copyright © 2013, A.D.A.M., Inc.

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