Anomalous left coronary artery from the pulmonary artery

 

Alternative Names

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

Definition

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.

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:

  • Abnormal heart rhythm
  • Enlarged heart
  • Heart murmur (rare)
  • Rapid pulse

Tests for anomalous left coronary artery include:

  • A test of the electrical activity in the heart (electrocardiogram)
  • A special dye injected into the blood vessels of the heart to see their structure and position (arteriography)
  • A thin tube (catheter) inserted in a blood vessel of the heart to measure blood pressure and oxygen levels (cardiac catheterization)
  • Cardiac magnetic resonance imaging (MRI)
  • Chest x-rays
  • Ultrasound of the heart (echocardiogram)

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:

  • Heart attack
  • Heart failure
  • Heart rhythm problems
  • Permanent damage to the heart

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

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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