Navigate Up

Heart Center - A-Z Index

#
J
Q
X
Z

Print This Page

Anomalous left coronary artery from the pulmonary artery

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) .

Click to download

Alternative Names

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

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:

  • 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.

Updated: 5/14/2014

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.


©  UPMC | Affiliated with the University of Pittsburgh Schools of the Health Sciences
Supplemental content provided by A.D.A.M. Health Solutions. All rights reserved.

For help in finding a doctor or health service that suits your needs, call the UPMC Referral Service at 412-647-UPMC (8762) or 1-800-533-UPMC (8762). Select option 1.

UPMC is an equal opportunity employer. UPMC policy prohibits discrimination or harassment on the basis of race, color, religion, ancestry, national origin, age, sex, genetics, sexual orientation, marital status, familial status, disability, veteran status, or any other legally protected group status. Further, UPMC will continue to support and promote equal employment opportunity, human dignity, and racial, ethnic, and cultural diversity. This policy applies to admissions, employment, and access to and treatment in UPMC programs and activities. This commitment is made by UPMC in accordance with federal, state, and/or local laws and regulations.

Medical information made available on UPMC.com is not intended to be used as a substitute for professional medical advice, diagnosis, or treatment. You should not rely entirely on this information for your health care needs. Ask your own doctor or health care provider any specific medical questions that you have. Further, UPMC.com is not a tool to be used in the case of an emergency. If an emergency arises, you should seek appropriate emergency medical services.

For UPMC Mercy Patients: As a Catholic hospital, UPMC Mercy abides by the Ethical and Religious Directives for Catholic Health Care Services, as determined by the United States Conference of Catholic Bishops. As such, UPMC Mercy neither endorses nor provides medical practices and/or procedures that contradict the moral teachings of the Roman Catholic Church.

© UPMC
Pittsburgh, PA, USA UPMC.com