Truncus Arteriosus

Definition

Truncus arteriosus is a rare type of congenital heart disease characterized by a single blood vessel arising from the right and left ventricles, instead of the normal two (pulmonary artery and aorta).

There are three subtypes of truncus arteriosus, depending on the specific anatomy of the single vessel.

Heart, section through the middle

Alternative Names

Symptoms

• Lethargy
• Poor feeding
• Shortness of breath (dyspnea)
• Rapid breathing (tachypnea)
• Fatigue
• Cyanosis (blue discoloration of skin)
• Delayed growth or growth failure
• Broadening of the finger tips (clubbing)

Signs and tests

The cardiologist or pediatrician usually hears a murmur when listening to the heart with a stethoscope.

• ECG shows signs of enlargement of the heart (ventricular hypertrophy).
• X-ray of the chest shows heart enlargement and fluid-filled lungs.
• Echocardiogram shows a ventricular septal defect (VSD) and a single truncal artery -- definitive diagnosis.
• Rarely, a heart catheterization is necessary to help with the diagnosis or planning of a treatment strategy.

Treatment

Surgery is needed to treat this condition. Two procedures are available: one is banding of the pulmonary arteries coming off the truncus; the other is complete repair. Complete repair appears to be the preferred option but as the child grows, repeat surgical procedures may be necessary.

Expectations (prognosis)

Complete repair usually affords good results, although re-operation is sometimes necessary as the patient grows. Untreated cases have a poor outcome, usually leading to death between the first and second years of life. Sometimes the diagnosis is missed until early adulthood; these patients generally need a heart and a lung transplant.

Complications

• Heart failure
• Pulmonary hypertension (high blood pressure in the lungs)

Calling your health care provider

Call your health care provider if your infant or child appears lethargic, does not eat well, appears excessively tired or mildly short of breath, or does not seem to be growing or developing normally.

If the child's skin, lips, or nail beds appear blue or if the child seems significantly short of breath, take the child to the emergency room or have the child examined promptly.

Prevention

There is no known prevention, but early treatment can often prevent serious complications.

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Review Date:6/27/2006
Reviewed By: Anne J. L. Chun, M.D., Assistant Professor of Pediatrics, Division of Pediatric Cardiology, New York University School of Medicine, New York, NY.Review provided by VeriMed Healthcare Network.