Persistent truncus arteriosus (patient information)

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Kalsang Dolma, M.B.B.S.[2]

Overview

Truncus arteriosus is a rare type of heart disease that occurs at birth (congenital heart disease), in which a single blood vessel (truncus arteriosus) comes out of the right and left ventricles, instead of the normal two vessels (pulmonary artery and aorta).

There are different types of truncus arteriosus.

What are the symptoms of Persistent truncus arteriosus ?

• Bluish skin (cyanosis)
• Delayed growth or growth failure
• Fatigue
• Lethargy
• Poor feeding
• Rapid breathing (tachypnea)
• Shortness of breath (dyspnea)
• Widening of the finger tips (clubbing)

What causes Persistent truncus arteriosus ?

In normal circulation, the pulmonary artery comes out of the right ventricle and the aorta comes out of the left ventricle, which are separate from each other. Coronary arteries (which supply blood to the heart muscle) come out of the aorta just above the valve at the entrance of the aorta.

In truncus arteriosus, a single artery comes out of the ventricles. There is usually also a large hole between the two ventricles (ventricular septal defect). As a result, the blue (without oxygen) and red (oxygen-rich) blood mix.

Some of this mixed blood goes to the lungs, some goes to the coronary arteries, and the rest goes to the body. Usually, too much blood is sent to the lungs.

If this condition is not treated, two problems occur:

• Too much blood circulation in the lungs may cause extra fluid to build up in and around them, making it difficult to breathe.
• The blood vessels to the lungs become permanently damaged. Over time, it becomes very hard for the heart to force blood to them. This is called pulmonary hypertension and it can be life-threatening.

When to seek urgent medical care?

Call your health care provider if your infant or child:

• Appears lethargic
• Appears overly tired or mildly short of breath
• Does not eat well
• Does not seem to be growing or developing normally

If the skin, lips, or nail beds look blue or if the child seems to be very short of breath, take the child to the emergency room or have the child examined promptly.

Diagnosis

A murmur is usually heard when listening to the heart with a stethoscope.

Tests include:

• ECG
• Echocardiogram
• Heart catheterization (only needed in rare cases to help with the diagnosis or in planning a treatment strategy)
• MRI of the heart
• X-ray of the chest

Treatment options

Surgery is needed to treat this condition. The surgery creates two separate arteries.

Usually the truncal vessel is kept as the new aorta. A new pulmonary artery is created using tissue from another source. The branch pulmonary arteries are sewn to this new artery. The hole between the ventricles is closed.

Where to find medical care for Persistent truncus arteriosus ?

Directions to Hospitals Treating Persistent truncus arteriosus

What to expect (Outlook/Prognosis)?

Complete repair usually provides good results. Another procedure may be needed as the patient grows. Untreated cases result in death, often during the first year of life.

Possible complications

Heart failure High blood pressure in the lungs (pulmonary hypertension) with pulmonary obstructive lung disease

Prevention

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

Sources

NLM