Show AllA baby with truncus arteriosus usually begins to have problems in the first week of life. Their oxygen levels are often slightly lower than normal resulting in cyanosis.
Because of the excessive amount of blood flow to the lungs with this anomaly, congestive heart failure (CHF) develops in the first week or two of life. On chest X-ray, the heart looks big and the lung fields look hazy indicating pulmonary overcirculation.
Signs of congestive heart failure are rapid breathing, shortness of breath, wheezing, grunting or very noisy breathing, nasal flaring, retractions, and restlessness.
The liver may be large due to a backup of blood or systemic congestion. Neck vein distention, poor feeding, and facial swelling are also seen.
Most often parents report rapid breathing, poor feeding, and a bluish color of the skin, especially around the mouth and nose. The signs and symptoms often increase when the infant eats.
While the diagnosis may be suspected by physical examination, the echocardiogram will confirm the presence of truncus arteriosus. The anatomy of the great vessels, the single, complex truncal valve and the ventricular septal defect are easily seen.
A cardiac catheterization may be done on rare occasions if anatomy appears very unusual, or the diagnosis is made later as information is needed regarding the pressures in the pulmonary arteries. In most cases, however, the echocardiogram gives enough information to plan for surgery.
Initial treatment begins with stabilizing the infant. Medications to control congestive heart failure such as diuretics are often begun.
Ensuring good nutrition may require the use of a feeding tube or intravenous hyperalimentation. Surgical correction is typically carried out in the first few weeks of life after the infant is maximally stabilized.
The surgical repair of truncus arteriosus requires the use of heart-lung bypass machine support. It involves three major components:
- Separating the pulmonary arteries from the main truncus (the truncus will remain as the first part of the aorta);
- closure of the ventricular septal defect using a patch
- creating a connection between the right ventricle and the pulmonary arteries using a valved conduit, usually a homograft pulmonary artery.
Most infants will require a period of very close monitoring in the Cardiac Intensive Care Unit while their heart function recovers from the major reconstruction.
The use of mechanical ventilation, special monitoring lines, and strong intravenous medications is typical during this period.
Gradually, as the heart function stabilizes, the supporting measures may be withdrawn and conversion to oral medications and attention to feeding dominates the management program.
Time in hospital following surgery may vary from one to three weeks in most cases.
Currently over 90 percent of children survive repair of truncus arteriosus. As the child grows, they will be followed by their cardiologist.
Typically, there will be no physical restrictions imposed on the child. As a child grows, the conduit that was used to connect the right ventricle and pulmonary arteries will not, and this will lead to obstruction to blood flow. The progression of this narrowing is easily followed by the cardiologist using physical examination and echocardiograms.
Recommendation for surgery to replace the right ventricle to pulmonary artery conduit with a larger one is usually made long before any symptoms would be evident.
This conduit will often need to be replaced two or three times during childhood to accommodate for growth. These operations are typically tolerated very well with a hospitalization of less than a week.
Problems with the truncal valve are more serious and can significantly affect the early and late mortality of these children. The more leaky (or narrowed) this valve is, the greater the chance that some intervention has to be done sooner than later (on average within 5 to 7 years) to prevent severe damage to the heart.
On occasion after an open-heart procedure, the child's hemodynamics may be tenuous. Added to that, not infrequently there is some swelling of the heart muscle from surgery and the inflammation induced by the heart-lung machine.
For that reason, at times surgeons elect to leave the chest open only to close it a few days later. In this way, the breastbone is prevented from pushing on the heart in the most critical and early hours after an operation. Despite this, the heart itself is not exposed; a layer of soft protective material is sewn to the skin edges to provide coverage of the heart, along with sterile bandages.
To minimize the slightly increased risk of infection, antibiotics are continued during the time the chest is open. Generally, the chest is closed in the intensive care unit under the same heavy sedation and analgesia that is provided to the infant during this time.
