Truncus arteriosus

Truncus arteriosus: Description, Causes and Risk Factors: Truncus arteriosusTruncus arteriosus is a rare type of heart disease that occurs at 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). When the fetus develops during pregnancy, the heart starts with a single large blood vessel coming from the heart called the truncus arteriosus. If fetal development progresses normally, the truncus divides into two arteries that carry blood out of the heart: The pulmonary artery, which is attached to the right bottom chamber (ventricle) of the heart and divides into two arteries carrying oxygen-poor blood to each side of the lungs.
  • The aorta, which is attached to the left bottom chamber (ventricle) of the heart and carries oxygen-rich blood to the body.
Sometimes the single large blood vessel fails to divide during fetal development and the baby is born with a heart that has one artery carrying blood out of it. This condition is known as truncus arteriosus or persistent truncus arteriosus (the trunk "persists"). The undivided trunk is attached to the heart as one artery straddling the bottom chambers and then divides into arteries taking blood to the lungs and body. The oxygen-poor blood from the right ventricle (bottom chamber) and the oxygen-rich blood from the left ventricle (bottom chamber) mix when ejected out into the trunk, and more blood than normal goes back to the lungs making it harder for the infant to breathe. In almost all cases, children with the congenital heart defect truncus arteriosus also have a large hole between the bottom chambers of the heart. This is called a ventricular septal defect (VSD). As a result of these abnormalities, the baby's blood is not as oxygenated as it should be when it circulates through the body. Truncus arteriosus occurs in less than one out of every 10,000 live births. It makes up 1 percent of all cases of congenital heart disease. Symptoms: Symptoms may include: Cyanosis.
  • Delayed growth or growth failure.
  • Fatigue.
  • Lethargy.
  • Poor feeding.
  • Rapid breathing (tachypnea).
  • Shortness of breath.
  • Widening of the finger tips (clubbing).
Diagnosis: Tests may include: EKG/ECG (electrocardiogram).
  • Echocardiogram.
  • 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.
  • MRI of the heart.
  • X-ray of the chest.
Treatment: 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.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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