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Truncated arteriosus is a rare congenital heart defect where the pulmonary and aortic valves do not separate, resulting in one large artery. Early repair is necessary to avoid permanent damage, and improved surgical techniques allow for one-step repair with a high success rate. Children with repaired truncus arteriosus require lifetime follow-up by a cardiologist.
Truncated arteriosus is a rare congenital heart defect in which the pulmonary and aortic valves fail to separate completely. This lack of separation results in one large artery, instead of two, leaving the heart. The arterial trunk also presents with a large ventricular septal defect. Children can also have a malformed thymus gland, which can affect the immune response to even the simplest of viruses.
This disorder requires early repair because the pressure on the lungs is significantly increased by the large ventricular septal defect and malformed valves. Too much blood gets to the lungs, creating high pressure that needs to be addressed early to avoid permanent damage. Also, too little oxygenated blood gets to the body, which can lead to failure to thrive, cyanosis or “blue baby”, clubbing of the extremities and exhaustion.
Until the 1980s, the arterial trunk was usually addressed in two surgeries. The first surgery placed a band around the pulmonary valve to reduce blood flow to the lungs. This gave the baby time to grow before repairing the ventricular septal defect and separating and suturing the pulmonary and aortic arteries.
However, lung bandaging is rarely used now, because improved surgical techniques and anesthesia delivery allow for repair soon after the baby is born. The one-step repair has a high success rate, even when done on babies as young as a few days old. Additional valve repairs may be required as the child gets older. The pulmonary valve is particularly prone to leaking over time and may need to be replaced. A bovine, porcine, or donor valve is used. Valve replacement has a low risk and is considered one of the simplest heart repairs.
Children with repaired truncus arteriosus require lifetime follow-up by a cardiologist. After a thorough examination during the first few months of a repair, cardiologists usually see the child once a year. During a regular visit, the cardiologist usually does an echocardiogram, or ultrasound of the heart, to evaluate the repair and make sure that the pulmonary valve leak is not significant. In some cases, pulmonary valve leakage after a repair should be evaluated by cardiac catheterization. This outpatient procedure is considered minimally invasive and does not require general anesthesia, although some form of conscious sedation may be used to keep the child comfortable and asleep throughout.
The child with repaired trunk artery is usually allowed to participate in normal activities, but the cardiologist may recommend abstaining from competitive sports, particularly as the child gets older. Children can also take low-dose aspirin to prevent blood clots that could lead to strokes. Most cardiologists also recommend prophylactic antibiotics before dental exams and procedures.
The arterial trunk is a significant and serious set of heart defects. It is sometimes linked to a condition called Di George, which involves other anatomical defects and can complicate health both before and after repair. Children without Di George are generally expected to have a normal life expectancy and quality of life.
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