CHDFNigeria
Truncus Arteriosus is a rare defect of the heart in which a single common blood vessel comes out of the heart, instead of the usual two vessels (the main pulmonary artery and aorta). Truncus arteriosus occurs in less than one out of every 10,000 live births. It can occur by itself or as part of certain genetic disorders e.g DiGeorge syndrome.
In Truncus arteriosus, blood leaves the heart in a single channel called Truncus. Normally blood coming into the heart and that leaving the heart doesn’t mix and this ensures proper oxygenation of the body. In truncus arteriosus, blood from both sides of the heart leaves the heart through a single channel which results in the mixing of blood and reduction in oxygenation of the body. The mixing is further worsen by an abnormal connection, VSD, between the left and right side of the heart.
Signs and symptoms
Clinical features of Truncus depends on age of the child. Typical ones includes:
Cyanosis (bluish discolouration of the body)
Heart murmurs
A bounding pulse (forceful)
Wide pulse pressure can also be observed
Abnormal fast breathing
Poor feeding
Extreme sleepiness
Diagnosis
Can be made before or after birth as with any other CHDs. Similar diagnostic investigations are ordered e.g.
Chest X-ray
ECG
Echocardiography
CT scan
Management of Truncus Arteriosus
Medications
Some babies with truncus arteriosus also will need medicines to help strengthen the heart muscle, lower their blood pressure, and help their body get rid of extra fluid E.g Furosemide, Spironolactone.
Nutrition
Nutritional support is necessary for children who show signs of poor feeding or weight gain.
Surgery
Surgery is needed to repair the heart and blood vessels. This is usually done in the first few months of life. Options for repair depend on how sick the child is and the specific structure of the defect. The goal of the surgery to repair truncus arteriosus is to create a separate flow of oxygen-poor blood to the lungs and oxygen-rich blood to the body.
References:
Nelson textbook of paediatric 20th Edition
Centre for disease control and prevention; https://www.cdc.gov/ncbddd/heartdefects/truncusarteriosus.html
