TAPVC in U.A.E

Total Anomalous Pulmonary Venous Connection (TAPVC) or Total Anomalous Pulmonary Venous Return (TAPVR) is a rare congenital disorder characterized by a malformation in all the four pulmonary veins that carry oxygenated blood from the lungs to the atrium. The malformation is such that the veins do not connect to the left atrium perfectly.

In case of TAPVC, the four pulmonary veins drain into the right atrium because of an anomalous connection. There are different TAPVC types, depending on where and how the pulmonary veins drain into the right atrium. The common TAPVC types include supracariac, cardiac, and infracardiac TAPVC.

All types of TAPVC are corrected with the help of a surgery, which is conducted during infancy itself. Additionally, all types of TAPVC almost always have an atrial septal defect (ASD), which requires a separated surgery for its closure. An ASD is a hole between the upper two chambers of the heart. As a result, some amount of oxygenated blood from the right atrium is transferred to the left atrium and out from the body.

TAPVC Symptoms

TAPVC is detected as soon as the baby is born or even before. The following are some of the main TAPVC symptoms:

• Low oxygen saturation
• Less blood volume in the left atrium and ventricle
• Obstruction of the pulmonary veins
• Congestion in the lungs
• Pulmonary hypertension
• Severe cyanosis (bluish appearance of the skin)
• Unstable blood pressure

TAPVC repair is conducted as soon as the condition is diagnosed, which may happen during pregnancy or after the birth of the baby. However, the exact time as to when the repair is conducted depends on the overall health of the baby. During TAPVC repair, the neonatal cardiac surgeons connect the pulmonary veins to the back of the left atrium to restore the normal flow of blood to the heart. Additionally, they also close off any abnormal connection between the pulmonary veins. The TAPVC repair procedure does not end there. The last step in the repair mostly includes the closure of the ASD. Prior to the surgery, the body of the child is connected to an artificial heart-lung machine.  The machine is disconnected as soon as the normal beating of the heart is restored after making doing all the corrective repairs.

TAPVC repair success rate is excellent with over 95 percent success rate in children who are treated electively. A child or an adult may undergo TAPVR surgery successfully, however, that does not mean that it is fully cured. They will have to attend follow-up appointments with the surgeon in the future on a regular basis. The success rate, on the other hand, is low when the procedure is conducted on an emergency basis in critically-ill infants. Even if infants do survive after undergoing emergency TAPVC repair procedure, they will be kept under a prolonged post-surgery intensive care. They are also put on a ventilator machine to assist breathing.

Even is the repair is successfully handled, the patient is required to attend follow-up session with the surgeon for the rest of your life. During the follow-up appointments, the surgeon will check the progress and keep a tap on health conditions that may develop as the child grows into an adult. Most of the children who undergo TAPVC repair and ASD closure are able to grow and develop normally. Abnormal cardiac rhythm and obstruction in the pulmonary veins are the two post-surgical complications that may rarely occur.