Learning about Total Anomalous Pulmonary Venous Connection (TAPVC)
March 11, 2020 | Contributed by R. Srivatsan, edited by Ananya Ravi
Throughout our journey of supporting heart disorder treatment, we come across various types of CHDs and this blog aims to bring awareness to one such defect.
Total Anomalous Pulmonary Venous Connection (TAPVC) is a birth defect of the heart. In a child with TAPVC, oxygen-rich blood does not return from the lungs to the left atrium. Instead, the oxygen-rich blood returns to the right side of the heart. Here, oxygen-rich blood mixes with oxygen-poor blood. This causes the child to get less oxygen than is needed for the body.
Babies with TAPVC usually have a hole between the right atrium and the left atrium (Atrial Septal Defect) that allows the mixed blood to get to the left side of the heart and pumped out to the rest of the body. Since the children require urgent surgery to cure the defect, this is considered a critical defect.
In some instances, not all the Pulmonary Veins terminate at the right atrium. One or more veins terminate to the left atrium. Hence, this condition is called Partial Anomalous Pulmonary Venous Return (PAPVR). Therefore, PAPVR is not as critical as TAPVC.
Types Of TAPVC:
- Supracardiac: In this condition, the Pulmonary Veins form an abnormal connection above the heart to the superior vena cava which supplies impure blood from the upper part of the body to the right atrium.
- Cardiac: In cardiac TAPVC, the pulmonary veins meet behind the heart and connect to the right atrium.
- Infracardiac: In this condition, the pulmonary veins come together and form abnormal connections below the heart.
Facts about TAPVC:
- The incidence of TAPVC has been reported as 0.008% per live births, with an approximate incidence of 2.2% in patients having CHD.
- In 1957, Darling and associates coined the different types of TAPVC
- TAPVC and PAPVR are difficult to be detected during pregnancy as much blood does not flow to the lungs in the foetus. Generally, such conditions are detected after birth.
- Bluish discoloration of the skin
- Breathing problems
- Weak pulse
- Poor feeding
- Children with TAPVC have a murmur.
- Although, in some cases, murmurs can be absent.
- Doctors will also suggest an Echocardiogram which can confirm the defect.
- Children with TAPVC require open-heart surgery to cure the defect.
- The timing of the surgery is dependent upon how sick the child is.
- To repair the defect, the Pulmonary Veins are connected to the left atrium, the Atrial Septal Defect is closed and any abnormal connections between the blood vessels are closed.
- The long term prognosis for children with TAPVC is good unless and until there are other complications.
- In some cases it has been observed that children undergoing TAPVC repair have Pulmonary Veins stenosis, requiring further interventions.
We have supported around 50 children with TAPVC. The smallest child supported by Genesis Foundation for TAPVC was 7 hours after birth at Amrita Institute of Medical Sciences in Kochi.
This is her story.
Two days old, and the firstborn of Priya and Muralitharan, baby of Priya had not even been named yet. She was born in Coimbatore. Throughout her pregnancy, Priya had consulted a gynaecologist at a private hospital. In her fifth month of pregnancy, a fetal echo was conducted, which revealed that the baby had a complex heart defect. Both the parents were counselled and Priya was required to have regular check-ups. The baby was delivered at the private hospital weighing 2.5kg and showed significant signs of breathing difficulty. She was stabilized and transferred to G Kuppuswamy Naidu Memorial Hospital (GKNM Hospital) in Coimbatore. An Echo was conducted, which confirmed the diagnosis – Total Anomalous Pulmonary Venous Connection (TAPVC). The only way to cure this defect was through open-heart surgery. It was naturally very difficult for the family to process all that had been happening to their new-born. Muralitharan is a helper in a private company where he earns an income of Rs 9000 per month. He took a loan of Rs 100,000 from a private money lender in order to arrange for funds for the treatment of his baby. But this was not enough. So, the hospital approached us for support. Thanks to the donations from The Luxor Foundation, we were able to pay the balance amount towards the successful surgery.Baby of Priya is now on her way to recovery.
In the past two years at Genesis Foundation, we have supported 22 males and 12 females below the age of one suffering from TAPVC and 2 girls and 2 boys over the age of one.
We are constantly at work to provide heart disorder treatment in India to the ones who are unfortunately unable to access such healthcare facilities and treatment.