A Simple Guide to Tetralogy of Fallot With an Absent Pulmonary Valve

Tetralogy of Fallot with an absent Pulmonary Valve is a rare defect, yet Genesis Foundation has been very successful at raising money with the help of our contributors who donate to Save Little Hearts.

Rakshita and Kirthik are two out of the many kids, whose quality of life was enhanced by timely surgeries supported by Genesis Foundation and its patrons.

Rakshita, the three-year old daughter of Kumutha and Suman from Kancheepuram, Tamil Nadu, was just a year old when she developed pneumonia. Her parents rushed her to the local district hospital where the doctors detected a heart murmur. She was then referred to a government hospital in Chennai where an echo done showed Tetralogy of Fallot. Moreover, her Pulmonary Valve was absent.  The child was given medication and asked to come after a year. However, six months later, Rakshita developed pneumonia again. This time her parents were referred to Sooriya Hospital in Chennai by a private doctor. Once the ECHO was done and the diagnosis confirmed, surgery was suggested. It seemed an impossible task with her father’s earnings of Rs 7000 a month. Kumutha then approached Genesis Foundation. The surgery was then performed with support from WPP India CSR Foundation. Now Rakshita leads a normal life learning and enjoying each day as it comes. Kirthik Muthu is a one-year old boy from Krishnapuram, Tamil Nadu. Kirthik is the only child to his parents; Aravind Raj, a tea stall worker who hardly makes Rs 6,000 per month and mother Seetha Lakshmi who is a housewife. Being a preterm baby, he was diagnosed dyspnea with cyanosis immediately after birth which was then treated. Later, due to feeding issues and poor weight gain, the baby was taken to a local hospital at five months of age. The baby was evaluated and diagnosed with Tetralogy of Fallot with Absent Pulmonary Valve and was advised total correction. Kirthik was then seen by Dr R Krishna Kumar in during a screening camp in Thirunelveli Royal Hospital and referred to Amrita Institute for further management and brifing on the surgery. With generous support extended by the WPP India CSR Foundation, Kirthik underwent surgery.  Pist recovery, Kirthik is a healthy and a very naughty boy as says his mother. But he is the apple of his parents eyes and they’re very grateful to all doctors and sponsors.

What is Tetralogy of Fallot with an Absent Pulmonary Valve?
Tetralogy of Fallot is a condition in which the pulmonary valve is either completely absent or poorly formed. The pulmonary valve is responsible for regulating the flow of blood to the lungs.

Image Caption: Difference between a normal heart and a heart with Tetralogy of Fallot.

Congenital absence of pulmonary valve syndrome (APV) is a unique variant of congenital heart diseases. This condition was first described by Dr. Chevers in 1847. There is an association of this defect with Tetralogy of Fallot (ToF) due to which this condition is often referred to as Tetralogy of Fallot or Absent pulmonary valve syndrome. It has been observed that almost 3% of children with Tetralogy of Fallot have no Pulmonary Valve.
The similarities of this congenital heart defect with tetralogy of Fallot makes the diagnosis challenging with the striking difference of the massively dilated pulmonary artery branches instead of the hypoplastic ones that are present in Fallot.

Symptoms
These are the most common symptoms observed in patients suffering from Tetralogy of Fallot.

  • Cyanosis (Bluish discoloration of skin)
  • Failure to thrive
  • Rapid Breathing
  • Respiratory Failure

Examination
Fortunately, Tetralogy of Fallot with an absent pulmonary valve can be diagnosed during pregnancy through the Fetal Echo test. Then post birth, a doctor may order the following tests for the diagnosis once a murmur has been confirmed. These tests include, but are not limited to:

  • Echocardiogram
  • Chest X-Ray
  • CT Scan of the chest

Treatment
Children who are diagnosed with the Tetralogy of Fallot and an absent pulmonary valve require open heart surgery.

Summary of Surgery
A surgery for Tetralogy of Fallot with an absent pulmonary valve entails various steps. These are highlighted in the image below.

Image Caption: A brief summary on Tetralogy of Fallot surgery.

Conclusion
The long-term prognosis for children being operated for Tetralogy of Fallot is good. Some children might require additional surgeries as and when they grow up, but they all have shown a good recovery rate. You can join hands with Genesis Foundation and donate to Save Little Hearts. Click here to know more.

– Contributed by R.Srivatsan, Edited by Monisa N.

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