In October, when eight-month-old Fathima Shamna underwent a surgery for Tetralogy of Fallot (TOF), a congenital heart disease, at the State-run Sri Jayadeva Institute of Cardiovascular Sciences, it was a unique experience for the doctor performing the operation.
P S Seetharama Bhat, Professor and Senior Consultant in Cardiothoracic Surgery at the institute, had operated upon the baby’s father Sirajuddin in 2009 and his first child Mohammed Hyaiz in 2019 for the same birth defect at the same hospital. While the father was operated upon when he was 19 years old, the first child underwent the surgery after he turned one.
Blue baby syndrome
TOF is a birth defect (commonly known as blue baby syndrome) that affects normal blood flow through the heart. It happens when a baby’s heart does not form correctly as it grows and develops in the mother’s womb.
“This is the first time in my career spanning 32 years that two children and their father have been operated on for the same birth defect. Such a high degree of genetic penetration is not common as only 10% of children born to patients with congenital heart disease develop the exact same defect,” Dr Bhat told The Hindu.
“Although the mother of these two children was cautioned about the congenital heart disease by her gynaecologist during the pregnancies, she refused to get it terminated. She hoped that her children could be saved by her husband’s doctor. Her faith only increased my responsibility,” he said.
Combination of four defects
Institute Director C N Manjunath said TOF is the most common cyanotic congenital heart disease. “It is a combination of four congenital heart defects. These defects usually result in a lack of oxygen-rich blood reaching the body,” he said.
Infants with TOF can have bluish discoloration of skin and nail beds — called cyanosis — because their blood does not carry enough oxygen. If timely surgical intervention is not done, babies can develop polycythaemia (blood thickening), abscess in the brain and bleeding disorders with a reduced lifespan, he said.
“Usually the optimal timing of surgery is between one to two years unless there are more symptoms. The outcome of the TOF surgery depends on the adequacy of the pulmonary arteries. The rate of surgical mortality is around 4%-7% depending on the medical centre and surgical expertise,” he said.
Rehnaaz, wife of Mr. Sirajuddin, said, “I had pinned my hopes on doctors at Jayadeva but was confident about a successful surgery when both my children were operated upon by the same doctor who treated my husband.”
