A 10-and-half-a-month-old baby from Kalyan has been diagnosed with Uhl’s Anomaly, an extremely rare heart disease that leads to cardiac failure, and is quite possibly the first reported case in India.
The British Heart Journal has reported only 80 cases across the world between 1900 and 1993. In all likelihood though, there could have been more cases that went undetected, both in India and elsewhere.
Uhl’s Anomaly is a fatal condition in which the muscles surrounding the lower right chamber of the heart are totally or partially absent, leading to cardiac failure.
Dr Sumitra Venkatash, a consultant paediatric cardiologist at Bai Jerbai Wadia Hospital in Parel who is treating the infant Swaroop Verma, said, “When his parents brought him to the hospital, he had a routine cough and cold. But, when we saw that he was having persistent breathing troubles, we advised the parents to have him admitted.”
From his X-ray report, doctors learnt that the child had an abnormally large sized heart. “Normally, the size of the heart is 50-55% of the size of the chest cavity. However, in Swaroop’s case, the size of his heart is nearly 63% of the size of his chest cavity,” said Dr Venkatash. While the chest X-ray gave doctors the first clue about his condition, subsequent tests like echocardiogram and a cardiac MRI confirmed Swaroop had Uhl’s Anomoly.
“We have lot of referral of cases of rare disorders. But our doctors have never come across any patient with this heart disorder,” said Dr Minnie Bodhanwala, CEO of Wadia Hospitals
Currently, the infant is on medication like sildenafil, popularly known as Viagra, and diuretics that are helping control his high blood pressure. However, the only procedure that can save the boy’s life in the coming years is a cardiac transplant. “A cardiac transplant is estimated to cost anywhere between Rs 20-30 lakhs for which the parents will need financial help. We have already registered his name at Fortis Hospital (Mulund). But, finding a match of his age and weight could turn out to be difficult,” said Dr Venkatash.