A two-month-old baby diagnosed with supravalvular aortic stenosis (SVAS) and supravalvular pulmonary stenosis (SVPS), a rare type of obstruction in blood flow to both large arteries of the heart, recently underwent a complex heart surgery at a private hospital in the city.
SVAS is the narrowing, or stenosis, of large blood vessels that carry blood from the heart to the body (aorta). It is a rare birth defect that occurs in one in 20,000 newborns.
“The two major arteries leaving the heart (one taking blood to the body and other taking blood to the lungs) in the baby had severe stenosis or narrowing, resulting in significant obstruction of blood flow. This is an extremely rare condition, especially having significant obstruction in both the great arteries to the extent that it reduced the function of the heart,” said Devananda N.S., consultant, Cardiothoracic Vascular Surgery, Heart and Lung Transplant Surgery, Manipal Hospitals, where the baby was treated.
The baby, who remained stable after the procedure, was discharged after two weeks of near-normal heart function.
The baby had developed breathing difficulty, excessive sweating, and feeding issues after 5-6 weeks after birth. When taken for a routine check-up, the paediatrician noticed a heart murmur and referred him for further investigation. On evaluation, an echocardiogram showed the baby had a rare congenital defect.
During the five-hour surgery, both the arteries had to be cut open at the place of obstruction and widened by grafting a piece of the pericardium (outer covering of the heart) to allow optimal blood flow through them, the doctor said.
After the surgery, a repeat echocardiogram was done to make sure the obstruction had been released completely without causing damage to the heart valve. “We were finally able to get rid of all the medications and discharged the child after two weeks. The right ventricle, which was quite dysfunctional, also recovered by 60% to 70% and is expected to recover completely by another month or two,” the doctor said.