Kid with rare heart disease goes under scalpel, successfully

Finally, it’s time to celebrate for Nobert and his family after Kevin, his six-year-old son, suffering from a very rare heart disease, congenital atresia of the left main coronary artery, was successfully operated by the doctors at Kokilaben Dhirubhai Ambani Hospital (KDAH) last week. Congenital atresia of the left coronary artery is an extremely rare disease with only 50 cases published in the world literature so far.

Kevin suffered from two attacks before his parents brought him to the KDAH. His last attack was three months ago. “The last attack was severe. He was in the school bus and suddenly, he blacked out. There was no pulse and he required cardiopulmonary resuscitation for 15 minutes,” said Nobert.

With no other symptoms, Kevin was initially evaluated for seizure disorders. “His cardiac examination was found to be normal. Kevin’s cardiac tests like Echocardiogram, ECG, Holter, etc were also normal. Doctors then opted for coronary angiography,” added Nobert. Though structurally and functionally the heart was normal, the coronary angiography revealed severe narrowing of the left coronary opening from the aorta. The left main coronary artery and its main branches were also very narrow in calibre.

Nobert later decided to bring Kevin to KDAH. The surgeons planned an open heart surgery to see if normal anatomy could be restored or at least whether a coronary artery bypass graft could be placed to augment the blood supply to the “blood starved” left ventricle.

Dr Suresh Rao, paediatric and congenital heart surgeon, KDAH said, “At surgery, to our complete surprise, we found that there was no opening of the left coronary artery in the aorta and a dimple was seen where the opening should have been. This has obviously been since the heart developed in the foetus. The artery was seen to be branching normally, but it was small in calibre. The rest of the visualised cardiac anatomy was normal.”

Though the doctors had the choice of restoring the normal anatomy or place a coronary artery bypass graft they went for coronary osteoplasty, which involved creation and enlargement of the coronary opening.

“Coronary bypass is not a good option for a children compared to coronary osteoplasty, which lasts longer. Thankfully, we didn’t have any hiccups and the operation was successful. We did his coronary angiogram again and he has a good sized left coronary ostium with good filling of the left main stem coronary, LAD and circumflex arteries.”
Photo Caption(s):Dr Suresh Rao, paediatric and congenital heart surgeon, KDAH, with Nobert and his son Kevin, who has since been discharged.