Man survives a rare heart defect

ALCAPA accounts for less than 1% of congenital heart anomalies

Enknvally Jayasimha is among the few ever known to have survived a heart defect that kills nearly all babies born within the first year.

At 43, Enknvally Jayasimha’s only concern is to live a few more years to ensure his children become financially independent. This father of three survived a rare congenital illness, but a close brush with death three years ago does not let him breathe easy.

Jayasimha is among the few ever known to have survived a heart defect that kills nearly all babies born within the first year. However, Jayasimha only discovered in middle age that the left side of his heart was oxygen-starved all his life due to an anatomical error.

Discovery of his congenital defect happened when an episode of cold and cough left him breathless one night in December 2014. With a throbbing heart, his wife rushed him to a doctor at Borabanda, who said he would not survive the night.

“I was told I would not make it if I did not immediately get advanced care. Multiple doctors I saw in one night had given up on me,” he said.

He was rushed to a private hospital at Banjara Hills, where doctors suspected his heart was failing due to a heart attack brought on by blocked coronary vessels. His financial condition ruled out critical-care medical treatment at the facility. He was moved to Osmania General Hospital.

“Our priority was to stabilise him. His heart rate was over 180 beats per minute. It looked liked he was having myocardial infarction. We shifted him to OGH,” said P. Praneeth, cardiologist at CARE Hospital where Jayasimha’s wife first rushed him.

At OGH, Nagula Praveen, who was working towards a higher medical degree, stabilised Jayasimha. As per protocol, the doctor performed an angiogram and to his surprise, could not access left coronary network of the heart.

“When we could not engage Jayasimha’s left system, we suspected it may have been completely blocked. But we also suspected an anomalous origin for his left coronary artery,” said Dr. Praveen.

The two sides of the heart were fed oxygenated blood by the aorta, the largest artery in the body, through the left and right coronary arteries. Jayasimha’s first angiogram led to suspicions of left coronary artery originating from elsewhere and not aorta, but it did not reveal just where.

In January 2015, a second angiogram showed that Jayasimha had Anomalous Left Coronary Artery Origin from Pulmonary Artery (ALCAPA). The left side of his heart, which pumps oxygenated blood to the body, was being supplied deoxygenated blood all his life. The ALCAPA accounts for less than 1% of congenital heart anomalies. Dr. Praneeth termed Jayasimha’s condition a ‘once-a-lifetime’ case a cardiologist may witness.

Jayasimha’s symptom-less survival with the ALCAPA adds to a growing body of evidence suggesting the importance of an alternate coronary circulation the heart evolves, called collaterals, which develop to circumvent a blocked artery. His condition was described in November last year in a paper published by Dr. Praveen in the journal IHJ Cardiovascular Case Reports.