South Florida man diagnosed with extremely rare heart defect doctors say they had only read about in textbooks

Most congenital heart defects are diagnosed shortly after birth, but one South Florida man spent more than seven decades unaware he was living with an extremely rare heart condition.

Carlos Perez had no idea he was born with a congenital heart defect until doctors began investigating an episode of atrial fibrillation several years ago.

"I had not a clue," Perez said.

After feeling dizzy and fatigued at home, Perez went to the hospital for an evaluation. During a series of tests, physicians made an unexpected discovery.

"One doctor came in the room and then two doctors and then a whole audience," Perez recalled. "They said, 'We've all read about this in medical school, but we've never seen one.'"

Doctors found Perez had an extremely rare five-chambered heart. A normal heart has four chambers. In Perez's case, a membrane divided his left atrium, effectively creating an extra chamber.

Dr. Tom Nguyen with Baptist Health Heart and Vascular Care compared the condition to a house with an unexpected wall separating one room from another.

"For him, he had a room with a wall across it," Nguyen said. "The reason why he lived through his 70s is that the wall had a little hole in it, and that allowed the blood to get through."

The condition, known as cor triatriatum, accounts for less than 0.1% of all congenital heart abnormalities. Most cases are identified in infancy or childhood because the membrane restricts blood flow, causing symptoms much earlier in life.

According to the Centers for Disease Control and Prevention, congenital heart defects are the most common type of birth defect in the United States, affecting nearly 1% of births, or about 40,000 babies each year. Advances in medicine have improved survival rates, and today, more adults than children are living with congenital heart disease.

Perez's doctors used robotic technology to perform a minimally invasive procedure to remove the membrane dividing his atrium. During the same operation, surgeons performed ablations to treat his atrial fibrillation and placed a clip designed to help regulate heart function.

"To me, it's fascinating. The mechanical complexity of the device and the skill needed to work it," Perez said.

The surgery marked a first-of-its-kind collaboration between the Baptist Health Miami Cardiac & Vascular Institute and the Nicklaus Children's Hospital Heart Institute.

Dr. Makoto Hashimoto, a cardiac surgeon, said the partnership could help pave the way for treating more adults with complex congenital heart conditions.

"I think this opened the door to treat more complicated adult congenital disease in the future," Hashimoto said.

Nguyen called the collaboration a turning point for health care in South Florida.

"It really is a turning point, more importantly for Miami and the health care for Miami," Nguyen said.

Perez was discharged from the hospital within a week. He stressed that he never experienced chest pain, noting that his symptoms included dizziness, fatigue, and very low blood pressure.

His message to others is simple: "Get checked out," he said.