Man's rare heart disorder went unnoticed for 67 years

Businessman has Heart Attack

A man in Florida only recently learned, at age 67, that his heart is different from others' in a major way: One of the chambers of his heart is divided in two, according to a recent report of his case.

The condition, known as a double-chambered right ventricle, is extremely uncommon, said Dr. Valeria Duarte, a cardiology fellow at the University of Florida who presented the case here Tuesday at a research meeting called the American Heart Association Scientific Sessions.

Even among those who have it, it's "very, vey rare to diagnose it in adulthood," Duarte told Live Science. The condition is usually diagnosed in infancy or early childhood, she said. It most frequently appears in people with other congenital heart defects, which are also typically diagnosed early in life, she said.

The man did indeed have another congenital defect that he was aware of — a condition called ventricular septal defect, which is a hole in the wall of the heart that separates the two lower chambers, or ventricles. Because the hole was small and not causing problems for the man, he had not had it repaired.

Still, he was unaware that he had a double-chambered right ventricle.

The symptoms of a double-chambered right ventricle can include fatigue and low blood pressure, but these symptoms can result from many other conditions, Duarte said. Both symptoms can result from lower blood flow in the vein leading from the heart's right ventricle to the lungs, or increased pressure in the right ventricle, she said. [Heart of the Matter: 7 Things to Know About Your Ticker]

While doubled-chambered right ventricles do not always cause problems, patients can suffer damage to the ventricle, putting themselves at risk of right ventricle failure, which can be fatal, Duarte said. Ventricle failure — like other types of heart failure — means that the chamber can no longer pump blood effectively.

When the man initially came in for his trouble breathing, the doctors listened to his heart and found a murmur (an unusual sound heard during a heartbeat).

Using an echocardiogram, the doctors detected that there was a muscle bundle, dividing the man's right ventricle into two parts. This can create problems with blood flow in the chamber, and pressure can build up on one side of the band, Duarte said. The echocardiogram also showed an enlarged right ventricle, she said.

An MRI confirmed the diagnosis, she said.

Although patients may be initially scared when they learn they have a double-chambered ventricle, they may feel reassured after doctors tell them that it can be repaired, Duarte said. This is the second patient with a double-chambered right ventricle that Duarte said she has seen.

The treatment for a double-chambered right ventricle is always surgery, Duarte said.

During the surgery, doctors also repaired the small hole in the man's heart. In addition, the doctors found an enlarged aorta, and they repaired that as well, she said.

Duarte said the man's surgery went well, and he has gone back to his normal life without significant symptoms. He does not need to take any medications for the condition, but he does need to have yearly echocardiograms to check his heart health.