Rare defect found in man who thought he was having heart attack

The remarkable case of an Indian man found to have five heart chambers instead of four surprised Dubai doctors, who had never encountered the rare condition before.

But medics say such discoveries are becoming more common, with lifestyle choices a contributing factor.

People starting families later in life and those in consanguineous marriages are at a greater risk of giving birth to children with heart defects, experts said.

The diagnosis involved a 51-year-old patient experiencing chest pains. The man went to Aster Hospital in Mankool, Dubai, because he thought he was having a heart attack.

He was found to have a condition called cor triatriatum sinister, resulting in the heart having three upper chambers rather than the usual two.

With so few other cases, doctors said there was little guidance in how to deal with the man’s symptoms.

“First we had to rule out an acute emergency and then evaluate what was the cause of his chest pain,” said cardiologist Dr Sachin Upadhyaya. “The most common cause is coronary artery disease and acute heart attack or angina.

“But when we did an electrocardiograph, everything was normal. The second step was cardiac imaging and the heart function was OK but we could see something unusual in the left atrium.

“We were careful, as knew there was something there, but none of us had encountered that in real practice before. This is so rare, there are no standard treatment guidelines.”

Doctors treated the man with medication, rather than surgery, and he is now recovering at home – although he needs regular check-ups. Congenital heart problems are usually picked up at birth through routine screening, making the discovery even more unusual in an otherwise healthy adult.

Such issues are more common in children born to parents in consanguineous relationships or with heart defects. Men are more likely than women to have the condition.

More common conditions are holes inside heart walls and narrowed or leaky valves. Severe cases can involve missing blood vessels or heart chambers, or a poorly formed heart.

They occur in fewer than 1 per cent of births.

Doctors said they seeing more cases due to parents starting families later in life.

“The incidence is not small, historically it has been around eight severe defects in 1,000 births worldwide,” said Dr Anil Bansal, a consultant cardiologist at Saudi German Hospital in Dubai.

“Because more people are having children later, it has now risen to around 9.5 in every 1,000 births.

“The age of parents and consanguinity, such as marriages among cousins, is a big issue.”

More than 85 per cent of babies born with a heart defect live to at least the age of 18 but those with more serious problems rarely survive into adulthood.

Surgery is often not a cure for congenital heart defects, with patients needing additional procedures during their lifetime or prolonged medication.

Typically, adults living with a defect face a life of health problems, issues with growth and eating, difficulty with exercise or development and a risk of sudden cardiac arrest or stroke.

As the Mankool patient lived a normal life, doctors said his case was more unusual.