The remarkable case of an Indian man found to have five heart chambers instead of four shocked Dubai doctors who had never encountered the rare condition in their careers.
But medics say such startling discoveries are gradually 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 extraordinary diagnosis reported this week involved a 51-year-old patient experiencing chest pains, who thought he was having a heart attack so visited Aster Hospital in Mankool, Dubai.
Some abnormalities that are not severe are compatible with normal life and occasionally there is a possibility of survival to age 50
Dr CG Venkitachalam,
Burjeel Hospital in Abu Dhabi
He was found to have a condition called cor triatriatum sinister, which affects fewer than 0.004 per cent of the population, resulting in three upper chambers of the heart rather than the usual two.
Doctors believe it is the same condition that ended the life of the star of the golden age of Indian cinema, Madhubala, who died aged 36 in 1969.
With few other medical cases from which to refer, doctors said there was little guidance in how to deal with the man’s symptoms after he arrived in hospital in Dubai.
Entering uncharted waters
“First we had to rule out an acute emergency and then evaluate what was the cause of his chest pain,” said Dr Sachin Upadhyaya, a cardiology specialist at Aster Hospital who treated the man, who did not want to be named.
“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 in any European Society of Cardiology or American Society of Cardiology guidelines.”
Doctors were able to treat the man with medication, rather than surgery, and he is now recovering at home – although he must return for regular check-ups.
Will such cases always be so rare?
Congenital heart problems are usually picked up at birth through routine screening, making the discovery all the more remarkable in an otherwise healthy adult.
Defects include structural heart problems and are more common in children born to parents in consanguineous relationships or in those whose parents had heart defects.
Men are more likely than women to have the condition.
More common examples of heart anomalies include holes inside heart walls and narrowed or leaky valves. Severe cases usually involve missing blood vessels or heart chambers or a poorly formed heart and occur in fewer than 1 per cent of births.
Despite the rarity of such problems, doctors said they are beginning to see 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, 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.
“These are rare defects but corrective surgery is relatively simple.”
One of the more common anomalies is an atrial septal defect where there is a hole in the dividing wall of the upper heart chambers.
A hole can vary in size and may close on its own or sometimes requires surgery.
More than 85 per cent of babies born with an anomaly live to at least the age of 18 but those with more serious defects 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 as adults.
Patient living a normal life
Typically, adults living with a defect face a life of health problems, including issues with growth and eating, as well as difficulty with exercise or developmental delays and even sudden cardiac arrest or stroke.
As the Mankool patient lived an otherwise normal life, doctors said his case was all the more unusual.
“This is an especially rare anomaly, particularly as this patient was 51, which is quite old to have this picked up,” said Dr CG Venkitachalam, a cardiologist at Burjeel Hospital in Abu Dhabi.
“Some abnormalities that are not severe are compatible with normal life and occasionally, like this, there is a possibility of survival to age 50.
“I have seen maybe just one patient in 30 years at that age.
“So if they're picked up early with screening, they can be rectified pretty early on and then the patient can live a long and healthy life.
“In this country, the recognition of this is pretty good and we pick them up very early, mostly before they leave the ICU.”
The view from The National
Key facilities
- Olympic-size swimming pool with a split bulkhead for multi-use configurations, including water polo and 50m/25m training lanes
- Premier League-standard football pitch
- 400m Olympic running track
- NBA-spec basketball court with auditorium
- 600-seat auditorium
- Spaces for historical and cultural exploration
- An elevated football field that doubles as a helipad
- Specialist robotics and science laboratories
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- Disruption Lab and Research Centre for developing entrepreneurial skills
Company%20Profile
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COMPANY PROFILE
Name: Rain Management
Year started: 2017
Based: Bahrain
Employees: 100-120
Amount raised: $2.5m from BitMex Ventures and Blockwater. Another $6m raised from MEVP, Coinbase, Vision Ventures, CMT, Jimco and DIFC Fintech Fund
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6. Arnaud Demare (Fra) Groupama-FDJ
7. Max Kanter (Ger) Movistar Team
8. Olav Kooij (Ned) Jumbo-Visma
9. Tom Devriendt (Bel) Intermarché-Wanty-Gobert Matériaux
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Power 165hp @ 6,000rpm
Torque 240Nm @ 1,400rpm 0-100kph: 9.2 seconds
Top speed 420 kph (governed)
Fuel economy, combined 35.2L / 100km (est)
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Engine: 4.0-litre flat-six
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The biog
Favourite hobby: I love to sing but I don’t get to sing as much nowadays sadly.
Favourite book: Anything by Sidney Sheldon.
Favourite movie: The Exorcist 2. It is a big thing in our family to sit around together and watch horror movies, I love watching them.
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Name: Kumulus Water
Started: 2021
Founders: Iheb Triki and Mohamed Ali Abid
Based: Tunisia
Sector: Water technology
Number of staff: 22
Investment raised: $4 million
What is 'Soft Power'?
Soft power was first mentioned in 1990 by former US Defence Secretary Joseph Nye.
He believed that there were alternative ways of cultivating support from other countries, instead of achieving goals using military strength.
Soft power is, at its root, the ability to convince other states to do what you want without force.
This is traditionally achieved by proving that you share morals and values.
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What's in the deal?
Agreement aims to boost trade by £25.5bn a year in the long run, compared with a total of £42.6bn in 2024
India will slash levies on medical devices, machinery, cosmetics, soft drinks and lamb.
India will also cut automotive tariffs to 10% under a quota from over 100% currently.
Indian employees in the UK will receive three years exemption from social security payments
India expects 99% of exports to benefit from zero duty, raising opportunities for textiles, marine products, footwear and jewellery
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UPI facts
More than 2.2 million Indian tourists arrived in UAE in 2023
More than 3.5 million Indians reside in UAE
Indian tourists can make purchases in UAE using rupee accounts in India through QR-code-based UPI real-time payment systems
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SCORES IN BRIEF
New Zealand 153 and 56 for 1 in 22.4 overs at close
Pakistan 227
(Babar 62, Asad 43, Boult 4-54, De Grandhomme 2-30, Patel 2-64)
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1. Fasting
2. Prayer
3. Hajj
4. Shahada
5. Zakat
EMERGENCY PHONE NUMBERS
Estijaba – 8001717 – number to call to request coronavirus testing
Ministry of Health and Prevention – 80011111
Dubai Health Authority – 800342 – The number to book a free video or voice consultation with a doctor or connect to a local health centre
Emirates airline – 600555555
Etihad Airways – 600555666
Ambulance – 998
Knowledge and Human Development Authority – 8005432 ext. 4 for Covid-19 queries
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