Medical

10 heart conditions with strange names and how they got them

Ever heard of Prinzmetal angina, Takotsubo cardiomyopathy, or even Kawasaki syndrome? Rick Karsan explains these conditions and how they got their names.

1. Takotsubo cardiomyopathy

Ventriculography showing cardiac morphology in systole in a patient with takotsubo cardiomyopathy

The condition was first described in Japan. The name Takotsubo is a Japanese word, meaning “a pot used for trapping octopus”. It refers to the shape of the heart in people with this condition (as shown on the bottom left section of the image above).

Also known as stress cardiomyopathy and colloquially as broken-heart syndrome, Takotsubo cardiomyopathy is a condition that results in a sudden weakness of heart muscle. This can be triggered by significant emotional stress, hence the name broken-heart syndrome. It can cause chest pain and breathlessness, similar to a heart attack but the condition is temporary and reversible.

2. Cardiac syndrome X

In the 70s, scientists were left scratching their heads when patients experiencing angina-like chest pains didn't appear to have narrowings or blockages in the coronary arteries – the usual cause of angina.

The condition got its name after a study of patients with chest pain carried out by researchers Robert Arbogast and Martial Bourassa produced some puzzling results. In one group, there were electrocardiogram (ECG) signs of coronary heart disease despite the fact they had normal coronary angiograms – this group was referred to as group X in the study. In 1973 Dr Harvey Kemp coined the term “syndrome X”, which later evolved into “cardiac syndrome X”. It is now more commonly known as microvascular angina, and thought to be an abnormality of the tiny arteries in the heart. It is a separate condition to Prinzmetal Angina (see below), although both conditions are more common in women than men.

3. Prinzmetal angina

This peculiar moniker comes from the name of American doctor Myron Prinzmetal, who published work on the condition in 1959.

Also known as variant angina, angina inversa or coronary artery spasm, this is a condition characterised by cycles of chest pain. The pain occurs due to the blood vessels supplying the heart going into spasm, preventing normal blood flow.

Treatment involves medications, including nitrates and calcium channel blockers. It is more common in women than men.

Coincidentally, Prinzmental was also one of the first cardiologists to explore the link between diet and coronary heart disease. Eating an unhealthy diet can contribute to the development of coronary heart disease. 

4. Kawasaki disease

I could make no diagnosis of this unusual sickness for which I could find no reference in any medical literature

Dr Tomisaku Kawasaki

The condition was first described in the 1960s by Japanese paediatrician Dr Tomisaku Kawasaki. When he spotted it in a 4-year-old boy in 1961. “I could make no diagnosis of this unusual sickness for which I could find no reference in any medical literature,” he said. When he saw his second case a year later, he “realised that I had seen two instances which did not exist in any medical textbook.”

It’s a rare condition which mainly affects children under the age of five. It is thought to be caused by an infection, although the exact cause is unknown. Symptoms are a combination of:

  • fever
  • reddened lips
  • rash
  • swelling of hands and feet
  • red eyes
  • lumps in the neck (swollen lymph nodes)

In Kawasaki disease the blood vessels, including those that supply the heart, can become inflamed. This can go on to increase the risk of heart complications either at the time or in later life.

Treatment involves aspirin and intravenous immunoglobulins (a solution of antibodies). The condition is most common in Japan, while in the UK, it’s rare – affecting around eight in every 100,000 children.

Kawasaki worked for 40 years as a paediatrician at the Red Cross Hospital in Hiroo, Tokyo, which is where he spotted the condition that gave his name. He is reported to have decided to become a paediatrician because: “Adult patients were full of complaints, but sick children said little. Basically, I preferred [treating the] children.”

5. Torsades de pointes

The term Torsades de pointes means ‘twisting of the points’ in French

Torsades de pointes is a dangerous abnormal heart rhythm that can lead to sudden cardiac death. It was first described by the French doctor François Dessertenne.

The name of this rare condition relates to the electrical waveform the heart produces on an ECG trace, where there is a twisting and writhing pattern It can cause palpitations, dizziness, light headedness and fainting, with a risk of death if the abnormal rhythm is prolonged. It can occur in connection with other conditions, such as long QT syndrome.

The term Torsades de pointes means ‘twisting of the points’ in French. The term is used for a movement in ballet, and for a twist in the hair or a decorative twist of a cord on clothing, as well as an ornamental motif imitating twisted hairs or threads, seen on classical architectural columns.

6. Tetralogy of Fallot

The strange name feeds back to the French doctor Etienne-Louis Arthur Fallot who described it in 1888. Tetralogy of Fallot is a congenital heart condition that occurs when babies are born with four structural defects of the heart. These are:

  1. Pulmonary stenosis - the pulmonary valve and/or artery is narrowed.
  2. Overriding aorta - the aorta (the main vessel which carries blood from the heart to the rest of the body) usually comes of the left side of the heart. However in TOF, its placed towards the right side, which means that the body receives a mixture of blood which is both high and low in oxygen.
  3. Ventricular septal defect - a hole between the two ventricles (lower chambers) of the heart.
  4. Right ventricular hypertrophy - the muscle on the right side of the heart is thickened excessively.

Babies with this condition usually have a heart operation early in life. Well-known people with the condition include the snowboarder and Olympic gold medallist Shaun White, and Australian cricketer Beau Casson – showing that you can go on to live a full and active life.

7. Barlow’s syndrome

In people with Barlow syndrome, one or more of the flaps of the mitral valve is floppy and don’t close properly

Named after the doctor who described it in 1966, South African professor John Brereton Barlow, the condition is a type of heart valve disease, which affects the mitral valve (which separates the upper and lower chambers on the left side of the heart, and helps control blood flow through the heart). In people with Barlow syndrome, one or more of the flaps of the mitral valve is floppy and don’t close properly.

Later, Dr John Michael Criley gave it a second and more literal name of mitral valve prolapse.

Also known as click-murmur syndrome, because of the sounds which can sometimes be heard with a stethoscope, it is one of the most common types of heart valve abnormality, though many patients have no symptoms.

As a student Barlow was nicknamed "canary" by his colleagues because he kept canaries outside his office, so perhaps the disease could have been called Canary syndrome.

8. Ebstein’s anomaly

The condition was named after Wilhelm Ebstein, a German doctor who first described the defect in 1866.

Ebstein’s anomaly is a congenital heart defect in which one of the heart valves (the tricuspid valve, between the right atrium and the right ventricle) is not formed properly, which can cause blood to leak backwards. As a result, the right atrium of the heart can become enlarged.

It can occur on its own or alongside other congenital heart problems, including atrial septal defect.

9. Eisenmenger syndrome

Eisenmenger syndrome is a rare complication of a heart defect that you are born with (congenital). It involves a heart abnormality that causes a hole to develop between the two chambers of your heart, allowing blood to flow incorrectly within the heart, at the same time as having pulmonary hypertension, which is increased blood pressure in the blood vessels in the lungs.

Symptoms may not arise until the person is in their teens or 20s, when cyanosis (blueish lips, fingers and toes) may be seen. Tiredness and breathing difficulties may occur, as well as palpitations, dizziness and chest pain. The main treatment is medication.

Eisenmenger syndrome was given its name by Dr Paul Wood after Dr Victor Eisenmenger, who first described the condition in 1897.

10. Kounis syndrome

Kounis syndrome is an episode of angina or even a heart attack triggered by an allergic reaction. The syndrome was named after Professor Nicholas G Kounis, a Greek cardiologist, who found that the syndrome is caused by chemicals, such as histamine, which are released by the body during an allergic reaction. These chemicals cause the arteries that supply the heart with blood to go into spasm, thus limiting the blood flow and causing angina-like chest pain.

The allergy can be triggered by bee or wasp stings, or medicines, and there is also some evidence linking Kounis syndrome with a general anaesthetic or stents.

In patients with existing fatty plaque in the coronary arteries, it can even cause the plaque to rupture, leading to a clot and causing a heart attack.

Treatment involves the use of medications to stop the allergic response, such as corticosteroids, antihistamines and adrenaline, if necessary. Meanwhile treatment for the heart problems will also be carried out.

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