Symptoms, causes, treatment and outlook

Cardiac achalasia is a swallowing disorder that falls into an unusual category. It is both very rare and one of the most common causes of motor-related swallowing difficulty.

The condition only affects approximately 1.6 people per 100,000 worldwideeven if these numbers seem to be increasing.

This article will explore what happens in people with cardiac achalasia, what causes this condition, and what can be done to treat it.

Cardiac achalasia is a rare condition that affects the esophagus. It is the tube that carries food and liquids from your mouth to your stomach.

At the base of this tube, leading to the stomach, is a ring-shaped muscle called the lower esophageal sphincter. This muscle is tightly closed when you are not eating to prevent food and stomach juices from bubbling up the esophagus and causing tissue damage. When you eat, this sphincter relaxes, allowing food to pass past and into the stomach for digestion.

In people with cardiac achalasia, there is a problem with the way this muscle opens and closes. This causes swallowing problems, which can lead to a host of other symptoms and complications.

The main symptom of cardiac achalasia is difficulty swallowing. The muscular opening that separates your throat from your stomach doesn’t open and close when it’s supposed to. This causes food to get trapped in the esophagus, where it can eventually back up into the mouth.

Not only can this difficulty in swallowing lead to nutritional deficiencies, but it can also cause a number of other symptoms like:

  • mild chest pain that comes and goes
  • regurgitation of food from the esophagus into the mouth
  • the movement of food from the esophagus into the lungs (aspiration)
  • cough at night
  • dry mouth and eyes

Malnutrition and weight loss can result from these symptoms, but people with cardiac achalasia can also develop problems such as pneumonia by aspirating (inhaling) food into their lungs. The risk of developing esophageal cancer also appears to be higher in people with this condition.

Nobody really knows what causes cardiac achalasia, but it could be damage to the nerves that help the throat, stomach, and other parts of the digestive system function. What damages these nerves and causes them to lose function is a mystery, but there are theories that genetics, autoimmune diseases, or even infections could play a role.

In terms of who develops this disease, there really are no patterns. Cardiac achalasia appears to affect mostly genders and races equally and is primarily a condition that occurs in adults. It is most common for this condition to develop in your twenties and fifties, with less than 2 to 5% cases appearing in children under 16 years of age.

When genetics is suspected as the cause in very rare forms of this condition, those assigned male at birth are twice as likely to be affected as those assigned female. People with the following characteristics are also more likely to be affected:

Other possible links to the development of this condition include things like:

Diagnosis of cardiac achalasia can take some time, simply due to the rarity of the condition. If you have trouble swallowing or regurgitating undigested food – the two most common early symptoms of the disease – your doctor will start by asking about your personal and family medical history, as well as any new symptoms you‘have.

A physical examination may be performed. With this condition, raising the arms and other movements can sometimes help move food into the stomach.

A physical examination alone is notThis is usually not enough to diagnose cardiac achalasia. Typically, the following tests will be done to better understand what is happening when you swallow:

  • X-ray imaging to examine the structure of your digestive system.
  • Imaging after drinking a barium solution (study of barium swallowing) to follow the path of swallowed food and liquids.
  • Esophageal manometry to measure the strength and efficiency of muscle contractions in the throat.
  • Upper gastrointestinal (GI) endoscopy for a direct view of your esophagus.

The main goal of treatment for cardiac achalasia is to relieve swallowing problems and prevent complications. Since the root cause of this condition isnot fully understood, there really isThere is no treatment to truly prevent or correct achalasia of the cardia.

Surgery and medications may be used depending on the severity of the condition and how well you tolerate the surgery.

Surgical treatments

For people at low risk of surgical complications, treatments may include the following procedures:

  • Pneumatic expansion. A balloon is inserted into the esophagus and dilated to widen the opening of the lower esophageal sphincter. People who undergo this procedure often need to repeat it in time.
  • Laparoscopic Heller myotomy (LHM). A surgical procedure called fundoplication that brings stomach tissue to the base of the esophagus, bypassing the lower sphincter. This is considered a first-line treatment for cardiac achalasia, as up to 97% of patients respond to this procedure.
  • Peroral endoscopic myotomy (POEM). A tube is passed through the mouth and a small incision is made to open and loosen the lower esophageal sphincter. This procedure is effective but often reserved for certain types of achalasia of the cardia, including when other treatments have failed.

Each of these procedures can carry the risk of surgical complications, the need for repeated treatment, and the development of gastroesophageal reflux disease (GERD) due to lower sphincter relaxation.

Medications

In some cases, drugs like muscle relaxants and isosorbide or nifedipine are used to relieve symptoms of cardiac achalasia. Dietary changes to the thickness and textures of foods and liquids may also help.

Botulinum toxin applications through an endoscopy tube are also an option. This treatment is generally reserved for people who cannot tolerate surgery or who have a less favorable state of health.

Cardiac achalasia is considered a chronic, incurable neurological disorder. Even with treatment, this condition will require lifelong monitoring and treatment.

Therapies like pneumatic dilation can help for a while, but 10-15% of people experience excessive dilation. This can result in the removal of all or part of the esophagus in 5% of people who receive this therapy.

Other surgeries don’t have these exact results, but even LHM and POEM tend to fail or require repeat treatment after about 5 years.

Even after surgery, some lifestyle and diet changes may be necessary, such as:

  • try to eat small meals
  • eat standing up
  • avoid lying flat
  • keep your head at a 30-45 degree angle to avoid aspiration

Although there is no cure for cardiac achalasia, the overall prognosis is good for most people with the condition. A typical life expectancy is possible with lifelong follow-up and on-the-spot treatments.

Cardiac achalasia is a chronic condition in which the muscle at the base of the throat where food enters the stomach is overstretched. This can cause food and liquids to back up into the throat.

Surgeries to loosen this muscle are a treatment option, but usually need to be repeated over time.

There is no cure for cardiac achalasia, but people with this disorder can usually live a normal lifespan with regular monitoring and treatment.

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