Cor Triatriatum

Overview

Cor triatriatum is a rare heart problem that a person is born with. In most cases, a thin sheet of tissue divides the upper left chamber of the heart, called the left atrium, into two parts. This extra tissue can partly block blood flow from the lungs to the rest of the body. Less often, the same kind of problem happens on the right side of the heart. Some people have only a small blockage and few or no symptoms. Others need treatment, often surgery, to open the way for blood to flow normally.

No. Cor triatriatum is very rare. It is seen in only a small number of people with congenital heart defects.

Symptoms

Common symptoms

• Shortness of breath, especially with exercise or activity
• Feeling very tired or easily worn out
• Fast or pounding heartbeat, called palpitations
• Cough, wheezing, or repeated chest infections
• Chest discomfort
• Swelling in the legs or ankles in some people

Symptoms in children

• Poor feeding or tiring quickly during feeds
• Poor weight gain or slow growth
• Fast breathing
• Sweating during feeds or activity
• Pale, grey, or bluish lips or skin
• Frequent lung infections

Symptoms in older adults

• Breathlessness with activity or when lying flat
• Unusual tiredness
• Irregular or fast heartbeat
• Swelling in the legs or ankles
• Cough or lung congestion
• Feeling dizzy or lightheaded

Causes

Main causes

• Cor triatriatum happens when the heart does not form normally during pregnancy. A thin membrane or sheet of tissue remains inside an upper heart chamber.
• In most cases, the exact reason this happens is not known.
• It is not caused by anything the person ate, drank, or did after birth.

Risk factors

• Having other congenital heart defects
• A family history of congenital heart defects, though this is not common
• It can be found in babies, children, or adults, but it is always present from birth

When to see a doctor

See a doctor urgently if:

• You or your child has new or worsening shortness of breath
• You have a fast, irregular, or pounding heartbeat that does not settle
• Your child is not feeding well, is sweating during feeds, or is not gaining weight
• You notice swelling in the legs or ankles that is getting worse

Book a routine appointment if:

• You have a known heart defect and your follow-up appointment is due
• You have mild breathlessness or tiredness that keeps coming back
• You want to ask about heart defects that run in your family

Diagnosis

A doctor will ask about symptoms, listen to the heart, and check the body. The main test is usually an echocardiogram, which uses sound waves to make pictures of the heart. This can show the extra membrane and how much blood flow is blocked. Sometimes other heart tests are needed.

Tests that may be done

• Echocardiogram, also called an ultrasound of the heart
• Electrocardiogram, or ECG, to check the heart rhythm
• Chest X-ray to look at heart size and lung fluid
• Cardiac MRI or CT scan for more detailed pictures
• Cardiac catheterization in some cases, using a thin tube to measure pressures in the heart

What to expect at your appointment

Most tests are painless and do not involve radiation, though CT scans use a small amount. Children may need a calm setting or mild sedation for some tests. A cardiologist, a doctor who specializes in the heart, will explain the results and whether treatment is needed.

Treatment

Treatment depends on how much the extra tissue blocks blood flow and whether there are symptoms. Some people only need regular checkups. Others need surgery to remove or open the membrane. A heart doctor will decide what is right for each person.

Self-care at home

• Keep all appointments with your heart doctor
• Take care of your general health and treat infections promptly
• Ask your care team before starting strenuous exercise or competitive sports
• Do not smoke, and avoid secondhand smoke
• Ask your doctor or pharmacist before taking any new medicine, including over-the-counter ones

Medical treatments

There is no medicine that removes the extra tissue. Medicines may be used to ease symptoms such as extra fluid or a fast heartbeat, but they do not fix the blockage. If the blockage is significant, surgery is usually the main treatment. Your care team will explain the choices in plain words and answer your questions.

When is surgery considered?

Surgery may be recommended if the membrane blocks blood flow, if there are symptoms, or if pressure builds up in the heart or lungs. The goal is to remove the blockage and let blood flow normally. Many people do well after surgery, but follow-up care is still important.

Living with this condition

Most people with cor triatriatum can go to school, work, and take part in daily life. If you have symptoms or are waiting for treatment, you may need to pace yourself and rest more. Your heart team can tell you what activity level is safe for you.

Lifestyle tips

• Keep regular follow-up appointments
• Stay active within the limits your care team recommends
• Avoid smoking and secondhand smoke
• Get enough sleep and manage stress
• Ask for help when you feel unwell or overwhelmed

Diet and exercise

Eat a balanced diet with plenty of vegetables, fruit, whole grains, and lean protein. Limit very salty foods if your doctor advises it. Exercise is helpful for many people, but ask your heart team what type and amount is safe for you, especially if you have symptoms or are recovering from surgery.

Mental health and emotional wellbeing

Living with a heart condition can feel worrying, especially for parents of children with heart defects. It is normal to feel anxious or sad at times. Talking with your care team, family, or a counselor can help. If you ever feel unable to cope or have thoughts of harming yourself, contact your local emergency number or a crisis support service right away.

Prevention

Cor triatriatum cannot be prevented. It is a heart defect that forms before birth, and it is not caused by anything a parent did during pregnancy.

Vaccines

There is no vaccine to prevent cor triatriatum. Your care team may recommend routine vaccinations to protect your general health and heart health. Ask your doctor or pharmacist which vaccines are right for you or your child.

Screening programmes

There is no routine screening for the general public. If someone in your family has a congenital heart defect, ask your doctor whether any extra checks are needed. Heart problems are sometimes seen during pregnancy ultrasound, but not all are found before birth.

Complications

If left untreated

• Heart failure, when the heart cannot pump blood well enough
• High blood pressure in the lungs, called pulmonary hypertension
• Irregular heartbeats
• Fluid build-up in the lungs
• Poor growth or feeding problems in children
• Rarely, blood clots or stroke

Long-term outlook

The outlook is often good, especially when the condition is found and treated. Many people live full lives after repair. Some need lifelong follow-up with a heart doctor. Your care team can give you the most useful advice for your situation, and regular checkups help catch problems early.

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