Organs On Left Side Of Body

Heterotaxy syndrome is a condition in which the internal organs are abnormally arranged in the chest and abdomen. The term “heterotaxy” is from the Greek words “heteros,” meaning “other than,” and “taxis,” meaning “arrangement.” Individuals with this condition have complex birth defects affecting the heart, lungs, liver, spleen, intestines, and other organs.

Heterotaxy syndrome

Heterotaxy syndrome is a condition in which the internal organs are abnormally arranged in the chest and abdomen. The term “heterotaxy” is from the Greek words “heteros,” meaning “other than,” and “taxis,” meaning “arrangement.” Individuals with this condition have complex birth defects affecting the heart, lungs, liver, spleen, intestines, and other organs.

In the normal body, most of the organs in the chest and abdomen have a particular location on the right or left side. For example, the heart, spleen, and pancreas are on the left side of the body, and most of the liver is on the right. This normal arrangement of the organs is known as “situs solitus.” Rarely, the orientation of the internal organs is completely flipped from right to left, a situation known as “situs inversus.” This mirror-image orientation usually does not cause any health problems, unless it occurs as part of a syndrome affecting other parts of the body. Heterotaxy syndrome is an arrangement of internal organs somewhere between situs solitus and situs inversus; this condition is also known as “situs ambiguus.” Unlike situs inversus, the abnormal arrangement of organs in heterotaxy syndrome often causes serious health problems.

Heterotaxy syndrome can alter the structure of the heart , including the attachment of the large blood vessels that carry blood to and from the rest of the body. It can also affect the structure of the lungs , such as the number of lobes in each lung and the length of the tubes (called bronchi) that lead from the windpipe to the lungs. In the abdomen, the condition can cause a person to have no spleen (asplenia) or multiple small, poorly functioning spleens (polysplenia). The liver may lie across the middle of the body instead of being in its normal position to the right of the stomach. Some affected individuals also have intestinal malrotation, which is an abnormal twisting of the intestines that occurs in the early stages of development before birth.

Depending on the organs involved, signs and symptoms of heterotaxy syndrome can include a bluish appearance of the skin or lips (cyanosis, which is due to a shortage of oxygen), breathing difficulties, an increased risk of infections, and problems with digesting food. The most serious complications are generally caused by critical congenital heart disease, a group of complex heart defects that are present from birth. Biliary atresia, a problem with the bile ducts in the liver , can also cause severe health problems in infancy.

The severity of heterotaxy syndrome varies depending on the specific abnormalities involved. Some affected individuals have only mild health problems related to the condition. At the other end of the spectrum, heterotaxy syndrome can be life-threatening in infancy or childhood, even with treatment.

Frequency

The prevalence of heterotaxy syndrome is estimated to be 1 in 10,000 people worldwide. However, researchers suspect that the condition is underdiagnosed, and so it may actually be more common than this. Heterotaxy syndrome accounts for approximately 3 percent of all congenital heart defects. For reasons that are unknown, the condition appears to be more common in populations in Asia than in North America and Europe. Recent studies report that in the United States, the condition occurs more frequently in children born to Black or Hispanic mothers than in children born to white mothers.

Causes

Heterotaxy syndrome can be caused by mutations in many different genes. The proteins produced from most of these genes play roles in determining which structures should be on the right side of the body and which should be on the left, a process known as establishing left-right asymmetry. This process occurs during the earliest stages of embryonic development. Dozens of genes are probably involved in establishing left-right asymmetry; mutations in at least 20 of these genes have been identified in people with heterotaxy syndrome.

In some cases, heterotaxy syndrome is caused by mutations in genes whose involvement in determining left-right asymmetry is unknown. Rarely, chromosomal changes such as insertions, deletions , duplications , and other rearrangements of genetic material have been associated with this condition.

Heterotaxy syndrome can occur by itself, or it can be a feature of other genetic syndromes that have additional signs and symptoms. For example, at least 12 percent of people with a condition called primary ciliary dyskinesia have heterotaxy syndrome. In addition to abnormally positioned internal organs, primary ciliary dyskinesia is characterized by chronic respiratory tract infections and an inability to have children (infertility). The signs and symptoms of this condition are caused by abnormal cilia , which are microscopic, finger-like projections that stick out from the surface of cells. It appears that cilia play a critical role in establishing left-right asymmetry before birth.

Studies suggest that certain factors affecting a woman during pregnancy may also contribute to the risk of heterotaxy syndrome in her child. These include diabetes mellitus; smoking; and exposure to hair dyes, cocaine, and certain chemicals such as pesticides and organic solvents.

Some people with heterotaxy syndrome have no identified gene mutations or other risk factors. In these cases, the cause of the condition is unknown.

Learn more about the genes associated with Heterotaxy syndrome

Additional Information from NCBI Gene:

Inheritance

Most often, heterotaxy syndrome is sporadic, meaning that only one person in a family is affected. However, about 10 percent of people with heterotaxy syndrome have a close relative (such as a parent or sibling) who has a congenital heart defect without other apparent features of heterotaxy syndrome. Isolated congenital heart defects and heterotaxy syndrome may represent a range of signs and symptoms that can result from a particular genetic mutation; this situation is known as variable expressivity. It is also possible that different genetic and environmental factors combine to produce isolated congenital heart defects in some family members and heterotaxy syndrome in others.

When heterotaxy syndrome runs in families, it can have an autosomal dominant , autosomal recessive , or X-linked pattern of inheritance, depending on which gene is involved. Autosomal dominant inheritance means that one copy of the altered gene in each cell is sufficient to cause the disorder. Autosomal recessive inheritance means that both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition. In X-linked inheritance, the mutated gene that causes the disorder is located on the X chromosome, one of the two sex chromosomes in each cell.

When heterotaxy syndrome occurs as a feature of primary ciliary dyskinesia, it has an autosomal recessive pattern of inheritance.

Other Names for This Condition

Heterotaxy
HTX
Ivemark syndrome
Left isomerism
Right isomerism
Situs ambiguus
Situs ambiguus viscerum
Visceral heterotaxy

Spleen

The spleen is a small organ inside your left rib cage, just above the stomach. It’s part of the lymphatic system (which is part of the immune system). The spleen stores and filters blood and makes white blood cells that protect you from infection. Many diseases and conditions can affect how the spleen works. A ruptured (torn) spleen can be fatal.

Overview

Your spleen is a fist-sized organ that lives inside your left ribcage.

What is the spleen?

Your spleen is a small organ that sits inside your left rib cage, just above your stomach. In adults, the spleen is about the size of an avocado. The spleen is part of your lymphatic system (which is part of your immune system). It does several important jobs to keep your body healthy.

Many different conditions, diseases, disorders and injuries affect how your spleen works. Providers usually treat the condition or illness that’s causing problems with the spleen. If necessary, healthcare providers can remove your spleen during a procedure called a splenectomy.

Function

What does the spleen do?

Stores blood.
Filters blood by removing cellular waste and getting rid of old or damaged blood cells.
Makes white blood cells and antibodies that help you fight infection.
Maintains the levels of fluid in your body.
Produces antibodies that protect you against infection.

Anatomy

What are the parts of the spleen?

There are two parts of the spleen. They each do different jobs. The types of tissue in the spleen are:

White pulp: As part of the immune system, the white pulp produces white blood cells. These blood cells make antibodies. Antibodies fight infection.
Red pulp: The red pulp acts like a filter. It removes waste from the blood and gets rid of old or damaged blood cells. Red pulp also destroys bacteria and viruses.

Conditions and Disorders

What conditions and disorders affect this system?

Many disorders, conditions, injuries and diseases can cause problems in the spleen. These problems include:

Enlarged spleen (splenomegaly): Several conditions can cause the spleen to swell and get too big. An enlarged spleen can cause pain and an uncomfortable feeling of fullness, even if you haven’t eaten much. Splenomegaly is a dangerous condition because the spleen can rupture (tear) or bleed. The spleen can become enlarged from:

Blood cancers, such as leukemia and Hodgkin’s lymphoma, and cancer in other parts of the body that metastasize (spread) to the spleen.
Blood clots in the spleen or the liver.
Certain types of anemia, including hemolytic anemia.
Cystic fibrosis (CF).
Infections, including mononucleosis (mono), syphilis, malaria and endocarditis (infection of the heart’s lining).
Liver problems, including cirrhosis.
Inherited metabolic disorders, such as Gaucher disease.
Inflammatory diseases, including sarcoidosis.
Protein disorders like amyloidosis.

Functional asplenia: This condition happens when your spleen doesn’t work as it should. It may overreact (hypersplenism) and destroy healthy red blood cells. Destroying too many blood cells can increase the risk of infection and lead to bruising and bleeding. Functional asplenia can result from:

An accident or trauma that damages the spleen.
Celiac disease.
Sickle cell disease.

Damaged or ruptured spleen: Your spleen can rupture (tear) from injuries and trauma. Car accidents and blows to the abdomen (belly) are common causes of spleen damage. This life-threatening injury can cause severe internal bleeding.

Symptoms of a ruptured spleen include:

A quick heartbeat.
Nausea.
Dizziness.
Pain under the ribs on the left side.

Care

How can I keep my spleen healthy?

To keep your spleen, lymphatic system and immune system working properly, you should drink plenty of water, exercise regularly and maintain a healthy weight. Eat a balanced diet with plenty of fruits and vegetables. By staying healthy, you’ll help your immune system protect you from infections and illness.

Additional Common Questions

Can you live without a spleen?

Although the spleen does many important jobs in the body, it is possible to live without one. Providers call this condition asplenia or living without a spleen.

Rarely, some people are born without a spleen. Sometimes, healthcare providers perform surgery to remove the spleen (splenectomy) because it’s damaged or diseased. Without the spleen, the liver takes over many of the spleen’s duties.

Splenectomy is also a treatment for different types of thrombocytopenia, including immune thrombocytopenia (ITP). These disorders cause low platelet levels in the body. Platelets are blood cells that help your blood clot.

What are the complications of a damaged or missing spleen?

People who live without a spleen have a higher risk of infection. If the spleen is missing or damaged, the body has a harder time protecting itself from bacteria and viruses. People who have other conditions that affect the immune system (such as cancer or HIV) are at a higher risk of infection.

If you’re living without a spleen or your spleen doesn’t work like it should, talk to your provider. You should stay up-to-date on vaccinations to help protect you from getting sick. Your provider may recommend taking daily antibiotics to prevent a bacterial infection. This may be important if you also have another condition that affects your immune system.

When should I call my doctor?

Call your provider if you have any symptoms of an enlarged or ruptured spleen, including:

Early satiety (a feeling of fullness after only eating a little bit).
Unexplained bruising or bleeding.
Pain under the left rib cage or tenderness when you touch the area.

How do I know if I’m at risk for spleen problems?

You have a higher risk of spleen problems if you have certain conditions. These include some blood cancers, Gaucher disease or cystic fibrosis. People with a rare condition called hereditary spherocytosis are at a higher risk of severe anemia and may need to have the spleen removed. Talk to your provider if you have a family history of these conditions.

A note from Cleveland Clinic

Your spleen is a small but important organ. It works hard to fight infection, remove old or damaged blood cells and keep fluids moving through your body. Many disorders, infections, injuries and diseases can cause problems in the spleen. Talk to your provider right away if you have pain in your rib cage on the left side. This could be a sign of a ruptured spleen, which is a life-threatening condition.