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Eisenmenger Syndrome

Table of Contents

Eisenmenger Syndrome

ES; Eisenmenger complex; Eisenmenger disease; Eisenmenger reaction; Eisenmenger physiology

Eisenmenger syndrome (ES) refers to the combination of a type of high blood pressure that affects the blood vessels of the lungs and heart (pulmonary hypertension), and abnormal blood flow through the heart.

Ventricular Septal Defect

Image by BruceBlaus

Summary

Eyzenmenger sindromu

Image by MrArifnajafov/Wikimedia

Eyzenmenger sindromu

Eyzenmenger sindromu anadangəlmə ürək qüsurlarında sistem arterial təzyiqin qüsur dəliyi vasitəsilə ağciyər arteriyasına ötürülməsi nəticəsində meydana çıxan ağciyər arteriyasının hipertenziyasıdır. Bu zaman ağciyərlərin damar sistemində, xüsusilə kiçik diametrli ağciyər arteriolalarında obstruktiv dəyişikliklər (damar divarının gedən struktiv dəyişikliklər nəticəsində qalınlaşması prosesi) gedərək onlarda diffuz daralmalar əmələ gəlir ki, bu da onların qandaşıma funksiyasının pozulmasına gətirib çıxarır. Eyzenmenger sindromunda ağciyər arteriyasındakı təzyiq sistem arterial təzyiqə bərabər olur. Nəticə etibarilə sağ mədəcikdə müqavimətin kəskin artması venoz qanın qüsur dəliyi vasitəsilə geri aorta sisteminə atılması, sağ-sol şuntu baş verir və sianoz inkişaf edir.

Image by MrArifnajafov/Wikimedia

What Is Eisenmenger Syndrome?

Eisenmenger syndrome (ES) refers to the combination of a type of high blood pressure that affects the blood vessels of the lungs and heart (pulmonary hypertension), and abnormal blood flow through the heart. ES most often occurs in people who are born with a heart defect (congenital heart defect) that does not get repaired in childhood. The most common type of heart defect associated with Eisenmenger syndrome is a ‘hole in the heart’ or ventricular septal defect, but other types of heart defects can lead to Eisenmenger syndrome. The symptoms include blueish skin color (cyanosis), rounded fingers and toes (clubbing), and shortness of breath. The symptoms of ES typically get worse over time.

Eisenmenger syndrome is not inherited in families. It is diagnosed based on the symptoms in a person with a heart defect that has not been repaired. Treatment for this syndrome includes medications for pulmonary hypertension and avoiding high-risk situations, such as pregnancy and high-altitudes. A heart-lung transplant is a treatment option for those who have severe ES.

Source: Genetic and Rare Diseases (GARD) Information Center

Additional Materials (2)

What is Eisenmenger syndrome? | Circulatory System and Disease | NCLEX-RN | Khan Academy

Video by khanacademymedicine/YouTube

Eisenmenger Syndrome Explained: A Comprehensive Guide

Video by Medical Centric/YouTube

10:01

What is Eisenmenger syndrome? | Circulatory System and Disease | NCLEX-RN | Khan Academy

khanacademymedicine/YouTube

4:09

Eisenmenger Syndrome Explained: A Comprehensive Guide

Medical Centric/YouTube

Symptoms

Chest Pain

Image by TheVisualMD

Chest Pain

A blood clot blocking a major artery in the lung is called a pulmonary embolism. In most cases, multiple clots travel to the lungs from another part of the body, particularly in deep veins in the legs. Large embolisms can cause tissue damage and impair the lungs' ability to supply oxygen to the rest of the body. Symptoms can include sudden shortness of breath, chest pain and a cough with signs of blood. Although anyone can develop pulmonary embolisms, such clots are often associated with prolonged bed rest, long periods of sitting in a cramped position and old age.

Image by TheVisualMD

What Are the Signs and Symptoms of Eisenmenger Syndrome?

Signs and symptoms of Eisenmenger syndrome include:

High blood pressure in the main blood vessels to the lungs (pulmonary hypertension)
Shortness of breath
Bluish skin, lips, finger and toes (cyanosis)
Rounded fingertips and toes (clubbing)
Chest pain
Feeling tired or dizzy

Other signs and symptoms include an abnormal heart rhythm (arrhythmia), stroke, coughing up blood (hemoptysis), and swelling of joints from excess uric acid (gout). Eisenmenger syndrome mainly develops in people who are born with a heart defect that does not get repaired. The symptoms usually develop before puberty but can also develop in young adulthood and typically get worse with time.

Source: Genetic and Rare Diseases (GARD) Information Center

Additional Materials (1)

Clubbing Fingers

Example of clubbing secondary to pulmonary hypertension in a patient with Eisenmenger's syndrome

Image by Ann McGrath/Wikimedia

Clubbing Fingers

Ann McGrath/Wikimedia

Cause

Ventricular Septal Defect Patch

Image by BruceBlaus

Ventricular Septal Defect Patch

A medical illustration depicting Ventricular Septal Defect Patch

Image by BruceBlaus

What Causes Eisenmenger Syndrome?

Eisenmenger syndrome (ES) is caused by a defect in the heart. The most common heart defect that can lead to ES is a ventricular septal defect (VSD), a hole between the two pumping chambers (the left and right ventricles) of the heart. Other heart defects that can lead to Eisenmenger syndrome include atrial septal defect (ASD) and patent ductus arteriosus (PDA). These heart defects allow blood that has already picked up oxygen from the lungs to flow back into the lungs, instead of going out to the rest of the body. This can eventually lead to increased pressure in the pulmonary artery (pulmonary hypertension), the main blood vessel that connects the heart and the lungs. Increased pulmonary hypertension leads to abnormal blood flow and results in less oxygen going to the body.

Over time, this abnormal blood flow can damage the small blood vessels in the lungs. This causes high blood pressure in the lungs. As a result, the blood backs up and does not go to the lungs to pick up oxygen. Instead, the blood goes from the right side to the left side of the heart (right to left shunt), and oxygen-poor blood travels to the rest of the body.

Source: Genetic and Rare Diseases (GARD) Information Center

Additional Materials (1)

the diagram shows a healthy heart and one suffering from ventricular septal defect.

the diagram shows a healthy heart and one suffering from ventricular septal defect.

Image by Mariana Ruiz LadyofHats

the diagram shows a healthy heart and one suffering from ventricular septal defect.

Mariana Ruiz LadyofHats

Inheritance

Zero / None / Null

Image by Clker-Free-Vector-Images/Pixabay

Zero / None / Null

Image by Clker-Free-Vector-Images/Pixabay

How Is Eisenmenger Syndrome Inherited?

Eisenmenger syndrome is not inherited in families. However, genetic factors may contribute to the chance to be born with a heart defect.

Source: Genetic and Rare Diseases (GARD) Information Center

Diagnosis

Pulse oximetry

Image by UusiAjaja

Pulse oximetry

Picture of a wrist mounted pulse oxymeter. The sensor is attached to the finger which in turn is attached to the device via a cable. The device shows the oxygenation of the blood, the pulse rate and the pulse waveform along with several indicators of device specific information.

Image by UusiAjaja

How Is Eisenmenger Syndrome Diagnosed?

Eisenmenger syndrome (ES) is diagnosed based on the symptoms and the presence of a heart defect. Additional blood work and other medical tests may be done to determine the extent of the symptoms. These tests may include pulse oximetry (checks blood oxygen levels), chest x-ray, EKG, pulmonary function tests, complete blood count (CBC), and a test for iron levels.

Source: Genetic and Rare Diseases (GARD) Information Center

Additional Materials (1)

Pulse Oximetry using the Tetherless Masimo Radius PPG

Tetherless Pulse Oximetry

Image by Phoenix573

Pulse Oximetry using the Tetherless Masimo Radius PPG

Phoenix573

Treatment

Medicating High Blood Pressure

Image by TheVisualMD

Medicating High Blood Pressure

If lifestyle changes don’t reduce blood pressure to an acceptable level, doctors will generally prescribe antihypertensive medications. There are a number of different types of antihypertensives available. The most commonly prescribed include: Thiazide diuretics. Often the first drug prescribed, diuretics dilate blood vessels and also decrease fluid volume in the body by helping the kidneys to eliminate salt and water. Angiotensin-converting enzyme (ACE) inhibitors. ACE inhibitors reduce hypertension by dilating arterioles (the smallest divisions of the arteries, linking the arteries and the capillaries). They do this by blocking the production of ACE, a naturally occurring substance that constricts blood vessels. Angiotensin II receptor blockers (ARBs). Like ACE inhibitors, ARBs dilate arterioles, but they accomplish this by preventing ACE from having any effect on the heart and blood vessels. ARBs are often prescribed for people who experience adverse side effects from ACE inhibitors.

Image by TheVisualMD

How Might Eisenmenger Syndrome Be Treated?

In addition, people with ES are advised to avoid situations that may make their symptoms worse, including pregnancy, high altitudes, and extreme physical exercise.

Oxygen therapy has been helpful for some people, but it may not prevent the symptoms from getting worse. People with severe symptoms may need a heart-lung transplant.

Source: Genetic and Rare Diseases (GARD) Information Center

Prognosis

Prognosis Icon

Image by mcmurryjulie/Pixabay

Prognosis Icon

Image by mcmurryjulie/Pixabay

What Is the Long-Term Outlook for People with Eisenmenger Syndrome?

The long-term outlook for someone with Eisenmenger syndrome depends on the severity of the symptoms and the presence of any other health conditions. Typically, the symptoms of ES get worse over time.

Source: Genetic and Rare Diseases (GARD) Information Center

Statistics

Populations - How Common

Image by geralt/Pixabay

Populations - How Common

Image by geralt/Pixabay

How Common Is Eisenmenger Syndrome?

The exact number of people with Eisenmenger syndrome (ES) is unknown. This condition occurs in about 1-6% of adults who are born with a heart defect. ES is more common in people who have a heart defect that was not detected or surgically repaired as a child.

Source: Genetic and Rare Diseases (GARD) Information Center

Related HealthJournals

Congenital Heart Defects

Congenital heart defects (CHD) are problems with the structure of the heart, and are the most common type of birth defect. The defects can involve the walls of the heart, the valves of the heart, and the arteries and veins near the heart. Learn more about the symptoms, causes, treatments, and how to manage congenital heart defects.

Ventricular Septal Defect

A ventricular septal defect is a hole in the wall between the ventricles, which are the two lower chambers of the heart. If the hole is large, this may make the heart and lungs work harder and may cause fluid to build up in the lungs. Learn more about ventricular septal defects.

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Eisenmenger Syndrome

Eisenmenger syndrome (ES) refers to the combination of a type of high blood pressure that affects the blood vessels of the lungs and heart (pulmonary hypertension), and abnormal blood flow through the heart.

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