Eisenmenger's syndrome is defined as the process in which a left-to-right shunt

Cardiac shunt

Cardiac shunts is when the blood flow follows a pattern in the heart that deviates from the normal circuit of the circulatory system. It may be described as right-left, left-to-right or bidirectional, or as systemic-to-pulmonary or pulmonary-to-systemic. The direction may be controlled by left...

 caused by a congenital heart defect

Congenital heart defect

A congenital heart defect is a defect in the structure of the heart and great vessels which is present at birth. Many types of heart defects exist, most of which either obstruct blood flow in the heart or vessels near it, or cause blood to flow through the heart in an abnormal pattern. Other...

 causes increased flow through the pulmonary vasculature, causing pulmonary hypertension

Pulmonary hypertension

In medicine, pulmonary hypertension is an increase in blood pressure in the pulmonary artery, pulmonary vein, or pulmonary capillaries, together known as the lung vasculature, leading to shortness of breath, dizziness, fainting, and other symptoms, all of which are exacerbated by exertion...

, which in turn, causes increased pressures in the right side of the heart and reversal of the shunt into a right-to-left shunt

Right-to-left shunt

A right-to-left shunt is a cardiac shunt which allows blood to flow from the right heart to the left heart. This terminology is used both for the abnormal state in humans and for normal physiological shunts in reptiles...

.

In adults, the most common causes of cyanotic congenital heart disease are Eisenmenger syndrome and tetralogy of Fallot. Eisenmenger's syndrome specifically refers to the combination of systemic-to-pulmonary communication, pulmonary vascular disease and cyanosis.
It can cause serious complications in pregnancy

Pregnancy

Pregnancy refers to the fertilization and development of one or more offspring, known as a fetus or embryo, in a woman's uterus. In a pregnancy, there can be multiple gestations, as in the case of twins or triplets...

, though successful delivery has been reported.

Etymology

Eisenmenger syndrome was so named

Eponym

An eponym is the name of a person or thing, whether real or fictitious, after which a particular place, tribe, era, discovery, or other item is named or thought to be named...

 by Dr. Paul Wood after Dr. Victor Eisenmenger, who first described the condition in 1897.

Etiology

A number of congenital heart defects can cause Eisenmenger syndrome, including atrial septal defect

Atrial septal defect

Atrial septal defect is a form of congenital heart defect that enables blood flow between the left and right atria via the interatrial septum. The interatrial septum is the tissue that divides the right and left atria...

s, ventricular septal defect

Ventricular septal defect

A ventricular septal defect is a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart.The ventricular septum consists of an inferior muscular and superior membranous portion and is extensively innervated with conducting cardiomyocytes.The membranous...

s, patent ductus arteriosus

Patent ductus arteriosus

Patent ductus arteriosus is a congenital disorder in the heart wherein a neonate's ductus arteriosus fails to close after birth. Early symptoms are uncommon, but in the first year of life include increased work of breathing and poor weight gain...

, and more complex types of acyanotic heart disease

Heart disease

Heart disease, cardiac disease or cardiopathy is an umbrella term for a variety of diseases affecting the heart. , it is the leading cause of death in the United States, England, Canada and Wales, accounting for 25.4% of the total deaths in the United States.-Types:-Coronary heart disease:Coronary...

.

Pathogenesis

The larger, more muscular, left side of the heart generates the higher pressures required to supply blood to the whole body. The smaller, right side of the heart generates the lower pressure required to circulate blood solely through the lungs. If a large anatomic defect exists between the two sides of the heart, blood will flow from the left side to the right side. This results in high blood flow and pressure travelling through the lungs. The increased pressure causes damage to delicate capillaries, which then are replaced with scar tissue. Scar tissue does not contribute to oxygen transfer, therefore decreasing the useful volume of the pulmonary vasculature. The scar tissue also provides less flexibility than normal lung tissue, causing further increases in blood pressure, and the heart must pump harder to continue supplying the lungs, leading to damage of more capillaries.

Eventually, due to increased resistance, pulmonary pressures may increase sufficiently to cause a reversal of blood flow, so blood begins to travel from the right side of the heart to the left side, and the body is supplied with deoxygenated blood, leading to cyanosis

Cyanosis

Cyanosis is the appearance of a blue or purple coloration of the skin or mucous membranes due to the tissues near the skin surface being low on oxygen. The onset of cyanosis is 2.5 g/dL of deoxyhemoglobin. The bluish color is more readily apparent in those with high hemoglobin counts than it is...

 and resultant organ damage.

The reduction in oxygen transfer reduces oxygen saturation

Oxygen saturation

Oxygen saturation or dissolved oxygen is a relative measure of the amount of oxygen that is dissolved or carried in a given medium. It can be measured with a dissolved oxygen probe such as an oxygen sensor or an optode in liquid media, usually water.It has particular significance in medicine and...

 in the blood, leading to increased production of red blood cells in an attempt to bring the oxygen saturation up. The excess of red blood cells is called Polycythaemia. Desperate for enough circulating oxygen, the body begins to dump immature red cells into the blood stream. Immature red cells are not as efficient at carrying oxygen as mature red cells, and they are less flexible, less able to easily squeeze through tiny capillaries in the lungs, and so contribute to death of pulmonary capillary beds. The increase in red blood cells also causes hyperviscosity syndrome

Hyperviscosity syndrome

Hyperviscosity syndrome is a group of symptoms triggered by increase in the viscosity of the blood. Symptoms of high blood viscosity include spontaneous bleeding from mucous membranes, visual disturbances due to retinopathy, and neurologic symptoms ranging from headache and vertigo to seizures and...

.

A person with Eisenmenger syndrome is paradoxically subject to the possibility of both uncontrolled bleeding due to damaged capillaries and high pressure, and random clots due to hyperviscosity and stasis

Stasis (medicine)

In medicine, stasis is the state in which the normal flow of a body liquid stops, for example the flow of blood through vessels or of intestinal contents through the digestive tract....

of blood.

Signs and Symptoms

Signs and symptoms of Eisenmenger syndrome include:

• Cyanosis, a blue tinge to the skin resulting from lack of oxygen
• High red blood cell count
• Swollen or clubbed finger tips (clubbing)
• Fainting, called syncope
• Heart failure
• Arrhythmia or irregular heart rhythms
• Bleeding disorders
• Coughing up blood
• Iron deficiency
• Kidney problems
• Stroke
• Gout, rare, due to increased uric acid resorption and production with impaired excretion
• Gallstones

Treatment

If the hole in the heart is identified before it causes pulmonary hypertension, it can normally be repaired through surgery preventing the disease. After pulmonary hypertension, a heart-lung transplant or a lung transplant with repair of the heart is the only cure.

Various medicines and therapies are used for treatment of the symptoms.

External links

• Mayo Clinic, "Detailed Description of Eisenmenger's Syndrome"
• Down's Heart Group, "Easily understood description of Eisenmenger's Syndrome and how it affects people with Down's Syndrome who have unoperated congenital heart defects."