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Encyclopedia > Eisenmenger's syndrome
Eisenmenger's syndrome
Classification & external resources
ICD-10 Q21.8
ICD-9 745.4
DiseasesDB 4143
eMedicine med/642 
MeSH D004541

Eisenmenger's syndrome or Eisenmenger's reaction is defined as the process in which a left-to-right shunt in the heart causes increased flow through the pulmonary vasculature, causing pulmonary hypertension, which in turn, causes increased pressures in the right side of the heart and reversal of the shunt into a right-to-left shunt. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // Q00-Q99 - Congenital malformations, deformations and chromosomal abnormalities (Q00-Q07) Congenital malformations of the nervous system (Q00) Anencephaly and similar malformations (Q01) Encephalocele (Q02) Microcephaly (Q03) Congenital hydrocephalus (Q04) Other congenital malformations of brain (Q05) Spina bifida (Q06) Other congenital malformations of spinal cord (Q07) Other congenital malformations of nervous... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... Human circulatory system. ... In medicine, pulmonary hypertension (PH) is an increase in blood pressure in the pulmonary artery or lung vasculature, leading to shortness of breath, dizziness, fainting, and other symptoms, all of which are exacerbated by exertion. ...

Contents

Diagnosis

Conditions needed for a person to be diagnosed with Eisenmenger's Syndrome are:

  1. an underlying heart defect that allows blood to pass between the left and right sides of the heart.
  2. pulmonary hypertension, or elevated blood pressure in the lungs
  3. polycythemia, an increase in the number of red blood cells
  4. the reversal of the shunt

Polycythemia is a condition in which there is a net increase in the total number of red blood cells in the body. ...

Etymology

Eisenmenger's syndrome was so named[1] by Dr. Paul Wood after Dr. Victor Eisenmenger, who first described[2] the condition in 1897. An eponym is the name of a person, whether real or fictitious, who has (or is thought to have) given rise to the name of a particular place, tribe, discovery, or other item. ... 1897 (MDCCCXCVII) was a common year starting on Friday (see link for calendar). ...


Etiology

A number of congenital heart defects can cause Eisenmenger's syndrome, including atrial septal defects, ventricular septal defects, patent ductus arteriosus, and more complex types of acyanotic heart disease. Atrial septal defects (ASD) are a group of congenital heart diseases that enables communication between atria of the heart and may involve the interatrial septum. ... A ventricular septal defect (or VSD) is a defect in the ventricular septum (the wall dividing the left and right ventricles of the heart). ... Patent ductus arteriosus (PDA) is a congenital heart defect wherein a childs ductus arteriosus fails to close after birth. ... Heart disease is an umbrella term for a number of different diseases which affect the heart and is the leading cause of death in the United States as of 2007. ...


Pathogenesis

The left side of the heart supplies to the whole body, and as a result has higher pressures than the right side, which supplies only deoxygenated blood to the lungs. If a large anatomic defect exists between the 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.


The reduction in oxygen transfer reduces oxygen saturation 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 polycythemia. 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. Oxygen saturation is a relative measure of the amount of oxygen that is dissolved or carried in a given medium. ... Polycythemia is a condition in which there is a net increase in the total number of red blood cells in the body. ... Hyperviscosity syndrome is an increase in the viscosity of the blood. ...


A person with Eisenmenger's 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 of blood. The rough places in the heart lining at the site of the septal defects/shunts tend to gather platelets and keep them out of circulation, and may be the source of random clots. The term stasis has several meanings: A state of stabilty, in which all forces are equal and opposing, therefore they cancel out each other. ...


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 and resultant organ damage. Cyanosis refers to the bluish coloration of the skin due to the presence of deoxygenated hemoglobin in blood vessels near the skin surface. ...


Treatment

In early childhood, surgical intervention can repair the heart defect, preventing most of the pathogenesis of Eisenmenger's syndrome. If treatment has not taken place, heart-lung transplant is required to fully treat the syndrome. If this option is not available, treatment is mostly palliative, using anticoagulants, pulmonary vasodilators such as bosentan, antibiotic prophylaxis to prevent endocarditis, phlebotomy to treat polycythemia, and maintaining proper fluid balance. These measures can prolong lifespan and improve quality of life. Palliative care is any form of medical care or treatment that concentrates on reducing the severity of the symptoms of a disease or slows its progress rather than providing a cure. ... Bosentan is a dual endothelin receptor antagonist important in the treatment of pulmonary artery hypertension (PAH). ... Prophylaxis refers to any medical or public health procedure whose purpose is to prevent, rather than treat or cure, disease. ... Ancient Greek painting in a vase, showing a physician (iatros) bleeding a patient. ...


References

  1. ^ Wood, P. Pulmonary hypertension with special reference to the vasoconstrictive factor. Br Heart J 1958;20:557-570. PMID 13584643
  2. ^ Eisenmenger V. Die angeborenen Defekte der Kammerscheidewände des Herzens. Zeitschr Klin Med 1897;32(Supplement):1-28.

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."

 
 

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