Eisenmenger's syndrome
| Eisenmenger's syndrome | |
|---|---|
| Classification and 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 caused by an acyanotic heart defect causes increased flow through the pulmonary vasculature, causing pulmonary hypertension,[1][2] which in turn, causes increased pressures in the right side of the heart and reversal of the shunt into a right-to-left shunt. 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,[3] though successful delivery has been reported.[4]
Contents
Etymology
Eisenmenger's syndrome was so named[5] by Dr. Paul Wood after Dr. Victor Eisenmenger, who first described[6] the condition in 1897.[7]
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.[1]
Pathogenesis
The left side of the heart supplies blood 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 Erythrocytosis. 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.
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.
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.
People who have had Eisenmenger Syndrome
- Michael Kutcher
- fraternal twin of actor Ashton Kutcher had an emergency heart transplant in 1991 due to a septal defect. He is now 32 and lives in Iowa selling retirement plans.
- Sue Ainsworth Hillgren
- was 47 years old at the time she died in 1988 -- believed to be among the oldest survivors of Eisenmenger Syndrome. Most patients die in their second or third decade of life. Ainsworth Hillgren, who also lived in Iowa, was a respected decorative artist and had worked as a guidance counselor, until her declining health forced her to retire at age 38.
- Daniel Mulder
- was born with congenital heart disease, pulmnary hypertension, and eisenmengers' syndrome --- living in southwestern Chicago suburbs is currently doing very well at the age of 53. This posted 9/7/2010 by Mr. Mulder.
References
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<references />, or <references group="..." />Teresa Mudd- Currently lives in Louisville Ky and was diagnosed with Eisenmenger Syndrome at the age of 57.
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."
bg:Синдром на Айзенменгер de:Eisenmenger-Reaktion es:Síndrome de Eisenmenger fr:Syndrome d'Eisenmenger it:Sindrome di Eisenmenger nl:Syndroom van Eisenmenger ja:アイゼンメンゲル症候群 nn:Eisenmengers syndrom
pl:Zespół Eisenmengera- ↑ 1.0 1.1 Lua error in package.lua at line 80: module 'Module:Citation/CS1/Suggestions' not found.
- ↑ Eisenmenger syndrome at Dorland's Medical Dictionary
- ↑ Siddiqui S, Latif N (2008). "PGE1 nebulisation during caesarean section for Eisenmenger's syndrome: a case report". J Med Case Reports. 2: 149. doi:10.1186/1752-1947-2-149. PMC 2405798 Freely accessible. PMID 18466628.
- ↑ Lua error in package.lua at line 80: module 'Module:Citation/CS1/Suggestions' not found.
- ↑ Wood, P. Pulmonary hypertension with special reference to the vasoconstrictive factor. Br Heart J 1958;20:557-570. PMID 13584643
- ↑ Eisenmenger V. Die angeborenen Defekte der Kammerscheidewände des Herzens. The condition was first mentioned by Hippocrates, the Greek physician. Zeitschr Klin Med 1897;32(Supplement):1-28.
- ↑ synd/3034 at Who Named It?
