Eisenmenger's syndrome

Eisenmenger syndrome
Classification and external resources
ICD-10	Q21.8
ICD-9	745.4 (CDC/BPA 745.410)
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 a congenital 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.

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.

Clubbing of fingers in a patient with Eisenmenger's syndrome; first described by Hippocrates, clubbing is also known as "Hippocratic fingers"

It can cause serious complications in pregnancy,^[3] though successful delivery has been reported.^[4]

Etymology

Eisenmenger 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 syndrome, including atrial septal defects, ventricular septal defects, patent ductus arteriosus, and more complex types of acyanotic heart disease.^[1]

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 and resultant organ damage.

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 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.

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 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 ^[8]
• 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. ^[9] 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. ^[10]

References

1. ^ ^{a b} Jensen AS, Iversen K, Vejlstrup NG, Hansen PB, Søndergaard L (April 2009). "[Eisenmenger syndrome]" (in Danish). Ugeskrift for Laeger 171 (15): 1270–5. PMID 19416617. 
2. ^ "Eisenmenger syndrome" at Dorland's Medical Dictionary
3. ^ Siddiqui S, Latif N (2008). "PGE1 nebulisation during caesarean section for Eisenmenger's syndrome: a case report". J Med Case Reports 2 (1): 149. doi:10.1186/1752-1947-2-149. PMC 2405798. PMID 18466628. http://www.jmedicalcasereports.com/content/2//149. 
4. ^ Makaryus AN, Forouzesh A, Johnson M (November 2006). "Pregnancy in the patient with Eisenmenger's syndrome". Mt. Sinai J. Med. 73 (7): 1033–6. PMID 17195894. http://www.mssm.edu/msjournal/73/7371033.shtml. 
5. ^ Wood, P (1958). "Pulmonary hypertension with special reference to the vasoconstrictive factor.". British heart journal 20 (4): 557–70. doi:10.1136/hrt.20.4.557. PMC 491807. PMID 13584643. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=491807. 
6. ^ 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.
7. ^ synd/3034 at Who Named It?
8. ^ Braunwald E. Heart Disease: A Textbook of Cardiovascular Medicine. P 1617-1618. Ann Intern Med 1998; 128:745-755
9. ^ "Eisenmenger syndrome". NIH MedLine Plus. 2010-02-5. http://www.nlm.nih.gov/medlineplus/ency/article/007317.htm. 
10. ^ "Eisenmenger Syndrome Treatment & Management". MedScape Reference. 2008-06-5. http://emedicine.medscape.com/article/154555-treatment. 

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."

• 00529 at CHORUS