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Eisenmenger syndrome

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Eisenmenger syndrome
Other namesES, Eisenmenger's syndrome, Eisenmenger reaction, tardive cyanosis.
Heart with a ventricular septal defect showing
Eisenmenger Syndrome due to a ventricular septal defect
SpecialtyMedical genetics, cardiology Edit this on Wikidata
TreatmentSupportive measures, transplant surgery

Eisenmenger syndrome or Eisenmenger's syndrome is defined as the process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect (typically by a ventricular septal defect, atrial septal defect, or less commonly, patent ductus arteriosus) causes pulmonary hypertension[1][2] and eventual reversal of the shunt into a cyanotic right-to-left shunt. Because of the advent of fetal screening with echocardiography early in life, the incidence of heart defects progressing to Eisenmenger syndrome has decreased.

Eisenmenger syndrome in a pregnant mother can cause serious complications,[3] though successful delivery has been reported.[4] Maternal mortality ranges from 30% to 60%, and may be attributed to fainting spells, blood clots forming in the veins and traveling to distant sites, hypovolemia, coughing up blood or preeclampsia. Most deaths occur either during or within the first weeks after delivery.[5] Pregnant women with Eisenmenger syndrome should be hospitalized after the 20th week of pregnancy, or earlier if clinical deterioration occurs.

Signs and symptoms

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Nail clubbing of fingers in a patient with Eisenmenger syndrome. First described by Hippocrates, clubbing is also known as "Hippocratic fingers".

Signs and symptoms of Eisenmenger syndrome include the following:[6]

One of the most severe and common complications of Eisenmenger syndrome is cardiac arrhythmia, especially supraventricular arrhythmias. Approximately 40% of patients diagnosed with Eisenmenger syndrome were also found to have these arrhythmias during routine ECG screenings. These arrhythmias have worse prognosis in patients with Eisenmenger syndrome, compared to the general population, and can be a source of sudden cardiac death.[9]

Causes

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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]

Diagnosis

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Diagnosis of Eisenmenger syndrome is typically conducted via transthoracic echocardiography, which facilitates the identification and evaluation of shunts, anatomical defects, and ventricular function. Following diagnosis, or in some cases of inconclusive diagnosis, a cardiac catheter may be used to both confirm the diagnosis and to assess the patient's pulmonary arterial pressure, an important predictive value for prognosis and treatment.[9]

Treatment

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If the inciting defect in the heart is identified before it causes significant pulmonary hypertension, it can normally be repaired through surgery, preventing the disease.[10] After pulmonary hypertension is sufficient to reverse the blood flow through the defect, however, the maladaptation is considered irreversible, and a heart–lung transplant or a lung transplant with repair of the heart is the only curative option. Transplantation is the final therapeutic option and only for patients with poor prognosis and quality of life. Timing and appropriateness of transplantation remain difficult decisions.[5] 5-year and 10-year survival ranges between 70% and 80%, 50% and 70%, 30% and 50%, respectively.[11][12][13] Since the average life expectancy of patients after lung transplantation is as low as 30% at 5 years, patients with reasonable functional status related to Eisenmenger syndrome have improved survival with conservative medical care compared with transplantation.[14]

Various medicines and therapies for pulmonary hypertension are under investigation for treatment of the symptoms.[15]

Antiarrhythmic drugs are important for many patients with Eisenmenger syndrome, as evidence suggests that arrhythmia-induced sudden cardiac death may be the leading cause of death among patients with the disease. These therapies generally aim to restore and maintain sinus rhythm, but the specific interventions chosen will depend on the nature of the patient's arrhythmia.[9]

Etymology

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Eisenmenger syndrome was named[16] by Paul Wood after Victor Eisenmenger, who first described[17] the condition in 1897.[18]

References

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  1. ^ a b Jensen AS, Iversen K, Vejlstrup NG, Hansen PB, Søndergaard L (April 2009). "Eisenmenger syndrome". Ugeskrift for Laeger (in Danish). 171 (15): 1270–1275. 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 Rep. 2 (1): 149. doi:10.1186/1752-1947-2-149. PMC 2405798. PMID 18466628.
  4. ^ Makaryus AN, Forouzesh A, Johnson M (November 2006). "Pregnancy in the patient with Eisenmenger's syndrome". Mt. Sinai J. Med. 73 (7): 1033–1036. PMID 17195894. Archived from the original on 2009-03-05. Retrieved 2008-12-20.
  5. ^ a b Oechslin, Erwin; Mebus, Siegrun; Schulze-Neick, Ingram; Niwa, Koichiro; Trindade, Pedro T; Eicken, Andreas; Hager, Alfred; Lang, Irene; Hess, John; Kaemmerer, Harald (2010). "The Adult Patient with Eisenmenger Syndrome: A Medical Update after Dana Point Part III: Specific Management and Surgical Aspects". Current Cardiology Reviews. 6 (4): 363–372. doi:10.2174/157340310793566127. ISSN 1573-403X. PMC 3083818. PMID 22043213.
  6. ^ "Eisenmenger syndrome | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 2021-10-25.
  7. ^ Mann, Douglas L.; Zipes, Douglas P.; Libby, Peter; Bonow, Robert O.; Braunwald, Eugene (2015). Braunwald's Heart Disease. Philadelphia: Saunders. pp. 1617–1618. ISBN 978-0-323-29429-4.
  8. ^ Vongpatanasin, W.; Brickner, M.E.; Hillis, L.D.; Lange, R.A. (1998-05-01). "The Eisenmenger syndrome in adults". Annals of Internal Medicine. 128 (9): 745–755. doi:10.7326/0003-4819-128-9-199805010-00008. ISSN 0003-4819. PMID 9556469.
  9. ^ a b c Diller, Gerhard-Paul; Gatzoulis, Michael A. (2007-02-27). "Pulmonary Vascular Disease in Adults With Congenital Heart Disease". Circulation. 115 (8): 1039–1050. doi:10.1161/CIRCULATIONAHA.105.592386. ISSN 0009-7322. PMID 17325254. S2CID 10479313.
  10. ^ "Eisenmenger syndrome". NIH MedLine Plus. 2010-02-05.
  11. ^ Goerler, Heidi; Simon, Andre; Gohrbandt, Bernhard; Hagl, Christian; Oppelt, Petra; Weidemann, Juergen; Haverich, Axel; Strueber, Martin (2007). "Heart-lung and lung transplantation in grown-up congenital heart disease: long-term single centre experience". European Journal of Cardio-Thoracic Surgery. 32 (6): 926–931. doi:10.1016/j.ejcts.2007.08.024. ISSN 1010-7940. PMID 17900918.
  12. ^ Waddell, Thomas K.; Bennett, Leah; Kennedy, Renee; Todd, Thomas R.J.; Keshavjee, Shaf H. (2002). "Heart-lung or lung transplantation for Eisenmenger syndrome". The Journal of Heart and Lung Transplantation. 21 (7): 731–737. doi:10.1016/s1053-2498(01)00420-x. ISSN 1053-2498. PMID 12100899.
  13. ^ Stoica, S.C.; McNeil, K.D.; Perreas, K.; Sharples, L.D.; Satchithananda, D.K.; Tsui, S.S.; Large, S.R.; Wallwork, J. (2001). "Heart-lung transplantation for Eisenmenger syndrome: early and long-term results". The Annals of Thoracic Surgery. 72 (6): 1887–1891. doi:10.1016/s0003-4975(01)03099-5. ISSN 0003-4975. PMID 11789765.
  14. ^ Beghetti, Maurice; Galiè, Nazzareno (Mar 2009). "Eisenmenger Syndrome". Journal of the American College of Cardiology. 53 (9): 733–740. doi:10.1016/j.jacc.2008.11.025. PMID 19245962.
  15. ^ Penalver, Jorge L (2019-04-15). "Eisenmenger Syndrome Treatment & Management: Approach Considerations, Oxygen Therapy, Pulmonary Vasodilator Therapy". Medscape Reference. Retrieved 2024-08-10.
  16. ^ Wood, P (1958). "Pulmonary hypertension with special reference to the vasoconstrictive factor". British Heart Journal. 20 (4): 557–570. doi:10.1136/hrt.20.4.557. PMC 491807. PMID 13584643.
  17. ^ V, Eisenmenger (1897). "Die angeborenen Defekte der Kammerscheidewand des Herzens". Z Klin Med. 32: 1–28.
  18. ^ synd/3034 at Who Named It?
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