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Lutembacher syndrome

MedGen UID:
9825
•Concept ID:
C0024164
•
Disease or Syndrome
Synonyms: Lutembacher Syndrome; Lutembacher's Syndrome; Lutembachers Syndrome; Syndrome, Lutembacher; Syndrome, Lutembacher's
SNOMED CT: Lutembacher syndrome (204319006); Lutembacher's syndrome (204319006)
 
Monarch Initiative: MONDO:0006839

Definition

A condition characterized by a combination of ostium secundum atrial septal defect and an acquired mitral valve stenosis. [from MONDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLutembacher syndrome

Professional guidelines

PubMed

Lutembacher syndrome: an ideal combination for percutaneous treatment.
Cheng TO
Chin Med J (Engl) 2006 Feb 20;119(4):351-2. PMID: 16537037

Recent clinical studies

Etiology

Does patent foramen ovale presence procure favourable outcomes in patients with severe rheumatic mitral stenosis?
Babur Guler G, Dogan AC, Kalkan AK, Demir AR, Uygur B, Birant A, Zencirkiran Agus H, Pastore MC, Memiç K, Uzun F, Cameli M, Erturk M
Int J Cardiovasc Imaging 2021 Oct;37(10):2871-2879. Epub 2021 Jun 9 doi: 10.1007/s10554-021-02257-5. PMID: 34109513
Lutembacher's syndrome: Is the mitral pathology always rheumatic?
Vaideeswar P, Marathe S
Indian Heart J 2017 Jan-Feb;69(1):20-23. Epub 2016 Jul 9 doi: 10.1016/j.ihj.2016.07.003. PMID: 28228300Free PMC Article
Successful percutaneous management of Lutembacher syndrome.
Goel S, Nath R, Sharma A, Pandit N, Wardhan H
Indian Heart J 2014 May-Jun;66(3):355-7. Epub 2014 May 15 doi: 10.1016/j.ihj.2014.03.016. PMID: 24973844Free PMC Article
Clinical efficiency and safety analysis of transcatheter interventional therapy for compound congenital cardiovascular abnormalities.
Song ZY, Shu MQ, Hu HY, Tong SF, Ran BL, Liu JP, Li YH, He GX
Clin Cardiol 2007 Oct;30(10):518-21. doi: 10.1002/clc.20149. PMID: 17929282Free PMC Article
Transcatheter treatment of Lutembacher syndrome: combined balloon mitral valvuloplasty and percutaneous atrial septal defect closure.
Ledesma M, Martinez P, Cázares MA, Feldman T
J Invasive Cardiol 2004 Nov;16(11):678-9. PMID: 15550747

Diagnosis

Lutembacher syndrome presenting as heart failure in a young Nigerian at Obafemi Awolowo University Teaching Hospitals Complex (OAUTHC), Ile-Ife: a case report.
Ojo OE, Akintomide AO, Adebayo RA, Akinyele OA, Akhionbare IG, Williams OT, Oguntola BO, Adesanya OS, Obasanjo AL
Pan Afr Med J 2022;41:342. Epub 2022 Apr 28 doi: 10.11604/pamj.2022.41.342.29903. PMID: 35909428Free PMC Article
Non-surgical treatment of Lutembacher syndrome: combined percutaneous transcatheter therapy.
Johny D, Subramanyam K, Bhandary RM, Rao A
BMJ Case Rep 2022 May 18;15(5) doi: 10.1136/bcr-2021-247984. PMID: 35584855Free PMC Article
Lutembacher syndrome with mitral valve calcification in a 31-year old male.
Adam AM, Godil A, Ali Mallick MS, Khan F, Rizvi AH, Makhdoom IM
J Pak Med Assoc 2018 Feb;68(2):340-342. PMID: 29786701
Lutembacher's syndrome: Is the mitral pathology always rheumatic?
Vaideeswar P, Marathe S
Indian Heart J 2017 Jan-Feb;69(1):20-23. Epub 2016 Jul 9 doi: 10.1016/j.ihj.2016.07.003. PMID: 28228300Free PMC Article
Lutembacher's Syndrome.
Bari MA, Haque MS, Uddin SN, Shamsuzzaman M, Khan GK, Sutradhar SR
Mymensingh Med J 2005 Jul;14(2):206-8. PMID: 16056214

Therapy

Lutembacher syndrome with mitral valve calcification in a 31-year old male.
Adam AM, Godil A, Ali Mallick MS, Khan F, Rizvi AH, Makhdoom IM
J Pak Med Assoc 2018 Feb;68(2):340-342. PMID: 29786701
Iatrogenic Lutembacher Syndrome after Percutaneous Mitral Commissurotomy.
Karamitsos TD, Nikolaidou C, Koutsakis A, Anastasiadis K, Karvounis C, Hadjimiltiades S
J Heart Valve Dis 2017 May;26(3):368-371. PMID: 29092127
Concomitant tuberculous meningitis and Lutembacher syndrome with multiple atrial septal defects and infective endocarditis.
Firdous S
J Coll Physicians Surg Pak 2012 Oct;22(10):666-8. doi: 10.2012/JCPSP.598600. PMID: 23058155
Clinical efficiency and safety analysis of transcatheter interventional therapy for compound congenital cardiovascular abnormalities.
Song ZY, Shu MQ, Hu HY, Tong SF, Ran BL, Liu JP, Li YH, He GX
Clin Cardiol 2007 Oct;30(10):518-21. doi: 10.1002/clc.20149. PMID: 17929282Free PMC Article
Giant left atrium secondary to tight mitral stenosis leading to acquired Lutembacher syndrome: a case report with emphasis on role of echocardiography in assessment of Lutembacher syndrome.
Ananthasubramaniam K, Iyer G, Karthikeyan V
J Am Soc Echocardiogr 2001 Oct;14(10):1033-5. doi: 10.1067/mje.2001.111265. PMID: 11593210

Prognosis

Does patent foramen ovale presence procure favourable outcomes in patients with severe rheumatic mitral stenosis?
Babur Guler G, Dogan AC, Kalkan AK, Demir AR, Uygur B, Birant A, Zencirkiran Agus H, Pastore MC, Memiç K, Uzun F, Cameli M, Erturk M
Int J Cardiovasc Imaging 2021 Oct;37(10):2871-2879. Epub 2021 Jun 9 doi: 10.1007/s10554-021-02257-5. PMID: 34109513
Transcatheter treatment of Lutembacher syndrome: combined balloon mitral valvuloplasty and percutaneous atrial septal defect closure.
Ledesma M, Martinez P, Cázares MA, Feldman T
J Invasive Cardiol 2004 Nov;16(11):678-9. PMID: 15550747
Cyanosis and clubbing in a patient with iatrogenic Lutembacher syndrome.
Essop MR, Essop AR, Bedhesi S, Sareli PE
Eur Heart J 1995 Mar;16(3):421-3. doi: 10.1093/oxfordjournals.eurheartj.a060927. PMID: 7789387
Long-term follow-up of cardiac rhythms after biatrial transseptal approach (Dubost's incision).
Kimura M, Kitasato K, Kamatani M, Fujino T
Fukuoka Igaku Zasshi 1992 Feb;83(2):57-61. PMID: 1592335
Value and limitations of Doppler echocardiographic determination of mitral valve area in Lutembacher syndrome.
Vasan RS, Shrivastava S, Kumar MV
J Am Coll Cardiol 1992 Nov 15;20(6):1362-70. doi: 10.1016/0735-1097(92)90249-m. PMID: 1430687

Clinical prediction guides

Does patent foramen ovale presence procure favourable outcomes in patients with severe rheumatic mitral stenosis?
Babur Guler G, Dogan AC, Kalkan AK, Demir AR, Uygur B, Birant A, Zencirkiran Agus H, Pastore MC, Memiç K, Uzun F, Cameli M, Erturk M
Int J Cardiovasc Imaging 2021 Oct;37(10):2871-2879. Epub 2021 Jun 9 doi: 10.1007/s10554-021-02257-5. PMID: 34109513
Earliest description by Johann Friedrich Meckel, Senior (1750) of what is known today as Lutembacher syndrome (1916).
Wiedemann HR
Am J Med Genet 1994 Oct 15;53(1):59-64. doi: 10.1002/ajmg.1320530113. PMID: 7802038
Long-term follow-up of cardiac rhythms after biatrial transseptal approach (Dubost's incision).
Kimura M, Kitasato K, Kamatani M, Fujino T
Fukuoka Igaku Zasshi 1992 Feb;83(2):57-61. PMID: 1592335
Value and limitations of Doppler echocardiographic determination of mitral valve area in Lutembacher syndrome.
Vasan RS, Shrivastava S, Kumar MV
J Am Coll Cardiol 1992 Nov 15;20(6):1362-70. doi: 10.1016/0735-1097(92)90249-m. PMID: 1430687
Pseudo-Wolff-Parkinson-White syndrome observed in a patient with Lutembacher's syndrome.
Ishizawa K, Sakurai T, Nagai M, Hirose K, Wakabayashi A
Jpn Heart J 1975 Mar;16(2):204-10. doi: 10.1536/ihj.16.204. PMID: 1117593

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