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cleft mitral valve

MedGen UID:
473080
•Concept ID:
C0344772
•
Congenital Abnormality; Disease or Syndrome
Synonym: Cleft mitral valve
SNOMED CT: Cleft leaflet of mitral valve (78495000); True cleft of mitral leaflet (78495000)
 
Monarch Initiative: MONDO:0019818
Orphanet: ORPHA95465

Definition

A rare, congenital, non-syndromic heart malformation characterized by a slit-like hole or defect in one of the mitral valve leaflets, which is usually thickened and distorted. It usually affects the anterior leaflet, but the cleft of posterior leaflet has also been described. Cleft mitral valve can be isolated or associated with other congenital heart anomalies. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVcleft mitral valve

Professional guidelines

PubMed

Clinical features and management of isolated cleft mitral valve in childhood.
Tamura M, Menahem S, Brizard C
J Am Coll Cardiol 2000 Mar 1;35(3):764-70. doi: 10.1016/s0735-1097(99)00588-4. PMID: 10716481

Recent clinical studies

Etiology

Balloon Valvuloplasty for Congenital Aortic Stenosis: Experience at a Tertiary Center in a Developing Country.
Charafeddine FA, Houssein HB, Kibbi NB, El-Rassi IM, Tabbakh AM, Abutaqa MS, Bulbul ZF, Younis NK, Arabi MT, Bitar FF
J Interv Cardiol 2021;2021:6681693. Epub 2021 Jan 12 doi: 10.1155/2021/6681693. PMID: 33519306Free PMC Article
Diagnosis of Isolated Cleft Mitral Valve Using Three-Dimensional Echocardiography.
Narang A, Addetia K, Weinert L, Yamat M, Shah AP, Blair JE, Mor-Avi V, Lang RM
J Am Soc Echocardiogr 2018 Nov;31(11):1161-1167. Epub 2018 Aug 7 doi: 10.1016/j.echo.2018.06.008. PMID: 30097300Free PMC Article
Isolated Mitral Cleft in Trisomy 21: An Initially 'Silent' Lesion.
Thankavel PP, Ramaciotti C
Pediatr Cardiol 2016 Feb;37(2):405-8. Epub 2015 Oct 20 doi: 10.1007/s00246-015-1291-5. PMID: 26481224
Effectiveness of mild therapeutic hypothermia following cardiac arrest in adult patients with congenital heart disease.
Young MN, Hollenbeck RD, Pollock JS, McPherson JA, Fredi JL, Piana RN, Mah ML, Fish FA, Markham L
Am J Cardiol 2014 Jul 1;114(1):128-30. Epub 2014 Apr 18 doi: 10.1016/j.amjcard.2014.04.012. PMID: 24819894
Characteristics and management of cleft mitral valve.
Fraisse A, Massih TA, Kreitmann B, Metras D, Vouhé P, Sidi D, Bonnet D
J Am Coll Cardiol 2003 Dec 3;42(11):1988-93. doi: 10.1016/j.jacc.2003.07.019. PMID: 14662264

Diagnosis

Cleft posterior mitral valve leaflet: The added value of 3-dimensional echocardiography.
Rizk J, Abdel-Hay MA
Echocardiography 2022 Feb;39(2):404-406. Epub 2022 Jan 11 doi: 10.1111/echo.15273. PMID: 35014733
Diagnosis of Isolated Cleft Mitral Valve Using Three-Dimensional Echocardiography.
Narang A, Addetia K, Weinert L, Yamat M, Shah AP, Blair JE, Mor-Avi V, Lang RM
J Am Soc Echocardiogr 2018 Nov;31(11):1161-1167. Epub 2018 Aug 7 doi: 10.1016/j.echo.2018.06.008. PMID: 30097300Free PMC Article
Isolated cleft of both the anterior and posterior mitral valve leaflets.
Aksu HU, Aslan M, Ozturk D, Yalcin AA, Yıldırım A
Echocardiography 2016 Nov;33(11):1793-1794. Epub 2016 Aug 22 doi: 10.1111/echo.13309. PMID: 27546525
Congenital discrete subaortic stenosis in pregnancy: case report and literature review.
Guglin M, Kristof-Kuteyeva O
J Heart Valve Dis 2013 May;22(3):439-44. PMID: 24151775
Characteristics and management of cleft mitral valve.
Fraisse A, Massih TA, Kreitmann B, Metras D, Vouhé P, Sidi D, Bonnet D
J Am Coll Cardiol 2003 Dec 3;42(11):1988-93. doi: 10.1016/j.jacc.2003.07.019. PMID: 14662264

Therapy

Balloon Valvuloplasty for Congenital Aortic Stenosis: Experience at a Tertiary Center in a Developing Country.
Charafeddine FA, Houssein HB, Kibbi NB, El-Rassi IM, Tabbakh AM, Abutaqa MS, Bulbul ZF, Younis NK, Arabi MT, Bitar FF
J Interv Cardiol 2021;2021:6681693. Epub 2021 Jan 12 doi: 10.1155/2021/6681693. PMID: 33519306Free PMC Article
Mitral valve with a single leaflet.
Bezgin T, Elveran A, Karagöz A, Çanga Y, Yılmaz F
Turk Kardiyol Dern Ars 2014 Jan;42(1):80-2. doi: 10.5543/tkda.2014.44380. PMID: 24481102
Isolated cleft in the posterior mitral valve leaflet: a congenital form of mitral regurgitation.
Wyss CA, Enseleit F, van der Loo B, Grünenfelder J, Oechslin EN, Jenni R
Clin Cardiol 2009 Oct;32(10):553-60. doi: 10.1002/clc.20608. PMID: 19911346Free PMC Article
Severe hemolytic anemia after repair of primum septal defect and cleft mitral valve.
Alehan D, Doğan R, Ozkutlu S, Elshershari H, Gümrük F
Turk J Pediatr 2001 Oct-Dec;43(4):329-31. PMID: 11765164

Prognosis

Effectiveness of mild therapeutic hypothermia following cardiac arrest in adult patients with congenital heart disease.
Young MN, Hollenbeck RD, Pollock JS, McPherson JA, Fredi JL, Piana RN, Mah ML, Fish FA, Markham L
Am J Cardiol 2014 Jul 1;114(1):128-30. Epub 2014 Apr 18 doi: 10.1016/j.amjcard.2014.04.012. PMID: 24819894
Congenital discrete subaortic stenosis in pregnancy: case report and literature review.
Guglin M, Kristof-Kuteyeva O
J Heart Valve Dis 2013 May;22(3):439-44. PMID: 24151775
Abnormal mitral valve anatomy in d-transposition of the great arteries: anatomic characterization and surgical outcomes.
Camarda JA, Harris SE, Hambrook J, Frommelt MA, Tweddell JS, Frommelt PC
Pediatr Cardiol 2013 Jan;34(1):70-4. Epub 2012 Jun 4 doi: 10.1007/s00246-012-0388-3. PMID: 22660521
Isolated cleft posterior mitral valve leaflet: an uncommon cause of mitral regurgitation.
Amin A, Davis M, Auseon A
Eur J Echocardiogr 2009 Jan;10(1):173-4. Epub 2008 Jul 29 doi: 10.1093/ejechocard/jen212. PMID: 18664488
Characteristics and management of cleft mitral valve.
Fraisse A, Massih TA, Kreitmann B, Metras D, Vouhé P, Sidi D, Bonnet D
J Am Coll Cardiol 2003 Dec 3;42(11):1988-93. doi: 10.1016/j.jacc.2003.07.019. PMID: 14662264

Clinical prediction guides

Effectiveness of mild therapeutic hypothermia following cardiac arrest in adult patients with congenital heart disease.
Young MN, Hollenbeck RD, Pollock JS, McPherson JA, Fredi JL, Piana RN, Mah ML, Fish FA, Markham L
Am J Cardiol 2014 Jul 1;114(1):128-30. Epub 2014 Apr 18 doi: 10.1016/j.amjcard.2014.04.012. PMID: 24819894
Atrioventricular canal defect in patients with RASopathies.
Digilio MC, Romana Lepri F, Dentici ML, Henderson A, Baban A, Roberti MC, Capolino R, Versacci P, Surace C, Angioni A, Tartaglia M, Marino B, Dallapiccola B
Eur J Hum Genet 2013 Feb;21(2):200-4. Epub 2012 Jul 11 doi: 10.1038/ejhg.2012.145. PMID: 22781091Free PMC Article
Factors predicting the progress of mitral valve disease in surgically treated adults with ostium primum atrial septal defects.
Agarwal V, Aggarwal SK, Voleti CD
J Thorac Cardiovasc Surg 2009 Mar;137(3):543-7. doi: 10.1016/j.jtcvs.2008.08.045. PMID: 19258062
Characteristics and management of cleft mitral valve.
Fraisse A, Massih TA, Kreitmann B, Metras D, Vouhé P, Sidi D, Bonnet D
J Am Coll Cardiol 2003 Dec 3;42(11):1988-93. doi: 10.1016/j.jacc.2003.07.019. PMID: 14662264
M mode and two dimensional echocardiography in mitral and aortic regurgitation: pre- and postoperative evaluation of volume overload of the left ventricle.
Kotler MN, Mintz GS, Parry WR, Segal BL
Am J Cardiol 1980 Dec 18;46(7):1144-52. doi: 10.1016/0002-9149(80)90285-4. PMID: 7192930

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