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Shone complex

MedGen UID:
501135
Concept ID:
C1868705
Disease or Syndrome
Synonyms: shone complex; Shone Syndrome; Shone syndrome; shone syndrome; shone's syndrome (greater than 3 sites)
SNOMED CT: Shone complex (41371000119100); Shone syndrome (41371000119100)
 
Monarch Initiative: MONDO:0020404
Orphanet: ORPHA99063

Definition

Shone complex is a rare congenital cardiac malformation characterized by a complex of four obstructive lesions of the left heart: supravalvular mitral membrane, parachute mitral valve, muscular or membranous subvalvular aortic stenosis and coarctation of aorta. Clinical manifestations include heart murmur, shortness of breath and increased load intolerance, left ventricular hypertrophy and dilatation of the left atrium. Partial forms, involving only two or three out of the four specific anomalies, are also described and occasionally other cardiovascular anomalies (e.g. bicuspid aortic valve, patent ductus arteriosus, ventricular septal defect) may be associated. [from ORDO]

Term Hierarchy

Professional guidelines

PubMed

Hopper RK, van der Have O, Hollander SA, Dipchand AI, Perez de Sa V, Feinstein JA, Tran-Lundmark K
Pediatr Transplant 2023 Mar;27(2):e14461. Epub 2023 Jan 2 doi: 10.1111/petr.14461. PMID: 36593638
Burrage MK, Lewis AJ, Miller JJJ
Cardiovasc Drugs Ther 2023 Apr;37(2):379-399. Epub 2022 Jul 26 doi: 10.1007/s10557-022-07355-7. PMID: 35881280Free PMC Article
Szilagyi PG, Schaffer S, Shone L, Barth R, Humiston SG, Sandler M, Rodewald LE
Pediatrics 2002 Nov;110(5):e58. doi: 10.1542/peds.110.5.e58. PMID: 12415064

Recent clinical studies

Etiology

Jain CC, Warnes CA, Egbe AC, Cetta F, DuBrock HM, Connolly HM, Miranda WR
Am J Cardiol 2020 Sep 1;130:137-142. Epub 2020 Jun 16 doi: 10.1016/j.amjcard.2020.06.024. PMID: 32703525
Vaikunth SS, Lui GK
Heart Fail Rev 2020 Jul;25(4):569-581. doi: 10.1007/s10741-019-09904-z. PMID: 31873841
Jin SC, Homsy J, Zaidi S, Lu Q, Morton S, DePalma SR, Zeng X, Qi H, Chang W, Sierant MC, Hung WC, Haider S, Zhang J, Knight J, Bjornson RD, Castaldi C, Tikhonoa IR, Bilguvar K, Mane SM, Sanders SJ, Mital S, Russell MW, Gaynor JW, Deanfield J, Giardini A, Porter GA Jr, Srivastava D, Lo CW, Shen Y, Watkins WS, Yandell M, Yost HJ, Tristani-Firouzi M, Newburger JW, Roberts AE, Kim R, Zhao H, Kaltman JR, Goldmuntz E, Chung WK, Seidman JG, Gelb BD, Seidman CE, Lifton RP, Brueckner M
Nat Genet 2017 Nov;49(11):1593-1601. Epub 2017 Oct 9 doi: 10.1038/ng.3970. PMID: 28991257Free PMC Article
Aslam S, Khairy P, Shohoudi A, Mercier LA, Dore A, Marcotte F, Miró J, Avila-Alonso P, Ibrahim R, Asgar A, Poirier N, Mongeon FP
Can J Cardiol 2017 Feb;33(2):253-259. Epub 2016 Sep 29 doi: 10.1016/j.cjca.2016.09.005. PMID: 27956040
St Louis JD, Bannan MM, Lutin WA, Wiles HB
Ann Thorac Surg 2007 Oct;84(4):1357-62; discussion 1362-3. doi: 10.1016/j.athoracsur.2007.05.003. PMID: 17888998

Diagnosis

Yang R, Greene CL
Semin Cardiothorac Vasc Anesth 2023 Dec;27(4):260-272. Epub 2023 Sep 26 doi: 10.1177/10892532231203372. PMID: 37750051
Kohli U, Jeevanandam V, Patel AR
Circ Cardiovasc Imaging 2021 Jun;14(6):e012317. Epub 2021 May 26 doi: 10.1161/CIRCIMAGING.120.012317. PMID: 34034510
Aslam S, Khairy P, Shohoudi A, Mercier LA, Dore A, Marcotte F, Miró J, Avila-Alonso P, Ibrahim R, Asgar A, Poirier N, Mongeon FP
Can J Cardiol 2017 Feb;33(2):253-259. Epub 2016 Sep 29 doi: 10.1016/j.cjca.2016.09.005. PMID: 27956040
Grimaldi A, Vermi AC, Ho SY, Pappalardo F, Castiglioni A, Benussi S, Zangrillo A, Alfieri O
Interact Cardiovasc Thorac Surg 2012 Apr;14(4):440-4. Epub 2012 Jan 25 doi: 10.1093/icvts/ivr169. PMID: 22279117Free PMC Article
St Louis JD, Bannan MM, Lutin WA, Wiles HB
Ann Thorac Surg 2007 Oct;84(4):1357-62; discussion 1362-3. doi: 10.1016/j.athoracsur.2007.05.003. PMID: 17888998

Therapy

Cleveland JD, Bansal N, Wells WJ, Wiggins LM, Kumar SR, Starnes VA
J Thorac Cardiovasc Surg 2023 Jan;165(1):262-272.e3. Epub 2022 Apr 25 doi: 10.1016/j.jtcvs.2022.04.015. PMID: 35599209
Schulz A, Buratto E, Wallace FRO, Fulkoski N, Weintraub RG, Brizard CP, Konstantinov IE
J Thorac Cardiovasc Surg 2022 Jul;164(1):186-196.e2. Epub 2022 Jan 26 doi: 10.1016/j.jtcvs.2022.01.022. PMID: 35227498
Latus H, Yerebakan C, Schranz D, Akintuerk H
J Thorac Cardiovasc Surg 2014 Nov;148(5):e226-8. Epub 2014 Jul 31 doi: 10.1016/j.jtcvs.2014.07.042. PMID: 25173115
Bartram U, Thul J, Bauer J, Wössmann W, Schranz D
J Heart Lung Transplant 2006 Oct;25(10):1263-7. Epub 2006 Sep 12 doi: 10.1016/j.healun.2006.06.011. PMID: 17045940

Prognosis

Cleveland JD, Bansal N, Wells WJ, Wiggins LM, Kumar SR, Starnes VA
J Thorac Cardiovasc Surg 2023 Jan;165(1):262-272.e3. Epub 2022 Apr 25 doi: 10.1016/j.jtcvs.2022.04.015. PMID: 35599209
Aslam S, Khairy P, Shohoudi A, Mercier LA, Dore A, Marcotte F, Miró J, Avila-Alonso P, Ibrahim R, Asgar A, Poirier N, Mongeon FP
Can J Cardiol 2017 Feb;33(2):253-259. Epub 2016 Sep 29 doi: 10.1016/j.cjca.2016.09.005. PMID: 27956040
Grimaldi A, Vermi AC, Ho SY, Pappalardo F, Castiglioni A, Benussi S, Zangrillo A, Alfieri O
Interact Cardiovasc Thorac Surg 2012 Apr;14(4):440-4. Epub 2012 Jan 25 doi: 10.1093/icvts/ivr169. PMID: 22279117Free PMC Article
Saroli T, Gelehrter S, Gomez-Fifer CA, van der Velde ME, Bove EL, Ensing GJ
Echocardiography 2008 Aug;25(7):727-31. Epub 2008 Apr 27 doi: 10.1111/j.1540-8175.2008.00685.x. PMID: 18445061
St Louis JD, Bannan MM, Lutin WA, Wiles HB
Ann Thorac Surg 2007 Oct;84(4):1357-62; discussion 1362-3. doi: 10.1016/j.athoracsur.2007.05.003. PMID: 17888998

Clinical prediction guides

Cleveland JD, Bansal N, Wells WJ, Wiggins LM, Kumar SR, Starnes VA
J Thorac Cardiovasc Surg 2023 Jan;165(1):262-272.e3. Epub 2022 Apr 25 doi: 10.1016/j.jtcvs.2022.04.015. PMID: 35599209
Digilio MC, Gnazzo M, Lepri F, Dentici ML, Pisaneschi E, Baban A, Passarelli C, Capolino R, Angioni A, Novelli A, Marino B, Dallapiccola B
Am J Med Genet A 2017 Nov;173(11):2912-2922. Epub 2017 Sep 8 doi: 10.1002/ajmg.a.38417. PMID: 28884922
Sivanandam S, Nyholm J, Wey A, Bass JL
Pediatr Cardiol 2015 Oct;36(7):1376-81. Epub 2015 Apr 8 doi: 10.1007/s00246-015-1168-7. PMID: 25851170
Grimaldi A, Vermi AC, Ho SY, Pappalardo F, Castiglioni A, Benussi S, Zangrillo A, Alfieri O
Interact Cardiovasc Thorac Surg 2012 Apr;14(4):440-4. Epub 2012 Jan 25 doi: 10.1093/icvts/ivr169. PMID: 22279117Free PMC Article

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