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Cardiac valvular dysplasia 2(CVDP2)

MedGen UID:
1823999
Concept ID:
C5774226
Disease or Syndrome
Synonym: CVDP2
 
Gene (location): ADAMTS19 (5q23.3)
 
Monarch Initiative: MONDO:0859572
OMIM®: 620067

Definition

Cardiac valvular dysplasia-2 (CVDP2) is characterized primarily by congenital stenosis and insufficiency of the semilunar valves, although mild insufficiency of the atrioventricular valves has been observed as well. Other features include subaortic stenosis and dilation of the ascending aorta and/or pulmonary artery in some patients (Wunnemann et al., 2020; Massadeh et al., 2020). For a discussion of genetic heterogeneity of CVDP, see CVDP1 (212093). [from OMIM]

Clinical features

From HPO
Chest pain
MedGen UID:
2992
Concept ID:
C0008031
Sign or Symptom
An unpleasant sensation characterized by physical discomfort (such as pricking, throbbing, or aching) localized to the chest.
Aortic regurgitation
MedGen UID:
8153
Concept ID:
C0003504
Disease or Syndrome
An insufficiency of the aortic valve, leading to regurgitation (backward flow) of blood from the aorta into the left ventricle.
Heart murmur
MedGen UID:
9171
Concept ID:
C0018808
Finding
An extra or unusual sound heard during a heartbeat caused vibrations resulting from the flow of blood through the heart.
Palpitations
MedGen UID:
14579
Concept ID:
C0030252
Finding
A sensation that the heart is pounding or racing, which is a non-specific sign but may be a manifestation of arrhythmia.
Pulmonary valve insufficiency
MedGen UID:
11031
Concept ID:
C0034088
Pathologic Function
The retrograde (backwards) flow of blood through the pulmonary valve into the right ventricle during diastole.
Tricuspid regurgitation
MedGen UID:
11911
Concept ID:
C0040961
Disease or Syndrome
Failure of the tricuspid valve to close sufficiently upon contraction of the right ventricle, causing blood to regurgitate (flow backward) into the right atrium.
Bicuspid aortic valve
MedGen UID:
57436
Concept ID:
C0149630
Congenital Abnormality
Aortic valve disease-2 (AOVD2) is characterized by bicuspid aortic valve (BAV) and dilation of the ascending aorta. Calcification of the valve and the aorta has been observed, and some patients exhibit coarctation of the aorta (Tan et al., 2012; Luyckx et al., 2019; Park et al., 2019). For a general phenotypic description and a discussion of genetic heterogeneity of aortic valve disease, see AOVD1 (109730).
Systolic heart murmur
MedGen UID:
115909
Concept ID:
C0232257
Finding
A heart murmur limited to systole, i.e., between the first and second heart sounds S1 and S2.
Subvalvular aortic stenosis
MedGen UID:
90950
Concept ID:
C0340375
Disease or Syndrome
A fixed form of obstruction to blood flow across the left-ventricular outflow tract related to stenosis (narrowing) below the level of the aortic valve.
Pulmonary artery dilatation
MedGen UID:
140900
Concept ID:
C0428851
Disease or Syndrome
An abnormal widening of the diameter of the pulmonary artery.
Ascending tubular aorta aneurysm
MedGen UID:
163631
Concept ID:
C0856747
Anatomical Abnormality
An abnormal localized widening (dilatation) of the tubular part of the ascending aorta.
Left ventricular diastolic dysfunction
MedGen UID:
696562
Concept ID:
C1273070
Disease or Syndrome
Abnormal function of the left ventricule during left ventricular relaxation and filling.
Dysplastic aortic valve
MedGen UID:
355656
Concept ID:
C1866207
Finding
A congenital malformation of the aortic valve characterized by leaflet deformation.
Pulmonic stenosis
MedGen UID:
408291
Concept ID:
C1956257
Disease or Syndrome
A narrowing of the right ventricular outflow tract that can occur at the pulmonary valve (valvular stenosis), below the pulmonary valve (infundibular stenosis), or above the pulmonary valve (supravalvar stenosis).
Increased left ventricular end-diastolic volume
MedGen UID:
1660169
Concept ID:
C4748648
Finding
Abnormally high volume of blood in the left ventricle at the end of diastole (just before systole).
Central cyanosis
MedGen UID:
636837
Concept ID:
C0521800
Finding
Generalized bluish discoloration of the body and the visible mucous membranes, which occurs due to inadequate oxygenation secondary to conditions that lead to an increase in deoxygenated hemoglobin or presence of abnormal hemoglobin.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Professional guidelines

PubMed

Leoni C, Blandino R, Delogu AB, De Rosa G, Onesimo R, Verusio V, Marino MV, Lanza GA, Rigante D, Tartaglia M, Zampino G
Am J Med Genet A 2022 Feb;188(2):431-445. Epub 2021 Oct 13 doi: 10.1002/ajmg.a.62529. PMID: 34643321
Latchford AR, Neale K, Phillips RK, Clark SK
Dis Colon Rectum 2012 Oct;55(10):1038-43. doi: 10.1097/DCR.0b013e31826278b3. PMID: 22965402
Allali S, Le Goff C, Pressac-Diebold I, Pfennig G, Mahaut C, Dagoneau N, Alanay Y, Brady AF, Crow YJ, Devriendt K, Drouin-Garraud V, Flori E, Geneviève D, Hennekam RC, Hurst J, Krakow D, Le Merrer M, Lichtenbelt KD, Lynch SA, Lyonnet S, MacDermot K, Mansour S, Megarbané A, Santos HG, Splitt M, Superti-Furga A, Unger S, Williams D, Munnich A, Cormier-Daire V
J Med Genet 2011 Jun;48(6):417-21. Epub 2011 Mar 17 doi: 10.1136/jmg.2010.087544. PMID: 21415077Free PMC Article

Recent clinical studies

Etiology

Leoni C, Blandino R, Delogu AB, De Rosa G, Onesimo R, Verusio V, Marino MV, Lanza GA, Rigante D, Tartaglia M, Zampino G
Am J Med Genet A 2022 Feb;188(2):431-445. Epub 2021 Oct 13 doi: 10.1002/ajmg.a.62529. PMID: 34643321
Bouhlel I, Ajmi H, Slim M, Gribaa R, Naffati E
Tunis Med 2021 Feb;99(2):291-297. PMID: 33899201Free PMC Article
Di Mauro M, Bezante GP, Di Baldassarre A, Clemente D, Cardinali A, Acitelli A, Salerni S, Penco M, Calafiore AM, Gallina S; Italian Study Group on Valvular Heart Disease Italian Society of Cardiology
Int J Cardiol 2013 Sep 30;168(2):707-15. Epub 2013 May 3 doi: 10.1016/j.ijcard.2013.04.043. PMID: 23647591
Bradić Z, Ivanović B, Marković D, Simić D, Janković R, Kalezić N
Acta Chir Iugosl 2011;58(2):39-43. doi: 10.2298/aci1102039b. PMID: 21879649
Fauchier JP, Fauchier L, Babuty D, Cosnay P
Pacing Clin Electrophysiol 1996 Feb;19(2):231-44. doi: 10.1111/j.1540-8159.1996.tb03315.x. PMID: 8834693

Diagnosis

Shanmugam S, Sennaiyan UN, Krishna MR
Fetal Pediatr Pathol 2022 Jun;41(3):529-532. Epub 2020 Oct 16 doi: 10.1080/15513815.2020.1831665. PMID: 33063582
Bouhlel I, Ajmi H, Slim M, Gribaa R, Naffati E
Tunis Med 2021 Feb;99(2):291-297. PMID: 33899201Free PMC Article
Matalon DR, Stevenson DA, Bhoj EJ, Santani AB, Keena B, Cohen MS, Lin AE, Sheppard SE, Zackai EH
Am J Med Genet A 2021 May;185(5):1486-1493. Epub 2021 Mar 8 doi: 10.1002/ajmg.a.62146. PMID: 33683002Free PMC Article
Di Mauro M, Bezante GP, Di Baldassarre A, Clemente D, Cardinali A, Acitelli A, Salerni S, Penco M, Calafiore AM, Gallina S; Italian Study Group on Valvular Heart Disease Italian Society of Cardiology
Int J Cardiol 2013 Sep 30;168(2):707-15. Epub 2013 May 3 doi: 10.1016/j.ijcard.2013.04.043. PMID: 23647591
Bradić Z, Ivanović B, Marković D, Simić D, Janković R, Kalezić N
Acta Chir Iugosl 2011;58(2):39-43. doi: 10.2298/aci1102039b. PMID: 21879649

Therapy

Okubo K, Gigli L, Trevisi N, Foppoli L, Radinovic A, Bisceglia C, Frontera A, D'Angelo G, Cireddu M, Paglino G, Mazzone P, Della Bella P
Circ Arrhythm Electrophysiol 2020 Aug;13(8):e008307. Epub 2020 Jul 12 doi: 10.1161/CIRCEP.119.008307. PMID: 32657137
Morrical BD, Dearani JA, Cabalka AK
Catheter Cardiovasc Interv 2019 Feb 1;93(2):E101-E104. Epub 2018 Oct 31 doi: 10.1002/ccd.27683. PMID: 30380204
Zhang Y, Cai AL, Ren WD, Guo YJ, Zhang DY, Sun W, Wang Y, Wang L, Qin Y, Huang LP
BMC Pregnancy Childbirth 2016 Jun 30;16:145. doi: 10.1186/s12884-016-0933-9. PMID: 27363399Free PMC Article
Lee T, Takeshima Y, Okizuka Y, Hamahira K, Kusunoki N, Awano H, Yagi M, Sakai N, Matsuo M, Iijima K
Gene 2013 Jan 10;512(2):456-9. Epub 2012 Nov 2 doi: 10.1016/j.gene.2012.10.060. PMID: 23124041
Brown ML, Dearani JA, Danielson GK, Cetta F, Connolly HM, Warnes CA, Li Z, Hodge DO, Driscoll DJ
J Am Coll Cardiol 2008 Aug 5;52(6):460-6. doi: 10.1016/j.jacc.2008.03.064. PMID: 18672167

Prognosis

Bouhlel I, Ajmi H, Slim M, Gribaa R, Naffati E
Tunis Med 2021 Feb;99(2):291-297. PMID: 33899201Free PMC Article
Porayette P, Fruitman D, Lauzon JL, Le Goff C, Cormier-Daire V, Sanders SP, Pinto-Rojas A, Perez-Atayde AR
Pediatr Dev Pathol 2014 May-Jun;17(3):209-16. Epub 2013 Nov 19 doi: 10.2350/13-08-1370-CR.1. PMID: 24251637
Fan K, Lee K, Lau CP
J Interv Card Electrophysiol 1999 Oct;3(3):239-45. doi: 10.1023/a:1009847707872. PMID: 10490480
Bonnet D, Saygili A, Bonhoeffer P, Fermont L, Sidi D, Kachaner J
Int J Cardiol 1997 Apr 18;59(2):113-8. doi: 10.1016/s0167-5273(97)02937-9. PMID: 9158161
Fauchier JP, Fauchier L, Babuty D, Cosnay P
Pacing Clin Electrophysiol 1996 Feb;19(2):231-44. doi: 10.1111/j.1540-8159.1996.tb03315.x. PMID: 8834693

Clinical prediction guides

Leoni C, Blandino R, Delogu AB, De Rosa G, Onesimo R, Verusio V, Marino MV, Lanza GA, Rigante D, Tartaglia M, Zampino G
Am J Med Genet A 2022 Feb;188(2):431-445. Epub 2021 Oct 13 doi: 10.1002/ajmg.a.62529. PMID: 34643321
Bouhlel I, Ajmi H, Slim M, Gribaa R, Naffati E
Tunis Med 2021 Feb;99(2):291-297. PMID: 33899201Free PMC Article
Okubo K, Gigli L, Trevisi N, Foppoli L, Radinovic A, Bisceglia C, Frontera A, D'Angelo G, Cireddu M, Paglino G, Mazzone P, Della Bella P
Circ Arrhythm Electrophysiol 2020 Aug;13(8):e008307. Epub 2020 Jul 12 doi: 10.1161/CIRCEP.119.008307. PMID: 32657137
Fauchier JP, Fauchier L, Babuty D, Cosnay P
Pacing Clin Electrophysiol 1996 Feb;19(2):231-44. doi: 10.1111/j.1540-8159.1996.tb03315.x. PMID: 8834693
Kasanuki H, Ohnishi S, Tanaka E, Hirosawa K
Jpn Circ J 1989 Dec;53(12):1565-70. doi: 10.1253/jcj.53.1565. PMID: 2632827

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