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https://doi.org/10.15836/ccar2021.307

Genetic and personalized approach to valvular heart disease

Katica Cvitkušić Lukenda orcid id orcid.org/0000-0001-6188-0708 ; General Hospital dr. Josip Benčević, Slavonski Brod
Domagoj Vučić orcid id orcid.org/0000-0003-3169-3658 ; General Hospital dr. Josip Benčević, Slavonski Brod
Marijana Knežević Praveček orcid id orcid.org/0000-0002-8727-7357 ; General Hospital dr. Josip Benčević, Slavonski Brod
Krešimir Gabaldo orcid id orcid.org/0000-0002-0116-5929 ; General Hospital dr. Josip Benčević, Slavonski Brod
Domagoj Mišković orcid id orcid.org/0000-0003-4600-0498 ; General Hospital dr. Josip Benčević, Slavonski Brod
Blaženka Miškić orcid id orcid.org/0000-0001-6568-3306 ; General Hospital dr. Josip Benčević, Slavonski Brod
Ana Livun orcid id orcid.org/0000-0002-6758-1677 ; University Hospital Dubrava


Puni tekst: engleski pdf 159 Kb

str. 307-307

preuzimanja: 301

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Sažetak

Ključne riječi

bicuspid aortic valve; calcified aortic valve disease; genetic testing; mitral valve prolapse

Hrčak ID:

261752

URI

https://hrcak.srce.hr/261752

Datum izdavanja:

1.9.2021.

Posjeta: 846 *



Valve diseases have a large share in the total morbidity and mortality of the adult population, and can be congenital or acquired. The last few decades have seen a predominance of degenerative (calcified) heart valve diseases due to a prolonged life expectancy in economically developed countries. The therapeutic approach has remained unchanged and in the case of severe dysfunction, the valve is replaced with a mechanical or biological prosthesis, balloon valvuloplasty or valve reconstruction. The findings in understanding the development of heart valves along with human genome sequencing have led to the discovery of a genetic basis in valvular diseases (1). Also, there are numerous evidence to suggest that heart valve diseases which develop in adulthood has its source in embryonic development. In this review authors will display the genetic basis of the two most common inherited valvular diseases: bicuspid aortic valve and mitral valve prolapse, as well as a review of the findings suggesting a genetic contribution to calcified aortic valve disease (2). In addition, this review will include a review of the guidelines and benefits of genetic testing, as well as highlight the need to include genetic counseling in families with proven or suspected malformations. Linking genetic information to the clinical phenotype (Table 1) and potential outcomes of surgical treatment leads to a personalized approach to each patient (3).

TABLE 1 Gene mutations associated with valvular heart disease.
LocationGeneInheritancePhenotype
Bicuspid aortic valve
Syndromic17q24.3KCNJ2ADAndersen syndrome
Nonsyndromic9q34.3
20q13.33
15q22.31
11q24.2
NOTCH1
GATA5
SMAD6
ROBO4
AD
AD, AR
AD
AD
AOVD1
CHTD5
AOVD2
AOVD3
Mitral valve prolapse
Syndromic15q21.1
9q22.33
3p24.1
7q21.3, 12q13.1,
2q32.2,9q34.3
FBN1
TGFBR1
TGFBR2
Collagen types I–III, V/ XI
AD
AD
AD
AR,AD
AD,AD
Marfan syndrome
Loeys-Dietz syndrome 1
Loeys-Dietz syndrome2
OI 1,Ehlers-Danlos syndrome, cardiac valvular type
NonsyndromicXq28Filamin AXLCardiac valvular dysplasia
Aortic valve stenosis
CAVD9q34.3NOTCH1ADAOVD1
AD – autosomal dominant, AR – autosomal recessive, AOVD – aortic valve disease, CHTD – congenital heart disease, OI – osteogenesis imperfecta, XL – X-linked, CAVD - calcified aortic valve disease.

LITERATURE

1 

Blue GM, Kirk EP, Giannoulatou E, Sholler GF, Dunwoodie SL, Harvey RP, et al. Advances in the Genetics of Congenital Heart Disease: A Clinician’s Guide. J Am Coll Cardiol. 2017 February 21;69(7):859–70. https://doi.org/10.1016/j.jacc.2016.11.060 PubMed: http://www.ncbi.nlm.nih.gov/pubmed/28209227

2 

Koenig SN, Lincoln J, Garg V. Genetic basis of aortic valvular disease. Curr Opin Cardiol. 2017 May;32(3):239–45. https://doi.org/10.1097/HCO.0000000000000384 PubMed: http://www.ncbi.nlm.nih.gov/pubmed/28157139

3 

Diz OM, Toro R, Cesar S, Gomez O, Sarquella-Brugada G, Campuzano O. Personalized Genetic Diagnosis of Congenital Heart Defects in Newborns. J Pers Med. 2021 June 16;11(6):562. https://doi.org/10.3390/jpm11060562 PubMed: http://www.ncbi.nlm.nih.gov/pubmed/34208491


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