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Left ventricular noncompaction 4(LVNC4)

MedGen UID:
462032
Concept ID:
C3150682
Disease or Syndrome
Synonym: LVNC4
 
Monarch Initiative: MONDO:0800350
OMIM®: 102540; 613424

Professional guidelines

PubMed

Khan RS, Pahl E, Dellefave-Castillo L, Rychlik K, Ing A, Yap KL, Brew C, Johnston JR, McNally EM, Webster G
J Am Heart Assoc 2022 Jan 4;11(1):e022854. Epub 2021 Dec 22 doi: 10.1161/JAHA.121.022854. PMID: 34935411Free PMC Article
Miszalski-Jamka K, Jefferies JL, Mazur W, Głowacki J, Hu J, Lazar M, Gibbs RA, Liczko J, Kłyś J, Venner E, Muzny DM, Rycaj J, Białkowski J, Kluczewska E, Kalarus Z, Jhangiani S, Al-Khalidi H, Kukulski T, Lupski JR, Craigen WJ, Bainbridge MN
Circ Cardiovasc Genet 2017 Aug;10(4) doi: 10.1161/CIRCGENETICS.117.001763. PMID: 28798025Free PMC Article
Meyers DE, Basha HI, Koenig MK
Tex Heart Inst J 2013;40(4):385-94. PMID: 24082366Free PMC Article

Recent clinical studies

Etiology

van Waning JI, Caliskan K, Hoedemaekers YM, van Spaendonck-Zwarts KY, Baas AF, Boekholdt SM, van Melle JP, Teske AJ, Asselbergs FW, Backx APCM, du Marchie Sarvaas GJ, Dalinghaus M, Breur JMPJ, Linschoten MPM, Verlooij LA, Kardys I, Dooijes D, Lekanne Deprez RH, IJpma AS, van den Berg MP, Hofstra RMW, van Slegtenhorst MA, Jongbloed JDH, Majoor-Krakauer D
J Am Coll Cardiol 2018 Feb 20;71(7):711-722. doi: 10.1016/j.jacc.2017.12.019. PMID: 29447731
Miszalski-Jamka K, Jefferies JL, Mazur W, Głowacki J, Hu J, Lazar M, Gibbs RA, Liczko J, Kłyś J, Venner E, Muzny DM, Rycaj J, Białkowski J, Kluczewska E, Kalarus Z, Jhangiani S, Al-Khalidi H, Kukulski T, Lupski JR, Craigen WJ, Bainbridge MN
Circ Cardiovasc Genet 2017 Aug;10(4) doi: 10.1161/CIRCGENETICS.117.001763. PMID: 28798025Free PMC Article
Meyers DE, Basha HI, Koenig MK
Tex Heart Inst J 2013;40(4):385-94. PMID: 24082366Free PMC Article
Ng D, Johnston JJ, Teer JK, Singh LN, Peller LC, Wynter JS, Lewis KL, Cooper DN, Stenson PD, Mullikin JC, Biesecker LG; NIH Intramural Sequencing Center (NISC) Comparative Sequencing Program
Circ Cardiovasc Genet 2013 Aug;6(4):337-46. Epub 2013 Jul 16 doi: 10.1161/CIRCGENETICS.113.000039. PMID: 23861362Free PMC Article
Towbin JA
Heart Fail Clin 2010 Oct;6(4):453-69, viii. doi: 10.1016/j.hfc.2010.06.005. PMID: 20869646

Diagnosis

Bagnall RD, Singer ES, Wacker J, Nowak N, Ingles J, King I, Macciocca I, Crowe J, Ronan A, Weintraub RG, Semsarian C
Circ Genom Precis Med 2022 Dec;15(6):e003686. Epub 2022 Oct 11 doi: 10.1161/CIRCGEN.121.003686. PMID: 36252119
de Frutos F, Ochoa JP, Navarro-Peñalver M, Baas A, Bjerre JV, Zorio E, Méndez I, Lorca R, Verdonschot JAJ, García-Granja PE, Bilinska Z, Fatkin D, Fuentes-Cañamero ME, García-Pinilla JM, García-Álvarez MI, Girolami F, Barriales-Villa R, Díez-López C, Lopes LR, Wahbi K, García-Álvarez A, Rodríguez-Sánchez I, Rekondo-Olaetxea J, Rodríguez-Palomares JF, Gallego-Delgado M, Meder B, Kubanek M, Hansen FG, Restrepo-Córdoba MA, Palomino-Doza J, Ruiz-Guerrero L, Sarquella-Brugada G, Perez-Perez AJ, Bermúdez-Jiménez FJ, Ripoll-Vera T, Rasmussen TB, Jansen M, Sabater-Molina M, Elliot PM, Garcia-Pavia P; European Genetic Cardiomyopathies Initiative Investigators
J Am Coll Cardiol 2022 Oct 11;80(15):1447-1461. Epub 2022 Aug 22 doi: 10.1016/j.jacc.2022.07.023. PMID: 36007715
Khan RS, Pahl E, Dellefave-Castillo L, Rychlik K, Ing A, Yap KL, Brew C, Johnston JR, McNally EM, Webster G
J Am Heart Assoc 2022 Jan 4;11(1):e022854. Epub 2021 Dec 22 doi: 10.1161/JAHA.121.022854. PMID: 34935411Free PMC Article
Srivastava S, Yavari M, Al-Abcha A, Banga S, Abela G
Heart Fail Rev 2022 Jul;27(4):1063-1076. Epub 2021 Jul 7 doi: 10.1007/s10741-021-10128-3. PMID: 34232438
Meyers DE, Basha HI, Koenig MK
Tex Heart Inst J 2013;40(4):385-94. PMID: 24082366Free PMC Article

Therapy

Kobayashi K, Akita T, Akita S, Ito H, Terazawa S, Mutsuga M, Usui A
Nagoya J Med Sci 2023 Aug;85(3):626-634. doi: 10.18999/nagjms.85.3.626. PMID: 37829491Free PMC Article
Yogasundaram H, Alhumaid W, Dzwiniel T, Christian S, Oudit GY
Can J Cardiol 2021 Apr;37(4):547-559. Epub 2021 Jan 22 doi: 10.1016/j.cjca.2021.01.016. PMID: 33493662
Nagai T, Horinouchi H, Kitayama A, Ikari Y
Echocardiography 2019 Apr;36(4):791-793. Epub 2019 Mar 4 doi: 10.1111/echo.14288. PMID: 30834555
Muser D, Liang JJ, Witschey WR, Pathak RK, Castro S, Magnani S, Zado ES, Garcia FC, Desjardins B, Callans DJ, Frankel DS, Marchlinski FE, Santangeli P
Heart Rhythm 2017 Feb;14(2):166-175. Epub 2016 Nov 24 doi: 10.1016/j.hrthm.2016.11.014. PMID: 27890738
Bhatia NL, Tajik AJ, Wilansky S, Steidley DE, Mookadam F
J Card Fail 2011 Sep;17(9):771-8. doi: 10.1016/j.cardfail.2011.05.002. PMID: 21872148

Prognosis

Bagnall RD, Singer ES, Wacker J, Nowak N, Ingles J, King I, Macciocca I, Crowe J, Ronan A, Weintraub RG, Semsarian C
Circ Genom Precis Med 2022 Dec;15(6):e003686. Epub 2022 Oct 11 doi: 10.1161/CIRCGEN.121.003686. PMID: 36252119
de Frutos F, Ochoa JP, Navarro-Peñalver M, Baas A, Bjerre JV, Zorio E, Méndez I, Lorca R, Verdonschot JAJ, García-Granja PE, Bilinska Z, Fatkin D, Fuentes-Cañamero ME, García-Pinilla JM, García-Álvarez MI, Girolami F, Barriales-Villa R, Díez-López C, Lopes LR, Wahbi K, García-Álvarez A, Rodríguez-Sánchez I, Rekondo-Olaetxea J, Rodríguez-Palomares JF, Gallego-Delgado M, Meder B, Kubanek M, Hansen FG, Restrepo-Córdoba MA, Palomino-Doza J, Ruiz-Guerrero L, Sarquella-Brugada G, Perez-Perez AJ, Bermúdez-Jiménez FJ, Ripoll-Vera T, Rasmussen TB, Jansen M, Sabater-Molina M, Elliot PM, Garcia-Pavia P; European Genetic Cardiomyopathies Initiative Investigators
J Am Coll Cardiol 2022 Oct 11;80(15):1447-1461. Epub 2022 Aug 22 doi: 10.1016/j.jacc.2022.07.023. PMID: 36007715
Khan RS, Pahl E, Dellefave-Castillo L, Rychlik K, Ing A, Yap KL, Brew C, Johnston JR, McNally EM, Webster G
J Am Heart Assoc 2022 Jan 4;11(1):e022854. Epub 2021 Dec 22 doi: 10.1161/JAHA.121.022854. PMID: 34935411Free PMC Article
van Waning JI, Caliskan K, Hoedemaekers YM, van Spaendonck-Zwarts KY, Baas AF, Boekholdt SM, van Melle JP, Teske AJ, Asselbergs FW, Backx APCM, du Marchie Sarvaas GJ, Dalinghaus M, Breur JMPJ, Linschoten MPM, Verlooij LA, Kardys I, Dooijes D, Lekanne Deprez RH, IJpma AS, van den Berg MP, Hofstra RMW, van Slegtenhorst MA, Jongbloed JDH, Majoor-Krakauer D
J Am Coll Cardiol 2018 Feb 20;71(7):711-722. doi: 10.1016/j.jacc.2017.12.019. PMID: 29447731
Meyers DE, Basha HI, Koenig MK
Tex Heart Inst J 2013;40(4):385-94. PMID: 24082366Free PMC Article

Clinical prediction guides

Khan RS, Pahl E, Dellefave-Castillo L, Rychlik K, Ing A, Yap KL, Brew C, Johnston JR, McNally EM, Webster G
J Am Heart Assoc 2022 Jan 4;11(1):e022854. Epub 2021 Dec 22 doi: 10.1161/JAHA.121.022854. PMID: 34935411Free PMC Article
Srivastava S, Yavari M, Al-Abcha A, Banga S, Abela G
Heart Fail Rev 2022 Jul;27(4):1063-1076. Epub 2021 Jul 7 doi: 10.1007/s10741-021-10128-3. PMID: 34232438
Mazzarotto F, Hawley MH, Beltrami M, Beekman L, de Marvao A, McGurk KA, Statton B, Boschi B, Girolami F, Roberts AM, Lodder EM, Allouba M, Romeih S, Aguib Y, Baksi AJ, Pantazis A, Prasad SK, Cerbai E, Yacoub MH, O'Regan DP, Cook SA, Ware JS, Funke B, Olivotto I, Bezzina CR, Barton PJR, Walsh R
Genet Med 2021 May;23(5):856-864. Epub 2021 Jan 26 doi: 10.1038/s41436-020-01049-x. PMID: 33500567Free PMC Article
van Waning JI, Caliskan K, Hoedemaekers YM, van Spaendonck-Zwarts KY, Baas AF, Boekholdt SM, van Melle JP, Teske AJ, Asselbergs FW, Backx APCM, du Marchie Sarvaas GJ, Dalinghaus M, Breur JMPJ, Linschoten MPM, Verlooij LA, Kardys I, Dooijes D, Lekanne Deprez RH, IJpma AS, van den Berg MP, Hofstra RMW, van Slegtenhorst MA, Jongbloed JDH, Majoor-Krakauer D
J Am Coll Cardiol 2018 Feb 20;71(7):711-722. doi: 10.1016/j.jacc.2017.12.019. PMID: 29447731
Meyers DE, Basha HI, Koenig MK
Tex Heart Inst J 2013;40(4):385-94. PMID: 24082366Free PMC Article

Recent systematic reviews

Hirono K, Takarada S, Miyao N, Nakaoka H, Ibuki K, Ozawa S, Origasa H, Ichida F
Open Heart 2022 May;9(1) doi: 10.1136/openhrt-2021-001908. PMID: 35613828Free PMC Article
Bardhi E, Faralli I, Deroma M, Galoppi P, Ventriglia F, Giancotti A, Perrone G, Brunelli R
J Matern Fetal Neonatal Med 2021 Sep;34(17):2910-2917. Epub 2019 Oct 1 doi: 10.1080/14767058.2019.1671337. PMID: 31570025
Kayvanpour E, Sedaghat-Hamedani F, Gi WT, Tugrul OF, Amr A, Haas J, Zhu F, Ehlermann P, Uhlmann L, Katus HA, Meder B
Clin Res Cardiol 2019 Nov;108(11):1297-1308. Epub 2019 Apr 12 doi: 10.1007/s00392-019-01465-3. PMID: 30980206
Grigoratos C, Barison A, Ivanov A, Andreini D, Amzulescu MS, Mazurkiewicz L, De Luca A, Grzybowski J, Masci PG, Marczak M, Heitner JF, Schwitter J, Gerber BL, Emdin M, Aquaro GD
JACC Cardiovasc Imaging 2019 Nov;12(11 Pt 1):2141-2151. Epub 2019 Mar 13 doi: 10.1016/j.jcmg.2018.12.029. PMID: 30878415

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