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Left ventricular noncompaction 9(LVNC9)

MedGen UID:
814475
•Concept ID:
C3808145
•
Disease or Syndrome
Synonym: LVNC9
 
Monarch Initiative: MONDO:0800346
OMIM®: 191010; 611878

Professional guidelines

PubMed

Genotype and Cardiac Outcomes in Pediatric Dilated Cardiomyopathy.
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
Diagnostic criteria for left ventricular non-compaction in cardiac computed tomography.
Fuchs TA, Erhart L, Ghadri JR, Herzog BA, Giannopoulos A, Buechel RR, Stämpfli SF, Gruner C, Pazhenkottil AP, Niemann M, Kaufmann PA, Tanner FC
PLoS One 2020;15(7):e0235751. Epub 2020 Jul 10 doi: 10.1371/journal.pone.0235751. PMID: 32649698Free PMC Article
Genetic Testing in Pediatric Left Ventricular Noncompaction.
Miller EM, Hinton RB, Czosek R, Lorts A, Parrott A, Shikany AR, Ittenbach RF, Ware SM
Circ Cardiovasc Genet 2017 Dec;10(6) doi: 10.1161/CIRCGENETICS.117.001735. PMID: 29212898Free PMC Article

Recent clinical studies

Etiology

Stroke and embolism in patients with left ventricular hypertrabeculation/noncompaction.
Schiebel K, Finsterer J, Lazarevic P, Stöllberger C
J Stroke Cerebrovasc Dis 2022 Sep;31(9):106623. Epub 2022 Jul 6 doi: 10.1016/j.jstrokecerebrovasdis.2022.106623. PMID: 35803121
MR -specific characteristics of left ventricular noncompaction and dilated cardiomyopathy.
Gregor Z, Kiss AR, Grebur K, Szabó LE, Merkely B, Vágó H, Szűcs A
Int J Cardiol 2022 Jul 15;359:69-75. Epub 2022 Apr 15 doi: 10.1016/j.ijcard.2022.04.026. PMID: 35436556
Catheter ablation of ventricular arrhythmias in left ventricular noncompaction cardiomyopathy.
Sánchez Muñoz JJ, Muñoz-Esparza C, Verdú PP, Sánchez JM, Almagro FG, Ruiz GE, Gimeno Blanes JR, Alberola AG
Heart Rhythm 2021 Apr;18(4):545-552. Epub 2020 Dec 17 doi: 10.1016/j.hrthm.2020.12.014. PMID: 33346135
Left Ventricular Noncompaction in Older Patients.
Tian T, Yang KQ, Mao Y, Zhou LL, Wang LP, Xiao Y, Yang YK, Zhang Y, Meng X, Zhou XL
Am J Med Sci 2017 Aug;354(2):140-144. Epub 2017 Apr 21 doi: 10.1016/j.amjms.2017.04.012. PMID: 28864371
Arrhythmias and left ventricular hypertrabeculation /noncompaction.
Stöllberger C, Finsterer J
Curr Pharm Des 2010;16(26):2880-94. doi: 10.2174/138161210793176518. PMID: 20632950

Diagnosis

Stroke and embolism in patients with left ventricular hypertrabeculation/noncompaction.
Schiebel K, Finsterer J, Lazarevic P, Stöllberger C
J Stroke Cerebrovasc Dis 2022 Sep;31(9):106623. Epub 2022 Jul 6 doi: 10.1016/j.jstrokecerebrovasdis.2022.106623. PMID: 35803121
Genotype and Cardiac Outcomes in Pediatric Dilated Cardiomyopathy.
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
Molecular analysis of dilated and left ventricular noncompaction cardiomyopathies in Egyptian children.
Mehaney DA, Haghighi A, Embaby AK, Zeyada RA, Darwish RK, Elfeel NS, Abouelhoda M, El-Saiedi SA, Gohar NA, Seliem ZS
Cardiol Young 2022 Feb;32(2):295-300. Epub 2021 May 26 doi: 10.1017/S1047951121002055. PMID: 34036930
Left Ventricular Noncompaction in Older Patients.
Tian T, Yang KQ, Mao Y, Zhou LL, Wang LP, Xiao Y, Yang YK, Zhang Y, Meng X, Zhou XL
Am J Med Sci 2017 Aug;354(2):140-144. Epub 2017 Apr 21 doi: 10.1016/j.amjms.2017.04.012. PMID: 28864371
Left Ventricular Noncompaction: A Distinct Genetic Cardiomyopathy?
Arbustini E, Favalli V, Narula N, Serio A, Grasso M
J Am Coll Cardiol 2016 Aug 30;68(9):949-66. doi: 10.1016/j.jacc.2016.05.096. PMID: 27561770

Therapy

A proposed strategy for anticoagulation therapy in noncompaction cardiomyopathy.
Chimenti C, Lavalle C, Magnocavallo M, Alfarano M, Mariani MV, Bernardini F, Della Rocca DG, Galardo G, Severino P, Di Lullo L, Miraldi F, Fedele F, Frustaci A
ESC Heart Fail 2022 Feb;9(1):241-250. Epub 2021 Dec 16 doi: 10.1002/ehf2.13694. PMID: 34918480Free PMC Article
Non compaction cardiomyopathy: Review of a controversial entity.
Lorca R, Rozado J, Martín M
Med Clin (Barc) 2018 May 11;150(9):354-360. Epub 2017 Nov 22 doi: 10.1016/j.medcli.2017.09.026. PMID: 29173988
Ventricular arrhythmias associated with left ventricular noncompaction: Electrophysiologic characteristics, mapping, and ablation.
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
Isolated noncompaction of the left ventricular myocardium in adults: a systematic overview.
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
Arrhythmias and left ventricular hypertrabeculation /noncompaction.
Stöllberger C, Finsterer J
Curr Pharm Des 2010;16(26):2880-94. doi: 10.2174/138161210793176518. PMID: 20632950

Prognosis

Stroke and embolism in patients with left ventricular hypertrabeculation/noncompaction.
Schiebel K, Finsterer J, Lazarevic P, Stöllberger C
J Stroke Cerebrovasc Dis 2022 Sep;31(9):106623. Epub 2022 Jul 6 doi: 10.1016/j.jstrokecerebrovasdis.2022.106623. PMID: 35803121
Genotype and Cardiac Outcomes in Pediatric Dilated Cardiomyopathy.
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
Clinical Characteristics and Prognosis of Fetal Left Ventricular Noncompaction in Japan.
Ozawa SW, Takarada S, Okabe M, Miyao N, Nakaoka H, Ibuki K, Ichida F, Hirono K; Fetal CM Study Collaborators
Circ J 2021 Dec 24;86(1):98-105. Epub 2021 Aug 3 doi: 10.1253/circj.CJ-20-1148. PMID: 34349074
Catheter ablation of ventricular arrhythmias in left ventricular noncompaction cardiomyopathy.
Sánchez Muñoz JJ, Muñoz-Esparza C, Verdú PP, Sánchez JM, Almagro FG, Ruiz GE, Gimeno Blanes JR, Alberola AG
Heart Rhythm 2021 Apr;18(4):545-552. Epub 2020 Dec 17 doi: 10.1016/j.hrthm.2020.12.014. PMID: 33346135
Left Ventricular Noncompaction in Older Patients.
Tian T, Yang KQ, Mao Y, Zhou LL, Wang LP, Xiao Y, Yang YK, Zhang Y, Meng X, Zhou XL
Am J Med Sci 2017 Aug;354(2):140-144. Epub 2017 Apr 21 doi: 10.1016/j.amjms.2017.04.012. PMID: 28864371

Clinical prediction guides

Characteristics and long-term survival of patients with left ventricular non-compaction cardiomyopathy.
Demir E, Bayraktaroğlu S, Çinkooğlu A, Candemir A, Candemir YB, Öztürk RO, Dadaş ÖF, Orman MN, Zoghi M, Akıllı A, Ceylan N, Gürgün C, Nalbantgil S
ESC Heart Fail 2022 Dec;9(6):4219-4229. Epub 2022 Sep 16 doi: 10.1002/ehf2.14081. PMID: 36111517Free PMC Article
Genotype and Cardiac Outcomes in Pediatric Dilated Cardiomyopathy.
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
Clinical Characteristics and Prognosis of Fetal Left Ventricular Noncompaction in Japan.
Ozawa SW, Takarada S, Okabe M, Miyao N, Nakaoka H, Ibuki K, Ichida F, Hirono K; Fetal CM Study Collaborators
Circ J 2021 Dec 24;86(1):98-105. Epub 2021 Aug 3 doi: 10.1253/circj.CJ-20-1148. PMID: 34349074
The role of multimodality imaging in the diagnosis of left ventricular noncompaction.
Rao K, Bhaskaran A, Choudhary P, Tan TC
Eur J Clin Invest 2020 Sep;50(9):e13254. Epub 2020 May 14 doi: 10.1111/eci.13254. PMID: 32329049
Left Ventricular Noncompaction: A Distinct Genetic Cardiomyopathy?
Arbustini E, Favalli V, Narula N, Serio A, Grasso M
J Am Coll Cardiol 2016 Aug 30;68(9):949-66. doi: 10.1016/j.jacc.2016.05.096. PMID: 27561770

Recent systematic reviews

Electrophysiological phenotyping of left ventricular noncompaction cardiomyopathy in pediatric populations: A systematic review.
Fitzsimons LA, Kneeland-Barber DM, Hannigan GC, Karpe DA, Wu L, Colon M, Randall J, Tucker KL
Physiol Rep 2024 May;12(9):e16029. doi: 10.14814/phy2.16029. PMID: 38684446Free PMC Article
Thromboembolic events in left ventricular non-compaction: comparison between children and adults - a systematic review and meta-analysis.
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
Electrophysiologic and electroanatomic characterization of ventricular arrhythmias in non-compaction cardiomyopathy: A systematic review.
Bhaskaran A, Campbell T, Virk S, Bennett RG, Kizana E, Kumar S
J Cardiovasc Electrophysiol 2021 May;32(5):1421-1429. Epub 2021 Apr 12 doi: 10.1111/jce.15026. PMID: 33792994
Prognostic Significance of Left Ventricular Noncompaction: Systematic Review and Meta-Analysis of Observational Studies.
Aung N, Doimo S, Ricci F, Sanghvi MM, Pedrosa C, Woodbridge SP, Al-Balah A, Zemrak F, Khanji MY, Munroe PB, Naci H, Petersen SE
Circ Cardiovasc Imaging 2020 Jan;13(1):e009712. Epub 2020 Jan 21 doi: 10.1161/CIRCIMAGING.119.009712. PMID: 31959004Free PMC Article
Meta-Analysis of the Prognostic Role of Late Gadolinium Enhancement and Global Systolic Impairment in Left Ventricular Noncompaction.
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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