U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Left ventricular noncompaction 10(LVNC10)

MedGen UID:
811617
Concept ID:
C3715165
Disease or Syndrome
Synonym: LVNC10
 
Gene (location): MYBPC3 (11p11.2)
 
Monarch Initiative: MONDO:0014163
OMIM®: 615396

Definition

Left ventricular noncompaction is a heart (cardiac) muscle disorder that occurs when the lower left chamber of the heart (left ventricle), which helps the heart pump blood, does not develop correctly. Instead of the muscle being smooth and firm, the cardiac muscle in the left ventricle is thick and appears spongy. The abnormal cardiac muscle is weak and has an impaired ability to pump blood because it either cannot completely contract or it cannot completely relax. For the heart to pump blood normally, cardiac muscle must contract and relax fully.

Some individuals with left ventricular noncompaction experience no symptoms at all; others have heart problems that can include sudden cardiac death. Additional signs and symptoms include abnormal blood clots, irregular heart rhythm (arrhythmia), a sensation of fluttering or pounding in the chest (palpitations), extreme fatigue during exercise (exercise intolerance), shortness of breath (dyspnea), fainting (syncope), swelling of the legs (lymphedema), and trouble laying down flat. Some affected individuals have features of other heart defects. Left ventricular noncompaction can be diagnosed at any age, from birth to late adulthood. Approximately two-thirds of individuals with left ventricular noncompaction develop heart failure. [from MedlinePlus Genetics]

Clinical features

From HPO
Primary dilated cardiomyopathy
MedGen UID:
2880
Concept ID:
C0007193
Disease or Syndrome
Familial dilated cardiomyopathy is a genetic form of heart disease. It occurs when heart (cardiac) muscle becomes thin and weakened in at least one chamber of the heart, causing the open area of the chamber to become enlarged (dilated). As a result, the heart is unable to pump blood as efficiently as usual. To compensate, the heart attempts to increase the amount of blood being pumped through the heart, leading to further thinning and weakening of the cardiac muscle. Over time, this condition results in heart failure.\n\nIt usually takes many years for symptoms of familial dilated cardiomyopathy to cause health problems. They typically begin in mid-adulthood, but can occur at any time from infancy to late adulthood. Signs and symptoms of familial dilated cardiomyopathy can include an irregular heartbeat (arrhythmia), shortness of breath (dyspnea), extreme tiredness (fatigue), fainting episodes (syncope), and swelling of the legs and feet. In some cases, the first sign of the disorder is sudden cardiac death. The severity of the condition varies among affected individuals, even in members of the same family.
Congestive heart failure
MedGen UID:
9169
Concept ID:
C0018802
Disease or Syndrome
The presence of an abnormality of cardiac function that is responsible for the failure of the heart to pump blood at a rate that is commensurate with the needs of the tissues or a state in which abnormally elevated filling pressures are required for the heart to do so. Heart failure is frequently related to a defect in myocardial contraction.
Syncope
MedGen UID:
21443
Concept ID:
C0039070
Sign or Symptom
Syncope is a syndrome in which loss of consciousness is of relatively sudden onset, temporary (usually less than 1 to 2 minutes), self-terminating, and of usually rapid recovery. Syncope leads to a generalized weakness of muscles with loss of postural tone, inability to stand upright, and loss of consciousness. Once the patient is in a horizontal position, blood flow to the brain is no longer hindered by gravitation and consciousness is regained. Unconsciousness usually lasts for seconds to minutes. Headache and drowsiness (which usually follow seizures) do not follow a syncopal attack. Syncope results from a sudden impairment of brain metabolism usually due to a reduction in cerebral blood flow.
Left ventricular noncompaction
MedGen UID:
450531
Concept ID:
C1960469
Disease or Syndrome
Left ventricular noncompaction is a heart (cardiac) muscle disorder that occurs when the lower left chamber of the heart (left ventricle), which helps the heart pump blood, does not develop correctly. Instead of the muscle being smooth and firm, the cardiac muscle in the left ventricle is thick and appears spongy. The abnormal cardiac muscle is weak and has an impaired ability to pump blood because it either cannot completely contract or it cannot completely relax. For the heart to pump blood normally, cardiac muscle must contract and relax fully.\n\nSome individuals with left ventricular noncompaction experience no symptoms at all; others have heart problems that can include sudden cardiac death. Additional signs and symptoms include abnormal blood clots, irregular heart rhythm (arrhythmia), a sensation of fluttering or pounding in the chest (palpitations), extreme fatigue during exercise (exercise intolerance), shortness of breath (dyspnea), fainting (syncope), swelling of the legs (lymphedema), and trouble laying down flat. Some affected individuals have features of other heart defects. Left ventricular noncompaction can be diagnosed at any age, from birth to late adulthood. Approximately two-thirds of individuals with left ventricular noncompaction develop heart failure.
Pulmonary arterial hypertension
MedGen UID:
425404
Concept ID:
C2973725
Disease or Syndrome
Pulmonary hypertension is defined mean pulmonary artery pressure of 25mmHg or more and pulmonary capillary wedge pressure of 15mmHg or less when measured by right heart catheterisation at rest and in a supine position.
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).

Professional guidelines

PubMed

Wanert C, El Louali F, Al Dybiat S, Nguyen K, Zaffran S, Ovaert C
Arch Cardiovasc Dis 2023 Jun-Jul;116(6-7):309-315. Epub 2023 May 16 doi: 10.1016/j.acvd.2023.04.008. PMID: 37246080
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
Thavendiranathan P, Dahiya A, Phelan D, Desai MY, Tang WH
Heart 2013 May;99(10):681-9. Epub 2012 Nov 26 doi: 10.1136/heartjnl-2012-302816. PMID: 23184013

Recent clinical studies

Etiology

Stöllberger C, Finsterer J
Rev Cardiovasc Med 2021 Dec 22;22(4):1413-1420. doi: 10.31083/j.rcm2204146. PMID: 34957781
Ichida F
J Cardiol 2020 Jan;75(1):1-9. Epub 2019 Oct 17 doi: 10.1016/j.jjcc.2019.09.011. PMID: 31629663
Brieler J, Breeden MA, Tucker J
Am Fam Physician 2017 Nov 15;96(10):640-646. PMID: 29431384
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
Towbin JA, Lorts A, Jefferies JL
Lancet 2015 Aug 22;386(9995):813-25. Epub 2015 Apr 9 doi: 10.1016/S0140-6736(14)61282-4. PMID: 25865865

Diagnosis

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
Ichida F
J Cardiol 2020 Jan;75(1):1-9. Epub 2019 Oct 17 doi: 10.1016/j.jjcc.2019.09.011. PMID: 31629663
Brieler J, Breeden MA, Tucker J
Am Fam Physician 2017 Nov 15;96(10):640-646. PMID: 29431384
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
Towbin JA, Lorts A, Jefferies JL
Lancet 2015 Aug 22;386(9995):813-25. Epub 2015 Apr 9 doi: 10.1016/S0140-6736(14)61282-4. PMID: 25865865

Therapy

Ivanov A, Dabiesingh DS, Bhumireddy GP, Mohamed A, Asfour A, Briggs WM, Ho J, Khan SA, Grossman A, Klem I, Sacchi TJ, Heitner JF
Circ Cardiovasc Imaging 2017 Sep;10(9) doi: 10.1161/CIRCIMAGING.117.006174. PMID: 28899950
Minamisawa M, Koyama J, Kozuka A, Miura T, Ebisawa S, Motoki H, Okada A, Izawa A, Ikeda U
J Cardiol 2016 Nov;68(5):431-438. Epub 2015 Dec 28 doi: 10.1016/j.jjcc.2015.11.008. PMID: 26740463
Towbin JA, Lorts A, Jefferies JL
Lancet 2015 Aug 22;386(9995):813-25. Epub 2015 Apr 9 doi: 10.1016/S0140-6736(14)61282-4. PMID: 25865865
Peters F, Khandheria BK, Botha F, Libhaber E, Matioda H, Dos Santos C, Govender S, Meel R, Essop MR
J Card Fail 2014 Oct;20(10):709-715. Epub 2014 Jul 29 doi: 10.1016/j.cardfail.2014.07.007. PMID: 25079299
Baquero GA, Colegrove DJ, Banchs JE
Tex Heart Inst J 2013;40(3):331-8. PMID: 23914034Free PMC Article

Prognosis

Wang Y, Jia H, Song J
Curr Cardiol Rep 2023 Oct;25(10):1299-1317. Epub 2023 Sep 15 doi: 10.1007/s11886-023-01944-0. PMID: 37721634Free PMC Article
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
Stöllberger C, Finsterer J
Rev Cardiovasc Med 2021 Dec 22;22(4):1413-1420. doi: 10.31083/j.rcm2204146. PMID: 34957781
Ichida F
J Cardiol 2020 Jan;75(1):1-9. Epub 2019 Oct 17 doi: 10.1016/j.jjcc.2019.09.011. PMID: 31629663
Stöllberger C, Finsterer J
Expert Rev Cardiovasc Ther 2016 Oct;14(10):1151-60. Epub 2016 Aug 1 doi: 10.1080/14779072.2016.1215244. PMID: 27442845

Clinical prediction guides

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
Stöllberger C, Finsterer J
Rev Cardiovasc Med 2021 Dec 22;22(4):1413-1420. doi: 10.31083/j.rcm2204146. PMID: 34957781
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
Sigvardsen PE, Fuchs A, Kühl JT, Afzal S, Køber L, Nordestgaard BG, Kofoed KF
Eur Heart J Cardiovasc Imaging 2021 Jan 1;22(1):67-74. doi: 10.1093/ehjci/jeaa110. PMID: 32386205
Ichida F
J Cardiol 2020 Jan;75(1):1-9. Epub 2019 Oct 17 doi: 10.1016/j.jjcc.2019.09.011. PMID: 31629663

Recent systematic reviews

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
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

Supplemental Content

Recent activity

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...