Left ventricular noncompaction

Is left ventricular noncompaction (LVNC) difficult to diagnose?

There are factors that affect the ease of diagnosing LVNC. Currently there is no clear genetic or laboratory test to diagnose this condition. Diagnosis relies heavily on Imaging studies, such as echocardiography. There are heart conditions that can look similar to LVNC on imaging studies, which can make correct diagnosis challenging. On the other hand, with new imaging technologies, people with no heart problems (no dangerous rhythms or heart weakness) are being identified as having sponginess or "hyper-trabeculation" in their hearts. This has raised some concern for inappropriate diagnosis or overdiagnosis of LVNC.

Last updated on 05-01-20

How might left ventricular noncompaction be treated?

There are no specific treatment guidelines for left ventricular noncompaction (LVNC). Medical management varies depending on clinical manifestations, left ventricular ejection fraction (LVEF), the presence or absence of arrhythmias, and the risk of thromboembolism.

Depending on the above factors, treatment might include: medications including diuretics, ACE inhibitors or Angiotensin II Receptor Blockers (ARBs), anticoagulants, and beta-blockers; an implantable cardioverter- defibrillator (ICD) or a pacemaker if an arrhythmia is present; and cardiac transplantation.

Last updated on 05-01-20

General Information

Left ventricular noncompation - Children's Cardiomyopathy Foundation

The Children's Cardiomyopathy Foundation has a Web page on cardiomyopathy, which includes information on left ventricular noncompaction. Click on Children's Cardiomyopathy Foundation to view the page.

Last updated on 04-27-20

Testing Resources

Heart Failure Society of America

Genetic Evaluation of Cardiomyopathy - A Heart Failure Society of America practice guideline.
Hershberger, et.al. Journal of Cardiac Failure; 15:83-97

Last updated on 04-27-20

Where To Start

Stanford - Left ventricular noncompaction

The Stanford Center for Inherited Cardiovascular Disease offers patient friendly information on left ventricular noncompaction. Click on the link above to view the information page.

Last updated on 04-27-20

Left ventricular noncompaction - Cardiomyopathy Association

The Cardiomyopathy Association has information on left ventricular noncompaction. Click on Cardiomyopathy Association to view the information page.

Last updated on 04-27-20

Cincinnati Children's Hospital Medical Center - left ventricular noncompaction

The Cincinnati Children's Hospital Medical Center Web site has information on left ventricular noncompaction. Click on Cincinnati Children's Hospital Medical Center to view the page.

Last updated on 04-27-20

Name: Cardiomyopathy Association Chiltern Court Asheridge Road
Unit 10 Chesham Buckinghamshire HP5 2PX
United Kingdom
Phone: +44 01494 791 224 Fax : +44 1923 249 987 Email: contact@cardiomyopathy.org Url: http://www.cardiomyopathy.org
Name: Children's Cardiomyopathy Foundation P.O. Box 547
Tenafly, NJ, 07670 , United States
Toll Free: 866-808-CURE (2873) Fax : 201-227-7016 Email: info@childrenscardiomyopathy.org Url: http://www.childrenscardiomyopathy.org/
Name: Heart Failure Society of America HFSA 6707 Democracy Blvd. Suite 925
Bethesda, MD, 20817, United States
Phone: 301-312-8635 Toll Free: 888-213-4417 Email: info@hfsa.org Url: http://www.hfsa.org/
Niemann M, Stork S, Weidemann F. Left ventricular noncompaction cardiomyopathy: An overdiagnosed disease Circulation. 2012; 126. e240-e243. Reference Link

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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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