- HCM: the disease
- How to screen for HCM
- Treatment Options
- HCM: Genetics
- Is a Cure Available?
- After a Loss
- HCM Spectrum Disorders
- Beckwith-Wiedemann (B-W) Syndrome
- Carnitine Deficiency
- Costello's Syndrome
- Danon Disease
- Fabry's Disease
- Forbes Disease
- Friedreich's Ataxia
- Left Ventricular Non Compaction
- Muscular Dystrophy With Associated HCM
- Noonan Syndrome
- Pompe Disease
- Wolff-Parkinson-White Syndrome
Left Ventricular Non Compaction
Left Ventricular Non-Compaction (LVNC) is a genetic condition, which is categorized under the general term of cardiomyopathy. This is when the lower left chamber of the heart, known as the left ventricle, contains bundles or pieces of muscle, called trabeculations that extend into the chamber. Normally, the trabeculations would become compacted and would transform the heart muscle from spongy to smooth and solid. LVNC occurs when compaction fails to take place. These trabeculations normally occur in the apex (bottom) of the heart but can also be seen in the left ventricle. Individuals with this disease may also have HCM, dilated cardiomyopathy or restrictive cardiomyopathy. LVNC is estimated to affect 1 in 1000 individuals per year however; this condition is more likely to occur than estimated due to the fact that some may not have symptoms. Initial diagnosis may be during any time in life however, LVNC is most commonly diagnosed in children and adults.
In children and adults who have heart failure or an abnormal heart rhythm, symptoms include: SOB (shortness of breath), lightheadedness, syncope (fainting), heart palpitations (feeling abnormal heart beats), unexplained weight gain or swelling. In newborns and babies, symptoms include excessive sweating with activity or feedings, and/or difficulty feeding which may lead to poor growth. Although some with LVNC experience no symptoms, there still may be a risk for complications. The most serious complication is cardiac arrest which is when the heart suddenly stops functioning. Most people with LVNC have a low risk for sudden cardiac arrest however, it is essential for anyone at risk to be monitored by a cardiologist with experience with LVNC.
Diagnosis is based on cardiac testing, family history, medical history and physical examination. When an individual is diagnosed with LVCN, there is a 20-40% chance that the underlying genetic cause will be identified. Most commonly, the only organ affected is the heart. Other genetic causes include syndromes, metabolic and mitochondrial disorders. LVNC usually develops before birth however; there is a chance that one may acquire or develop later in life.
Treatment for individuals is focused on treating associated types of heart muscle diseases such as HCM or a poorly beating heart. The primary goal is to improve cardiac function and prevent symptoms. Medications might be prescribed to treat symptoms, reverse heart damage and to prevent ongoing damage to the heart muscle. If an individual has decreased heart function, an anticoagulant (blood thinner) may be recommended in order to prevent blood clots to form between the trabeculations. Lifestyle changes such as moderate exercise may be recommended. Some people with LVNC are at an increased risk for sudden cardiac arrest and in these situations, an implantable cardioverter- defibrillator (ICD) may be recommended. A pacemaker may be combined with the ICD. In some cases, if the condition is severe, a heart transplant may be deemed necessary.
Figure 1: Image of a heart with Left Ventricular Non-Compaction with trabeculations.
“Cardiomyopathy.” Left Ventricular Non-Compaction Cardiomyopathy (LVNC), www.cincinnatichildrens.org/service/c/cardiomyopathy/types/left-ventricular-non-compaction-cardiomyopathy.
“Left ventricular non-Compaction .” Left ventricular non-Compaction cardiomyopathy - Cardiomyopathy UK, www.cardiomyopathy.org/left-ventricular-noncompaction/intro.
“Left ventricular noncompaction - Genetics Home Reference.” U.S. National Library of Medicine, National Institutes of Health, ghr.nlm.nih.gov/condition/left-ventricular-noncompaction#synonyms.
Am J Cardiol. 2016 Sep 15;118(6):901-905. doi: 10.1016/j.amjcard.2016.06.033. Epub 2016 Jun 27.