Left ventricular noncompaction
Left Ventricular Noncompaction Left Ventricular Noncompaction (LVNC) is a rare cardiomyopathy characterized by the presence of prominent trabeculations and deep intertrabecular recesses in the left ventricle of the heart. This condition can lead to various cardiac complications, including heart failure, arrhythmias, and thromboembolic events.
Etiology[edit | edit source]
LVNC is believed to result from an arrest in the normal process of myocardial compaction during embryonic development. This arrest leads to the persistence of a spongy myocardial layer, which is typically seen in the early stages of heart development.
Genetics[edit | edit source]
LVNC can be familial or sporadic. Familial cases often follow an autosomal dominant inheritance pattern, although autosomal recessive and X-linked forms have also been reported. Mutations in several genes, including MYH7, ACTC1, and TNNT2, have been associated with LVNC.
Clinical Presentation[edit | edit source]
Patients with LVNC may present with a variety of symptoms, ranging from asymptomatic to severe heart failure. Common symptoms include:
- Dyspnea
- Fatigue
- Palpitations
- Syncope
Diagnosis[edit | edit source]
The diagnosis of LVNC is primarily based on imaging studies. Echocardiography is the most commonly used modality, but cardiac MRI can provide more detailed information. Diagnostic criteria include:
- A two-layered myocardium with a noncompacted to compacted ratio >2:1 in end-systole.
- Prominent trabeculations and deep intertrabecular recesses.
Management[edit | edit source]
Management of LVNC focuses on treating symptoms and preventing complications. This may include:
- Medications for heart failure, such as beta-blockers and ACE inhibitors.
- Anticoagulation therapy to prevent thromboembolic events.
- Implantable cardioverter-defibrillators (ICDs) for patients at risk of sudden cardiac death.
Prognosis[edit | edit source]
The prognosis of LVNC varies widely depending on the severity of the condition and the presence of complications. Some patients remain asymptomatic, while others may experience progressive heart failure and require heart transplantation.
Research and Future Directions[edit | edit source]
Ongoing research is focused on better understanding the genetic basis of LVNC and developing targeted therapies. Advances in imaging techniques may also improve diagnostic accuracy and risk stratification.
See Also[edit | edit source]
NIH genetic and rare disease info[edit source]
Left ventricular noncompaction is a rare disease.
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Contributors: Prab R. Tumpati, MD
