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left ventricular noncompaction
ICD-10 Codes
Description
Left Ventricular Noncompaction (LVNC) Cardiomyopathy
Left ventricular noncompaction cardiomyopathy, also known as LVNC, is a rare and serious heart condition that affects the left ventricle, which is the main pumping chamber of the heart. In people with LVNC, the two-layered myocardium (heart muscle) has an abnormally thick sponge-like, trabecular layer [1]. This results in a poorly compacted or "noncompacted" left ventricle.
Characteristics and Symptoms
LVNC is characterized by:
- A heart that has not fully packed together its individual tissue layers [5]
- Pieces of muscle extending into the chamber of the left ventricle [3]
- Trabeculations (small, finger-like projections) in the myocardium, which can lead to blood flow problems [8]
Symptoms of LVNC may include:
- Breathlessness
- Fatigue (extreme tiredness)
- Feeling dizzy or light-headed
- Palpitations
- Loss of consciousness [7]
Diagnosis and Complications
LVNC is a congenital pathology, meaning it is present at birth. It can be diagnosed through various tests, including echocardiography, cardiac MRI, and electrocardiogram (ECG) [9]. If left untreated, LVNC can lead to serious complications, such as heart failure, arrhythmias, and sudden death.
References
[1] DP Singh · 2022 · Cited by 44 [3] Left ventricular noncompaction cardiomyopathy (LVNC) happens when the left ventricle has pieces of muscle that extend into the chamber. [5] Left ventricular noncompaction (LVNC) is an heart disorder characterized by a heart that has not fully packed together its individual tissue layers. [7] Breathlessness · Fatigue (extreme tiredness) · Feeling dizzy or light-headed · Palpitations · Loss of consciousness. [8] Left ventricular non-compaction (LVNC) is a congenital pathology that directly affects the lining walls of myocardial tissue, causing trabeculations with blood ... [9] May 5, 2022 — Isolated left ventricular noncompaction in adults: Clinical manifestations and diagnosis.
Additional Characteristics
- A heart condition affecting the left ventricle with an abnormally thick sponge-like layer
- Characterized by a poorly compacted or 'noncompacted' left ventricle
- Symptoms include breathlessness, fatigue, dizziness, palpitations, and loss of consciousness
- Diagnosed through tests like echocardiography, cardiac MRI, and electrocardiogram (ECG)
- Can lead to complications such as heart failure, arrhythmias, and sudden death
Signs and Symptoms
Common Signs and Symptoms of Left Ventricular Noncompaction (LVNC)
Left ventricular noncompaction (LVNC) is a rare heart condition characterized by the abnormal development of the left ventricle, which can lead to various symptoms. Here are some common signs and symptoms associated with LVNC:
- Shortness of breath: This is one of the most common symptoms of LVNC, often experienced during exercise or physical activity [1][2].
- Fatigue: Patients with LVNC may feel tired or exhausted easily, even after minimal physical exertion [3][4].
- Chest pain: Some individuals with LVNC may experience chest pain or discomfort, which can be a sign of heart problems [5].
- Palpitations: Fluttery or pounding heartbeats are common in people with LVNC, and can be a cause for concern [6][7].
- Dizziness or lightheadedness: Patients with LVNC may feel dizzy or light-headed, especially when standing up quickly [8].
In some cases, individuals with LVNC may experience more severe symptoms, such as:
- Fainting (syncope): Sudden loss of consciousness can occur in people with LVNC, particularly during physical activity [9].
- Sudden cardiac death: In rare instances, LVNC can lead to sudden cardiac death, which is a life-threatening condition [10].
It's essential for individuals with suspected LVNC to seek medical attention promptly, as early diagnosis and treatment can significantly improve outcomes.
References:
[1] Context 1 [2] Context 2 [3] Context 4 [4] Context 5 [5] Context 7 [6] Context 2 [7] Context 8 [8] Context 4 [9] Context 9 [10] Context 5
Additional Symptoms
- Shortness of breath
- Palpitations
- Dizziness or lightheadedness
- Sudden cardiac death
- chest pain
- syncope
- fatigue
Diagnostic Tests
Diagnostic Tests for Left Ventricular Noncompaction (LVNC)
Left ventricular noncompaction (LVNC) is a rare cardiomyopathy characterized by the presence of prominent trabeculations and deep intertrabecular recesses in the left ventricle. The diagnosis of LVNC typically involves a combination of clinical evaluation, imaging studies, and genetic testing.
Imaging Studies
- Echocardiography: Echocardiogram is the most common initial test that identifies the characteristic features of LVNC, such as prominent trabeculations and deep intertrabecular recesses [2]. It can also provide information on the left ventricular function and size.
- Cardiac Magnetic Resonance Imaging (MRI): Cardiac MRI is a highly sensitive and specific imaging modality for diagnosing LVNC. It can provide detailed images of the heart's structure and function, including the presence of trabeculations and intertrabecular recesses [3].
- Computed Tomography (CT) Scan: CT scan may also be used to evaluate the left ventricle and diagnose LVNC.
Additional Diagnostic Tests
- Echocardiography
- Computed Tomography (CT) Scan
- Cardiac Magnetic Resonance Imaging (MRI)
Treatment
Medications Used to Treat Left Ventricular Noncompaction (LVNC)
Left ventricular noncompaction (LVNC) is a rare cardiomyopathy characterized by the presence of prominent trabeculations and deep intertrabecular recesses in the left ventricle. While there are limited treatment options for LVNC, various medications can be used to manage its symptoms.
- Blood Thinners (Anticoagulants): Blood thinners such as warfarin or apixaban may be prescribed to reduce the risk of blood clots and stroke in patients with LVNC [1].
- Digoxin: Digoxin is a medication that can enhance myocardial contractility in children with LVNC and left heart systolic dysfunction [2].
- Beta Blockers: Beta blockers such as metoprolol or carvedilol may be used to help control heart beats and lower blood pressure in patients with LVNC [6].
- Heart Failure Medications: Drugs such as ACE inhibitors, ARBs, or hydralazine may be prescribed to manage heart failure symptoms in patients with LVNC [3][7].
Other Treatment Options
In addition to medications, other treatment options for LVNC may include:
- Implantable cardioverter-defibrillators (ICDs) to prevent sudden cardiac death
- Antiarrhythmic medications to control abnormal heart rhythms
- Anticoagulation therapy to reduce the risk of blood clots
It's essential to note that the specific treatment plan for LVNC will depend on individual patient factors, including the severity of symptoms and underlying health conditions.
References:
[1] - [8] are citations from the search results provided in the context.
Recommended Medications
- ACE inhibitors
- Antiarrhythmic medications
- ARBs
- Anticoagulation therapy
- digoxin
- Digoxin
- warfarin
- Warfarin
- carvedilol
- metoprolol
- Metoprolol
- apixaban
- hydralazine
- Hydralazine
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Differential Diagnosis of Left Ventricular Noncompaction (LVNC)
Left Ventricular Noncompaction (LVNC) is a rare cardiomyopathy characterized by the presence of prominent trabeculations and deep intertrabecular recesses in the left ventricle. The differential diagnosis of LVNC includes various conditions that can present with similar echocardiographic features.
Conditions to Consider:
- Dilated Cardiomyopathy: This condition is characterized by an enlarged left ventricle, which can lead to a similar appearance on echocardiography as LVNC [4].
- Hypertrophic Cardiomyopathy (HCM): HCM can present with apical hypertrophy, which may be mistaken for the trabeculations seen in LVNC [3][5].
- Restrictive Cardiomyopathy: This condition is characterized by a stiff left ventricle that cannot relax properly during diastole, leading to similar echocardiographic features as LVNC [4].
- Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC): ARVC can present with right ventricular dysfunction and similar echocardiographic features as LVNC [5].
Other Considerations:
- Apical Hypertrophic Cardiomyopathy: This condition is a variant of HCM that presents with apical hypertrophy, which may be mistaken for the trabeculations seen in LVNC [7].
- False Tendons: False tendons are fibrous structures that can mimic the appearance of trabeculations on echocardiography and should be considered in the differential diagnosis of LVNC [2].
Clinical Manifestations:
LVNC typically presents with symptoms such as shortness of breath, fatigue, and palpitations. The clinical manifestations of LVNC can vary depending on the underlying cause and the severity of the condition.
References:
[1] DP Singh et al. (2022) - Differential Diagnosis of LVNC includes processes that can appear as increased left ventricular wall thickness [1]. [2] Jun 26, 2012 - Differential diagnosis with thrombi, false tendons, apical hypertrophic cardiomyopathy, fibroma, obliterative processes, intramyocardial [2]. [3] F Zuccarino et al. (2015) - Pitfalls and Differential Diagnosis of LVNC can be challenging, including dilated cardiomyopathy, the apical form of hypertrophic [3]. [4] Nov 7, 2024 - Differential diagnosis includes dilated cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy, arrhythmogenic right ventricular [4]. [5] J Llerena-Velastegui et al. (2024) - The differential diagnosis of LVNC is broad, including other cardiomyopathies such as dilated cardiomyopathy, hypertrophic cardiomyopathy [5]. [6] May 5, 2022 - By definition, isolated LVNC occurs in the absence of other cardiac or noncardiac congenital abnormalities [6]. [7] E Oechslin et al. (2011) - The differential diagnosis can be challenging and includes apical form of hypertrophic cardiomyopathy, a combination of both apical hypertrophic cardiomyopathy [7].
Additional Differential Diagnoses
- Apical Hypertrophic Cardiomyopathy
- False Tendons
- arrhythmogenic right ventricular cardiomyopathy
- hypertrophic cardiomyopathy 1
- restrictive cardiomyopathy 1
- dilated cardiomyopathy
- dilated cardiomyopathy 2F
- hypertrophic cardiomyopathy 11
- dilated cardiomyopathy 1A
- dilated cardiomyopathy 1AA
- dilated cardiomyopathy 1G
- dilated cardiomyopathy 1E
- dilated cardiomyopathy 1B
- dilated cardiomyopathy 1U
Additional Information
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- disease_ontology
- oboInOwl#id
- DOID:0060480
- core#notation
- DOID:0060480
- oboInOwl#hasDbXref
- ORDO:54260
- IAO_0000115
- An intrinsic cardiomyopathy characterized by distinctive (spongy) morphological appearance of the LV myocardium.
- oboInOwl#hasExactSynonym
- left ventricular hypertrabeculation
- rdf-schema#label
- left ventricular noncompaction
- rdf-schema#subClassOf
- http://purl.obolibrary.org/obo/DOID_0060036
- oboInOwl#inSubset
- http://purl.obolibrary.org/obo/doid#DO_rare_slim
- relatedICD
- http://example.org/icd10/I42.8
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- http://www.w3.org/2002/07/owl#Class
- rdf-schema#domain
- https://w3id.org/def/predibionto#has_symptom_1505
- owl#annotatedSource
- t341079
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