Lenegres Disease, also known as LEH (Lenègre’s hypertrophic cardiomyopathy), is a rare heart condition characterized by thickening and fibrosis of the left ventricle. It was first described by a French cardiologist named Paul Lenegre in 1963. The thickening and scarring of the heart muscle makes it harder for the heart to pump blood effectively.
Causes And Risk Factors
The exact cause of Lev’s disease is unknown, but it is believed to be related to aging processes in the heart. Some potential risk factors include:
– Age – Lev’s disease usually affects middle-aged to elderly individuals, typically ages 50-70. It is very rare in younger people.
– Gender – Men are more likely to develop Lev’s disease than women by about a 3:1 ratio.
– Family history – In some cases, there may be a genetic component, as Lev’s disease has been reported to run in families on rare occasions. However, no single gene has been identified as causative.
– Other factors like smoking, high blood pressure, obesity, and high cholesterol levels may also potentially increase risk, but their roles are not well understood.
Symptoms
In the early stages of Lenegres Disease, people often do not experience any noticeable symptoms. As the condition progresses, common symptoms can include:
– Shortness of breath or breathlessness during physical exertion or when lying down. This is often the first noticeable symptom.
– Fatigue or weakness.
– Chest pain or discomfort, especially with exertion.
– Dizziness or fainting.
– Palpitations or abnormal heartbeats.
– Swelling in the legs, ankles, or abdomen from fluid buildup.- Irregular pulse.
Diagnosis
If a person experiences symptoms suggestive of Lenegres Disease, their doctor will perform a physical exam and ask about their medical history and symptoms. Diagnostic tests that may be used include:
– Electrocardiogram (ECG) – May show signs of left ventricular hypertrophy.
– Echocardiogram – Reveals thickened heart muscle and an abnormal left ventricle shape. Considered the gold standard test.
– Cardiac MRI – Provides detailed images of the heart that can help distinguish Lev’s disease from other types of hypertrophic cardiomyopathy.
– Cardiac catheterization – Involves inserting a thin tube into the heart and can assess heart pressures and rule out blockages in the coronary arteries.
Treatment And Management
Unfortunately, there is no cure for Lev’s disease, as the changes to the heart muscle are permanent. Treatment focuses on symptom management and preventing progression. Lifestyle modifications are recommended such as quitting smoking, following a heart-healthy diet, losing weight if overweight, limiting alcohol intake, and managing stress.
Medications May Include:
– Diuretics to help eliminate excess fluid from congestive heart failure.
– Beta-blockers to help slow the heartbeat and reduce workload on the heart.
– Angiotensin Converting Enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) to reduce blood pressure and protect the kidneys.
– Calcium channel blockers as another option to lower blood pressure.
More advanced procedures such as cardiac resynchronization therapy (CRT), an implantable defibrillator (ICD), or heart transplantation may be needed in severe cases with worsening symptoms not controlled by medications. Regular monitoring by a cardiologist is also important.
Prognosis
The prognosis for people with Lenegres Disease varies significantly depending on the severity of symptoms and how quickly the condition progresses. Many patients remain stable for years on medication. Those with mild symptoms have a relatively normal life expectancy.
However, in cases with worsening heart failure, the 5-year mortality rate approaches 40-50%. Risk factors for poorer outcomes include older age, male gender, presence of heart failure symptoms, and associated coronary artery disease. With proper management, many people can maintain good quality of life despite having Lev’s disease.
Lenegres Disease is a rare type of non-obstructive hypertrophic cardiomyopathy defined by thickening and fibrosis of the left ventricle. It usually develops slowly over many years. While there is no cure, treatments aimed at symptom relief and prevention of progression can successfully manage the condition for most patients. With medical care and lifestyle modifications, individuals with Lev’s disease can live healthy and fulfilling lives.
*Note:
1. Source: Coherent Market Insights, Public sources, Desk research
2. We have leveraged AI tools to mine information and compile it
About Author - Alice Mutum
Alice Mutum is a seasoned senior content editor at Coherent Market Insights, leveraging extensive expertise gained from her previous role as a content writer. With seven years in content development, Alice masterfully employs SEO best practices and cutting-edge digital marketing strategies to craft high-ranking, impactful content. As an editor, she meticulously ensures flawless grammar and punctuation, precise data accuracy, and perfect alignment with audience needs in every research report. Alice's dedication to excellence and her strategic approach to content make her an invaluable asset in the world of market insights. LinkedIn