Lenegres Disease, also known as Lev’s disease, is a rare cardiac condition characterized by fibrosis and degeneration of the Bundle of His and conduction pathways in the heart. This results in conduction disturbances and possible heart block. The disease was first described by French cardiologist Paul Lenegre in the 1960s.
Causes And Risk Factors
The exact cause of Lenegres Disease is unknown but it is believed to be related to fibrosis or thickening and hardening of heart tissues over time. Men are more commonly affected than women. Risk factors may include aging, high blood pressure, coronary artery disease or prior heart attacks. In some cases, it may be associated with other genetic conditions affecting connective tissues in the body.
Symptoms
The primary symptoms of Lev’s disease include dizziness or fainting episodes due to slowed heart rate. This occurs when electrical impulses traveling through damaged conduction pathways are impaired or blocked. Other symptoms may include fatigue, lightheadedness, chest pain and palpitations. Symptoms often worsen with exertion or stress. In advanced cases, it can cause complete heart block requiring a pacemaker.
Diagnosis
If a patient reports symptoms suggestive of conduction abnormalities, doctors will conduct a physical exam and take a detailed medical history. Testing may include an electrocardiogram (ECG) to check for abnormal heart rhythms and blocks in electrical conduction. A Holter monitor worn for 24-48 hours can detect intermittent conduction problems. An echocardiogram uses ultrasound to image the heart muscles and valves. A cardiac MRI may reveal scarring of the conduction system tissues. A coronary angiogram can rule out underlying coronary artery disease.
Treatment And Prognosis
Treatment focuses on managing the symptoms of Lenegres Disease. Medications may be used to slow the heart rate and control arrhythmias in mild cases. A pacemaker is often required for complete heart block or frequent fainting spells. Cardiac resynchronization therapy may benefit some patients. With appropriate treatment, most people can manage their symptoms well and live normal lifespans. However, in rare advanced cases, a heart transplant may be necessary. Regular follow-up with a cardiologist is important for monitoring the condition and adjusting therapies as needed.
Complications
Possible complications from longstanding Lev’s disease include further deterioration of conduction pathways leading to complete heart block. This can cause hemodynamic instability and potential cardiac arrest without a pacemaker. Other risks include heart failure due to loss of normal synchronized heart contractions over time.
Prognosis And Outlook
With proper treatment and management, many individuals with Lenegres Disease have a normal life expectancy. Symptoms can often be well-controlled with medications and/or a pacemaker. Regular follow-up care is important for monitoring the condition and adjusting therapies if the conduction defects worsen. While it cannot be cured, treatment aims to preserve quality of life by alleviating symptoms and minimizing risks of further complications. With new research, greater understanding of this rare condition’s causes and more individualized therapies, the prognosis for people with Lev’s disease continues to improve.
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1. Source: Coherent Market Insights, Public sources, Desk research
2. We have leveraged AI tools to mine information and compile it