What Is Idiopathic Cardiomyopathy?

Idiopathic cardiomyopathy mainly refers to cardiomyopathy caused by hereditary factors, and also includes mixed cardiomyopathy caused by both hereditary and non-hereditary factors. Patients often present with palpitations, shortness of breath, dyspnea, and edema, and in severe cases, syncope or sudden cardiac death may occur. Treatment options include drug therapy, ablation therapy, device therapy, and surgical treatment. The prognosis is related to the related genes, the severity of cardiac function and arrhythmia, and treatment factors.

Basic Information

Visiting department

cardiology

Whether inherited

Yes

Disease characteristics

Heart palpitations, shortness of breath, dyspnea, and edema. In severe cases, syncope or sudden cardiac death may occur.

Causes of Idiopathic Cardiomyopathy

Idiopathic cardiomyopathy is mostly autosomal dominant, but also inherited by autosomal recessive inheritance, mitochondrial inheritance, and X-linked inheritance. The disease can be divided into familial dilated cardiomyopathy (FDCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), restricted cardiomyopathy (RCM), left ventricular myocardial densification (LVNC), and hereditary transthyroidism And other types of amyloid cardiomyopathy (ATTRm).

Clinical manifestations of idiopathic cardiomyopathy

Idiopathic cardiomyopathy can cause arrhythmia, heart failure, thromboembolism and corresponding symptoms, such as dizziness, palpitations, chest tightness, dark Mongolia, dyspnea, loss of appetite, abdominal distension, edema, pain in the embolism area, etc., and severe heart disease Sudden death.

Idiopathic cardiomyopathy

X- ray inspection

The heart can appear generally enlarged, spherical, with an X-ray appearance similar to the pericardial effusion; or the left ventricle, left atrium, or right ventricle may be enlarged, or there may be no enlarged heart. Patients may also have pulmonary congestion and pulmonary interstitial edema, and pleural effusion is also common.

2. ECG examination

An electrocardiogram can detect arrhythmias, as well as changes in atrial or ventricular enlargement.

3. Echocardiography

Echocardiography can observe the size, structure, and function of the heart. It can be found that the wall thickens, the heart cavity enlarges, and the wall motion is abnormal. There can also be ventricular diastolic dysfunction, endocardial thickening, ventricular occlusion and other manifestations.

4. Heart failure marker detection

Heart failure markers include brain natriuretic peptides and N-terminal brain natriuretic peptide precursors, both of which are elevated, suggesting the presence of heart failure in patients.

5. Genetic testing

Genetic testing finds related gene mutations to help confirm the diagnosis, and also to determine the patient's typing and prognosis based on the type of genetic mutation.

6. Endocardial myocardial biopsy

Endocardial myocardial biopsy can observe the pathological changes of endocardium and myocardium, and provide a reference for diagnosis and differential diagnosis.

Idiopathic cardiomyopathy diagnosis

1. According to the patient's clinical manifestations, biochemical indicators, electrocardiogram, echocardiogram and other imaging examinations, a certain type of cardiomyopathy can be initially diagnosed.

2. Exclude the causes that may cause this type of cardiomyopathy, such as ischemia, infections, abnormal hemodynamics, systemic inflammation, immune or metabolic diseases.

3. Endocardial myocardial biopsy and genetic testing can help further define the diagnosis.

Idiopathic cardiomyopathy treatment

The treatment principles of idiopathic cardiomyopathy are to improve cardiac remodeling and heart failure, control arrhythmias, and prevent embolism and sudden death.

Drug treatment

(1) Patients with heart failure can be treated with diuretics, cardiotonics, vasodilators, beta-blockers, and renin-angiotensin-aldosterone system antagonists. Anticoagulation therapy can be performed if necessary.

(2) Patients with hereditary transthyretin amyloid cardiomyopathy can be treated with clozaconic acid.

2. Surgical treatment

Patients with poor drug treatment and severe illness may consider heart transplantation to improve prognosis and quality of life.

3. Other treatments

(1) Ablation treatment is feasible for patients with severe arrhythmia.

(2) Patients at high risk of sudden death can be implanted with cardioversion defibrillator or cardiac resynchronization therapy.

Idiopathic cardiomyopathy prognosis

The prognosis is related to the patient's mutant genes, clinical cardiac function, comorbidities, and treatment.

Idiopathic cardiomyopathy prevention

Genetic testing and prenatal testing are helpful to assess the risks associated with patients and family members. It is recommended to go to a professional genetic evaluation center for genetic counseling and genetic testing.