The 2023 ESC Guidelines for the Management of Cardiomyopathy have made a new description of the classification of cardiomyopathy, and according to morphological and functional characteristics, cardiomyopathy is mainly divided into five types: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), non-dilated left ventricular cardiomyopathy (NDLVC), arrhythmogenic right ventricular cardiomyopathy (ARVC) and restrictive cardiomyopathy (RCM).
Hypertrophic cardiomyopathy (HCM) is a myocardial disease of unknown etiology, characterized by asymmetrical hypertrophy of the ventricular wall, smaller intraventricular cavity, obstruction of left ventricular blood filling, and decreased diastolic compliance of the left ventricle.
Systolic and diastolic dysfunction, as well as a smaller chamber of the ventricle, can hold less blood, causing a range of symptoms.
Causes of hypertrophic cardiomyopathy:
50%-55% are hereditary: the disease is hereditary, autosomal dominant.
Calcium regulation disorders: Clinical studies and experiments have demonstrated that patients with hypertrophic cardiomyopathy have calcium regulation disorders, especially the elevated calcium concentration in myocardial cells is related to ventricular diastolic function.
The main symptoms of hypertrophic cardiomyopathy:
Dyspnea on exertion: the most common symptom, mainly due to elevated left ventricular diastolic filling pressure and elevated left ventricular pressure returning to the pulmonary circulation.
Dull pain in the precordial area: about 40% of patients with HCM have uncomfortable chest pain and do not respond well to nitroglycerin but have normal coronary angiography.
Palpitations: associated with decreased cardiac function and arrhythmias. Patients with HCM often have arrhythmias.
Syncope: 1 3 patients may experience syncope after sudden standing and exercise, which resolves spontaneously in a moment, often during exercise in children and young adults.
Sudden death: is one of the leading causes of sudden death in adolescents and athletes.
Heart failure: symptoms of left and right heart failure may occur in advanced stages.
Dizziness: often worse with exertion.
Diagnosis and process of hypertrophic cardiomyopathy: In the "2023 Heart Health Day Proposal", a number of experts, including Academician Ge Junbo and Professor Fan Huimin, jointly proposed to promote the "five early systems" of cardiovascular diseases: early prevention, early screening, early control, early **, early ** and full management.
Laboratory tests:
1) Biological markers: B-type natriuretic peptide NT-PROBNP and cardiac troponin and markers reflecting inflammation, fibrosis and cell death: ECG of HCM patients can show ST-T changes and abnormal Q waves. More than half of HCM patients have different levels of serum cardiac troponin, which is related to cardiac function.
2) Blood routine and blood biochemical examination: blood routine, blood biochemical index, iron metabolism index, etc. should be used as routine examination items for HCM patients.
ECG:
Routine ECG, Holter testing. More than 90% of patients with hypertrophic cardiomyopathy have ECG changes.
Echocardio**:
The thickness of the ventricular wall, the size of the ventricular cavity, and the presence and severity of outflow tract obstruction can be accurately measured.
Hypertrophic cardiomyopathy**:
Hypertrophic cardiomyopathy is based on drugs, interventional or surgical procedures if necessary.
Lifestyle interventions.
Because HCM is a common cause of sudden death in adolescents and athletes, experts recommend that HCM should avoid strenuous exercise, but patients with HCM generally lack exercise to prevent the aggravation of myocardial hypertrophy and sudden deathOn the other hand, a decrease in cardiorespiratory fitness is one of the markers of adverse events in HCM patients;Therefore, we should develop good living habits, regular physical examinations, maintain an optimistic and positive attitude, moderate and medium-intensity exercise, avoid strenuous exercise, improve cardiopulmonary function, and improve physical function.
Drugs**. There is no specific drug for hypertrophic cardiomyopathy in China. At present**, the main focus is to improve symptoms, reduce complications and prevent the occurrence of sudden death.
Alleviate left ventricular outflow tract obstruction: Receptor blockers or non-dihydropyridine calcium-channel blockers improve ventricular relaxation and increase ventricular diastolic filling time.
For heart failure**: diuretics, ace inhibitors, receptor blockers, reduce water and sodium retention, and reduce cardiac workload.
For atrial fibrillation: amiodarone (paroxysmal atrial fibrillation works well).
Interventional & Surgical**.
For obstructive cardiomyopathy, if the drug does not achieve a good effect, it is recommended to carry out interventional or surgical surgery in time, which can effectively reduce symptoms, improve the survival rate of patients, and reduce the sudden death rate, so as to achieve a better effect.
Percutaneous septal myocardial chemical ablation: This intervention is to inject alcohol into the ventricular septal branch of the coronary artery to cause necrosis of part of the myocardium and reduce myocardial hypertrophy.
Radiofrequency ablation of the ventricular septum: radiofrequency ablation is performed on the endocardial surface to target the site where the obstruction is most pronounced**.
Partial ventricular septal myocardial resection: For patients who are ineffective with drugs, part of the myocardium can be removed through this surgery, local hypertrophy can be improved, and symptoms such as poor breathing and shortness of breath can be effectively improved, but compared with other surgeries, this operation is more invasive and risky.
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