Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy is an inherited heart muscle disease medical science distinguishes between an obstructive and a non-obstructive form. Patients with the nonobstructive form are often involuntary for a long time or even for life.

What is hypertrophic cardiomyopathy?

The group of cardiomyopathies summarizes diseases of the heart muscle. Cardiomyopathies are associated with mechanical or electrical dysfunction of the heart. However, these are not necessarily associated with pathologically altered ventricles. This disease is a congenital disease of the heart muscle. In addition to asymmetric thickening of the left ventricle, the disease is associated with the separation of the ventricles. With its prevalence, familial hypertrophic cardiomyopathy is a relatively common heart disease. Inheritance is in autosomal dominant mode. Two forms of heart disease can be distinguished: hypertrophic cardiomyopathy with dynamic obstruction and a form without dynamic obstruction. The disease was described by Liouville and Holopao in the mid-nineteenth century. It has been recognized as a clinical entity since Brooke's description in the 20th century.

Causes of hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy is caused by genetic inheritance. All proteins code for functional genes in the cardiac regime. In more than 50 percent of cases, the genetic defect is in the structure of the so-called beta-myosin heavy chain. Structurally different point transitions in β-myosin and α-tropomyosin result in structural changes in proteins such as myosin-binding protein C or troponin-T. Hypertrophic cardiomyopathy is also called Sarokrem's disease. Most point mutations affecting the gene MYH7 at the locus on chromosome 14 cause branching disorders in hypertrophied cardiomyocytes in sarcoid. Due to the growth of lateral branches, the parallel arrangement is absent. The interstitium is recreated by connective tissue. Phenotypic expression depends on individual variation fewer environmental factors and modifier genes The disease is usually silent until the age of 13 years.

Symptoms of hypertrophic cardiomyopathy

A quarter of patients suffer from myocardial thickening of the left ventricle, which is located in the extraocular tract. Hence, inhibition occurs during exercise or rest as well. This results in high pressure on the narrowing of the aorta and the left ventricle. Obstruction is co-ventricular in about ten percent of patients. As the muscle thickens, the muscles stiffen the ventricle thus filling to a limited volume during the flaccid phase and blood backs up into the pulmonary veins causing dyspnea. As the disease progresses due to forced pumping, muscle stiffness increases. A suction is created in the narrow outflow region, also known as the Venture effect. Thus, leakage in the sense of mitral regurgitation occurs frequently. Under stress, cardiac arrhythmias occur, which can cause brief damage or sudden cardiac death. Hypertrophic cardiomyopathy accounts for many sudden deaths in young adults. Patients with the nonobstructive form are often sensitive. When symptoms are present, they are sensory symptoms such as shortness of breath, dizziness, or laryngitis.

Hypertrophic cardiomyopathy diagnosis and disease progression

In the diagnosis of hypertrophic cardiomyopathy, the most important thing is to rule out reactive myocardial hypertrophy due to exercise (athlete's heart) or due to hypertension. Diseases of the aortic valve should be considered for the differential diagnosis. Physical examination reveals a systolic murmur that increases with patient exertion. This symptom is observed during the Valsalva maneuver. ECG ideally provides evidence of left ventricular hypertrophy by showing Q-spike and repolarization dysfunction. On echocardiography, in addition to septal hypertrophy, displacement of the mitral valve leaflet can be observed, restricting the outflow tract. The resting gradient is measured in terms of the pressure jump between the left ventricle and the aorta. Magnetic resonance imaging dramatically depicts the distribution patterns and patchy appearance of the myocardium in the lesion. It also provides visualization of flow acceleration and possible evidence of past septal embolization. Cardiac catheterization measures pressure on the heart to determine myocardial stiffness. Molecular genetic testing confirms the diagnosis. Many asymptomatic patients have rather mild weakness and therefore a good prognosis. Forms with left ventricular outflow tract obstruction often progress to heart failure and therefore have a worse pre-disease.

Complications of hypertrophic cardiomyopathy

Complications of hypertrophic cardiomyopathy arise from possible symptoms and findings. For example, cardiac arrhythmias can become dangerous, in which case it becomes necessary to take medication to treat the arrhythmias. In addition, the risk of sudden cardiac death, death from heart failure, and scarring are relatively high, making hypertrophic cardiomyopathy the most complex and severe form of all cardiomyopathies. Sudden cardiac death occurs in one percent of cases and affects young patients. Symptoms are usually mild, hypertrophic cardiomyopathy makes identification difficult. It is also noted that there is a high risk for family members suffering from the same disease. In older affected individuals, heart failure plays a greater role as the most common complication. As the disease progresses, the left external track becomes increasingly strained and may narrow. It can strengthen the heart muscle. A stiff ventricle results in atrial fibrillation, which affects 25 percent of patients. Overall, hypertrophic cardiomyopathy is a curable disease that runs its course without complications in most cases and has good treatment prospects. Life expectancy is not affected; This occurs only in advanced diseases.

Treatment and therapy of hypertrophic cardiomyopathy

Although hypertrophic cardiomyopathy is not curable to date, it can now be treated to remarkably good effect. Therefore, diagnosis improves before diagnosis. Conservative treatment measures include drugs such as beta-blockers to down-regulate the left ventricle. Antiarrhythmic drugs reduce cardiac arrhythmias. Patients are advised to avoid competitive sports and sports with sudden maximum emphasis. Interventional measures may include catheter treatment of septal hypertrophy. Treatment of septal hypertrophy is performed by trans coronary diffusion or percutaneous transluminal septal myocardial ablation. By means of cardiac catheterization, the interventricular ramus is occluded with an anterior balloon. When the gradient of the extraocular tract drops, pure alcohol is chased through the balloon and forced into the obstructed region, inducing an infarct.

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