Pathology of hypertrophic cardiomyopathy

2021-01-28 12:00 AM

Questioning helps determine personal family history (sudden death, age of parental death). Suggestive signs such as angina may be detected

Define

Obstructive cardiomyopathy (BCTTN) is an enlarged all or part that obstructs the systolic ejection line. The most common form of septal hypertrophy and is caused by an obstruction of the left ventricle chamber.

Reason

Genetics: In 55% of cases, the dominant chromosome may be caused by:

Disorders of the catecholamines.

Long-term elevation of cellular calcium causes heart and blood vessel muscle dysfunction.

In 45% of cases: The disease occurs alone and with no known cause (2/3 men).

Sometimes you are born with no family origin.

Pathophysiology

Left ventricular systolic function: disorders and decreased left ventricular ejection activity, through which there are 2 explanations:

The gradient in the left ventricle is a consequence of hyperactive left ventricular premature emptying. 75% of systolic blood flow is expelled in the first third of systole. Anterior mitral valve (SAM) movement is only a result of left ventricular twisting.

Anterior displacement of the mitral valve (Venturi efficiency) due to an increase in speed through a reduced volume thrust chamber.

Left ventricular diastolic dysfunction:

Abnormalities of left ventricular dilation and filling.

The above disorders may improve with calcium inhibitors (Verapamil).

Anterior valve leaf movement during hypertrophic cardiomyopathy systole.

Implementing the quadrants

Detection situation: Occurs in young people, average age: 25-30 years, no hypertension, asymmetric wall hypertrophy

Systemic examination: (cardiopulmonary) because the patient usually tolerates well in some half of cases.

Functional signs: (nonspecific) detected by questioning.

Shortness of breath, exertion, nervousness, tachycardia, and atypical chest pain occur with exertion.

Questioning helps determine personal family history (sudden death, age of parental death). Suggestive signs such as: angina (30%), fainting, fainting (25%) but pulmonary oedema, an arrhythmia is less common.

Physical examination:

Normal blood pressure.

Palpation of the anterior heart area is marked with a tip, left.

Cardiopulmonary: Systolic (90% of cases) type of ejection between the systole, in the middle of the heart, spreads out the tip, the intensity changes over time, increased during exercise, ventricular ectopic, during the test Valsalva, reduced on inhalation, normal heart sounds, has normal T4. There was a complete systolic blowing due to a mitral regurgitation with decreased intensity when inhaled Nitrite d 'Amyle or during the Valsalva procedure.

ECG:

Heart rate: Usually sinus rhythm, in addition to atrial fibrillation (5-10%), ventricular arrhythmia.

Left ventricular hypertrophy (60%), sometimes 2 ventricles.

Angioplasty: Strong Q wave (<0.04 "), deep (50%) in V5, V6, D1, aVL.

Location and nature of murmurs in hypertrophic cardiomyopathy

Sometimes there is a wide Q wave, suggestive of infarction, easily mistakenly treated when there is angina.

Location and nature of murmurs in hypertrophic cardiomyopathy

Common repolarization disorder with ST depression, negative T waves.

Conduction disorder: Bloc is not completely left-branching; block of left-branching is anterior.

Chest X-ray:

The heart is normal, but when the balloon is large, it is necessary to find a double-leaf opening with left atrial dilatation. The aorta is small, translucent, and is not calcified.

Cardiogram:

Limited value. May help to differentiate ejection bleed from the blower. Help find the horse galloping and T2 split paradox.

Echocardiography:

2-sided ultrasound:

Hypertrophic properties: Abnormal density in the wall, distribution of hypertrophy, damage coordinated with the right ventricle.

Clogging mechanism: Deviation of the 2-leaf valve, twisting left ventricle.

Cardiac chamber size (dilated left atrium).

Normal recovery index usually increases.

Heart valve condition: S u move valve 2 leaves first.

The pericardium is normal.

Anterior systolic mitral valve (SAM) motion.

Early mid-systolic closure of a "butterfly" type aural valve. However, these 3 marks are infrequent and nonspecific.

Cardiac Doppler:

Color Doppler: Shows an increase in the rate in midterm left ventricular ejection chamber, combined mitral regurgitation.

Vascular doppler: Shows special variations of left ventricular filling.

Continuous Doppler: High value and allows to determine maximum gradient in the left ventricular chamber, mitral aperture and pulmonary artery pressure.

Other specific tests:

To support preoperative treatment or when there is a suspicion of coordinated coronary injury such as information technology, coronary angiography.

Measure 24-48 continuous EKG Holter.

High amplified electrocardiogram finds a slow voltage.

Electrocardiogram when too tight narrow.

Radioisotopes by Thallium, IRM ... rarely used.

Evolution

Heart failure.

Arrhythmia: atrial fibrillation, atrial extrasystole, ventricular ectopic, ventricular tachycardia

Other complications alternated: endocarditis, an embolism accident.

Sudden death due to tachycardia, ventricular obstruction, accounting for 2-3%.

Special clinical form

Hypertrophic cardiomyopathy does not have a gradient in the left ventricle and requires the use of pharmacological tests, based on family history, for treatment.

Muzzle: have a milder prognosis, seen in men at the average age of 35, have negative T waves, giant deep before the heart region.

Hypertrophic cardiomyopathy in older hypertensive people.

Previous hypertension has no family history of obstructive cardiomyopathy, and its prognosis is better with early treatment.

Differential diagnosis

clinical

Aortic Stenosis: Based on heart tone and ultrasound characteristics.

Bilateral cleft: Or a combination of obstructive cardiomyopathy, based on Doppler's treatment. Other: with ventricular opening, pulmonary stenosis.

ECG.

Need to distinguish Q waves in coronary failure, if needed angiography.

Supersonic

When there is left ventricular hypertrophy, ultrasound needs to find the cause (hypertension, aortic stenosis).

There may be a combination of obstructive cardiomyopathy and hypertensive heart disease.

Treatment

Drugs: Beta-blockers, calcium inhibitors, disopyramide, amiodarone.

Surgical treatment.

Treatment of complications such as pulmonary oedema, atrial fibrillation, ventricular arrhythmias.

Preventive antibiotics.

Obstructive cardiomyopathy has symptoms in young people

Medical treatment is primarily a beta-blocker (and/or verapamil).

Surgery needs to discuss when the pressure in the left ventricle is above 50mm in the basic state.

Hypertrophic cardiomyopathy in elderly hypertensive patients

The goal of treatment is to control hypertension, reduce left ventricular volume.

Medical treatment is the main thing, using drugs in increasing doses, monitoring the response by ultrasound.

Treatment of complications:

Acute pulmonary edema: Use a diuretic, a blocker ((celiprolol, propranolol) and / or verapamil.

Atrial fibrillation: Electric shock, digital, antiarrhythmic type IC, Amiodarone, anti-vitamin K (used only for chronic atrial fibrillation and dilated left atrium).

Ventricular arrhythmias: Treatment usually less effective, can be used alone or in combination amiodarone blockers β and Verapamil.

Preventive antibiotics:

When applying the procedure to prevent infectious endocarditis.