Hypertrophic Obstructive Cardiomyopathy Flashcards
Causes
Form of cardiomyopathy that may cause cause death both acutely and chronically
Young athlete: sudden cardiac death most commonly due to arrythmia (ventricular tachyarrythmias)
can also be due to outflow tract obstruction while most common in young athletes can present at any age
Chronic: progressive heart failure most commonly takes form of diastolic filling defects can also be due to systolic dysfunction
Types
Separated into obstructive and nonobstructive subtypes
Obstructive disease - about 70% of patients characterized by >30 mmHg gradient in the left ventricular outflow tract
Non-obstructive - smaller gradient across outflow tract
Genetics
50% of cases are are familial (inherited)
Inherited as autosomal dominant trait due to mutations in myocyte contractile elements
Symptoms
Like other cardiomypathies, may present with
Acute sudden symptoms
Slow, chronically-progressing symptoms
Symptoms include
Chest pain
Dizziness
Syncope
Lightheadedness
Dyspnea
Physical Exam
Typical signs of CHF
Obstructive type associated with preload dependent maneuvers
Increased murmur intensity with valsalva
Decreased murmur intensity with hand grip due to changing gradient across outflow tract
Evaluation
Echocardiography diagnostic gold standard may show
Asymmetrically thickened LV walls
Thickening of the intraventricular septum
Banana-like LV chamber
May show normal EF
Differential
Other cardiomyopathies, and other causes of CHF
Treatment
Medications to improve cardiac function
Medications include
Beta-blockers
CCBs
Disopyramide
Avoid certain medications including
Inotropes (i.e. digoxin)
Vasodilators
Overdiuresis
Prognosis
Excellent in some people who remain asymptomatic for life
Poor in others, resulting in sudden death
Prevention
Screen close relatives
Treat known hypertension to reduce risk of sudden death
Complications
Dilated cardiomyopathy
CHF
Arrhythmias
Syncope-related injury
