Hypertrophic obstructive cardiomyopathy Flashcards
What is cardiomyopathy?
Cardiomyopathy refers to disorders of the heart muscle.
‘Cardio-‘ refers to the heart, ‘myo-‘ refers to muscle, and ‘-pathy’ refers to disorder or disease.
What is hypertrophic obstructive cardiomyopathy (HOCM)?
HOCM is a condition where the left ventricle becomes hypertrophic, leading to thickening of the muscle, especially in the septum, causing obstruction of blood flow.
What are the key risks associated with HOCM?
HOCM is associated with increased risks of:
* Heart failure
* Myocardial infarction
* Arrhythmias
* Sudden cardiac death (often during exertion)
What is the genetic cause of HOCM?
HOCM is an autosomal dominant genetic condition caused by a defect in genes for sarcomere proteins. It occurs in about 1 in 500 people.
What are the common symptoms of HOCM?
Symptoms include:
* Asymptomatic in most patients
* Shortness of breath
* Fatigue
* Dizziness
* Syncope
* Chest pain
* Palpitations
* Severe cases may lead to heart failure symptoms like cough, orthopnoea, and oedema.
What examination findings are commonly seen in HOCM?
Examination findings include:
* Ejection systolic murmur (louder with Valsalva manoeuvre)
* Fourth heart sound
* Thrill at the lower left sternal border
* Atrial fibrillation (irregular pulse)
* Mitral regurgitation (pan-systolic murmur)
* Heart failure signs
What are the investigations used to diagnose HOCM?
Investigations include:
* ECG: May show left ventricular hypertrophy.
* Chest x-ray: Usually normal, may show pulmonary oedema in heart failure.
* Echocardiogram or cardiac MRI: Used to establish diagnosis.
* Genetic testing: Can identify the affected genes.
What are the treatment options for HOCM?
Treatment options depend on severity and symptoms, including:
* Beta blockers
* Surgical myectomy (removal of obstructive heart muscle)
* Alcohol septal ablation (minimally invasive procedure)
* Implantable cardioverter defibrillator (for those at risk of sudden cardiac death or arrhythmias)
* Heart transplant (in severe cases)
What lifestyle advice is given to HOCM patients?
Patients should avoid:
* Intense exercise
* Heavy lifting
* Dehydration
* ACE inhibitors and nitrates are avoided as they can worsen LVOT obstruction.
What are the outcomes and complications of HOCM?
Outcomes include:
* Minimal symptoms and normal lifespan (in most patients)
* Arrhythmias (e.g., atrial fibrillation)
* Mitral regurgitation
* Heart failure
* Sudden cardiac death
What is dilated cardiomyopathy?
Dilated cardiomyopathy is a condition where the heart muscle becomes thin and dilated. It can be genetic or secondary to other conditions (e.g., myocarditis).
What is alcohol-induced cardiomyopathy?
Alcohol-induced cardiomyopathy is a type of dilated cardiomyopathy caused by long-term alcohol use.
What is restrictive cardiomyopathy?
Restrictive cardiomyopathy is when the heart becomes rigid and stiff, impairing ventricular filling during diastole.
What is arrhythmogenic cardiomyopathy?
Arrhythmogenic cardiomyopathy is a genetic condition where heart muscle is replaced with fibrofatty tissue, increasing the risk of ventricular arrhythmias and sudden cardiac death.
What is Takotsubo cardiomyopathy (Broken Heart Syndrome)?
Takotsubo cardiomyopathy is a condition involving rapid onset of left ventricular dysfunction, often triggered by severe emotional stress. It usually resolves spontaneously.
