Cardiomyopathy Flashcards
What is the pathology of hypertrophic obstructive cardiomyopathy (HOCM). (2)
Left ventricular hypertrophy, especially involving the septum.
Causes LV outflow obstruction from asymmetric septal hypertrophy.
There is varying degrees of myocardial fibrosis.
Is family history important for HOCM.
Yes.
Approximately 50% of patients have a positive family history.
What percentage of patients with HOCM have a positive family history.
Approximately 50%.
How many mutations have been associated with HOCM.
Multiple.
What other condition is strongly associated with HOCM.
Fredrich’s ataxia.
What are the symptoms of HOCM. (78)
Many are asymptomatic. Dyspnoea. Fatigue. Palpitations. Angina. Syncope. CCF. Sudden death (may be the first manifestation).
What are the clinical signs of HOCM. (7)
a wave in JVP. Double apical impulse. Ejection systolic murmur (harsh). Systolic thrill at lower left sternal edge. Pansystolic murmur at the apex. 4th heart sound. Jerky pulse.
What ECG changes are seen in a patient with HOCM. (6)
LV hypertrophy. Deep Q waves (inferior and lateral leads). Progressive T wave inversion. Arrhythmias (AF, WPW). Ventricular ectopics. VT.
What is the prevalence of HOCM.
0.2%.
What is the mode of inheritance of HOCM.
Autosomal dominant condition.
What percentage of HOCM cases are sporadic.
50%.
What should you ask a patient you suspect of having HOCM.
If there is a family history of sudden death.
What may be seen on the echo of a patient with HOCM. (4)
Asymmetrical septal hypertrophy.
Small LV capacity with hypercontractile posterior wall.
Midsystolic closure of aortic valve.
Systolic anterior momement of mitral valve.
What is the mortality of HOCM. (2)
5.9% if 14.
What are some poor prognostic factors for HOCM. (3)
What are the forms of cardiomyopathy. (3)
Dilated cardiomyopathy.
Hypertrophic cardiomyopathy.
Restrictive cardiomyopathy.
What are some causes of restrictive cardiomyopathy. (7)
Idiopathic. Amyloidosis. Haemochromatosis. Sarcoidosis. Scleroderma. Loffler's eosinophilic endocarditis. Endomycocardial fibrosis.
What is the presentation of restrictive cardiomyopathy. (5)
Presents like constrictive pericarditis.
Features of RVF predominate:
Raised JVP with prominent x and y descents.
Hepatomegaly.
Oedema.
Ascites.
What is dilated cardiomyopathy.
It is characterised by dilatation of the ventricular chambers and systolic dysfunction with preserved wall thickness.
What is restrictive cardiomyopathy.
A rare condition in which there is normal or decreased volume of both ventricles with bi-atrial enlargement, normal wall thickness, normal cardiac valves and impaired ventricular filling with restrictive physiology by near normal systolic function.
What is the prevalence of dilated cardiomyopathy.
1 in 2500.
0.2%.
What are the associations of dilated cardiomyopathy. (8)
Alcohol. Raised BP. Haemochromatosis. Viral infection. Autoimmune. Peri- or post partum. Thyrotoxicosis. Congenital (X linked).
What is the presentation of dilated cardiomyopathy. (7)
Fatigue. Dyspnoea. Pulmonary oedema. RVF. Emboli. AF. VT.
What are the physical signs of dilated cardiomyopathy. (11)
Raised pulse. Low BP. Raised JVP. Displacced diffuse apex. S3 gallop. Mitral or tricuspid regurgitation. Pleural effusion. Oedema. Jaundice. Hepatomegaly. Ascites.
What is the mortality of dilated cardiomyopathy.
Variable, 40% in 2 years.