cardiomyopathy Flashcards
types of cardiomyopathy - which is most common? worst?
DCM - 60% HOCM RCM - worst, transplantation within years arrhythmogenic non-compaction
causes of DCM
1) idiopathic (50%)
2) myocarditis (40%)
3) genetic (35%) - neuromuscular, mitchondrial
4) tachycardia
5) cardiotoxics
6) endocrine
major complications of DCM
arrhythmias
emboli
CCF
most common gene mutation involved in DCM
TTN (titin)
most common viral causes of DCM
adenovirus, enterovirus, CMV, influenza
doxorubicin causes what kind of cardiomyopathy?
DCM - non-linear dose related (cumulative 400-500mg/m2
most common cause of SCD in paeds
HOCM
genetic causes of HOCM
- % of HOCM cases
- age of presentation
- ECG vs hypertrophy as first sign of HOCM
- 30-60% cases of HOCM
- depends on mutation - some in childhood, some later
- ECG may precede the hypertrophy!
HOCM - most associated syndrome
Noonan’s
what is the LVOT in HOCM dependent on?
LV volume! - less volume > inc LVOT
causes of RCM
- Most idiopathic
- Primary – Endomyocardial fibrosis, Loeffler’s, primary RCM (genetic)
- Systemic infiltrative disease – scleroderma, amyloidosis, sarcoidosis
- Metabolic – GSD, Fabry’s disease, haemochromatosis
- Malignancies or radiation therapy
RCM - what is the abnormality
Diastolic dysfunction with normal ventricular size and systolic function
Only the atria are markedly dilated (biatrial enlargement) – characteristic finding
Arrhythmogenic RV cardiomyopathy - hallmarks
Myocardium of the RV is partially or totally replaced by fibrous or adipose tissue
echo - systolic bulging pathognomonic
classic mcq - baby presents at 12 weeks with heart failure - ?2 major causes
VSD
ALCAPA
what med is CI in HOCM
digoxin - increases the obstruction by increasing contractiliy
