Inherited Cardiac Conditions Flashcards
what are the 4 types of cardiomyopathy
Hypertrophic
Dilated/Idiopathic
Arrhthmogenic Right Ventricular
Restrictive
what are signs of HCM
forceful and jerky pulse
4th heart sound
Mid systolic murmur
what are the signs of RCM
rapid x and y descent of JVP
3rd and 4th heart sound
hepatomegaly
ascites
what are the signs of HCM on ECG
LVH
Progressive T wave inversion
deep Q waves
how is RCM diagnosed
Cardiac catherterization
what are the pathological effects of cardiomyopathy
LVOT obstruction
Reduced ejection fraction
Mitral regurgitation
Rhythm disturbances
what are the histological features of dilated cardiomyopathy
- myocardial cell hypertrophy increased length without increased diameter
- increased interstitial fibrosis.
what are signs of dilated cardiomyopathy
Increased systolic and diastolic volume with low Ejection Fraction
S3
Gallop rhythm
what happens in ARVC
Heart muscle is replaced by fibrous and fatty tissue - RV
what are signs of ARVC
- ventricular arrhythmias of RV origin,
- sudden death
what are ECG characteristics of ARVC
- T-wave inversion beyond lead V1
- QRS duration in V1 > or = 110 msec
- a longer QRS duration in the right then left precordial leads.
what is RCM
walls of the ventricles become stiff, but not necessarily thickened abnormal filling with blood
what are physiological effects of RCM
Reduced EDV, normal ESV, increased Left ventricular filling pressure, normal EF
where is mutations seen in HCM
- sarcomeric genes
- mitochondrial DNA
where are mutations seen in ARVC
cardiac ryanodine receptor
what drugs are used in the treatment of CM
ACE Inhibitors Angiotensin Receptor Blockers (ARB) Beta-Blockers and Rate Limiting Calcium Antagonists Diuretics Anticoagulation Anti arrhythmics
what is the most common arrhthymia linked with sudden unexpected death
Long QT syndrome
what is LQTS
congenital disorder
syncope, seizures, sudden death
what is the average QT prolongation
> 440ms in males
> 460ms in females
what is Torsades de pointes
distinctive polymorphic ventricular tachycardia
the QRS amplitude varies
QRS complexes twist around the baseline
what are the ECG changes seen in Brugada syndrome
A.Fib
ST elevation and RBBB in V1-V3
what is structurally normal heart
when no cause for sudden death is found
Arrhythmia is the presumed mode of death
what are the features of Patent Ductus Arteriosus
Continous “machine-like” murmur
Collapsing pulse
Wide pulse pressure
Heaving apex beat
what are the features of a Ventricular Septal Defect
Pan-systolic murmur
systolic thrill and/or left parasternal heave
what are complications of VSD
Aortic regurgitation
Infective endocarditis
Right heart failure
what is coarctation of the aorta
narrowing of the descending aorta
what are features of coarctation of the aorta
Radio-femoral delay
Mid or Late systolic murmur that is maximal over back
Notching of the ribs
what is coarctation of the aorta associated with
Turner’s syndrome
Bicuspid aortic value
Berry aneurysms
what are the characteristics of Tetralogy of Fallot (TOF)
- Ventricular Septal Defect
- Right ventricular hypertrophy
- Right ventricular outflow tract obstruction + Pulmonary stenosis
- Overriding aorta
what are the features of TOF
- cyanosis
- right-to-left shunt
- ejection systolic murmur
what is the CXR presentation of TOF
‘boot-shaped’ heart
