Inherited Cardiac Conditions Flashcards

(31 cards)

1
Q

what are the 4 types of cardiomyopathy

A

Hypertrophic
Dilated/Idiopathic
Arrhthmogenic Right Ventricular
Restrictive

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2
Q

what are signs of HCM

A

forceful and jerky pulse
4th heart sound
Mid systolic murmur

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3
Q

what are the signs of RCM

A

rapid x and y descent of JVP
3rd and 4th heart sound
hepatomegaly
ascites

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4
Q

what are the signs of HCM on ECG

A

LVH
Progressive T wave inversion
deep Q waves

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5
Q

how is RCM diagnosed

A

Cardiac catherterization

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6
Q

what are the pathological effects of cardiomyopathy

A

LVOT obstruction
Reduced ejection fraction
Mitral regurgitation
Rhythm disturbances

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7
Q

what are the histological features of dilated cardiomyopathy

A
  • myocardial cell hypertrophy increased length without increased diameter
  • increased interstitial fibrosis.
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8
Q

what are signs of dilated cardiomyopathy

A

Increased systolic and diastolic volume with low Ejection Fraction
S3
Gallop rhythm

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9
Q

what happens in ARVC

A

Heart muscle is replaced by fibrous and fatty tissue - RV

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10
Q

what are signs of ARVC

A
  • ventricular arrhythmias of RV origin,

- sudden death

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11
Q

what are ECG characteristics of ARVC

A
  • T-wave inversion beyond lead V1
  • QRS duration in V1 > or = 110 msec
  • a longer QRS duration in the right then left precordial leads.
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12
Q

what is RCM

A

walls of the ventricles become stiff, but not necessarily thickened abnormal filling with blood


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13
Q

what are physiological effects of RCM

A

Reduced EDV, normal ESV, increased Left ventricular filling pressure, normal EF

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14
Q

where is mutations seen in HCM

A
  • sarcomeric genes

- mitochondrial DNA

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15
Q

where are mutations seen in ARVC

A

cardiac ryanodine receptor

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16
Q

what drugs are used in the treatment of CM

A
ACE Inhibitors
Angiotensin Receptor Blockers (ARB)
Beta-Blockers and Rate Limiting Calcium Antagonists
Diuretics 	
Anticoagulation
Anti arrhythmics
17
Q

what is the most common arrhthymia linked with sudden unexpected death

A

Long QT syndrome

18
Q

what is LQTS

A

congenital disorder

syncope, seizures, sudden death

19
Q

what is the average QT prolongation

A

> 440ms in males

> 460ms in females

20
Q

what is Torsades de pointes

A

distinctive polymorphic ventricular tachycardia
the QRS amplitude varies
QRS complexes twist around the baseline

21
Q

what are the ECG changes seen in Brugada syndrome

A

A.Fib

ST elevation and RBBB in V1-V3

22
Q

what is structurally normal heart

A

when no cause for sudden death is found

Arrhythmia is the presumed mode of death

23
Q

what are the features of Patent Ductus Arteriosus

A

Continous “machine-like” murmur
Collapsing pulse
Wide pulse pressure
Heaving apex beat

24
Q

what are the features of a Ventricular Septal Defect

A

Pan-systolic murmur

systolic thrill and/or left parasternal heave

25
what are complications of VSD
Aortic regurgitation Infective endocarditis Right heart failure
26
what is coarctation of the aorta
narrowing of the descending aorta
27
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
28
what is coarctation of the aorta associated with
Turner's syndrome Bicuspid aortic value Berry aneurysms
29
what are the characteristics of Tetralogy of Fallot (TOF)
- Ventricular Septal Defect - Right ventricular hypertrophy - Right ventricular outflow tract obstruction + Pulmonary stenosis - Overriding aorta
30
what are the features of TOF
- cyanosis - right-to-left shunt - ejection systolic murmur
31
what is the CXR presentation of TOF
'boot-shaped' heart