Inherited Cardiac Conditions Flashcards

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
Q

what are complications of VSD

A

Aortic regurgitation
Infective endocarditis
Right heart failure

26
Q

what is coarctation of the aorta

A

narrowing of the descending aorta

27
Q

what are features of coarctation of the aorta

A

Radio-femoral delay
Mid or Late systolic murmur that is maximal over back
Notching of the ribs

28
Q

what is coarctation of the aorta associated with

A

Turner’s syndrome
Bicuspid aortic value
Berry aneurysms

29
Q

what are the characteristics of Tetralogy of Fallot (TOF)

A
  • Ventricular Septal Defect
  • Right ventricular hypertrophy
  • Right ventricular outflow tract obstruction + Pulmonary stenosis
  • Overriding aorta
30
Q

what are the features of TOF

A
  • cyanosis
  • right-to-left shunt
  • ejection systolic murmur
31
Q

what is the CXR presentation of TOF

A

‘boot-shaped’ heart