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CARDIO > old congential + connective tissue diseases > Flashcards

old congential + connective tissue diseases Flashcards

1
Q

what problems would a bicuspid aortic valve have for a patient?

A

no problems at birth
eventually develop stenosis +/- regurg
predisposes to IE/SBE

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2
Q

what is ASD?

A

atrial septaldefect
hole connects the atria
secundum defects high in septum are commonest
often asymptomatic until adulthood

LV compliance lowers with age so L to R shunt

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3
Q

what are the symptoms of ASD?

A

dyspnoea
pulmonary HTN
arrhythmia
chest pain

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4
Q

what are the signs of ASD?

A

AF
raised JVP
pulmonary ESM
PHT -> TR or PR

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5
Q

what are the complications of ASD?

A

paradoxical emboli

eisenmenger’s syndrome (raised RA pressure- R to L shunt- cyanosis)

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6
Q

what investigations would you do for ASD?

A

ECG- secundum: RAD
CXR- pulmonary plethora
ECHO- diagnostic

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7
Q

what is the treatment for ASD?

A

transcatheter closure

recommended in adults if high pulmonary to systemic blood flow ratio >/1.5:1

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8
Q

what is coarctation of the aorta?

A

congenital narrowing of aorta
usually occurs just distal to origin of left subclavian
M>F

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9
Q

what assocations are there with coarctation of the aorta?

A

bicuspid aortic valve

turner’s syndrome

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10
Q

what are the signs of coarctation of the aorta?

A

radio-femoral delay + weak femoral pulse
HTN
systolic murmur/bruit heard best over L scapula

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11
Q

what are the complications of coarctation of the aorta?

A

HF

IE

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12
Q

what investigations would you do + results for coarctation of the aorta?

A

CXR- rib notching
ECG- LV strain
CT angiogram

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13
Q

what is the treatment of coarctation of the aorta?

A

balloon dilatation + stenting

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14
Q

what is VSD?

A

ventricular septal defect

hole connects ventricles

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15
Q

what are the causes of VSD?

A

congenital

acquired- post MI

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16
Q

what is the presentation of VSD?

A

severe HF in infancy

or incidental in later life

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17
Q

what are the signs of VSD?

A

1) small holes which are haemodynamically less significant give louder murmurs

harsh, panystolic murmur at left sternal edge
systolic thrill
left parasternal heave

2) larger holes -> PHT

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18
Q

what are the complications of VSD?

A

IE
PHT
eisenmneger’s

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19
Q

what would you see on ECG for VSD?

A

small- normal

large- LVH + RVH

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20
Q

what would you see on ECG for VSD?

A

small- mild pulm oedema

large- cariomegaly + marked pulmonary plethora

21
Q

what is the management of VSD?

A

surgical closure indicated if symptomatic with large shunt

22
Q

what is fallot’s tetralogy?

A

most common congenital cyanotic heart defect

abnormal separation of truncus arteriosus inot aorta and pulmonary arteries

23
Q

what is the pathology of fallot’s tetralogy?

A

1) VSD
2) pulmonary stenosis
3) RVH
4) overriding aorta

24
Q

what is associated with fallot’s tetralogy?

A

di george- CATCH-22

25
Q

what is the presentation of fallot’s tetralogy?

A

infants- hypercyanotic episodes, squatting, clubbing

adult- often asymptomatic
unoperated- cyanosis, ESM of PS
repaired- dyspnoea, palps, RVF

26
Q

what is seen on ECG for fallot’s tetralogy?

A

RVH

RBBB

27
Q

what is seen on CXR for fallot’s tetralogy?

A

coeur en sabot (clog shaped heart)

28
Q

what is seen on ECHO for fallot’s tetralogy?

A

anatomy + degree of stenosis

29
Q

what is the treatment of fallot’s tetralogy?

A

surgical usually before 1yo
closure of VSD
correction of pulmonary stenosis

30
Q

what is the epidemology of marfan’s syndrome?

A

autosomal dominant
spontaneous mutation in 25%
M=F
prevalence = 1/5000

31
Q

what is the pathophysiology of marfans

A

mutation in FBN1 gene on ch5 which encodes fibrillar 1 glycoprotein

fibrillin 1 is essential component of elating
histology- cystic medial necrosis

32
Q

what are the categories of presenation in marfans?

A

cardiac
ocular
MSK

33
Q

what are the cardiac presentations of marfans

A

aortic aneurysm +dissection
aortic root dilatation -> regurg
MV prolpase +/- regurgitate

34
Q

what are the ocular presentations of marfans

A

lens dislocation: superotemporal

35
Q

what are the MSK presentations of marfans

A 
high arched palate
arachnodactyly 
arm-span>height
pectus excavatum
scoliosis
pes planus
joint hypermobility
36
Q

what are the complications of marinas?

A

ruptured aortic aneurysm
spontaneous pneumothorax
diaphragmatic hernia
hernias

37
Q

how do you diagnose marfans

A

2/3 organ systems must be involved

38
Q

what are the ddx of marfans

A

MEN-2b
homocystinuria
EDS

39
Q

what investigations would you do in marfans

A 
slit lamp exam
CXR
ECG
ECHO
MRI
genetic testing- FBN-1 mutation
40
Q

what would you see on slit lamp exam in marfans

A

ectopia lentis

41
Q

what would you see on CXR in marfans

A

widened mediastinum
scoliosis
pneumothorax

42
Q

what would you see on ECG in marfans

A

arrhythmias- premature atrial + ventriuclar ectopics

43
Q

what would you see on ECHO in marfans

A

aortic root dilatation-> AR

MVP + MR

44
Q

what could you see on MRI for marfans

A

dural ectasia (dilatation of neural canal)

45
Q

what is the management of marfans

A

1) refer to ortho, cardio + optho
2) lifestyle- reduce cardiointensive sports
3) bb slow dilatation of aortic root
4) regular cardiac echo- surgery when aortic root >/5cm wide

46
Q

what is the pathogenesis of EDS?

A

rare heterogenous group of collagen disorders
6 subtypes with varying severity
most common types 1 + 2 are autosomal dominant

47
Q

how does EDS present?

A 
hyperelastic skin
hypermobile joints
cardiac- MVP, AR, MR + aneurysms
fragile blood vessels- easy bruising, GI bleeds
poor healing
48
Q

what are ddx for EDS?

A

cutis laxa- loose skin + hypermobile joints
pseudoxanthoma elasticum- skin laxity
marfans

CARDIO (10 decks)

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