Congenital Heart Disease Flashcards

1
Q

enlargement vs. dilatation

A

enlargement is a term used to describe an increase in volume in a chamber that is unrelated to failure of the myocardium

dilatation is when enlargement can be attributable to failure

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2
Q

hemodynamic changes of the valves due to pressure

A

mostly thickening at the line of closure and edge

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3
Q

hemodynamic changes of the valves due ot flow

A

generalized thickening of the valve

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4
Q

complex

A

a single abnormality or group of abnormalities that have a tendency to be associated

includes the effects of the abnormalities on the economy of the heart

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5
Q

categories of congenital heart disease

A

shunt

obstruction (left and right)

shunt with obstruction

other complexes

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6
Q

types of shunts

A

atrial septal defect

ventricular septal defect

common AV orifice

patend ductus arteriosus

aortico pulmonary septal defect

total anomalous pulmonary venous drainage

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7
Q

atrial septal defects

A

fossa ovalis or secundum type - common

ostium primum type

sinus venosus or proximal type of atrial septal defect - uncommon

coronary sinus type of atrial septal defect - rare

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8
Q

secundum type ASD

A

defect in the fossa ovalis

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9
Q

hemodynamics of secundum type ASD

A

RA, RV, and PA pressures are normal in childhood and rarely elevated

LA and LV pressrues are normal

R -> L shunt at the atrial level

increased pulmonary flow

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10
Q

secundum type ASD pathologic complex

A

RA and RV are hypertrophied and dilated

dilatation of tricuspid and pulmonary orifices and pulmonary trunk

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11
Q

primum type ASD

A

defect in distal to fossa ovalis, close to mitral and tricuspid valves

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12
Q

hemodynamics of primum type ASD

A

RV and PA pressures are normal or slightly elevated

RA, LA, and LV pressures are normal

L -> R shunt at trial level, slight R -> L

increased pulmonary flow

increased pulmonary vascular resistance and pulmonary hypertension may develop in adult life

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13
Q

pathologic complex of primum type ASD

A

cleft aortic leaflet of mitral valve

RA and RV hypertrophied and dilated

dilatation of tricuspid and pulmonic orifices

LA and LV are normal

LV hypertrophy present if mitral regurgitation or subaortic stenosis

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14
Q

ventricular septal defect

A

can occur anywhere in the ventricular septum

predilection for the defect to occur beneath the aortic valve, confluent in part with the membranous septum and extending anteriorly to some extent

most common is called subaortic, in part membranous and in part perimembranous type

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15
Q

hemodynamics of VSD

A

RV and PA pressures normal if defect is small, increased if large

RA and LA pressures normal or elevated

LV pressure normal

L -> R shunt

increased pulmonary flow

increased pulmonary vascular resistance and pulmonary hypertension may develop causing R -> L shunt and cyanosis if defect is large

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16
Q

pathologic complex of VSD

A

RA hypertrophied

RV hypertrophied and dilated

LA and LV hypertrophied and dilated

dilatation of pulmonic orifice and pulmoanry trunk

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17
Q

large VSD defect

A

additional pressure hypertrophy of the RV

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18
Q

patent ductus arteriosus

A

communication between the aorta and left pulmonary artery distal to isthmus

19
Q

hemodynamics of PDA

A

RV and PA pressures normal or elevated

LA and LV pressures normal or elevated

RA pressure normal

L -> R shunt at ductus level

increased pulmonary flow

if pulmonary vascular resistance is high, may have bidirectional shunt at ductus level

20
Q

pathological complex of PDA

A

without pulmonary hypertension - LA and LV hypertrophied and dilated

dilatation of PA with pulmonary hypertension

RA and RV hypertrophied and dilated

LA and LV vary

21
Q

pulmonary hypertension

A

flow increases beyond the distensibility of the lung vasculature

vasoconstriction of the vascular bed

secondary pathologic changes in the inima or media of the muscular arteries and arterioles of the lungs restricting the pulmonary bed

most common in large VSD, then PDA, and least common in ASD

22
Q

Eisenmenger complex

A

the reversal of a left-to-right shunt due to pulmonary hypertension

usually happens at the ventricular level or at the ductal level but not very common at the atrial septal defect level

23
Q

obstructive lesions without shunts

A

isolated pulmonary stenosis

isolated aortic stenosis

coarctation of the aorta

24
Q

isolated pulmonary stenosis

A

usually consists of a diaphragm-liek structure with an attempted formation of cusps with a central opening, which may be minute or small

uncommonly, the valve is failry well formed, but the cusps are agglutinated at the commissures - the annulus is quite small

25
Q

hemodynamics of isolated pulmonary stenosis

A

RV systolic pressure elevated

PA pressure normal or low

no shunts except R -> L may occur via patent foramen ovale or in severe PS

26
Q

pathologic complex of IPS

A

stenosis usually valvular or valvular and infundibular - rarely infundibular alone

RA and RV are hypertrophied

LA and LV are normal

poststenotic dilatation of PA often present

27
Q

congenital isolated aortic stenosis

A

valvular or ring

suprevalvular

subaortic

valve cusps may be bicuspid or unicuspid and may show irregular thickening termed as dysplastic valve

28
Q

hemodynamics of IAS

A

LV pressure elevated

RA, RV, and LA pressures normal unless LV fails

usually no shunts

normal flows

severe stenosis has large LV -> aortic systolic gradient and decreased pulse pressure

29
Q

pathologic complex of IAS

A

stenosis valvular, subvalvular, or supravalvular

LV hypertrophied

LA usually hpertrophied

RA and RV normal

30
Q

supravalvular aortic stenosis

A

two types

one consists of thickening and accentuation at the normal supravalvular aortic ridge at the upper margins of the sinuses of valsalva

the other consists of ridge thickening about a centimeter above the sinuses of valsalva

31
Q

subaortic stenosis

A

fibro elastic tissue beneath the aortic valve extending from the anterior ventricular septum to the aortic leaflet of the mitral valve

32
Q

coarctation of the aorta

A

narrowing of the transverse arch in the region of the isthmus

the isthmus is the segment between the origin of the left subclavian artery and ductus arteriosus or ligamentum arteriosum

33
Q

adult coarctation

A

constrictive narrowin of aorta in region of ligamentum arteriosum

34
Q

hemodynamics of adult coarctation

A

LV and proximal aortic pressures elevated

normal or low distal aortic pressure

RA, RV, and LA pressures normal, unless LV fails

usually no shunts

normal flows

pressure in arms greater than in legs

collateral vessels may cause abnormal pulses in upper thorax

35
Q

pathologic complex of adult coarctation

A

LA and LV hypertrophy

dilatation of ascending aorta

RA and RV normal

various form of narrowing of aorta

36
Q

fetal coarctation

A

non-constricting long narrowing of aorta with pulmonary hypertension

usually accompanied by patent foramen ovale and patent ductus arteriosus

37
Q

hemodynamics of fetal coarctation

A

RV and PA pressures elevated

L -> R shunt at atrial level

R -> L shunt at ductus level

increased pulmonary flow

cyanosis of lower extremities may be present

38
Q

pathologic complex of fetal coarctation

A

RA and RV hypertrophy and dilated

LA and LV atrophied

dilation of PA

hypoplasia of aorta

ASD, usually PDA

39
Q

tetralogy of fallot

A

infundibular pulmonary stenosis

right ventricular hypertrophy

ventricular septal defect

overriding aorta

**ventricular septal defect is a U shaped deformity of the ventricular septum confluent with the aortic valve

40
Q

types of tetralogy of fallot

A

cyanotic - common type

acyanotic

41
Q

hemodynamics of tetralogy of fallot

A

high RV systolic pressure

low PA pressure

normal LV, RA, and LA pressure

large R->L shunt

small L->R shunt at ventricular level

rarely large L->R shunt

decreased pulmonary flow, rarely increased

42
Q

pathologic complex for tetralogy of fallot

A

RA and RV hypertrophied

LA and LV normal or atrophied

43
Q

cyanotic tetralogy of fallot

A

pressure hypertrophy of the RA and RV with significant infundibular pulmonary obstruction

LA and LV have a tendency to be smaller than normal

the RV is contracting against systemic and infundibular resistance, decreased pulmonary flow, and predominant right to left shunt at the ventricular level

44
Q

acyanotic tetralogy of fallot

A