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Flashcards in Congenital Heart Disease Deck (44):
1

enlargement vs. dilatation

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

2

hemodynamic changes of the valves due to pressure

mostly thickening at the line of closure and edge

3

hemodynamic changes of the valves due ot flow

generalized thickening of the valve

4

complex

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

5

categories of congenital heart disease

shunt

obstruction (left and right)

shunt with obstruction

other complexes

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types of shunts

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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atrial septal defects

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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secundum type ASD

defect in the fossa ovalis

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hemodynamics of secundum type ASD

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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secundum type ASD pathologic complex

RA and RV are hypertrophied and dilated

dilatation of tricuspid and pulmonary orifices and pulmonary trunk

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primum type ASD

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

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hemodynamics of primum type ASD

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

13

pathologic complex of primum type ASD

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

14

ventricular septal defect

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

15

hemodynamics of VSD

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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pathologic complex of VSD

RA hypertrophied

RV hypertrophied and dilated

LA and LV hypertrophied and dilated

dilatation of pulmonic orifice and pulmoanry trunk

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large VSD defect

additional pressure hypertrophy of the RV

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patent ductus arteriosus

communication between the aorta and left pulmonary artery distal to isthmus

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hemodynamics of PDA

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

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pathological complex of PDA

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

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pulmonary hypertension

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

Eisenmenger complex

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

obstructive lesions without shunts

isolated pulmonary stenosis

isolated aortic stenosis

coarctation of the aorta

24

isolated pulmonary stenosis

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

hemodynamics of isolated pulmonary stenosis

RV systolic pressure elevated

PA pressure normal or low

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

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pathologic complex of IPS

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

congenital isolated aortic stenosis

valvular or ring

suprevalvular

subaortic

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

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hemodynamics of IAS

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

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pathologic complex of IAS

stenosis valvular, subvalvular, or supravalvular

LV hypertrophied

LA usually hpertrophied

RA and RV normal

30

supravalvular aortic stenosis

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

subaortic stenosis

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

32

coarctation of the aorta

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

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adult coarctation

constrictive narrowin of aorta in region of ligamentum arteriosum

34

hemodynamics of adult coarctation

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

pathologic complex of adult coarctation

LA and LV hypertrophy

dilatation of ascending aorta

RA and RV normal

various form of narrowing of aorta

36

fetal coarctation

non-constricting long narrowing of aorta with pulmonary hypertension

usually accompanied by patent foramen ovale and patent ductus arteriosus

37

hemodynamics of fetal coarctation

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

pathologic complex of fetal coarctation

RA and RV hypertrophy and dilated

LA and LV atrophied

dilation of PA

hypoplasia of aorta

ASD, usually PDA

39

tetralogy of fallot

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

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types of tetralogy of fallot

cyanotic - common type

acyanotic

41

hemodynamics of tetralogy of fallot

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

pathologic complex for tetralogy of fallot

RA and RV hypertrophied

LA and LV normal or atrophied

43

cyanotic tetralogy of fallot

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

acyanotic tetralogy of fallot