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Flashcards in Congenital Heart Disease Deck (44):

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


hemodynamic changes of the valves due to pressure

mostly thickening at the line of closure and edge


hemodynamic changes of the valves due ot flow

generalized thickening of the valve



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


categories of congenital heart disease


obstruction (left and right)

shunt with obstruction

other complexes


types of shunts

atrial septal defect

ventricular septal defect

common AV orifice

patend ductus arteriosus

aortico pulmonary septal defect

total anomalous pulmonary venous drainage


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


secundum type ASD

defect in the fossa ovalis


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


secundum type ASD pathologic complex

RA and RV are hypertrophied and dilated

dilatation of tricuspid and pulmonary orifices and pulmonary trunk


primum type ASD

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


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


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


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


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


pathologic complex of VSD

RA hypertrophied

RV hypertrophied and dilated

LA and LV hypertrophied and dilated

dilatation of pulmonic orifice and pulmoanry trunk


large VSD defect

additional pressure hypertrophy of the RV


patent ductus arteriosus

communication between the aorta and left pulmonary artery distal to isthmus


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


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


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


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


obstructive lesions without shunts

isolated pulmonary stenosis

isolated aortic stenosis

coarctation of the aorta


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


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


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


congenital isolated aortic stenosis

valvular or ring



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


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


pathologic complex of IAS

stenosis valvular, subvalvular, or supravalvular

LV hypertrophied

LA usually hpertrophied

RA and RV normal


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


subaortic stenosis

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


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


adult coarctation

constrictive narrowin of aorta in region of ligamentum arteriosum


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


pathologic complex of adult coarctation

LA and LV hypertrophy

dilatation of ascending aorta

RA and RV normal

various form of narrowing of aorta


fetal coarctation

non-constricting long narrowing of aorta with pulmonary hypertension

usually accompanied by patent foramen ovale and patent ductus arteriosus


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


pathologic complex of fetal coarctation

RA and RV hypertrophy and dilated

LA and LV atrophied

dilation of PA

hypoplasia of aorta

ASD, usually PDA


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


types of tetralogy of fallot

cyanotic - common type



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


pathologic complex for tetralogy of fallot

RA and RV hypertrophied

LA and LV normal or atrophied


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


acyanotic tetralogy of fallot