Congenital Heart Disease Flashcards
Congenital heart disease
This is a general term used to describe _______ of the _____ or ______ that are present from _____
abnormalities
heart or great vessels
birth
Congenital heart disease
• Usually arises from faulty embryogenesis during the ____ week of gestation (max ____ week)
3rd
5th
Congenital heart disease
few are associated with live birth
T/F
F
Most are associated with live birth
Congenital heart disease
some are not compartible with intrauterine survival
T/F
T
AETIOLOGY of congenital heart disease
•____% unknown
•__________ ,with genetic and environmental factors playing a part
• associated with chromosomal disorders such as ——— and ______ syndrome
90
Multifactorial
Downs and Turners
AETIOLOGY of congenital heart disease
• Infection such as _______
• Hypoxia
•__________
•_______ exposure to mother (especially between __-__ week)
Rubella
Thalidomide
Radiation
3-8
SHUNTS- These are __________ between the chambers or blood vessels;
• -left to right shunts
• -right to left shunts
abnormal communications
Initial left to right shunt
• - ____
• -_____
• __________________
• -APVD
• ______________ SYNDROME
• Right to left shunt
• -______
• -____
ASD; PDA
PERSISTENT TRUNCUS ARTERIOSUS (PTA)
HYPOPLASTIC LEFT HEART
TOF; TA
What congenital heart defect has the highest Relative Incidence
VSD
ATRIAL SEPTAL DEFECT
• ASD refers to an abnormal opening in ________ allowing communication of blood between the ________ and _______
atrial septum
right and left atrium
ATRIAL SEPTAL DEFECT is the same as a patent foramen ovale which occurs in about ___% of normal individuals
T/F
F
different from a patent foramen ovale
30
ATRIAL SEPTAL DEFECT
• There 3 main types ;
• - _______ type
• -_________ type ASD
• -___________ type
sceundum
primum
sinus venosus
ATRIAL SEPTAL DEFECT
• There 3 main types ;
• -sceundum type ,located _______
• -primum typeASD, _________
• -sinus venosus type, near the ———
highup
lowerdown
SA node
ATRIAL SEPTAL DEFECT: Secundum type ASD
•_____ % of all ASDs
• Results from defiency or fenestration of the ___________,__________, or _________
90
embryonic septum primum, deficiency of septum secundum or both
ATRIAL SEPTAL DEFECT: Secundum type ASD
• Aperture may be _____,_______, or_______
• Larger defect creates a ________
single, multiple or fenestrated
single atrial chamber
ATRIAL SEPTAL DEFECT: Primum type ASD
• ____% of all ASDs
• Occurs (low or high?) in the atrium
5
Low
ATRIAL SEPTAL DEFECT: Primum type ASD
• (Antero or Posterò?) -(inferior or superior ?) to the fossa ovalis and adjacent to the ________
Antero ; inferior
AV node
ATRIAL SEPTAL DEFECT: Primum type ASD
• associated with ___________ leaflet and can be associated with _______ defect
left anterior mitral
partial AV
ATRIAL SEPTAL DEFECT: Sinus venosus
•___% of all ASDs
• Located (low or high ?) in atrial septum, near _______ which may saddle it
5
High
entrance of SVC
ATRIAL SEPTAL DEFECT: Sinus venosus
• Associated with anomalous connections of ______________ to the right atrium
right pulmonary veins
ATRIAL SEPTAL DEFECT: Clinical features
•_____ to ______ shunt
• Initially (cyanotic or acyanotic?)
• Increased _______ blood flow
Left to right
Acyanotic
pulmonary
ATRIAL SEPTAL DEFECT: Clinical features
• Small ASDs are (poorly or well?) tolerated, (asymptomatic or symptomatic?)
•Large ASDs usually manifest around age ____
• _________ is a late consequence because the ________________________
Well; asymptomatic
30
Pulmonary hypertension
lungs cant tolerate increased blood flow
ATRIAL SEPTAL DEFECT: Clinical features
• When pulmonary hypertension occurs, it results in
• - _______
• -_______ difficulty
• -________ failure
•__________ is rare
•_________ embolus or _______ occurs when blood flow is reversed
cyanosis
respiratory
Cardiac
Infective endocarditis; Paradoxical
brain abscess
__________ is the most common congenital anomaly
VENTRICULAR SEPTAL DEFECT
VENTRICULAR SEPTAL DEFECT
It is associated with other anomalies
•_____% are isolated
• It may present at birth, later in life or in adulthood
30
VENTRICULAR SEPTAL DEFECT
• It may be
• - ________(___%),and (single or multiple?)
• - __________(____%)
muscular; 10; multiple
membranous;90
Ventricular septal defect
Functional severity depends on the _____ and presence or absence of _______
size
pulmonary stenosis
VENTRICULAR SEPTAL DEFECT: Small defects
• are ‘ ________ ’
•____% close spontaneously
• are (poorly or well?) tolerated
muscular
50
Well
VENTRICULAR SEPTAL DEFECT
•____________ and _________ are present from birth
• Small defects produce ________ and predisposition to ________
Right ventricular hypertrophy and pulmonary hypertension
jet streams
infective endocarditis
PATENT DUCTUS ARTERIOSUS
• This occurs when the normal _________ channels during intra-uterine life remains open after birth
aorto-pulmonary vascular
PATENT DUCTUS ARTERIOSUS
• Increased ______ after birth and changes in _____ contribute to the constriction and closing of the DA, normally
oxygen tension
Prostaglandin
PATENT DUCTUS ARTERIOSUS
• After birth, it may remain open, especially in ______ babies due to ________
•___-___% are isolated, others have ____
premature
low oxygen levels
85-90; VSD
PATENT DUCTUS ARTERIOSUS: Clinical presentation
• Presents with a _______(____) murmur
• (Acyanosis or Cyanosis?) initially
machinery (pan systolic)
Acyanosis
PATENT DUCTUS ARTERIOSUS: Clinical presentation
eventual Pulmonary hypertension with reversal of shunt , leading to:
•_______,________,______
•____________ failure
Cyanosis, finger clubbing, polycythaemia
Right ventricular
PATENT DUCTUS ARTERIOSUS • Clinical presentation
• Acyanotic heart disease is converted to cyanotic heart disease because ________________________ which ought not to be so.
the pulmonary tension is higher than the systemic tension
PATENT DUCTUS ARTERIOSUS • Clinical presentation
• Red blood cells are produced in increased amount because ______________ leading to polycythaemia
the body senses hypoxia
PATENT DUCTUS ARTERIOSUS • Clinical presentation
• Right ventricular ______ over time leads to ______ which causes heart failure
hypertrophy
dilation
ATRIOVENTRICULAR SEPTAL DEFECT
• Failure of fusion of the ___________________________ results in (complete or incomplete?) closure of the _______ and the inadequate formation of the ____________ and __________ leaflets
superior and inferior endocardial cushions
Incomplete ; AV septum
septal tricuspid and anterior mitral
ATRIOVENTRICULAR SEPTAL DEFECT
• The commonest abnormality is
• -_____ AV septal defects
• -_______ AV septal defect and a ________
partial
complete; large common AV valve
ATRIOVENTRICULAR SEPTAL DEFECT: Partial AV septal defects
•involves ________ and _____________
• Causing ______ insufficiency
Primum ASD and left anterior mitral leaflet
mitral valve
ATRIOVENTRICULAR SEPTAL DEFECT: Complete AV septal defect and a large common AV valve
• in this , ________________
• It is associated with _________
All 4 valves communicate
Down’s syndrome
EISENMENGER’S SYNDROME
•_______________ heart disease
• These are cases of _____,____,____,_____
• With _________ and reversal of flow
Late cyanotic
VSD, ASD, PDA and AVSD
pulmonary hypertension
EISENMENGER’S SYNDROME
• Usually the original lesions are (reparable or irreparable?) at this stage
• Therefore, the repair should be done before ___________________
irreparable
pulmonary hypertension builds up
CYANOTIC CONGENITAL HEART DISEASES
List 5
• TETRALOGY OF FALLOT
• TRANSPOSITION OF GREAT VESSELS
• TRUNCUS ARTERIOSUS
• TRICUSPID ATRESIA usually with ASD
• TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION
most common CYANOTIC CONGENITAL HEART DISEASE is ?
TETRALOGY OF FALLOT
TETRALOGY OF FALLOT
• It has 4 components
• 1- _________
• 2- ________
• 3- ____________
• 4- ______________
membranous VSD
overiding aorta
subpulmonary stenosis
right ventricular hypertrophy
TETRALOGY OF FALLOT
• Embryology results from ______________ of the _____________
anterior-superior displacement
infundibular septum
TETRALOGY OF FALLOT: Morphology
•_____ shaped heart
•___ size of _______
•________ stenosis, _______ stenosis or atresia of valves and arteries
Boot
VSD ; aortic orifice
Subpulmonary
pulmonary valve
TETRALOGY OF FALLOT: Morphology
•_______ is required for survival if there is __________ of _______ valves
• Other features include ______ or ________ insufficiency
•_____________ in 20% of cases
PDA
atresia of pulmonary valves
ASD or aortic valve
Right aortic arch
TETRALOGYOFFALLOT: Clinical Features
• Depends on the severity of ________
pulmonary obstruction
TETRALOGYOFFALLOT: Clinical Features
Those with _____ and mild pulmonary obstruction have left to right shunts and are refered to as ________
large VSD
Pink Tetralogy
TETRALOGYOFFALLOT: Clinical Features
Others will present with _______, __________ , ______ and in the very severe forms the baby is usually found in the _______ position
cyanosis
polycythemia
finger clubbing
squatting
TETRALOGY OF FALLOT
good Prognosis
• If there is ______
- The ______ are _______
no atresia
lungs are protected
TRANSPOSITION OF GREAT VESSELS
• The _______ arises from the Left ventricle and the _____ from the
Right ventricle
pulmonary trunk
aorta
IN TRANSPOSITION OF GREAT VESSELS
_____________ blood is circulated throughout the body
deoxygenated
TRANSPOSITION OF GREAT VESSELS
• If _____ is present, the degree of cyanosis is ___eased
• If _____ is absent, the baby may _______
PDA; decr
PDA; die soon after birth
TRANSPOSITION OF GREAT VESSELS
• There are 2 types of this condition
• 1) _______
• 2)__________
Complete
Incomplete
TRANSPOSITION OF GREAT VESSELS: Complete TGA
•results from the abnormal
formation of __________
• The aorta arises from the _____, lies (anterior or posterior?) and to the (left or right?) of the pulmonary trunk
truncal aortic septae
Right ventricle ; anterior
Right
TRANSPOSITION OF GREAT VESSELS
• Complete TGA
It occurs commonly in offsprings of _______ mothers
diabetic
Complete TGA is incompartible with life
T/F
T
Transposition of Great vessels
TGA with associated ______ has stable
shunt while those with ——- have unstable shunt
VSD
PDA
TRUNCUS ARTERIOSUS
• This occurs when there is a failure of _____ and ______ of the _______ of the ____ and _____ that would ordinarily divide into the aorta and pulmonary Trunk respectively
fusion and descent
spiral ridges
truncus and cornua
TRUNCUS ARTERIOSUS
• When the _____ fails to completely divide, the aorta and pulmonary trunk are left undivided at their outflow
spiral septum
TRUNCUS ARTERIOSUS
The truncus overrides only the left ventricle
T/F
F
The truncus overrides both ventricles
TRUNCUS ARTERIOSUS
The persistent truncus are always accompanied by _______________
membranous VSD
TRUNCUS ARTERIOSUS
• The truncal valve resembles the _____ and ______ valves and may have __,___, or ___ cusps
tricuspid and aortic
2, 4 or 5
TRUNCUS ARTERIOSUS
• May be associated with other defects such as:
•___________
•_________ pulmonary arteries
• Absence of the _________
•_________ hypoplasia
Right aortic arch
Hypoplastic
ductus arteriosus
Pulmonary
TRICUSPIDATRESIA
This occurs when there is __________________ with a (smaller or larger?) than normal _____ valve
an unusual division of the atrio-ventricular canal
Larger ; mitral
TRICUSPIDATRESIA
• Circulation is maintained through a _____ or a ______
• May be associated with hypoplasia of the ________
VSD; PDA
right ventricle
TRICUSPIDATRESIA
•_______ is usually present to channel blood from the right atrium to the left atrium
• This is necessary for the _____ to function
ASD
VSD
TRICUSPIDATRESIA
May be associated with ______________ syndrome
hypoplastic right heart
TOTAL ANOMALOUS PULMONARY VENOUS CONNECTIONS
• The ________ do not return to the ____
• They connect to the ________, _______ or some other anomalous sites
• The blood is thus returned to the _______
pulmonary veins; left atrium
innominate veins
coronary sinus
right atrium
In TOTAL ANOMALOUS PULMONARY VENOUS CONNECTIONS
• There is overload of the _____ side of the heart
•_______ provide free flow of oxygenated bld to the left side of the heart
right
An ASD
OBSTRUCTIVE CONGENITAL LESIONS
______________
________________
_______________
• COARCTATION OF THE AORTA
• PULMONARY STENOSIS
• AORTIC STENOSIS
Narrowing of the aorta is a rare structural abnormality
T/F
F
Common
COARCTATION OF THE AORTA
• _________ of the aorta is a common structural abnormality
• M:F–___:____
Narrowing
3-4:1
COARCTATION OF THE AORTA
Associated with ________ Syndrome or ______ aneurysm in the brain
Turner’s
Berry
COARCTATION OF THE AORTA
• There are 2 classical forms
• 1. _________ or ______
• 2. _______ or ________
Infantileorpreductal
Adult or postductal
Ductal refers to _________
ductus arteriosus
COARCTATION OF THE AORTA: Clinical Features
•This depends on the ____________
severity of the coarctation
COARCTATION OF THE AORTA: Clinical Features
• -PreductalCoarctation
• Usually presents in _______
• Majority (will or will not?) survive the neonatal period without surgical intervention
• ______ is necessary for survival
childhood
Will not
PDA
COARCTATION OF THE AORTA: Clinical Features
PreductalCoarctation
• Cyanosis of the ______ due to the delivery of ________ through the ____
• ________ vessels are spared
lower extremities
unsaturated blood
PDA
Upper limb
COARCTATION OF THE AORTA: Clinical Features
Postductal coarctation
• The ductus is closed and narrowing is ____________
• Majority of the children are (symptomatic or asymptomatic?)
• Xterized by _______ of the upper limb and lower BP of lower limb
beyond origin of carotid vessels
assymptomatic
hypertension
COARCTATION OF THE AORTA: Clinical Features
Postductal coarctation
•Features of arterial insufficiency (esp.in the ____)
• __________ develops between the precoarctation arterial branches and post coarctation arteries (internal mammary aa, arterial bld flow from branches of subclavian aa are directed to thoracic aorta)
Lower limb
collateral circulation
COARCTATION OF THE AORTA: Clinical Features
Postductal coarctation
• X-rays show _____ of the ______ of the ribs, due to ______ that involve the _______
notching; undersurface
collaterals
intercostal arteries
COARCTATION OF THE AORTA: Clinical Features
• _______
• _________ due to LVH
• Untreated, death comes by the age of ___ years due to ______
Murmurs
Cardiomegaly
40; CCF
COARCTATION OF THE AORTA: Clinical Features
• Intracranial _______
• Infective ________
• Vascular ______
• Aortic _______ (due to degenerative changes)
haemorrhage
endocarditis
rupture
dissection
PULMONARY STENOSIS
May be isolated or associated with
• -_______
• -________
• -____________ valve
TOF
Transposition
Dysplastic tricuspid
PULMONARY STENOSIS
• When the valve is completely atretic, there is a __________ and ______
hyperplastic right ventricle and an ASD
PULMONARY STENOSIS
• In pulmonary atresia, blood passes through _____ to ______ and into the lungs
an ASD to PDA
PULMONARY STENOSIS
• The pulmonary trunk is hypoplastic if there is a __________ as in _____
subpulmonary stenosis
TOF
PULMONARY STENOSIS is Associated with RVH
T/F
T
AORTIC STENOSIS AND ATRESIA
• This is usually associated with ______ of the _________ tract with ______________ syndrome
underdevelopment
left ventricular outflow
hypoplastic left ventricular
AORTIC STENOSIS AND ATRESIA
• Isolated in ___% of cases
80
AORTIC STENOSIS AND ATRESIA
• there are 3 major types
• 1-_____
• 2- ________
• 3- _______
valvular
subvalvular
supravalvular
AORTIC STENOSIS AND ATRESIA
Supra valvular stenosis is narrowing __________.
Usually ________.
Also seen in ______ syndrome ( ________ in ________ )
above the aortic valve
inherited
William; hypercalcaemia in children
AORTIC STENOSIS AND ATRESIA
________ is the most common form .
Valvular
AORTIC STENOSIS AND ATRESIA
Subvalvular stenosis includes obstruction by a _________ or by _____________
fibrous band
asymmetric muscular hypertrophy.
A unicuspid aortic valve is fatal
T/F
T
