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‫بسم هللا الرحمن الرحيم‬
Cardiovascular System
CMD-312
Congenital Anomalies of The
Heart & Blood Vessels
Adviced by:
Dr. masood ahmad
By:
MOHAMMED ALI ALANZ
Objectives:
List the developmental anomalies of the heart
1 and main arteries arising from aortic arch.
2
Describe atrial and ventricular septa defect.
3
Describe the developmental anomalies of the
main arteries arising from the aortic arch
Introduction
• The Heart:
• It’s a conical muscular organ having apex
directed downward, forwards and to the left,
and base directed backward and upwards .
• divided into 4 chambers : 2 atria (Right, left)
2 ventricles (Right, left).
• The 2 atria separated from ventricles by atrioventricular sulcus or coronary sulcus .
• The 2 ventricles are separated from each other
by anterior & posterior interventricular groove .
• Congenital anomalies of the heart and blood
vessels: is a general term used to describe
abnormalities of the heart or great vessels
that are present from birth. Most such
disorders arise from faulty embryogenesis
during gestational weeks 3 through 8, when
major cardiovascular structures develop .
classification of the congenital heart
defect :
• dependent on :
1. Dependent on the anatomical structure
defect .
–
–
–
–
–
Malposition
Atrial septal defect
Ventrical septal defect
Truncal defect
Defect in the heart vessels
2. Dependent on the time of developmental .
•Most major defects arise during weeks 6 and 7
of development .
3. Dependent on the presence of cyanosis:
Cyanotic.
Acynotic.
4. Dependent on the type of shunt:



Left-to-right shunt.
Right-to-left shunt.
obstructive (no shunt).
* Classification of congenital heart
diseases:
Acyanotic
Cyanotic
With shunts (Left-to-right shunt)
With shunts (right-to-left shunt)
Atrial septal defect
Fallot's tetralogy
Ventricular septal defect
Transposition of the great vessels
Patent ductus arteriosus
Partial anomalous venous drainage
Without shunts(Obstructive)
Without shunts (Obstructive)
Severe pulmonary stenosis
Coarctation of the aorta
Tricuspid atresia
Congenital aortic stenosis
Pulmonary atresia
Hypoplastic left heart
Congenital Heart Defect and Great
vessels:
1. Dextrocradia
2. Ectopia cordis
3. Atrial septal defect
4. Ventricular septal defect
5. Truncus arteriosus
6. Transposition of the great arteries
7. Unequal division of the truncus arteriosus
8. Corbiloculare
9. Undivided truncus arteriosus
10. Aortic or pulmonary stenosis and atresia
11. Transposition of the ascending aorta and pulmonary
trunk
Atrial septal :• Develpent :
- Roof of primitive atrium ----) septum primum
- b/w septum primum &A.V cushions (foramen
primum)
- Septum primum grow down & deganart ---)
foramen secundum
- Roof of primitive atrium right side ----)
septum secundum
• The septum primum will close the foramen
primum .
• b/w margin of septum primum & septum
secundum form a foramen ovale --)close at
birth
* Atrial Septal Defect :
 Definition:
 An ASD is an abnormal opening in the atrial
septum that allows communication of blood
between the left and right atria (not to be
confused with a patent foramen ovale, present
in up to one-third of normal individuals).
• Three major types of ASDs, classified
according to their location in the septum.
* Atrial Septal Defect :
 These three types are:
 Ostium secundum defect (90%): defect located at and
resulting from a deficient or fenestrated oval fossa.
 Ostium primum defect (5%): adjacent to the AV valves
and are usually associated with a cleft anterior mitral
leaflet.
 Sinus venosus defect (5%): are located near the
entrance of the superior vena cava. They are commonly
accompanied by anomalous connections of right
pulmonary veins to the superior vena cava or right
atrium.
Ostium secundum defect
Ostium primum defect
Sinus venosus defect
Ventricular septal
• Developent :
• It have 2 part
- Muscular part :
floor of primitive ventricle ----)muscular septum
has 2 horns ventral reaches rt. Bulbar &
ventral A.V cushions
b/w free edge of muscular septum &
atrioventricular cushions form a
interventricular foramen
• Membranous part :
- Bulbar & A.V cushions ----) membranous
septum ----)close the interventricular foramen
Ventricular septal defect
• Definition :
– VSD is abnormal opening in the ventricular septum
allowing shunting of blood ; abnormal
communication between left and right ventricle .
• Most VSDs occur in the membranous part of
the interventrecular sptum
• VSDs are associated with other cardiac
malformations ,esp.tetraology of fallot.
VSDs are due to :
1) complete absence of the membranous part of
the septum (Membranous VSD)
2) complete absence the muscular part of the
septum (Muscular VSD)
3) complete absence of both membranous and
muscular parts (Cor trilocolare biatriatum)
4) Partial defect of the membranous part of the
septum (Rogers disease )
Membranous VSD
Muscular VSD
Tetralogy of the Fallot :• This is classic group of four cardiac defect consists
of the following :
– Pulmonary stenosis .( obstruction to right ventricular out
flow )
– Ventricular septal defect .
– Overriding ( Dextroposition )of the aorta .
– Right ventricular hypertrophy .
The pulmonary trunk is usually small and there may
be varying degrees of pulmonary artery stenosis as
well.
Transposition of The Great Arteries
( TGA) :• Characterized by aorta arising from right
ventricle and pulmonary artery from the left
ventricle and often associated with other
cardiac abnormalities
• The defect result from the aorta lies anterior
& the right of the pulmonary trunk and arise
interiorly from the morphologic right
ventricle + the pulmonary trunk arise from
the morphologic left ventricle .
• TGA often associated with other cardiac
anomalies ASD.VSD
Aortic stenosis & atresia:• In this anomaly the edges of the valve are
usually fused to form a dome with a narrow
opening
• The valvular stenosis cause extra work for
the heart lead to hypertrophy of LV &
abnormal heart sound ( murmurs)
• Aortic atresia is present when obstruction of
the aorta or its valve is complete .
Aortic Arch Anomalies:
•
•
•
•
•
Patent ductus arteriosus
Coarctation of the Aorta
Double Aortic Arch
Right Arch of the Aorta
Abnormal origin of the right subcluvian
artery
Patent ductus arteriosus
• Before birth the ductus arteriosus is allow the
passage of the blood from pulmonary trunk
to arch of aorta due to nonfunctional lungs
• After birth by few hours (+)O2 tension in
blood ---)bradykinin ---)contracion&clossure
of ductus arteriosus
• At 3mont tunica intema ---) fibrous tissue ----)
fibrosis of duct ---)ligamentum arteriosum
• The ductus arteriosus (DA) is the vascular
connection between the pulmonary artery
and the aortic arch
• … the blood passes through the ductus
arteriosus from the pulmonary artery to the
arch of aorta
Coarctation of the aorta:• Carctation = constriction
• It is characterized by a constriction of varying
length to the aorta .
• the site of defect may be preductal
coarctation or postductal coarctation .
Preductal
Ductal
Postductal
Narrowing is just superior to ductus
arteriosus. It is associated with PDA
Narrowing of the arch is opposite to
ductus arteriousus. There is discrete
ridge like infolding of the aorta just
opposite the closed DA
Narrowing is just inferior to the ductus
arteriosus
Double Aortic Arch
• It is characterized by vascular ring around the
trachea and esophagus .
• It is result from failure of the distal part of the
right dorsal aorta to disappear that lead to right
and left arches form .
• Usually the right arch of the aorta is the large
one and passed posterior to the trachea and
esophagus .
• It is cause varying degree of compression of
trachea and esophagus .
Right arch of the aorta
• When the entire right dorsal aorta persists and the distal part of the left
dorsal aorta involutes , a right aortic arch result .
• There are two main types :
• right arch of the aorta without a retroesophageal component :
• the DA ( or ligamentum arteriosum )pass from the right pulmonary artery
to the right arch of the aorta .
• right arch of the aorta with a retroesophageal component :
– A small left arch of the aorta probably involuted , leaving the right arch of the
aorta posterior to the esophagus
• The DA( or ligamentum arteriosum ) attaches to the distal part of the arch
of the aorta and forms a ring which may constrict the esophagus and
trachea .
Abnormal origin of the right
subcluvian artery
• It occurs when the right 4th aortic arch and the
right dorsal aorta cranial to the seventh cervical
intersegmental artery abnormally regress .
• The right subclavian artery will come to lie on
the left side just inferior to the left subclavian
artery.
• The abnormal artery must cross the midline
posterior of the trachea and oesophagus to
supply the right upper limb and then constrict
them .
Thank you
Any question?
summary
• congenital heart defect : Dependent on
anatomical structure defect .
– Atrial septal defect
– Ventrical septal defect
Defect in the heart vessels:
–
–
–
–
Patent ductus arteriosus
Coarctation of the Aorta
Double Aortic Arch
Right Arch of the Aorta
References
• Longman’s Embryology by sadler
• Clinical Anatomy for medical students by Richard S.
Snell
• Before we are born by Moore
• Dividson Medicine
• Some web site for the picture