Download Congenital Anomalies of the heart

DR RANIA GABR

Discuss the congenital anomalies related to the
heart development.
 Congenital

volume load
Heart Defect
Acyanotic
pressure load
Cyanotic
↑ pulmonary flow
↓ pulmonary flow
left-to-right shunts obstr. ventric. outflow
•
atrial septal defect
• pulmonary valve stenosis
transpos. of gr. Vessels
tetralogy of Fallot
•
ventricular septal defect
aortic valve stenosis
single ventricle
pulmonary atresia
•
AV canal
coarctation of aorta
truncus arteriosus
tricuspid atresia
•
patent ductus arteriosus
total anomalous pulm.
total anomalous pulm.
return w/o obstruction
return w/ obstruction
Atrial
Septal defects:
 Septum
Primum Defect: Patent foramen ovale
Defect in the interatrial septum
Due to absence of the septum
Primum (Patent foramen ovale)
 Septum
Secondum Defect:
Defect in the interatrial septum
Due to absence of the septum
Secondum (Patent foramen
Secondum)
 Complete
absence of the
interatrial septum (Core
Triloculare
Biventriculare):
There is complete absence
of both septum primum
and septum secondum. i.e.
there is a common atrium.
 Patent
 The
osteum primum:
foramen primum may fail
to close inspite of formation of
the foramen secondum .
 This will cause disturbance in
the valvular mechanism of the
interatrial septum.
 Premature
closure
of foramen ovale:
Rarely , it is closed
BEFORE birth.
It results in marked
enlargement of the Right
atrium and ventricle.
 Persistent
atrioventricular
canal:
 The A-V cushions and the
septum intermedium fail to
develop.
 The A-V canal remains divided
into rt and lt parts.
 There is usually:
1- Patent foramen primum
2- IV septal defect
3- Abnormalities in the leaflets of
the valves guarding the A-V canal.
Ventricular
Septal defects:
Usually in the
membranous part
Tetralogy of Fallot:
Four co-occurring heart
defects:
• Pulmonary stenosis
• Ventricular septal defect
• Overriding aorta
(dextroposition)
• Right ventricular
hypertrophy
 Eisenmenger's
syndrome:
Characteristics of
Eisenmenger's syndrome
summarized as:
•
•
•
•
persistent truncus arteriosus
ventricular septal defect
left-right ventricular shunt
right ventricle hypertrophy
 Congenital
Aortic valve
stenosis:
 Occurs
due to fusion of the
cusps of the aortic valve .
 Very narrow aortic orifice
will cause Lt ventricular
hypertrophy.
 Regurge of blood from the
aorta to the left ventricle
will increase the lt ventr.
Hypertrophy.
 Congenital
Aortic
valve atresia:
 Under
developed lt
ventricle
 Narrow ascending aorta
 Patent ductus arteriosus to
carry the blood into the
aorta
 Pulmonary

atresia
The pulmonary artery is
underdeveloped, the right
ventricle very small, and
also sometimes the tricuspid
valve. The condition is also
sometimes referred to as
hypoplastic right heart.
 Transposition
of the
great arteries:
 Most
common cyanotic
neonatal heart defect
• Failure of
aorticopulmonary septum to
take a spiraling course
• Fatal without PDA, ASD, &
VSD
 Single
ventricle:
A univentricular heart may be
best described as a defect that
encompasses two complete
atria that provide venous
inflow into a dominant
ventricle (either right or left)
via a malformed AV connection.
 Ectopia
 The
Cordis:
heart is bulging
outside the chest due to
wide separation in the 2
parts of the sternum
Dextrocardia:
 The
heart and great
vessels are reversed as in
mirror image
 It might be a separate
condition or part of Situs
inversus
Situs
inversus