Download Congenital Heart Disease

Congenital Heart
Disease
Cheston M. Berlin, Jr., M.D.
Department of Pediatrics
Prevalence of Congenital Heart
Disease
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Overall prevalence: 1% (8-10 per 1,000
live births) over 25,000 new patients per
annum
#1 Cause of birth defect related deaths
Acyanotic Congenital Heart Disease:
65% (VSD, ASD, PDA)
Cyanotic Congenital Heart Disease: 35%
Stephen Cyran, M,D
Congenital Heart Disease

Ask two questions:
– Is the patient cyanotic (blue)?
– Is a shunt (blood flow) present?
Occurs in 1% of newborns
 Most can be fixed
 Patients need to be followed into adult
life

Congenital Heart Disease
CYANOSIS
+
+
SHUNT
-
-
NORMAL HEART
2 right
Pulmonary veins
2 left
svc
Right atrium
Left atrium
ivc
Right ventricle
Pulmonary artery
Left ventricle
Aorta
Atrial Septal Defect - ASD
O.5 per thousand births
 Patent foramen ovale - has flap, only
right to left shunt. Must exist for life to
continue in some defects.
 For both Ostium Secundum and Ostium
Primum - increased L
R shunt
 Surgical closure effective

Atrial Septal Defect
2 right
Pulmonary veins
2 left
svc
Right atrium
Left atrium
ivc
Right ventricle
Pulmonary artery
Left ventricle
Aorta
Ventricular Septal Defect - VSD
2.2 per 1,000 births
 Small lesions very common - 3-5% spontaneous closure
 Increased pulmonary blood flow - may
lead to pulmonary hypertension
 Surgical closure effective
 If high in ventricular wall, will involve the
atria - endocardial cushion defect

Ventricular Septal Defect
2 right
Pulmonary veins
2 left
svc
Right atrium
Left atrium
ivc
Right ventricle
Pulmonary artery
Left ventricle
Aorta
AV Canal (endocardial cushion
defect)
2 right
Pulmonary veins
2 left
svc
Right atrium
Left atrium
ivc
Right ventricle
Pulmonary artery
Left ventricle
Aorta
Coarctation of the Aorta
0.3 per 1,000 births
 May be silent - hypertension in the
upper extremities is the clue
 Surgery effective

Coarctation of the aorta
2 right
Pulmonary veins
2 left
svc
Right atrium
Left atrium
ivc
Right ventricle
Pulmonary artery
Left ventricle
Aorta
Forms of Congenital Aortic Coarctation
Stephen Cyran, M,D.
Left ventricle
Coarctation
(post ductal)
a
o r
t a
Patent Ductus Arteriosus
30-40% in premature infants
 0.4 per 1,000 in term infants
 May cause heart failure in the newborn
period
 Presence may be life saving other
cardiac defect prevents L to R shunt
 Surgery routine - ligation

Patent ductus arteriosus
2 right
Pulmonary veins
2 left
svc
Right atrium
Left atrium
ivc
Right ventricle
Pulmonary artery
Left ventricle
Aorta
Conotruncus
Most distal portion of the primitive heart
tube
 Determines normal separation of the
aorta and pulmonary outflow vessels
 Determines their normal alignment with
the ventricles

Conotruncus, Cont’d
Proliferation of cells within the
conotruncal cushion
 Migration of cells into the conotruncus
from the cardiac neural crest
 Resorption of the subaortic conus
 Leftward movement of the conotruncus

Truncus Arteriosus
Common outlet vessel
 VSD always present permitting mixing
of pulmonary with systemic blood.

Truncus arteriosus
2 right
Pulmonary veins
2 left
svc
Right atrium
Left atrium
ivc
Right ventricle
Truncus
Left ventricle
Transposition of the Great
Vessels
Complex group of lesions
 The pulmonary artery arises from the
left ventricle, and the aorta from the
right ventricle
 Incompatible with life unless shunting
occurs to permit oxygenated blood to
reach the systemic circulation
 Surgery difficult, but effective

Transposition of the
great vessels
2 right
Pulmonary veins
2 left
svc
Right atrium
Left atrium
ivc
Right ventricle
Aorta
Left ventricle
Pulmonary artery
Tetralogy of Fallot
0.5 per 1,000 live births
 Overriding aorta
 Ventricular Septal Defect
 Pulmonary stenosis
 Hypertropied wall of right ventricle
 May need temporary shunt before total
surgical correction

Tetralogy of Fallot
2 right
Pulmonary veins
2 left
svc
Right atrium
Left atrium
ivc
Right ventricle
Pulmonary artery
Left ventricle
Aorta
Total Anomalous Pulmonary
Venous Return
O.1 per 1,000 births
 All pulmonary venous blood returns to
the right side of the heart, usually the
right atrium
 Must have shunt for survival
 30% of patients have other lesions
 Surgery may be complex: depends on
anatomy

Total anomalous pulmonary
venous return
2 right
Pulmonary veins
2 left
Normal site
svc
Right atrium
Left atrium
ivc
Right ventricle
Pulmonary artery
Left ventricle
Aorta
Pulmonary atresia
TGV