Prepared by

Dr. Majid Al-Homiedan

CYANOTIC CONGENITAL
HEART DISEASE WITH
DECREASED PULMONARY

DDx od Cyanotic Congenital Heart Diseases with decreased

pulmonary vascularity
Tetralogy of Fallot
 Most common cause of cyanotic congenital heart disease beyond the
immediate neonatal period. Consists of (1) high ventricular septal
defect, (2) obstruction to right ventricular outflow (usually infundibular
stenosis), (3) overriding of the aortic orifice above the ventricular
defect, and (4) right ventricular hypertrophy.
 Decreased pulmonary vascularity; flat or concave pulmonary outflow
tract; enlargement of the right ventricle; right aortic arch in
approximately 25% of cases.

DDx od Cyanotic Congenital Heart Diseases with decreased

pulmonary vascularity
Pseudotruncus
Arteriosus
 Single large arterial trunk receives the outflow of blood
from both ventricles. The pulmonary arteries are absent,
so the pulmonary circulation is supplied by bronchial or
other collateral vessels.
 Decreased pulmonary vascularity; flat or concave
pulmonary outflow tract; enlargement of the right
ventricle; right aortic arch in approximately 40% of cases

DDx od Cyanotic Congenital Heart Diseases with decreased

pulmonary vascularity
Trilogy of Fallot
 Combination of pulmonary
valvular stenosis with an
intact ventricular septum
and an interatrial shunt
(patent foramen ovale or
true atrial septal defect).
Increased pressure on the
right side of the heart due
to pulmonary stenosis
causes the interatrial shunt
to be right to left.
 Decreased pulmonary
vascularity; poststenotic
dilatation of pulmonary
artery; heart size often
normal (usually some
evidence of right
ventricular hypertrophy).
DDx od Cyanotic Congenital Heart Diseases with decreased
pulmonary vascularity
Tricuspid
atresia/stenosis
 Decreased pulmonary
vascularity (usually
some degree of
pulmonary stenosis);
striking enlargement
of the right atrium if
small atrial shunt;
large left ventricle;
small right ventricle.

DDx od Cyanotic Congenital Heart Diseases with decreased

pulmonary vascularity
Ebstein's anomaly
 Downward displacement of
an incompetent tricuspid
valve into the right
ventricle.
 Decreased pulmonary
vascularity; flat or concave
pulmonary outflow tract;
characteristic squared or
boxed appearance of the
heart (bulging of the right
heart border by the
enlarged right atrium);
narrow vascular pedicle
and small aortic arch.

DDx od Cyanotic Congenital Heart Diseases with decreased

pulmonary vascularity
Uhl's disease
 Focal or complete
absence of the right
ventricular
myocardium (the right
ventricle becomes a
thin-walled fibroelastic
bag that contracts
poorly and cannot
effectively empty
blood from the right
side of the heart).
 Radiographic pattern
identical to that in
DDx od Cyanotic Congenital Heart Diseases with decreased
pulmonary vascularity