DDx od Cyanotic Congenital Heart Diseases with decreased pulmonary vascularity

CYANOTIC CONGENITAL HEART DISEASE WITH DECREASED PULMONARY VASCULARITY

Prepared byDr. Majid Al-Homiedan

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 Ebstein's anomaly.