Infundibular pulmonary stenosis

Case contributed by Dr Vincent Tatco

Presentation

Decreased exercise tolerance. Prior echocardiogram showed congenital heart disease.

Patient Data

Age: 30 years old
Gender: Female

The walls of the infundibulum is markedly thickened. A linear structure, likely a thin membrane, was seen attached to the anterior wall of the thickened infundibulum and the ventricular side of the interventricular septum. Cine images show a narrow jet across the narrowed infundibulum consistent with a severe infundibular pulmonary stenosis. The main pulmonary trunk is dilated.

The left brachiocephalic vein does not join the right and is seen to course inferiorly as a persistent left superior vena cava that drains into the dilated coronary sinus.

Case Discussion

This is a case of infundibular pulmonary stenosis, which is a congenital heart defect with an obstruction to the right ventricular outflow tract at the subvalvular level. An isolated pulmonary stenosis occurs in 8 to 12% of all congenital heart defects. Pulmonary stenosis is often associated with many other congenital heart disease, such as tetralogy of Fallot and single ventricle. An isolated infundibular pulmonary stenosis is rare. 

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