Pediatric Pulmonary Stenosis

  • Etiology:
    — Can occur within the ventricle (infundibular stenosis) or at pulmonary valve (valvular stenosis) or in pulmonary artery (peripheral stenosis)
    — Isolated valvular stenosis is much more common and is due to fusion of 3 cusps of the valve
  • Imaging CXR:
    — Post stenotic dilation of main pulmonary artery especially in left pulmonary artery because jet of blood shoots straight into it
    — Heart is normal is size as is the pulmonary vascularity
    — Right ventricular hypertrophy does not change the size of right ventricle but an upturned cardiac apex can be seen due to superior displacement of the left ventricle by the hypertrophied right ventricle
  • DDX:
  • Complications: Stenosis leads to increased right ventricle end systolic pressure and right ventricular hypertrophy and then right ventricular dilation when the right ventricle decompensates
  • Treatment:
  • Clinical: Usually asymptomatic with a systolic thrill associated with an ejection type murmur heard best over pulmonary area

Radiology Cases of Pulmonary Stenosis

Radiology Cases of Valvular Pulmonary Stenosis

CXR and angiogram of valvular pulmonary stenosis
CXR AP (left) shows prominence of the main pulmonary artery. AP image from a right ventricular injection of an angiogram (above right) shows a dilated main pulmonary artery while the lateral (below right) from the same injection shows doming of the pulmonary valve and a narrow jet of contrast entering the dilated main pulmonary artery.

Radiology Cases of Peripheral Pulmonary Stenosis of the Left Pulmonary Artery

CXR of left pulmonary artery stenosis
CXR AP shows markedly decreased pulmonary vascularity in the left lung when compared to the right lung. The lungs are clear.