Valvar Pulmonary Stenosis Differential Diagnoses

Updated: Dec 28, 2020
  • Author: Syamasundar Rao Patnana, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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Diagnostic Considerations

Important considerations

Exclude associated congenital anomalies and to detect the presence of cyanosis or a ductal-dependent lesion is a major error.

Do not fail to diagnose a serious congenital heart defect, such as tetralogy of Fallot is problematic.

Note the following:

  • Acyanotic patients with tetralogy of Fallot and those with a mild obstruction of the right ventricular outflow tract may have similar presentations and physical findings.

  • Tetralogy of Fallot is a lesion that is surgically correctable and that can be corrected safely, even in the neonatal period.

  • A tet spell, or hypercyanotic spell, is potentially lethal but frequently aborted with simple maneuvers. Such spells and can occur in previously acyanotic patients with tetralogy of Fallot ("pink tets").

  • Echocardiography can reliably confirm the precise diagnosis and help in differentiating valvar pulmonary stenosis from tetralogy of Fallot.

Echocardiography should not be withheld if complex anatomy is suspected.

Complications associated with balloon pulmonary valvuloplasty, such a rupture or perforation of the pulmonary artery or right ventricular outflow tract, are uncommon but can occur. This possibility should be explained to all parents.

Other problems to be considered

Also consider the following conditions in patients with suspected valvar pulmonary stenosis:

  • Complex congenital heart disease associated with findings of pulmonary stenosis

  • Infundibular and/or subpulmonary stenosis

  • Supravalvar pulmonary stenosis

  • Double-chambered right ventricle

  • Syndrome of absent pulmonary valves

Differential Diagnoses