Pulmonary stenosis

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  1. Subvalvular (Right ventricular outflow tract)
  2. Valvular
  3. Supravalvular (pulmonary truncus)
  4. Peripheral (at the bifurcation)
  5. Peripheral in the pulmonary artery

Isolated PS occurs with a frequency of 9% of all congenital cardiac abnormalities and, combined with other cardiac abnormalities, with a frequency of nearly 21%.

All forms lead to an obstruction of blood flow from the right ventricle to the pulmonary arteries. Depending on how severe the stenosis is a compensatory hypertrophy of the right ventricle developes.

Pathophysiology:

Light to middle-degree pulmonary stenoses often do not show themselves clinically and the development of the child is normal. As a rule, larger stenoses lead to right ventricular hypertrophy and heart failure. Symptoms include:

  • Dyspnea and tachypnea
  • Hepatomegaly due to blood congestion
  • Cyanosis may occur due to a persisting foramen ovale and R–>L shunt

Diagnosis:
In an ECG (signs of right hypertrophy), X-ray, ECHO.
Therapy:
Balloon dilatation or surgical correction.

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