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Posts Tagged ‘av canal defect’

ASD is the most common acyanotic heart disease. Clinically distinguishing  ostium primum (OP-ASD ) from ostium secundum (OS- ASD) is not an easy  task . A Wide fixed split pf S2  , a short systolic  murmur and PA pulsations  in left second space, a loud P2 and a hyperdyanmic RV occur in both .  The following features might give a clue for OP -ASD .

  • While  OS-ASD is  often an  isolated anomaly , isolated  OP- ASD is very rare.It usually occur as a part of partial or complete AV canal .
  • Early onset of symptoms
  • Early onset of pulmonary arterial hypertension( PAH )
  • Extra murmur of  1.VSD 2.MR (Cleft mitral valve ) may be present
  • Biventricular enlargement (MR/VSD)
  • ECG -Left axis deviation -Structural defect in left bundle ?

Confirmation is by Echocardiography and angiogram is rarely required today . Documentation of  classical goose neck deformity of LV outflow confirms the diagnosis.

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