Rarely a patient may be found with both pulmonary arterial hypertension (PAH) and pulmonary arteriovenous fistulae (PAVF).
In some patients the pulmonary arterial hypertension appears to precede the development of pulmonary arteriovenous fistulae. In others the PAH appears to be a complication of PAVF.
The type of pulmonary arteriovenous fistulae needs to be determined by imaging studies, but it is often diffuse and multiple.
The presence and extent of pulmonary arterial hypertension needs to be established by echocardiography with or without additional studies (right heart catheterization, etc).
It is important to see if the patient has:
(1) risk factors for PAVF (such as hereditary hemorrhagic telangiectasia)
(2) risk factors for pulmonary arterial hypertension
(3) clinical findings that may suggest which occurred first
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