Unknown atrial septal defect was diagnosed during the course of treatment for portopulmonary hypertension: A case report

Portopulmonary hypertension (POPH) is a condition defined by the development of pulmonary arterial hypertension (PAH) that is associated with liver disease or portal hypertension. Untreated POPH has a poor prognosis. POPH can be treated with PAH-targeted therapy and can sometimes resolve with liver transplantation (LT); however, poorly controlled POPH can preclude LT owing to an increased peri-operative risk. We report a case of POPH that was diagnosed as an unknown atrial septal defect during PAH-targeted therapy. As observed in our case, the same patient may have several causes of PAH. It is important to confirm an unknown shunt disease using echocardiography during PAH-targeted therapy.

Learning objective: It is difficult to detect unknown shunt disease in patients who are diagnosed with pulmonary arterial hypertension (PAH) owing to other causes. We report a case of portopulmonary hypertension that was diagnosed as an unknown atrial septal defect during PAH-targeted therapy. This report highlights the same patient may have several causes of PAH. It is important to confirm an unknown shunt disease using echocardiography during PAH-targeted therapy.