Objectives: Pulmonary arterial hypertension (PAH) in its advanced stages is complicated by right heart failure and often pericardial effusion. The optimal treatment of large or hemodynamically significant pericardial effusions in this group has not been defined.
Methods: All patients followed at the Johns Hopkins Hospital for PAH during a 1-year period that underwent pericardiocentesis or pericardial window placement were identified. Charts were analyzed for patient characteristics, echocardiographic data, and type/outcome of procedure.
Results: Six patients were identified; five underwent therapeutic drainage. Pericardiocentesis was performed in four cases; two had surgical pericardial windows. Two patients died after pericardiocentesis and one patient died after surgery. All patients died within 13 hours of the procedure.
Conclusion: We found a high mortality related to pericardial fluid drainage in patients with PAH. The pathophysiologic explanation for these deaths remains unclear, but clinicians should consider conservative management in this situation if possible.