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Some patients with severe pulmonary hypertension receive continuous infusions at home of prostacyclins, such as epoprostanol (flolan). These are generally delivered via a pump that the patient wears, which is attached to an indwelling catheter. As with any indwelling device, they are at risk for infection and other complications, including malfunction.
Interruption of delivery of the medication can result in rapid cardiovascular collapse, sometimes within minutes. In this instance, the medication should be resumed as quickly as possible (by a traditional IV if the catheter is not functional), and the patients should be treated as one would approach a patient with decompensated right heart failure.
I once saw a patient in the ED whose listed chief complaint was "medication refill", but was actually there for dislodgement of her prostacyclin catheter (thankfully she was ok). With more patients receiving devices they are dependent upon (insulin pumps, AICDs, prostacyclin catheters), be wary of chief complaints such as "medication refill" or "device malfunction."
Bottom Line: Interruption of continuous prostacyclin therapy for pulmonary hypertension can be rapidly fatal and should be addressed immediately.
References
Farber HW, Gin-sing W. Practical considerations for therapies targeting the prostacyclin pathway. Eur Respir Rev. 2016;25(142):418-430.
Maron BA, Zamanian R, Waxman AB, eds. Contemporary pharmacotherapies involving nitric oxide, prostacyclin, and endothelin receptor signaling pathways. In: Maron BA, Zamanian R, Waxman AB, eds. Pulmonary Hypertension: Basic Science to Clinical Medicine. Cham, Springer International Publishing, 2016; pp. 257–270