Catheter-Related Venous Thrombosis in Pulmonary Arterial Hypertension Patients on Intravenous Prostacyclin Therapy

Abstract

Rationale: Intravenous prostacyclin (PG) therapies are used for managing severe pulmonary arterial hypertension (PAH). Most PAH patients respond to PG therapies, but some experience complications associated with presence of indwelling catheters. We observed development of venous thromboses in patients and present our experience. Methods: A retrospective chart review of PAH patients on long-term intravenous PG therapy was conducted between 2008 and 2017.Demographic, hemodynamic, six-minute walk distance (6MWD), WHO/NYHA functional class (FC) and outcome data were collected. Results: Six of 63 patients on intravenous PG therapy were identified with venous thromboses. Mean age was 44±15 years (mean ±SD, range 31 to 70 years). All patients were female. Duration of PAH diagnosis 15±7 years; duration of IV PG therapy7.4±3.8 years. One patient had antiphospholipid antibody syndrome; hypercoagulable workup was negative for other five patients. Diagnostic right heart catheterization showed mean pulmonary artery pressure of 53.6±5.4 mmHg and thermodilution cardiac output was 4.94±1.4 l/min. Echocardiogram showed RAP of 8.3±6 mmHg. Development of thrombosis occurred 4±2 years after initiation of therapy. most common site of thrombosis was subclavian vein followed by femoral vein. All patients were on anticoagulation therapy with warfarin or a novel oral anticoagulation agent prior to development of thrombosis. Because of repeated catheter infections and poor vascular access, two of three patients were successfully transitioned to oral therapies without significant change in FC. Conclusion: A small percentage of patients on intravenous PG therapy for PAH develop thrombosis in areas of venous access. There does not appear to be a difference in occurrence of thrombosis in patients who have idiopathic PAH or associated PAH. Anticoagulation did not prevent initial or subsequent thrombosis. Development of recurrent thrombosis limits vascular access available for infusion of intravenous PG, thereby necessitating transition to alternative therapy. Transitioning off intravenous PG could affect patient's symptoms and FC. Funding Statement: authors state that no funding was received for this research. Declaration of Interests: authors state: The authors have no conflicts to disclose. Ethics Approval Statement: A waiver of consent was granted by the Institutional Review Board.