Pulmonary Vascular Disease: Pulmonary Vascular Disease |

First Highly Promising Experiences With Implantations of a Gas Driven Pump for Prostacyclin Therapy in Patients Even With Severe Pulmonary Hypertension and Concomitant Hematologic Malignancy FREE TO VIEW

Regina Steringer-Mascherbauer, MD; Uwe Fröschl, MD; Josef Aichinger, MD; Reinhold Fuegger, MD
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KH Elisabethinen, Linz, Austria

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A502. doi:10.1016/j.chest.2016.02.524
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SESSION TITLE: Pulmonary Vascular Disease

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, April 17, 2016 at 11:45 AM - 12:45 PM

PURPOSE: Pulmonary hypertension (PH) is a progressive fatal disease requiring aggressive specific therapy. As parenteral prostanoid administration with external pumps is associated with local side effects for subcutaneous treprostinil and rare but severe catheter-related infections for intravenous prostanoids, the availability of an implantable pump for intravenous treprostinil represents a significant progress. This surgical approach requires careful interdisciplinary interaction as PH patients carry significantly elevated anesthesia risks, especially with such relevant comorbidities.

METHODS: At our specialized PH center data are documented in ELPHREG (Elisabethinen Linz Pulmonary Hypertension Registry).

RESULTS: Between March 2012 and April 2015 three patients with severe PH and concomitant hematological malignancy were implanted at our center. According to our standard operating procedures all patients were uptitrated with subcutaneous treprostinil. Eligibility for anesthesia and pump implantation were independently assessed by the PH specialist, the anesthesiologist and the surgeon. All implantations at our center are performed by a dedicated surgical team. No perioperative complications were observed. In patient 3 a postoperative bleeding episode was managed during hospital stay. No other complications, especially no infections were observed. Patient 1 deceased 12 months after pump implantation due to the underlying malignancy. Currently a fourth patient, a 65 year old female with a diagnosis of post polycythemia vera myelofibrosis is on the waiting list for implantation as thrombocytopenia - a known side effect of ruxolitinib - is for now a contraindication for surgery.

CONCLUSIONS: The coincidence of life-threatening diseases provides an extraordinary challenge. Given intensive cooperation between all departments involved in the patient management and profound experience with the implantation procedure this treatment option can safely be offered even to patients with severe hematologic comorbidities as lymphomas. Implantation of an infusion pump for intravenous treprostinil in patients with severe hematologic comorbidity has not been reported before.

CLINICAL IMPLICATIONS: The treatment of such patients has to be restricted to expert centers, implantations to surgically experienced specialised PH centers.

DISCLOSURE: The following authors have nothing to disclose: Regina Steringer-Mascherbauer, Uwe Fröschl, Josef Aichinger, Reinhold Fuegger

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