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Intravenous Prostacylin Therapy in Pulmonary Hypertension

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Conference: 2014 International PHA Conference and Scientific Sessions

Release Date: 06.22.2014

Presentation Type: Abstracts

File Download: 2014 Conference Abstract - Hansdottir S

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Background: Portopulmonary hypertension (PoPH) is found in 2-12.5 % of patients with liver disease. Prostacyclin (PC) therapy has not been well studied in this population. The purpose of this analysis is to describe efficacy and safety of intravenous (IV) PC therapy in PoPH. 
Methods: A single center, retrospective analysis of patients with PoPH managed with IV PC over a 5 year period (2008-2013). Four patients (1 male, 3 females- mean age 50 years) were identified. Etiologies included portal vein thrombosis, hepatitis C, and primary sclerosing cholangitis. Three patients were on epoprostenol (mean maximal dose=44ng/kg/min) and one on treprostinil (max dose=97ng/kg/min). Two patients were on background sildenafil. Mean treatment time was 3.3 years. Statistical comparison prior to and on PC treatment was done using a paired, two tailed Student’s T-test.

Results:

 

Before
Mean (SD)

On treatment
Mean (SD)

p-value

Hemodynamics

 

 

 

mRA (mmHg)

18 (6.5)

12.5 (5.8)

0.487

mPAP (mmHg)

66.3 (24.0)

52.8 (24.9)

0.042*

CO (thermodilution in L/min)

4.4 (2.3)

7.7 (2.7)

0.081

PVR (Wood units)

15.3 (11.3)

6.5 (7.6)

0.048*

Clinical Parameters

 

 

 

NT proBNP

3530 (2306)

568 (601)

0.105

6-MWT distance

159 (159)

361 (122)

0.076

WHO  FC I

 

1 (25%)

 

WHO  FC II

 

2 (50%)

 

WHO  FC III

1 (25%)

1 (25%)

 

WHO  FC IV

3 (75%)

 

 

WHO: World Health Organization. FC: Functional class.

* Statistically significant (p< 0.05)

One patient had normalization of hemodynamics and underwent a liver transplantation, one patient was able to transition to inhaled therapy and two remain on IV PC therapy. Complications included 2 mechanical catheter issues and 2 catheter related infections.

Conclusions: All patients in this study responded favorably to PC therapy with improvement in symptoms by at least one WHO FC and clinically significant improvement in exercise capacity. In spite of the small size of the study changes in mPAP and PVR were statistically significant. All developed catheter related problems, only one of which was severe (sepsis in a patient on immunosuppression). We conclude that PC therapy can be done safely and successfully in this highly complex population. Close monitoring and early intervention of catheter related complications is essential.