Conference: 2014 International PHA Conference and Scientific Sessions
Release Date: 06.22.2014
Presentation Type: Abstracts
File Download: 2014 Conference Abstract - James Tarver
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Recognition of PAH is still delayed in many patients, resulting in their presentation with advanced stage disease (Functional Class III - IV) and advanced RV dysfunction. RV function directly correlates with survival in patients with PAH.
Background: Recognition of PAH is still delayed in many patients, resulting in their presentation with advanced stage disease (Functional Class III - IV) and advanced RV dysfunction. RV function directly correlates with survival in patients with PAH. Many patients who present with such advanced disease are unable to recover RV function. Evidence of RV improvement often lags behind symptom improvement.
Case History: A 52 y.o. male was transferred from an outside institution after presenting with dyspnea, exertional syncope & elevated cardiac enzymes. He had similar symptoms 2 years earlier & was diagnosed with PAH but was untreated after leaving AMA. RV dysfunction was described at that time. An echo prior to transfer revealed RVSP (estimated at 80 mmHg) with RV dilation & septal flattening consistent with RV pressure and volume overload, & a small, compressed LV. RHC at our facility revealed RA – 14 mmHg, PA – 75/33, mean 46 mmHg PCWP mean – 5 mmHg, CO – 2.2 l/min, CI – 1.0 l/min/m2. PVR – 1501 dynes, and no vasodilator response to IV adenosine. IV Treprostinil was initiated with a rapid titration protocol (2ng/kg/min increasing by 2 ng/kg/min q 12 hours) in addition to oral Tadalafil. He experienced rapid improvement in symptoms, and within 7 days was ambulating at a normal pace without symptoms. Follow up echocardiogram 6 days after initiation of IV Treprostinil(dose=16 ng/kg/min) revealed complete resolution of the RV pressure and volume overload, with substantial improvement in RV function (Fractional Area Change 11.6%-->34%, 3D RVEF – 39%). and reduction in TR velocity (4.19 m/s-->3.43 m/s). His IV Treprostinil was converted to SQ administration prior to discharge. He continues to do well clinically with improving 6MWT (currently 315m) on a dose of 31 ng/kg/min
Type: Case Study