Pulmonary Vascular Disease Program
The Pulmonary Vascular Disease Program at Jefferson specializes in diagnosing and treating individuals with pulmonary hypertension (PH), with a special emphasis on those with pulmonary arterial hypertension (PAH). All patients are seen at the Jefferson Jane and Leonard Korman Lung Center in the Walnut Towers offices. Patients may be referred to the Center by other physicians or request a second opinion on their own. Pulmonary hypertension is a complex and potentially fatal disease, often associated with a variety of other diseases yet undetected. Because there is often delay in either diagnosis or institution of optimal therapies and delays in treatment have been shown to lead to worsened survival, it is imperative that patients to be seen as soon as possible at a comprehensive clinical center with dedicated expertise with this condition.
Patients undergo a thorough physician evaluation and as necessary, echocardiograms, exercise testing, nurse teaching and medication starts - all on-site. Our busy practice includes referrals for PH related to connective tissue disorders (such as scleroderma and lupus), drugs/toxins, advanced liver disease, HIV infection, sickle cell disease, pulmonary fibrosis, sarcoidosis, pulmonary embolism, and sleep apnea.
Right heart catheterizations (also known as “Swan-Ganz catheterization” or “pulmonary artery catheterization”) with acute pulmonary vasodilator trials using inhaled nitric oxide are performed regularly by Jefferson physicians at Thomas Jefferson University Hospital’s cardiac catheterization laboratory. We are particularly pleased by the success our patients have had with the inhaled and newer parenteral (intravenous and subcutaneous) prostacyclins, coordinated by our nursing staff with close collaboration with the specialty pharmacies.
We are currently enrolling PAH patients in the Symphony study, a multi-center, open-label, single-arm, Phase 3b study of macitentan in patients with PAH to psychometrically validate the PAH-SYMPACT instrument. (update).
We are currently enrolling PAH patients in the BEAT trial, a multicenter, double-blind, randomized, placebo-controlled, Phase 3 study to assess the efficacy and safety of an oral prostacyclin added on to inhaled treprostinil (Tyvaso®). (update).
We recently completed our contribution to the PROSPECT Registry to prospectively describe use of room temperature-stable IV Epoprostenol. ("RTS Epoprostenol") in patients with PAH. (update).
We are currently following PAH patients enrolled in the GRIPHON trial, a global study of a novel oral prostacyclin analogue aiming to reduce morbidity and mortality caused by PAH. [update].
We are currently following PAH patients enrolled in the ASPIRE Registry, a post-marketing observational study to assess respiratory tract adverse events in PAH patients treated with inhaled Tyvaso (treprostinil). (update).
Our clinical research interests include new oral therapies for PAH, sleep apnea evaluation in PH, right ventricular function in sickle cell disease, improved ways to measure the exercise tolerance in PAH, catheter extraction of pulmonary embolism and pathogenesis of PAH in advanced liver disease.
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