PURPOSE: Implementation of the 2004 ACCP Recommendations for the Management of Pulmonary Arterial Hypertension (PAH) was studied in quality enhancement research initiative (QuERI) in PAH 517 patients among 52 US specialists.
METHODS: Physicians were asked to enroll PAH patients (known or newly diagnosed) and provide data on their medical management.
RESULTS: PAH was idiopathic in 37%, familial in 3%, had associated conditions in 50%: CTD in 28%, drug exposure in 9%, shunt in 7%, portal hypertension in 4%, HIV in 3% and venous/capillary involvement in <1%. WHO class was available in 471 patients: 9% were class I, 39% class II, 47% class III, and 5%class IV. Data on disease-specific treatments was available in 450 patients (87%). Epoprostenol/inhaled iloprost/treprostinil (PGI) was used in 33.7% overall and alone in 8.0%. Sildenafil (SID) was used in 37.3% overall and alone in 8.8%. Bosentan (BOS)was used in 53.1% and alone in 26.0%. Combination therapy was: PGI + SID in 16.9%; BOS + SID in 18.2%; PGI + BOS in 15.6%; and PGI + BOS + SID in 6.7%. 19.8% were on no therapy. All of these therapies were more frequently used as symptom class worsened. Among 96 patients on CCB, only 27 had acute vasoreactivity testing performed; only 6 of these were reported to have ACCP-defined vasoreactivity (22% of those tested for vasoreactivity and only 6.3% of the total population). Recommended PAH therapy of anticoagulants (warfarin) was used in 38% of class II, 52% of class III and 44% of class IV (44% overall). Diuretics were used in 40%, 53%, and 72% of class II, III, and IV, respectively. Modulators of renin system were used in 21% of patients and digoxin in 16%.
CONCLUSION: Medical management of PAH is complex with frequent use of multiple therapies. Disease specific therapies and diuretics are used more frequently as symptoms worsened. Vasoreactivity testing to direct CCB treatment was not performed in the majority of patients.
CLINICAL IMPLICATIONS: Greater adherence to ACCP guidelines for vasoreactivity testing is recommended.
DISCLOSURE: Vallerie McLaughlin, No Financial Disclosure Information; No Product/Research Disclosure Information