PURPOSE: Implementation of the 2004 ACCP Recommendations for the Diagnosis and Differential Assessment of Pulmonary Arterial Hypertension (PAH) was measured as part of a quality enhancement research initiative (QuERI) in 517 patients with PAH among 52 US specialist physicians.
METHODS: Physicians were asked to enroll PAH patients (known or newly diagnosed) and provide data on recommended diagnostic tests.
RESULTS: (Median, 25th and 75th percentile): Patients enrolled were 55 years old (44, 66), 77% female, with a BMI of 27.5 kg/m2 (23, 33), BP was 118/70 mmHg (104,130/61,78), and heart rate was 80 bpm (72,92). Most were functional class II or greater (91%) and 40% were using supplemental oxygen. PAH was reported as idiopathic in 37%, familial in 3%,and was associated with conditions in 50% as follows: connective tissue disorders (CTD) in 28%, drug exposure in 9%, congenital systemic-to-pulmonary shunt in 7%, portal hypertension in 4%, HIV in 3% and associated with significant venous or capillary involvement in <1%; the rest having been classified as “other”. WHO class was available in 471 patients: 42 were class I (9%), 182 class II (39%), 222 class III (47%), and 25 class IV (5%).The frequency of recommended diagnostic tests was: chest x-ray: 82%, ECG: 72%, echocardiogram: 89%, right heart catheterization (RHC): 77%, pulmonary function testing: 77%, and V/Q scanning in only 52%. CTD screen: 53%, HIV: 36%, CBC: 89%, liver function: 93%. All three tests: CXR, ECG, and echocardiogram were performed in 344 out of 517 (67%) patients.
CONCLUSION: Physicians treating PAH report multiple etiologies requiring a comprehensive and multi-pronged approach to the diagnostic evaluation. This preliminary assessment suggests that certain essential diagnostic tests maybe underutilized. ACCP guidelines recommend V/Q scanning to exclude correctable causes of PAH. ACCP guidelines also recommend HIV testing in all patients evaluated for PAH. These guidelines are not universally followed based on this review.
CLINICAL IMPLICATIONS: Stricter adherence to guidelines may result in more optimal management of these high-risk patients.
DISCLOSURE: Ronald Oudiz, No Financial Disclosure Information; No Product/Research Disclosure Information