Conference: 2012 International PHA Conference and Scientific Sessions
Release Date: 06.22.2012
Presentation Type: Abstracts
Roderick Deaño, MD, MPH1; Adaani Frost, MD2; Scott Visovatti, MD3; Cherylanne Glassner, BS4; Vallerie V. McLaughlin3 Mardi Gomberg-Maitland, MD, MSc1; Melvyn Rubenfire, MD3
1. U of Chicago Medical Center, USA
2. Baylor College of Medicine, USA
3. U of Michigan, USA
4. U of Chicago, USA
BACKGROUND: Pulmonary arterial hypertension (PAH, Group 1) is a rare disorder with a poor prognosis. The pathogenesis and response to treatment differs from other causes of pulmonary hypertension (PH) including pulmonary venous (Group 2), hypoxic and respiratory (Group 3), and chronic thromboembolic (Group 4). A heart catheterization is necessary to establish the diagnosis and prognosis. Because of the complexity, it should be managed by experienced physicians preferably in specialized tertiary PH centers. We assessed the accuracy of the diagnosis of PH in patients referred to PH centers and the frequency of use of PAH specific medications despite an uncertain or incorrect diagnosis.
METHODS: Information on new consented sequential PH patient referred from three tertiary PH centers (U of Michigan, Baylor, and U of Chicago) were collected and entered onto a secure internet database. Descriptive analysis was performed via STATA 11.
RESULTS: Of 141 referred PH patients evaluated over a 10 month period, 71% were female with a mean age of 56. 55% were functional class III/IV. 95% were physician referred; 80% by specialists. The most common diagnoses pre-referral was group 1 (40%), followed by ‘unknown’ (30%), group 3 (16%) and group 2 (9.2%). 30% had been initiated on PAH specific drugs pre-referral including: prostacyclins (3%), endothelial antagonists (12.1%), and phosphodiesterase inhibitors (23.4%); 21.3% without a right heart catheterization. At the completion of the evaluation, 41% of the patients were diagnosed as group 1, 20.6% did not have PH, 20% were group 2, and 13.5% group 3. Pre-referral 30.5% of the patients were diagnosed incorrectly or given a diagnosis of “unknown” and of the 42 patients initiated on PH-specific medications, 42% were initiated on PH medications inappropriately. Of the 41% of total patients diagnosed with group 1 post-referral, one fifth (21%) % were classified as ‘unknown’ pre-referral. Of the 20% of total patients diagnosed with group 2 PH post-referral, nearly half (46%) were classified as “unknown” 28.6% as group 2, 14% as group 3 and 10.7% as group 1 pre -referral.
CONCLUSION: Patients referred to PH centers are often misdiagnosed and misclassified and frequently initiated on medications inappropriately.