• Roderick C Deaño, Cherylanne Glassner-Kolmin, Melvyn Rubenfire, Adaani Frost, Scott Visovatti, Vallerie V McLaughlin, and Mardi Gomberg-Maitland.
• Department of Medicine, University of Chicago, Chicago, Illinois 60637, USA.
• JAMA Intern Med. 2013 May 27;173(10):887-93.

ImportancePulmonary hypertension (PH) is a fatal disease. Although the prognosis of pulmonary arterial hypertension (PAH) has improved with targeted therapies, the outcome is dependent on early detection and an accurate diagnosis.ObjectiveTo determine the accuracy of PH diagnoses in patients referred to PH centers and the frequency of PAH-specific medication use despite an uncertain or incorrect diagnosis.DesignMulticenter, descriptive, cross-sectional study. During a 10-month period in 2010 and 2011, data on newly referred patients were collected and entered into a secure Internet database.SettingThree large tertiary PH centers.ParticipantsOne hundred forty consecutive patients newly referred to PH centers were invited to participate, and all consented to do so.ResultsOf 140 patients referred with a mean age of 56 years, 95 (68%) were referred by cardiologists or pulmonologists and 86 (61%) had disease classified as World Health Organization functional class III or IV. Fifty-six of the prereferral diagnoses (40%) were PAH, 42 (30%) unknown, and 22 (16%) PH secondary to lung disease or hypoxia. Of the 98 patients who received a definitive diagnosis before referral, 32 (33%) received a misdiagnosis. Fifty-nine patients underwent catheterization of the right and/or left side of the heart for the first time at the tertiary center. Of the 38 patients who underwent catheterization of the right side alone, 14 (37%) received a different diagnosis after undergoing the procedure; of the 21 patients who underwent catheterization of both sides of the heart, 11 (52%) received a different diagnosis after undergoing the procedures. Forty-two patients (30%) had started receiving PAH-specific medications before referral, with 24 of the prescriptions (57%) contrary to published guidelines.Conclusions And RelevancePatients referred to PH centers for diagnosis and treatment are often referred late (with functional class III or IV disease), receive misdiagnoses, and are inappropriately prescribed medications. A reevaluation of educational efforts is required to improve awareness and the care and outcome of patients diagnosed as having PH.

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