IMPORTANCE: Pulmonary 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. OBJECTIVE: To 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. DESIGN: Multicenter, 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. SETTING: Three large tertiary PH centers. PARTICIPANTS: One hundred forty consecutive patients newly referred to PH centers were invited to participate, and all consented to do so. RESULTS: Of 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 RELEVANCE: Patients 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.
IMPORTANCE: Pulmonary 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. OBJECTIVE: To 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. DESIGN: Multicenter, 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. SETTING: Three large tertiary PH centers. PARTICIPANTS: One hundred forty consecutive patients newly referred to PH centers were invited to participate, and all consented to do so. RESULTS: Of 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 RELEVANCE: Patients 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.
Authors: Anna R Hemnes; Aaron W Trammell; Stephen L Archer; Stuart Rich; Chang Yu; Hui Nian; Niki Penner; Mitchell Funke; Lisa Wheeler; Ivan M Robbins; Eric D Austin; John H Newman; James West Journal: Circulation Date: 2014-10-31 Impact factor: 29.690
Authors: Mei-Sing Ong; Mary P Mullen; Eric D Austin; Peter Szolovits; Marc D Natter; Alon Geva; Tianxi Cai; Sek Won Kong; Kenneth D Mandl Journal: Circ Res Date: 2017-06-13 Impact factor: 17.367
Authors: Kari R Gillmeyer; Ming-Ming Lee; Alissa P Link; Elizabeth S Klings; Seppo T Rinne; Renda Soylemez Wiener Journal: Chest Date: 2019-05 Impact factor: 9.410
Authors: Kari R Gillmeyer; Kyung M Lee; Qing Shao; Donald R Miller; Bradley A Maron; Elizabeth S Klings; Seppo T Rinne; Renda Soylemez Wiener Journal: Ann Am Thorac Soc Date: 2019-08
Authors: Kari R Gillmeyer; Ming-Ming Lee; Alissa P Link; Elizabeth S Klings; Seppo T Rinne; Renda Soylemez Wiener Journal: Chest Date: 2018-11-22 Impact factor: 9.410