Referral of Patients With Pulmonary Hypertension Diagnoses to Tertiary Pulmonary Hypertension Centers: The Multicenter RePHerral Study

Study Questions:

What is the accuracy of pulmonary hypertension (PH) diagnoses in patients referred to PH centers, and the frequency of PAH-specific medication use despite an uncertain or incorrect diagnosis?


This was a 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 of three large tertiary PH centers. All 140 consecutive patients newly referred to PH centers were invited to participate, and consented to do so.


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.


The authors concluded that patients referred to PH centers for diagnosis and treatment are often referred late, receive misdiagnoses, and are inappropriately prescribed medications.


This study suggests that patients referred to PH centers for diagnosis and treatment are referred with advanced disease and are often misdiagnosed, misclassified, and treated inappropriately with PAH medications or undertreated in cases of severe PAH disease. Current guidelines recommend that physicians should refer patients early in the diagnostic process to PH centers prior to medication initiation, even if the prereferral diagnosis is incomplete. Since present efforts have not succeeded in promoting early diagnosis, early referral, or the importance of the diagnostic and treatment algorithm, we need to re-evaluate our approaches to improve the care of patients with PH.

Keywords: Uncertainty, Diagnostic Errors, Cross-Sectional Studies, Early Diagnosis, Referral and Consultation, Diagnosis, Differential, Hydrazines, Catheterization, Hypertension, Pulmonary, Cardiovascular Diseases, Databases, Factual, Lung Diseases

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