PURPOSE: Pulmonary hypertension (PH) can be associated with decreased functional capacity and poor prognosis in patients with parenchymal lung diseases. Yet, little attention has been given to current patterns of managing patients with PH and PLD.
METHODS: An 18-question survey was submitted electronically to all members of the Pulmonary Vascular Disease, Interstitial and Diffuse Lung Disease, Cardiovascular Medicine and Surgery, and Clinical Pulmonary Medicine Networks of ACCP. Survey responses were analyzed to determine current beliefs and practice patterns.
RESULTS: The survey was sent to 2821 members; 453 (16%) responded. 72% of respondents believed that PH occurs in >20% of IPF patients and only 45% of respondents believed that PH occurs in >20% of COPD patients. Most (95%) respondents reported testing for PH in patients with parenchymal lung diseases using transthoracic echocardiography (TTE) or right-heart catheterization (RHC). Most respondents believed that PH could occur in the absence of severe compromise in pulmonary function (70%) and hypoxia (50%). Approximately 30% of physicians reported not performing RHC to confirm a diagnosis of PH before beginning therapy. Most respondents (92%) felt that medical therapy was effective and prescribed vasoactive medications. The medication of first choice was bosentan or sildenafil in patients with IPF or sarcoidosis and sildenafil in patients with COPD. Most respondents believed that treating PH in these patients improves quality of life (63%) and dyspnea (67%), but were less sure about the impact on functional capacity and survival.
CONCLUSION: Practicing physicians believe that PH occurs in a significant proportion of patients with parenchymal lung diseases. Approximately 30% of physicians do not perform RHC to confirm this diagnosis prior to initiating therapy. Despite little supportive evidence, most physicians treat with vasoactive medications and believe that medical therapy can confer benefit. This study highlights the need for more studies to assess the true impact of currently available medications in patients with PH and parenchymal lung diseases.
CLINICAL IMPLICATIONS: PH in LD requires further study.
DISCLOSURE: Omar Minai, Consultant fee, speaker bureau, advisory committee, etc. Actelion, Gilead, United Therapeutics, and Pfizer; No Product/Research Disclosure Information