Advances in the field of pulmonary hypertension have led to the emergence of several treatment options for patients with idiopathic pulmonary arterial hypertension aimed at slowing disease progression and improving quality of life. These advances, however, have progressed more rapidly than our understanding of how to use these novel therapies clinically. Although there are no current therapy guidelines or evidence-based recommendations directing therapy, clinicians must make many decisions as to when and how to re-evaluate patients after treatment initiation, to define an adequate response and to plan ongoing management. This survey-based study assessed the attitudes of Canadian physicians caring for patients with idiopathic pulmonary arterial hypertension, and aimed to generate a consensus opinion regarding the methods of reassessment and therapeutic goals after treatment initiation.
Many treatment options are now available for patients with idiopathic pulmonary arterial hypertension (IPAH). Data regarding the optimal combination of therapies are lacking, as is consensus on how to assess response to therapy and when to change therapeutic regimens.
To gather the opinions of Canadian pulmonary hypertension (PH) experts regarding standard practice in the care of IPAH patients after therapy is initiated.
Canadian PH physicians were surveyed using short questionnaires to assess their opinions and practices in the care of IPAH patients. A Delphi forecasting approach was used to gain consensus among Canadian physicians on the most important clinical parameters to consider when assessing patients after the initiation of therapy.
Twenty-six of 37 Canadian PH experts who were invited to participate completed the study. All endorsed the use of combination therapy for IPAH patients despite the lack of universal provincial coverage for this approach. By consensus, WHO functional class, 6 min walk distance and hospitalization for right heart failure were the most important clinical parameters. The most highly rated physical examination parameters were jugular venous pressure, peripheral edema, the presence of ascites and body weight.
The overall approach to care of IPAH patients is similar across PH centres in Canada. A limited number of clinical and physical examination parameters were considered to be most important to reassess patients after therapy is initiated. These parameters, along with definition of threshold values, will facilitate the development of standard practice guidelines for IPAH patients in Canada.