Abstract
Editor:
Clinical practice guidelines represent important efforts to offer practicing healthcare professionals guidance in the management of various clinical conditions, such as pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). While they summarize “the science” into an understandable, albeit comprehensive format, it is obvious that adherence to guidelines has encountered impediments in both PAH and CTEPH. It is therefore not surprising that our findings described in the international survey of PAH management 1 parallel those reported earlier by Ryan et al. 2 and that our survey on CTEPH showed similar gaps in adherence to clinical practice guidelines. 3
Our study's unique characteristic is that it integrates in a more detailed fashion and at a large scale what previous regional or national studies reported on the diagnosis and management of PAH. For example, patients still tend to have significant limitations at the time of diagnosis, with a large proportion of patients being New York Heart Association functional class 3 or 4. These findings are coupled with a significant proportion of patients receiving a diagnosis from physicians not affiliated with a pulmonary hypertension center (33% and 71% in the United States and Argentina, respectively). Furthermore, there is still an underutilization of right heart catheterization for the definitive diagnosis (with over 50% of patients with PAH in Argentina not undergoing the test, one of the reasons being lack of proper facilities, equipment, or training). Nevertheless, the main reason listed for not performing this pivotal diagnostic test was not lack of infrastructure or capabilities but that “other tests indicated that the patient had PAH.” This, I should say, is a shockingly worrisome statement, because all guidelines strongly emphasize that the right heart catheterization is crucial for making an accurate diagnosis. The guidelines also took into account the fact that all clinical trials that proved the efficacy of available therapies for PAH selected those patients with PAH with a confirmatory right heart catheterization. This implies that available therapies are not tested for patients with suspected PAH, which is all that other tests can affirm.
We need to acknowledge the lack of proof that adherence to our guidelines improves patient care and outcomes, unlike the experience with common cardiovascular diseases. Nevertheless, in the era of evidence-based medicine, guidelines are based on the most current scientific evidence and support strategies that are shown to be associated with better outcomes and deserve consideration.
We therefore commend Ryan et al. 2 for their initial report, and we hope that ours, by complementing and expanding on their findings, will keep the interest and discussion going and stimulate clinical researchers to find solutions to a devastating disease.