Current organization of specialist pulmonary hypertension clinics: results of an international survey.

Optimal pulmonary hypertension (PH) management relies on a timely, accurate diagnosis and follow-up in specialized clinics by multidisciplinary teams that have clearly defined responsibilities and protocols. Internationally agreed criteria for expert center staff are lacking, particularly with respect to nurses, who often act as a central component of the team. This survey aimed to evaluate the current organization of PH clinics and the role of nurses. The survey (35 questions) was online February-December 2015 and was advertised at international PH nurse meetings and through international PH organizations to their corresponding clinics. In total, 126 healthcare professionals from 32 countries responded. According to respondents, 54% of clinics managed >200 patients, of whom 49% had a pulmonary arterial hypertension (PAH) diagnosis, on average. In terms of staff, 66% had a dedicated program administrator, 35% had one full-time nurse coordinator/practitioner/specialist, and 57% had a nurse attend outpatient clinic alongside a physician. Crucially, not all centers had a nurse in their team. The role of a nurse coordinator/practitioner/specialist varied with 51% taking patient histories/examinations and 66% managing outpatients. In 34% of clinics, nurses were involved in their own research. Protocols were available for PH therapies (81%), management of heart failure (37%) and pain (26%), and referring patients who did not have PAH/chronic thromboembolic PH back to their specialist (62%). Not all clinics are meeting all of the standards outlined in the latest guidelines with key areas of improvement being level of support from/for nurses, clear protocols, and referral pathways.

Volume Management in Pulmonary Arterial Hypertension Patients: An Expert Pulmonary Hypertension Clinician Perspective.

Fluid volume management in patients with pulmonary arterial hypertension (PAH) is essential in preventing right ventricular failure. Volume overload may be caused by disease progression, indiscretion of dietary sodium and fluid intake, or medication side effects, and is a frequent complication in patients with PAH. Healthcare professionals (HCPs) who care for patients with PAH have a key role in monitoring, preventing, and managing volume overload. Volume management techniques in patients with PAH include managing diuretic use and electrolyte imbalances, and monitoring fluid retention that can occur from the use of endothelin receptor antagonists or calcium channel blockers. Healthcare providers can create volume management protocols as well as patient educational materials. Patients should be educated to self-monitor their daily weights, incorporate dietary restrictions, and recognize symptoms associated with volume overload. Tools to help HCPs with volume management in patients with PAH are provided in this article.Funding Actelion Pharmaceuticals US, Inc.

Pulmonary Arterial Hypertension: A Focus on Infused Prostacyclins.

Pulmonary arterial hypertension (PAH) is characterized by vasoconstriction and cell proliferation in the pulmonary vasculature. Guideline-driven interventions with infused prostacyclin treatment are the mainstay for patients with advanced symptoms. Infused prostacyclin therapy is complex. It is critical to manage prostacyclin therapy with precision because boluses or interruptions can be fatal. Education of patients and inpatient staff nurses is necessary to prevent negative outcomes. Nurses are an essential part of the multidisciplinary team caring for patients with PAH. The diagnostic evaluation and treatment of PAH are reviewed here, and challenges associated with the care of patients on prostacyclin therapy are discussed.