Introduction: Pulmonary hypertension (PH) is a complex, life-threatening disease. Development of pulmonary hypertension centers is recommended by pulmonary hypertension best practice guidelines and patient organizations to successfully care for patients. However, very little is published on the characteristics of these centers or the daily management required to care for this complex patient population. Methods: This article, written by nurses who have extensive experience in managing patients with pulmonary hypertension, details the collaboration and workflows needed at a
Pulmonary arterial hypertension is a serious, life-altering condition. Patients who are diagnosed are often of child-bearing potential. Given the well-documented risks associated with this condition during pregnancy, as well as risks to the fetus from medications used to treat this disorder, patients should be strongly advised against pregnancy. Despite this, patients still become pregnant, leading to the question of whether care providers are counseling patients and their partners about the risks of pregnancy, methods of contraception, and issues of intimacy on a regular basis. We have conducted a survey of pulmonary hypertension specialist physicians and allied health care professionals on their practice patterns related to counseling on intimacy, contraception, and pregnancy prevention. Most respondents indicated they are counseling on these issues to varying degrees, but our survey pointed to several areas where improvements can be made. The most significant barrier to counseling for all respondents was lack of time. Survey respondents reported that a large percentage of the pregnancies seen in their practices were either intentional or due to contraceptive noncompliance. We review specific practical approaches to initiate reproductive health counseling as well as ways to integrate this important aspect of pulmonary arterial hypertension care into regular practice routines and documentation. Protocols regarding pregnancy avoidance and pulmonary arterial hypertension should be developed and become standard procedure.
SUMMARYAims: Tadalafil, a once-daily phosphodiesterase type 5 inhibitor (PDE-5I), offers clinicians an alternative to sildenafil, a 3-times-daily (t.i.d.) PDE-5I for treatment of pulmonary arterial hypertension (PAH). However, there are limited data describing the risks and benefits or recommended methodology of switching patients from sildenafil to tadalafil. Methods: Chart reviews were conducted on all World Health Organization group 1 patients on sildenafil for ≥3 months who transitioned to tadalafil with documented clinic visits and 6-min walk tests on both drugs. Most patients were transitioned by discontinuing sildenafil after the evening dose and initiating tadalafil 40 mg/day the next day. Data collected included demographics, PAH etiology, diagnostic hemodynamics, 6-min walk distance (6MWD), PDE-5I side effects, and concomitant medications. Data on B-type natriuretic peptide (BNP) levels were available for most patients also receiving endothelin receptor antagonists (ERAs). Results: Medical records from 98 patients were evaluated. Most patients (92%) were on sildenafil for >1 year, and 78% were receiving sildenafil 80-100 mg t.i.d. Ninetyseven percent of patients (95/98) were successfully transitioned and maintained on 40 mg/ day. With a mean duration on tadalafil therapy of 243 AE 127 days at the time of analysis, 6MWD was unchanged. Patient-reported adverse events included headache (4%) and heartburn (2%). There was minimal change in BNP levels in the subset of patients receiving an ERA concomitantly. Conclusions: Transition from sildenafil to tadalafil 40 mg/day appears feasible without clinical deterioration or intolerable side effects. This study provides guidance to physicians considering transition from sildenafil to tadalafil for selecting patients.
Performing longitudinal and consistent risk assessments for patients with pulmonary arterial hypertension (PAH) is important to help guide treatment decisions to achieve early on and maintain a low‐risk status and improve patient morbidity and mortality. Clinical gestalt or expert perception alone may over or underestimate a patient's risk status. Indeed, regular and continued use of validated risk assessment tools more accurately predict patients' survival. Effective PAH risk assessments are often underutilized even though many seasoned clinicians will attest to using these tools routinely. We present recommendations based on real‐world experience in varied clinical practice settings around the United States for overcoming barriers to facilitate regular, serial formal risk assessment. Expert advanced practice provider clinicians from mid to large‐size medical centers collaborated to formulate recommendations based on multiple discourses and discussions. Enlisting the help of support staff, such as medical assistants and nurses, to fill in available risk parameters in risk assessment tools can save time for providers and increase efficiency, as can technology‐based solutions such as integrating risk assessments into electronic medical records. Modified, abbreviated risk assessment tools can be applied to a patient's clinical scenario when all of a patient's data are not available to complete a more comprehensive assessment. Initial discussions regarding the overall meaning and prognostic importance of risk scores may assist patients to take on a more active role in terms of informed decision‐making regarding their care. A collaborative approach can help clinics establish consistent use of risk assessment.
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