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Pulmonary arterial hypertension (PAH) is a rare and debilitating disease characterized by abnormal proliferation and contraction of pulmonary vascular smooth muscle cells. The resulting increase in pressure and pulmonary vascular resistance results in progressive right heart failure, low cardiac output, and ultimately death if left untreated. PAH is defined by a persistent elevation in pulmonary artery pressure with normal left-sided pressures, differentiating it from left-sided heart disease. Symptoms progress from shortness of breath and decreasing exercise tolerance to right heart failure, with peripheral edema and marked functional limitation. Exercise-induced syncope, worsening symptoms at rest, and intractable right heart failure indicate critical disease. PAH may be idiopathic with no identifiable cause or associated with collagen vascular diseases, drugs, HIV, liver disease, and/or congenital heart disease. Familial or genetically mediated PAH accounts for a small percentage of cases. Advances in the understanding of pathobiological pathways that contribute to vascular proliferation and remodeling have resulted in new therapies that improve quality of life and survival. Emerging therapies focus on the nitric oxide, prostacyclin, and endothelin pathways. Nursing interventions are critical to ensure patients' success with these expensive and complex treatments and their optimal adjustment to living with PAH.

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.

This study contributes to the nurse/physician collaboration literature in that it was longitudinal, used a reliable and valid instrument, and surveyed nurses in medical/surgical units as well as the ICU. Some of the difficulties and benefits of research in today's practice setting are illustrated.

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.

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