Lisa Parfitt

Bosentan in pulmonary hypertension associated with fibrotic idiopathic interstitial pneumonia.

RATIONALE Pulmonary hypertension (PH) associated with fibrotic idiopathic interstitial pneumonia (IIP; idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia) confers important additional morbidity and mortality. OBJECTIVES To evaluate the safety and clinical efficacy of the dual endothelin-1 receptor antagonist bosentan in this patient group. METHODS In a randomized, double-blind, placebo-controlled study, 60 patients with fibrotic IIP and right heart catheter confirmed PH were randomized 2:1 to bosentan (n = 40) or placebo (n = 20). The primary study endpoint was a fall from baseline pulmonary vascular resistance index (PVRi) of 20% or more over 16 weeks. MEASUREMENTS AND MAIN RESULTS Sixty patients (42 men; mean age, 66.6 ± 9.2 yr), with a mean pulmonary artery pressure of 36.0 (± 8.9) mm Hg, PVRi 13.0 (± 6.7) Wood Units/m(2) and reduced cardiac index of 2.21 (± 0.5) L/min/m(2) were recruited to the study. Accounting for deaths and withdrawals, paired right heart catheter data were available for analysis in 39 patients (bosentan = 25, placebo = 14). No difference in the primary outcome was detected, with seven (28.0%) patients receiving bosentan, and four (28.6%) receiving placebo achieving a reduction in PVRi of greater than or equal to 20% (P = 0.97) at 16 weeks. There was no change in functional capacity or symptoms between the two groups at 16 weeks, nor any difference in rates of serious adverse events or deaths (three deaths in each group). CONCLUSIONS This study shows no difference in invasive pulmonary hemodynamics, functional capacity, or symptoms between the bosentan and placebo groups over 16 weeks. Our data do not support the use of the dual endothelin-1 receptor antagonist, bosentan, in patients with PH and fibrotic IIP. Clinical trial registered with www.clinicaltrials.gov (NCT 00637065).

Disease targeting therapies in patients with Eisenmenger syndrome: response to treatment and long-term efficiency.

OBJECTIVES To examine long-term efficacy of disease targeting therapies (DTT) in patients with Eisenmenger syndrome. METHODS All adult patients with Eisenmenger syndrome treated with DTT at our institution were included. Functional class (FC), oxygen saturation and 6-minute walk test distance (6 MWTd) were analysed retrospectively. RESULTS Between 2002 and 2010, 79 Eisenmenger patients (21 males, 16 with Down syndrome) aged 34 ± 10 years (range 17-68 years) were included. Median follow-up was 3.3 years (range 0.2 to 8.9 years). 6 MWTd increased early after initiation of DTT, with a plateau after approximately 3 years and no obvious trend towards a deterioration on average during longer-term follow-up. Two patients died during follow-up and escalation of treatment was required in 18 patients after a median period of 2.5 years. Escalation of therapy was also associated with an increase in 6 MWTd. In addition, FC improved on DTT and oxygen saturation, increased, both at rest and peak exercise. This effect was more pronounced in the patients with the lowest baseline oxygen saturation at rest. CONCLUSIONS Long-term DTT is safe and improves objective exercise capacity and subjective symptoms. Response to DTT was typically observed early after initiation of DTT and was, on average, maintained longer-term. However, 1 in 5 patients required escalation of DTT, with time, due to symptomatic deterioration and this was associated with an afresh improvement in 6 MWTd.

The use of sildenafil to treat pulmonary hypertension associated with interstitial lung disease.

Background and objective:  Limited data suggest a benefit following sildenafil treatment in patients with pulmonary hypertension (PH) and interstitial lung disease (ILD). The role of sildenafil in the management of PH in ILD is not clear. We report our experience of ILD patients with PH after 6‐month sildenafil therapy.

The spectrum of pulmonary arterial hypertension in adults with congenital heart disease: management from a physician and nurse specialist perspective

Congenital heart disease (CHD) is one of the most common causes of pulmonary arterial hypertension (PAH), with a prevalence nearing that of idiopathic PAH in registries. Despite similarities in pulmonary vascular pathology, PAH-CHD differs from idiopathic PAH and other types of PAH. In fact, PAH-CHD is a term that includes a wide spectrum of conditions and pathophysiologies, ranging from Eisenmenger syndrome to milder forms of PAH with systemic-to-pulmonary shunts, to those with previously repaired defects. Significant expertise in both CHD and PAH is required to manage these patients. We provide an overview of the modern management of the wide spectrum of PAH-CHD in adults, both from a physician and clinical nurse specialist perspective.

Intravenous prostacyclin for pulmonary hypertension: Patient's perspective on complications

Introduction: Intravenous (IV) prostacyclin therapy in patients with severe pulmonary arterial hypertension can be associated with life-threatening complications. Patient education and confidence in local healthcare services is critical to ensuring prompt treatment of such complications. We evaluated patient9s understanding of potential complications and their attitudes towards local healthcare services. Methods: Patients established on IV prostacyclin therapy were identified. Patients were counselled prior to receiving IV prostacyclin and subsequently completed a questionnaire assessing their level of understanding. Results: Ten patients were identified and agreed to participate; median distance from home to their specialist centre was 35 miles. All patients agreed or strongly agreed that they were confident in managing their pump. 8 patients reported not being aware of how long it was safe to keep their pump disconnected. 3 patients were not aware of the correct course of action to take in the event of pump failure and 3 patients did not know their correct dose. Only 3 patients felt confident in their local emergency department being able to manage any complications. Conclusions: Patients seem to require additional education. Strategies to ensure prompt recognition of complications and enhance patient confidence with local healthcare is necessary. We present a patient information card, which may assist achieving these goals. .