Continuous Intravenous Epoprostenol for Pulmonary Hypertension Due to the Scleroderma Spectrum of Disease

Badesch, David B.; Tapson, Victor F.; McGoon, Michael D.; Brundage, Bruce H.; Rubin, Lewis J.; Wigley, Fredrick M.; Rich, Stuart; Barst, Robyn J.; Barrett, Pamela S.; Kral, Kenneth M.; Jöbsis, Maria M.; Loyd, James E.; Murali, Srinivas; Frost, Adaani E.; Girgis, Reda E.; Bourge, Robert C.; Ralph, David; Elliott, C. Gregory; Hill, Nicholas S.; Langleben, David; Schilz, Robert; McLaughlin, Vallerie V.; Robbins, Ivan M.; Groves, Bertron Μ.; Shapiro, Shelley; Medsger, Thomas A.; Gaine, Seán; Horn, Evelyn M.; Decker, James C.; Knobil, Katharine

doi:10.7326/0003-4819-132-6-200003210-00002

Cited by 968 publications

(563 citation statements)

References 58 publications

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“…The prevalence of resting PAH in CTD is ϳ2.3-10 cases per million (5), typically mostly SSc patients (5-50%) (6)(7)(8)(9)(10)(11)(12), patients with mixed CTD (21-29%) (13), and patients with systemic lupus erythematosus (5-43%) (14)(15)(16). Based on right heart catheterization, the prevalence of resting PAH in SSc is likely 7.9-20% (7,17).…”

Section: Methodsmentioning

confidence: 99%

Exercise‐induced pulmonary hypertension associated with systemic sclerosis: Four distinct entities

Saggar

Khanna

Fürst

et al. 2010

Arthritis & Rheumatism

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Objective. Exercise-induced pulmonary hypertension (PH) may represent an early but clinically relevant phase in the spectrum of pulmonary vascular disease. There are limited data on the prevalence of exerciseinduced PH determined by right heart catheterization in scleroderma spectrum disorders. We undertook this study to describe the hemodynamic response to exercise in a homogeneous population of patients with scleroderma spectrum disorders at risk of developing pulmonary vascular disease. Methods.Patients with normal resting hemodynamics underwent supine lower extremity exercise testing. A classification and regression tree (CART) analysis was used to assess combinations of variables collected during resting right heart catheterization that best predicted abnormal exercise physiology, applicable to each individual subject.Results. Fifty-seven patients who had normal resting hemodynamics underwent subsequent exercise right heart catheterization. Four distinct hemodynamic groups were identified during exercise: a normal group, an exercise-induced pulmonary venous hypertension (ePVH) group, an exercise out of proportion PH (eoPH) group, and an exercise-induced PH (ePH) group. The eoPH and ePVH groups had higher pulmonary capillary wedge pressure (PCWP) than the ePH group (P < 0.05). The normal and ePH groups had exercise PCWP <18 mm Hg, which was lower than that in the ePVH and eoPH groups (P < 0.05). During submaximal exercise, the transpulmonary gradient and pulmonary vascular resistance (PVR) were elevated in the ePH and eoPH groups as compared with the normal and ePVH groups (P < 0.05). CART analysis suggested that resting mean pulmonary artery pressure (mPAP) >14 mm Hg and PVR >160 dynes/seconds/cm ؊5 were associated with eoPH and ePH (positive predictive value 89% for mPAP 14-20 mm Hg and 100% for mPAP >20 mm Hg).Conclusion. We characterized the exercise hemodynamic response in at-risk patients with scleroderma spectrum disorders who did not have resting PH. Four distinct hemodynamic groups were identified during exercise. These groups may have potentially different prognoses and treatment options.

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Section: Methodsmentioning

confidence: 99%

Exercise‐induced pulmonary hypertension associated with systemic sclerosis: Four distinct entities

Saggar

Khanna

Fürst

et al. 2010

Arthritis & Rheumatism

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“…Intravenous treatment with PGI2, its analogues such as iloprost [31], and PGE1 [32] have been shown to be benefi cial; however, intravenous administration is a drawback. Oral PGs have been shown to be ineff ective in their current form [34].…”

Section: Drug Treatmentmentioning

confidence: 99%

ESVM guidelines – the diagnosis and management of Raynaud’s phenomenon

Belch

Carlizza

Carpentier

et al. 2017

Vasa

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Abstract. Regarding the clinical diagnosis of Raynaud’s phenomenon and its associated conditions, investigations and treatment are substantial, and yet no international consensus has been published regarding the medical management of patients presenting with this condition. Most knowledge on this topic derives from epidemiological surveys and observational studies; few randomized studies are available, almost all relating to drug treatment, and thus these guidelines were developed as an expert consensus document to aid in the diagnosis and management of Raynaud’s phenomenon. This consensus document starts with a clarification about the definition and terminology of Raynaud’s phenomenon and covers the differential and aetiological diagnoses as well as the symptomatic treatment.

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“…PGI 2 (epoprostenol) is efficacious in patients with PAH. Continuous IV infusion with epoprostenol results in improvement in the exercise capacity and cardiopulmonary hemodynamics, and benefits survival in patients with PAH secondary to scleroderma [25]. It is now considered to be a first-line therapy in patients with severe PAH.…”

Section: Prostacyclin Analoguesmentioning

confidence: 99%

Diagnosis and treatment of systemic and localized scleroderma

Khanna¹

2011

Expert Review of Dermatology

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Continuous Intravenous Epoprostenol for Pulmonary Hypertension Due to the Scleroderma Spectrum of Disease

Abstract: Continuous epoprostenol therapy improves exercise capacity and cardiopulmonary hemodynamics in patients with pulmonary hypertension due to the scleroderma spectrum of disease.

Cited by 968 publications

References 58 publications

Exercise‐induced pulmonary hypertension associated with systemic sclerosis: Four distinct entities

Exercise‐induced pulmonary hypertension associated with systemic sclerosis: Four distinct entities

ESVM guidelines – the diagnosis and management of Raynaud’s phenomenon

Diagnosis and treatment of systemic and localized scleroderma

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