Hemodynamic Effects of Epoprostenol in Patients With Systemic Sclerosis and Pulmonary Hypertension

Strange, Charlie; Bolster, Marcy B.; Mazur, Joe; Taylor, Marian H; Gossage, James R.; Silver, Richard M.

doi:10.1378/chest.118.4.1077

Cited by 46 publications

(22 citation statements)

References 33 publications

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“…Intravenously administered epoprostenol or iloprost may increase intrapulmonary shunt in ILD patients, thus aggravating hypoxaemia. Therefore, the use of inhaled iloprost or NO, or orally administered agents such as sildenafil or bosentan, may be considered in these acute and life-threatening situations, although these agents have not been approved for this clinical indication [129][130][131][132][133]. In addition, volume management, oxygen supplementation, and noninvasive ventilation are applied as needed.…”

Section: Treatment Of Ph In the Context Of Ildmentioning

confidence: 99%

Pulmonary hypertension in interstitial lung disease: Fig. 1—

2008

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In the lungs, parenchymal and vascular remodelling share pathomechanisms that may explain the relatively high prevalence (30-40%) of pulmonary hypertension (PH) in interstitial lung disease (ILD) patients. Notably, PH significantly contributes to exercise limitation and dismal prognosis of ILD patients. The absence of specific clinical symptoms commonly leads to delayed diagnosis. Besides clinical judgment and out-of-proportion reduction in diffusing capacity, severe hypoxaemia or exercise oxygen desaturation, echocardiography and biomarkers such as B-type natriuretic peptide (BNP) and N-terminal pro-hormone BNP are potentially helpful tools in identifying PH. However, right heart catheterisation is still necessary to confirm the diagnosis. Management of PH in ILD comprises treatment of the underlying disease process and long-term oxygen therapy. Affected patients should be listed for lung transplantation without delay, when appropriate. However, due to age and comorbidities only a minority of ILD patients will be eligible for lung transplantation. In the absence of satisfactory therapies for many ILDs, and considering the clinical burden of PH in affected patients, specific vasomodulatory therapies presently approved for PAH may be promising options for ILD patients. Consequently, there is an urgent need for adequately designed clinical trials to assess the effectiveness of specific PH therapy in the context of ILDs.

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Section: Treatment Of Ph In the Context Of Ildmentioning

confidence: 99%

Pulmonary hypertension in interstitial lung disease: Fig. 1—

2008

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“…However, treatment with vasoactive agents in patients with lung fibrosis carries the risk of worsening hypoxemia due to the inhibition of hypoxic vasoconstriction in low-ventilation/perfusion lung units [91,92,93]. …”

Section: Treatmentmentioning

confidence: 99%

Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis: A Review

2011

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Idiopathic pulmonary fibrosis (IPF) is a progressive diffuse parenchymal disease with a poor prognosis. Pulmonary hypertension (PH) often complicates the course of IPF and may even be found in patients with preserved lung function. Possible pathogenetic mechanisms of PH in IPF include vascular destruction, pulmonary hypoxic vasoconstriction and vascular remodeling due to overexpression of cytokines and growth factors. PH in IPF patients is associated with decreased exercise capacity and a worse prognosis. Due to its prognostic significance, it seems important to investigate for PH in these patients. As the symptoms of PH in IPF are nonspecific, the development of PH in a patient with known IPF can be easily overlooked. Noninvasive methods provide clues for the diagnosis, but their sensitivity is limited. Doppler echocardiography is a useful tool for the detection of PH which also provides additional information regarding associated cardiac abnormalities. However, right heart catheterization remains the gold standard diagnostic test. Therapeutic options for PH in IPF are limited. Long-term oxygen administration for the correction of hypoxemia should be recommended. The availability of new pharmacological agents in the treatment of PH has raised the possibility of therapy in patients with IPF and associated PH. Whether these PH-targeted therapies may be of benefit in this patient group, in terms of improving functional outcomes and survival, remains uncertain.

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“…Skleroderma spektrumu hastalarından PAH tanısı ile takip edilen hastalarda PVT pozitifliğinin hayli nadir görüldüğü bildirilmiştir (15). Ayrıca özellikle skleroderma spektrumu hastalarında sol ventrikül disfonksiyonu olan hastalarda iNO ile ciddi pulmoner ödem tablosunun gelişebileceği unutulmamalıdır (1,16). Diğer PH gruplarında PVT ile prognoz ve tedavinin planlanması ile ilgili kısıtlı ve çeliş-kili veriler bulunmakta, güncel kılavuzlar, önerilen gruplar dışın-daki PH gruplarında PVT testi ile tedavi planlamasını önerme-mektedir (1, 2).…”

Section: Pulmoner Vazoreaktivite Testiunclassified

Pulmonary vasoreactivity testing in diagnosis and prognosis of pulmonary hypertension

Mutlu¹,

Hünük²,

Kıvrak³

2010

Anadolu Kardiyol Derg

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ABS TRACTRight heart catheterization (RHC) is required for confirmation of the diagnosis of pulmonary hypertension (PH) and to asses the prognosis and etiology. Pulmonary vasoreactivity testing (PVT) is one of the most crucial parts of RHC especially in the suitable patient subsets of pulmonary arterial hypertension (PAH) defined by the most recent guidelines. PVT has substantial importance in defining the most appropriate treatment modality. The aim of this review is to provide a comprehensive review of the literature about PVT and the agents used in PVT, to emphasize the importance of this procedure when done with correct indications with a correct technique and to contribute in the accurate management of PH patients.(Ana do lu Kar di yol Derg 2010; 10: Suppl 1; 43-9)

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Hemodynamic Effects of Epoprostenol in Patients With Systemic Sclerosis and Pulmonary Hypertension

Cited by 46 publications

References 33 publications

Pulmonary hypertension in interstitial lung disease: Fig. 1—

Pulmonary hypertension in interstitial lung disease: Fig. 1—

Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis: A Review

Pulmonary vasoreactivity testing in diagnosis and prognosis of pulmonary hypertension

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