Changes in right heart haemodynamics and echocardiographic function in an advanced phenotype of pulmonary hypertension and right heart dysfunction associated with pulmonary fibrosis

Saggar, Rajeev; Khanna, Dinesh; Vaidya, Anjali; Derhovanessian, Ariss; Maranian, Paul; Duffy, Erin; Belperio, John A.; Weigt, S. Samuel; Dua, Shiv; Shapiro, Shelley; Goldin, Jonathan G.; Abtin, Fereidoun; Lynch, Joseph P.; Ross, David J.; Forfia, Paul R.; Saggar, Rajan

doi:10.1136/thoraxjnl-2013-204150

Cited by 63 publications

(68 citation statements)

References 30 publications

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“…For categorical outcomes, an estimate for the absolute effect size was additionally obtained by multiplying the RR for each agent with the median placebo response rate for that outcome. 25 Differences between direct and indirect evidence were assessed using tests of model consistency by including trial design as an additional covariate in the model. 26 Third, we ranked drugs in order of their efficacy and tolerability using the surface under the cumulative ranking (SUCRA).…”

Section: Resultsmentioning

confidence: 99%

Comparative Effectiveness of Pharmacologic Interventions for Pulmonary Arterial Hypertension

Jain

Khera

Girotra

et al. 2017

Chest

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BACKGROUND:We conducted a systematic review and network meta-analysis to examine comparative efficacy and tolerability of pharmacologic interventions for pulmonary arterial hypertension (PAH).METHODS: MEDLINE, the Cochrane Register, EMBASE, CINAHL, and clinicaltrials.gov were searched (January 1, 1990 to March 3, 2016. Randomized controlled trials (RCTs) studying the approved pharmacologic agents endothelin receptor antagonists (ERA), phosphodiesterase inhibitors (PDE5i), the oral/inhaled (PO/INH) and IV/subcutaneous (SC) prostanoids, and riociguat and selexipag, alone or in combination, for pulmonary arterial hypertension (PAH) and reporting at least one efficacy outcome were selected.RESULTS: Thirty-one RCTs with 6,565 patients were selected. In network meta-analysis, when compared with a median placebo rate of 14.5%, clinical worsening was estimated at 2.8% with riociguat (risk ratio [RR], 0.19; 95% CI, 0.05-0.76); at 3.9% with ERA þ PDE5i (RR, 0.27; 95% CI, 0.14-0.52), and at 5.7% with PDE5i (RR, 0.39; 95% CI, 0.24-0.62). For improvement in functional status, when compared with 16.2% in the placebo group, improvement in at least one New York Heart Association/World Health Organization (NYHA/WHO) functional class was estimated at 81.8% with IV/SC prostanoids (RR, 5.06; 95% CI, 2.3211.04), at 28.3% with ERA þ PDE5i (RR, 1.75; 95% CI, 1.05-2.92), and at 25.2% with ERA (RR, 1.56; 95% CI, 1.22-2.00). Differences in mortality were not significant. Adverse events leading to discontinuation of therapy were highest with the PO/INH prostanoids (RR, 2.92; 95% CI, 1.68-5.06) and selexipag (RR, 2.06; 95% CI, 1.04-3.88) compared with placebo.CONCLUSIONS: Currently approved pharmacologic agents have varying effects on morbidity and functional status in patients with PAH. Future comparative effectiveness trials are warranted with a focus on a patient-centered approach to therapy. REGISTRATION: PROSPERO CRD42016036803CHEST 2017; 151(1):90-105 KEY WORDS: comparative efficacy; network meta-analysis; pulmonary arterial hypertension ABBREVIATIONS: 6MWD = 6-min walk distance; ERA = endothelin receptor antagonist; FDA = Food and Drug Administration; GRADE = Grading of Recommendations Assessment, Development, and Education; NYHA = New York Heart Association; PAH = pulmonary arterial hypertension; PDE5i = phosphodiesterase-5 inhibitor; RCT = randomized controlled trial; RR = risk ratio; SUCRA = surface under the cumulative ranking area; WHO = World Health Organization; WMD = weighted mean difference Pulmonary arterial hypertension (PAH) or World Health Organization (WHO) group 1 pulmonary hypertension is a progressive disease associated with significant morbidity and a 5% to 15% annual mortality rate. [1][2][3] In recent years, a number of drug classes to treat PAH have been approved for clinical use. These include endothelin receptor antagonists (ERA), phosphodiesterase-5 inhibitors (PDE5i), parenteral and nonparenteral prostacyclins, a soluble guanylate cyclase stimulator, and a prostacyclin-receptor agonist. Although randomized ...

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Section: Resultsmentioning

confidence: 99%

Comparative Effectiveness of Pharmacologic Interventions for Pulmonary Arterial Hypertension

Jain

Khera

Girotra

et al. 2017

Chest

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“…Retrospective, small case series of patients with sarcoidosis-associated pulmonary hypertension also reported an acute reduction in PVR and sustainable clinical improvement to long-term intravenous epoprostenol (mean follow-up of 29 months) [73], and improvement in PVR and cardiac output/index with parenteral prostacyclin as monotherapy or in combination with oral vasodilators (mean follow-up of 12.7 months) [74]. Finally, 12-week treatment with parenteral treprostinil improved right heart haemodynamics and echocardiographic parameters in a small group of patients with pulmonary fibrosis with severe pulmonary hypertension (mPAP ⩾35 mmHg) [75].…”

Section: Therapymentioning

confidence: 99%

Pulmonary vascular and cardiac impairment in interstitial lung disease

et al. 2017

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@ERSpublications Development of pulmonary hypertension is a major adverse factor in the natural history of interstitial lung disease http://ow.ly/nJB0302XAmD ABSTRACT Pulmonary vascular and cardiac impairment is increasingly appreciated as a major adverse factor in the natural history of interstitial lung disease. This clinically orientated review focuses on the current concepts in the pathogenesis, pathophysiology and implications of the detrimental sequence of increased pulmonary vascular resistance, pre-capillary pulmonary hypertension and right heart failure in interstitial lung disease, and provides guidance on its management.

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“…5 The study by Saggar et al builds on this concept further by studying pulmonary fibrosis patients with advanced PH, defined by a mean pulmonary artery pressure (mPAP) ≥35 mm Hg. 6 Patients in this study also had evidence of significant right-sided cardiac dysfunction. Although the number of patients studied was relatively small, it is encouraging that a salutary response to PH therapy across multiple domains was demonstrated.…”

Section: Steven D Nathanmentioning

confidence: 85%

Pulmonary hypertension complicating pulmonary fibrosis: bad and ugly, but good to treat?

Nathan¹

2014

Thorax

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Changes in right heart haemodynamics and echocardiographic function in an advanced phenotype of pulmonary hypertension and right heart dysfunction associated with pulmonary fibrosis

Cited by 63 publications

References 30 publications

Comparative Effectiveness of Pharmacologic Interventions for Pulmonary Arterial Hypertension

Comparative Effectiveness of Pharmacologic Interventions for Pulmonary Arterial Hypertension

Pulmonary vascular and cardiac impairment in interstitial lung disease

Pulmonary hypertension complicating pulmonary fibrosis: bad and ugly, but good to treat?

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