167 research outputs found
Right Heart Pulmonary Circulation Unit Involvement in Left-Sided Heart Failure: Diagnostic, Prognostic, and Therapeutic Implications
No full text: Although long neglected, the right heart (RH) is now widely accepted as a pivotal player in heart failure (HF) either with reduced or preserved ejection fraction. The chronic overload of the pulmonary microcirculation results in an initial phase characterized by right ventricular (RV) hypertrophy, right atrial dilation, and diastolic dysfunction. This progresses to overt RH failure when RV dilation and systolic dysfunction lead to RV-pulmonary arterial (RV-PA) uncoupling with low RV output. In the context of its established relevance to progression of HF, clinicians should consider assessment of the RH with information from clinical assessment, biomarkers, and imaging. Notably, no single parameter can predict prognosis alone in HF. Assessments simultaneously should encompass RV systolic function, pulmonary pressures, an estimation of RV-PA coupling, and RH morphologic features. Despite a large volume of evidence indicating the relevance of RH function to the clinical syndrome of HF, evidence-based management strategies are lacking. Targeting RH dysfunction in HF should be an objective of future investigations, being an unmet need in the current management of HF
Supplemental Material, Supp_Material - Diagnosis, Treatment and Follow Up of Acute Pulmonary Embolism: Consensus Practice from the PERT Consortium
No full textSupplemental Material, Supp_Material for Diagnosis, Treatment and Follow Up of Acute Pulmonary Embolism: Consensus Practice from the PERT Consortium by Belinda Rivera-Lebron, Michael McDaniel, Kamran Ahrar, Abdulah Alrifai, David M. Dudzinski, Christina Fanola, Danielle Blais, David Janicke, Roman Melamed, Kerry Mohrien, Elizabeth Rozycki, Charles B. Ross, Andrew J. Klein, Parth Rali, Nicholas R. Teman, Leoara Yarboro, Eugene Ichinose, Aditya M. Sharma, Jason A. Bartos, Mahir Elder, Brent Keeling, Harold Palevsky, Soophia Naydenov, Parijat Sen, Nancy Amoroso, Josanna M. Rodriguez-Lopez, George A. Davis, Rachel Rosovsky, Kenneth Rosenfield, Christopher Kabrhel, James Horowitz, Jay S. Giri, Victor Tapson, Richard Channick and the PERT Consortium in Clinical and Applied Thrombosis/Hemostasis</p
Chronic Thromboembolic Pulmonary Hypertension
No full textSince the last World Symposium on Pulmonary Hypertension in 2008, we have witnessed numerous and exciting developments in chronic thromboembolic pulmonary hypertension (CTEPH). Emerging clinical data and advances in technology have led to reinforcing and updated guidance on diagnostic approaches to pulmonary hypertension, guidelines that we hope will lead to better recognition and more timely diagnosis of CTEPH. We have new data on treatment practices across international boundaries as well as long-term outcomes for CTEPH patients treated with or without pulmonary endarterectomy. Furthermore, we have expanded data on alternative treatment options for select CTEPH patients, including data from multiple clinical trials of medical therapy, including 1 recent pivotal trial, and compelling case series of percutaneous pulmonary angioplasty. Lastly, we have garnered more experience, and on a larger international scale, with pulmonary endarterectomy, which is the treatment of choice for operable CTEPH. This report overviews and highlights these important interval developments as deliberated among our task force of CTEPH experts and presented at the 2013 World Symposium on Pulmonary Hypertension in Nice, France
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The Liver-Lung Connection in Portopulmonary Hypertension
No full textPortopulmonary hypertension, a subtype of World Health Organization Group 1 pulmonary arterial hypertension (PAH) that develops in the setting of portal hypertension, is the third most common cause of associated PAH and affects approximately 5-6% of patients evaluated for liver transplant. POPH is pathologically indistinct from idiopathic PAH(1), but is associated with a significantly lower 5-year survival of 40%(2).
POPH can complicate or preclude liver transplantation due to an elevated risk of perioperative death(8). Because of this increased risk, patients on the liver transplant list undergo yearly echocardiograms to screen for POPH, and patients with a known diagnosis of POPH undergo repeated right heart catheterizations every 3 months to ensure hemodynamics are acceptable for transplant(9, 10). Since 2006, patients with treated POPH who meet certain hemodynamic criteria have been eligible to receive MELD exception points, or waitlist priority upgrades, in order to expedite liver transplant(10, 11), but potentially important factors, such as severity of liver disease and POPH, are not included in the score and may affect survival.
The pathogenesis of POPH, characterized by pulmonary vasoconstriction and vascular remodeling, is poorly understood. There is no association between the presence of POPH and severity of liver disease or portal hypertension(3-5), but an increased prevalence of spontaneous portosystemic shunts in patients with POPH(6) as well as the development of POPH in patients with congenital portosystemic shunts with normal liver function(7) suggests that vasoactive factors from the splanchnic circulation contribute to disease pathogenesis. Identification of these factors and improved understanding of the role of the liver in the pathogenesis of POPH could lead to novel targeted therapies for this disease.
The overall goal of my research is to define the liver-lung connection in POPH and to determine the role of the portal circulation in disease pathogenesis and the role of liver transplant in the management of POPH. To address these goals, we have performed two studies. First, we performed a prospective multicenter case-control study in patients with liver disease to identify novel biomarkers of POPH and to compare biomarker gradients across the systemic and pulmonary circulation. We identified higher levels of macrophage migration inhibitory factor (MIF), a pleiotropic inflammatory cytokine, in both the systemic and pulmonary circulation of patients with POPH and found that MIF was correlated with POPH disease severity. Additionally, MIF in conjunction with echocardiogram could improve the screening of patients with liver disease for POPH. In the second study, we performed a retrospective analysis of the United Network for Organ Sharing (UNOS) national database to identify significant predictors of waitlist mortality in patients with POPH. We characterized waitlist and post-transplant outcomes in patients with POPH and identified both initial MELD score and pulmonary vascular resistance as significant predictors of waitlist mortality. Future directions will include prospective study to identify the short-term and long-term effects of liver transplant on pulmonary hemodynamics in patients with POPH.Portopulmonary hypertension, biomarker, macrophage migration inhibitory factor, waitlist mortalit
Pulmonary hypertension associated with lung diseases
No full textPulmonary hypertension (PH) associated with chronic lung disease (CLD) is both common and underrecognised. The presence of PH in the setting of lung disease has been consistently shown to be associated with worse outcomes. Recent epidemiological studies have advanced understanding of the heterogeneity of this patient population and shown that defining both the specific type of CLD as well as the severity of PH (i.e. deeper phenotyping) is necessary to inform natural history and prognosis. A systematic diagnostic approach to screening and confirmation of suspected PH in CLD is recommended. Numerous uncontrolled studies and one phase 3 randomised, controlled trial have suggested a benefit in treating PH in some patients with CLD, specifically those with fibrotic interstitial lung disease (ILD). However, other studies in diseases such as COPD-PH showed adverse outcomes with some therapies. Given the expanding list of approved pharmacological treatments for pulmonary arterial hypertension, developing a treatment algorithm for specific phenotypes of PH-CLD is required. This article will summarise existing data in COPD, ILD and other chronic lung diseases, and provide recommendations for classification of PH-CLD and approach to the diagnosis and management of these challenging patients
Effect of macitentan on hospitalizations: Results from the SERAPHIN trial
No full textOBJECTIVES:
This study sought to evaluate the effect of macitentan on hospitalization of patients with symptomatic pulmonary arterial hypertension (PAH).
BACKGROUND:
PAH is a progressive, life-threatening disease often requiring hospitalization.
METHODS:
In the multicenter, double-blind, randomized, event-driven, phase III SERAPHIN (Study with an Endothelin Receptor Antagonist in Pulmonary arterial Hypertension to Improve cliNical outcome) trial, patients with symptomatic PAH were randomized (1:1:1) to receive placebo or 3 mg or 10 mg of macitentan. Effects of macitentan on the risk, rate, and number of hospital days for all-cause and PAH-related hospitalizations were compared with those for placebo. Risk and causes of hospitalizations unrelated to PAH were investigated.
RESULTS:
Of 742 randomized patients, 250 received placebo, 250 received 3 mg of macitentan, and 242 received 10 mg of macitentan; the overall median duration of treatment was 115 weeks. Risk of all-cause hospitalization was reduced by 18.9% (p = 0.1208) and 32.3% (p = 0.0051) in the macitentan 3-mg and 10-mg arm, respectively. Rates of all-cause hospitalizations and numbers of hospital days were reduced by 20.5% (p = 0.0378) and 30.6% (p = 0.0278), respectively, with 3 mg of macitentan and by 33.1% (p = 0.0005) and 31.0% (p = 0.0336), respectively, with 10 mg of macitentan. Risk of PAH-related hospitalizations were reduced by 42.7% (p = 0.0015) and 51.6% (p < 0.0001) in the macitentan 3-mg and 10-mg arms, respectively. Rate of PAH-related hospitalizations and numbers of hospital days were reduced by 44.5% (p = 0.0004) and 53.3% (p = 0.0001), respectively, with 3 mg of macitentan, and reduced by 49.8% (p < 0.0001) and 52.3% (p = 0.0003), respectively, with 10 mg of macitentan. Risk of non-PAH-related hospitalization was similar between treatment arms.
CONCLUSIONS:
Macitentan 10 mg significantly reduced the risk and rate of all-cause hospitalization, which was driven by reductions in the risk and rate of PAH-related hospitalization. (Study of Macitentan [ACT-064992] on Morbidity and Mortality in Patients With Symptomatic Pulmonary Arterial Hypertensio
A Right Heart Catheterization for the Diagnosis of Pulmonary Hypertension: Yes, But How?
No full textSCOPUS: ed.jDecretOANoAutActifinfo:eu-repo/semantics/publishe
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