https://caltcm.memberclicks.net/assets/documents/acc_aha_chf_2013_guidelines.pdf

2013 ACCF/AHA Guideline for the Management of Heart Failure: A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines

This document is an international evidence-based guideline on the diagnosis and management of idiopathic pulmonary fibrosis, and is a collaborative effort of the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Association. It represents the current state of knowledge regarding idiopathic pulmonary fibrosis (IPF), and contains sections on definition and epidemiology, risk factors, diagnosis, natural history, staging and prognosis, treatment, and monitoring disease course. For the diagnosis and treatment sections, pragmatic GRADE evidence-based methodology was applied in a question-based format. For each diagnosis and treatment question, the committee graded the quality of the evidence available (high, moderate, low, or very low), and made a recommendation (yes or no, strong or weak). Recommendations were based on majority vote. It is emphasized that clinicians must spend adequate time with patients to discuss patients' values and preferences and decide on the appropriate course of action.

An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-Based Guidelines for Diagnosis and Management

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Modern Applied Statistics With S

ALAT
: alanine aminotransferase
ASAT
: aspartate aminotransferase
APAH
: associated pulmonary arterial hypertension
BAS
: balloon atrial septostomy
BMPR2
: bone morphogenetic protein receptor 2
BNP
: brain natriuretic peptide
BPA
: balloon pulmonary angioplasty
BREATHE
: Bosentan

/pdf/2015-esc-ers-guidelines-for-the-diagnosis-and-treatment-of-y26yabnnom.pdf

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT).

on Statement: Society of Echocardiography is accreditedby theAccreditationCouncil for edical Education to provide continuingmedical education for physicians. n Society of Echocardiography designates this educational activity for of 1.0 AMA PRA Category 1 Credits . Physicians should only claim credit te with the extent of their participation in the activity. CCI recognize ASE’s certificates and have agreed to honor the credit hours registry requirements for sonographers. Society of Echocardiography is committed to ensuring that its educational ll sponsored educational programs are not influencedby the special interests ation or individual, and itsmandate is to retain only those authors whose fists canbeeffectively resolved tomaintain thegoals andeducational integrity y. While a monetary or professional affiliation with a corporation does not fluence an author’s presentation, the Essential Areas and policies of the ire that any relationships that could possibly conflict with the educational activity be resolved prior to publication and disclosed to the audience. f faculty and commercial support relationships, if any, have been indicated. ience: is designed for all cardiovascular physicians and cardiac sonographers with erest and knowledge base in the field of echocardiography; in addition, reschers, clinicians, intensivists, and other medical professionals with a spein cardiac ultrasound will find this activity beneficial.

https://www.nationaljewish.org/getattachment/programs/directory/cardiology/pulmonary-hypertension-center/Echo-Guidelines-for-RV-JASE-2010.pdf.aspx

Guidelines for the Echocardiographic Assessment of the Right Heart in Adults: A Report from the American Society of Echocardiography Endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography

Background:

Patients with Systemic Sclerosis-associated pulmonary hypertension (SScPAH) have worse survival than patients with idiopathic PAH (IPAH). We hypothesized that the RV adapts differently in SScPAH vs IPAH.

Methods:

We examined 58 prospectively obtained cardiac MRIs (cMRI) in 34 SScPAH and 24 IPAH patients all diagnosed by right heart catheterization (RHC). cMRI and RHC measurements were recorded for all patients. Regression analysis was used to assess the association between diagnosis and RV measurements after adjusting for age and RV load.

Results:

Clinical and demographic variables are shown in Table 1. Univariate regression showed RV mass index (RVMi) varied linearly with measures of RV load. ANCOVA analysis suggested an interaction between diagnosis and RVMi adjusting for PVR and age (p=0.09). A spline model showed a significantly lower rate of increase in RVMi (0.51vs2.03g/m2*WU) in SScPAH vs IPAH at PVR>7 WU.

Conclusion:

RVMi varies linearly with measures of RV load. After adjusting for differences in RV load and age, SScPAH patients had a significantly decreased rate of hypertrophy with increasing PVR that was most prominent at PVR>7WU. This difference in adaptive hypertrophy may, in part, explain poorer survival in SScPAH despite lower measures of load.

View this table:

Table 1: Demographic and Catheterization Data





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https://erj.ersjournals.com/content/erj/42/Suppl_57/4835.full.pdf

Response to increased load in systemic sclerosis-associated PAH and idiopathic PAH

The addition of sildenafil to bosentan therapy in the treatment of pulmonary arterial hypertension

Background: Right ventricular (RV) contractility has been shown to be significantly worse in systemic sclerosis-associated pulmonary arterial hypertension (SSPAH) versus idiopathic PAH (IPAH), despite similar afterloads. Speckle-based strain may be a useful tool in conjunction with conventional 2D echocardiography (2DE) for detection of regional and global RV dysfunction between groups. Aim: To determine differences in conventional and novel measures of RV function between IPAH and SSPAH. Methods: 55 well-characterized PAH patients (23 IPAH, 32 SSPAH) underwent right heart catheterization (RHC) within 5 hours of 2DE. 2DE analysis included tricuspid annular plane systolic excursion (TAPSE) and fractional area change (FAC), as well as novel basal, mid, apical and global RV longitudinal systolic strain (RVLSS). Results: SSPAH patients were more commonly female and older. There were no significant differences in hemodynamic parameters by RHC, or 2D based TAPSE or FAC between SSPAH and IPAH. In contrast, RVLSS was significantly diminished in SSPAH both globally (-12.6 ± 5.1 vs -15.6 ± 4.3%, p=0.03), and regionally, mostly due to a decreased basal RVLSS in SSPAH (-15.5 ± 7.9 vs -24.8 ± 8%, p=0.02). There was no correlation between abnormalities in RVLSS and invasive measures of mean pulmonary arterial pressure, and pulmonary vascular resistance. Conclusion: Under similar afterload, conventional 2DE measures of RV function were not different between SSPAH and IPAH, while speckle-based strain revealed differences in global RV contractility mostly due to decrease in basal RVLSS in SSPAH. These findings are suggestive of unique pattern of RV contractility in SSPAH not detected by 2DE alone.

Right ventricular strain differences between idiopathic and systemic sclerosis Pulmonary Hypertension

We thank Ms. Deborah McCollister for her feedback on our recent article titled “Updating clinical endpoint definitions.” McCollister judiciously points out some of the challenges raised with the re...

/pdf/updating-clinical-endpoints-in-pulmonary-arterial-1zp1sxtfg8.pdf

Updating clinical endpoints in pulmonary arterial hypertension: when challenges are welcome

1. Lederer DJ, Martinez FJ. Idiopathic pulmonary fibrosis. N Engl J Med 2018;379:797–798. 2. Pardo A, Selman M. The interplay of the genetic architecture, aging, and environmental factors in the pathogenesis of idiopathic pulmonary fibrosis. Am J Respir Cell Mol Biol 2021;64:163–172. 3. Ley B, Collard HR, King TE Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011;183: 431–440. 4. Johannson KA, Ley B, Collard HR. Models of disease behavior in idiopathic pulmonary fibrosis. BMC Med 2015;13:165. 5. King TE Jr, Tooze JA, Schwarz MI, Brown KR, Cherniack RM. Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. Am J Respir Crit Care Med 2001;164:1171–1181. 6. Ley B, Ryerson CJ, Vittinghoff E, Ryu JH, Tomassetti S, Lee JS, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med 2012;156:684–691. 7. Wells AU, Desai SR, Rubens MB, Goh NS, Cramer D, Nicholson AG, et al. Idiopathic pulmonary fibrosis: a composite physiologic index derived from disease extent observed by computed tomography. Am J Respir Crit Care Med 2003;167:962–969. 8. du Bois RM, Weycker D, Albera C, Bradford WZ, Costabel U, Kartashov A, et al. Ascertainment of individual risk of mortality for patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011;184: 459–466. 9. Collard HR, Brown KK, Martinez FJ, Raghu G, Roberts RS, Anstrom KJ. Study design implications of death and hospitalization as end points in idiopathic pulmonary fibrosis. Chest 2014;146:1256–1262. 10. Bowman WS, Echt GA, Oldham JM. Biomarkers in progressive fibrosing interstitial lung disease: optimizing diagnosis, prognosis, and treatment response. Front Med (Lausanne) 2021;8:680997. 11. Molyneaux PL, Fahy WA, Byrne AJ, Braybrooke R, Saunders P, Toshner R, et al. CYFRA 21-1 predicts progression in idiopathic pulmonary fibrosis: a prospective longitudinal analysis of the PROFILE cohort. Am J Respir Crit Care Med 2022;205:1440–1448. 12. Vercauteren IM, Verleden SE, McDonough JE, Vandermeulen E, Ruttens D, Lammertyn EJ, et al. CYFRA 21-1 in bronchoalveolar lavage of idiopathic pulmonary fibrosis patients. Exp Lung Res 2015;41:459–465. 13. Stock CJW, Hoyles RK, Daccord C, Kokosi M, Visca D, De Lauretis A, et al. Serum markers of pulmonary epithelial damage in systemic sclerosis-associated interstitial lung disease and disease progression. Respirology 2021;26:461–468. 14. Bowman WS, Newton CA, Linderholm AL, Neely ML, Pugashetti JV, Kaul B, et al. Proteomic biomarkers of progressive fibrosing interstitial lung disease: a multicentre cohort analysis. Lancet Respir Med [online ahead of print] 18 Jan 2022; DOI: 10.1016/ S2213-2600(21)00503-8. 15. Maher TM, Oballa E, Simpson JK, Porte J, Habgood A, Fahy WA, et al. An epithelial biomarker signature for idiopathic pulmonary fibrosis: an analysis from the multicentre PROFILE cohort study. Lancet Respir Med 2017;5:946–955. 16. Jenkins RG, Simpson JK, Saini G, Bentley JH, Russell AM, Braybrooke R, et al. Longitudinal change in collagen degradation biomarkers in idiopathic pulmonary fibrosis: an analysis from the prospective, multicentre PROFILE study. Lancet Respir Med 2015;3:462–472. 17. Sadatsafavi M, Adibi A, Puhan M, Gershon A, Aaron SD, Sin DD. Moving beyond AUC: decision curve analysis for quantifying net benefit of risk prediction models. Eur Respir J 2021;58:58.

https://doi.org/10.1164/rccm.202203-0537ed

Paul M. Hassoun

Papers

Response to increased load in systemic sclerosis-associated PAH and idiopathic PAH

The addition of sildenafil to bosentan therapy in the treatment of pulmonary arterial hypertension

Right ventricular strain differences between idiopathic and systemic sclerosis Pulmonary Hypertension

Updating clinical endpoints in pulmonary arterial hypertension: when challenges are welcome

Promises and Pitfalls of Multiomics Approaches to Pulmonary Arterial Hypertension