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
Right ventricular (RV) adaptation is the principal determinant of outcomes in pulmonary arterial hypertension (PAH), however RV function is challenging to assess. RV responses to hemodynamic stressors are particularly difficult to interrogate without invasive testing. This study sought to identify metabolomic markers of in vivo right ventricular function and exercise performance in PAH.


METHODS
Consecutive subjects with PAH (n=23) underwent rest and exercise right heart catheterization with multi-beat pressure volume loop analysis. Pulmonary arterial blood was collected at rest and during exercise. Mass spectrometry-based targeted metabolomics were performed, and metabolic associations with hemodynamics and comprehensive measures of RV function were determined using sparse partial least squares regression. Metabolite profiles were compared to pro-BNP measurements for accuracy in modeling ventriculo-arterial parameters.


RESULTS
Thirteen metabolites changed in abundance with exercise, including metabolites reflecting increased arginine bioavailability, precursors of catecholamine and nucleotide synthesis, and branched chain amino acids. Higher resting arginine bioavailability predicted more favorable exercise hemodynamics and pressure-flow relationships. Subjects with more severe PAH augmented arginine bioavailability with exercise to a greater extent than subjects with less severe PAH. We identified relationships between kynurenine pathway metabolism and impaired ventriculo-arterial coupling, worse RV diastolic function, lower RV contractility, diminished RV contractility with exercise, and RV dilation with exercise. Metabolite profiles outperformed pro-BNP in modeling RV contractility, diastolic function, and exercise performance.


CONCLUSIONS
Specific metabolite profiles correspond to RV functional measurements only obtainable via invasive pressure-volume loop analysis and predict RV responses to exercise. Metabolic profiling may inform discovery of RV functional biomarkers.

Metabolic profiling of in vivo right ventricular function and exercise performance in pulmonary arterial hypertension

OBJECTIVE
Patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) experience worse survival and derive less benefit from pulmonary vasodilator therapies than patients with idiopathic PAH (IPAH). We sought to identify differential metabolism in CTD-PAH versus IPAH patients that might underlie these observed clinical differences.


METHODS
Adult subjects with CTD-PAH (n=141) and IPAH (n=165) from the PVDOMICS (Pulmonary Vascular Disease Phenomics) Study were included. Detailed clinical phenotyping was performed at cohort enrollment, including broad-based global metabolomic profiling of plasma samples. Subjects were followed prospectively for ascertainment of outcomes. Supervised and unsupervised machine learning algorithms and regression models were used to compare CTD-PAH versus IPAH metabolomic profiles and to measure metabolite-phenotype associations and interactions. Gradients across the pulmonary circulation were assessed using paired mixed venous and wedged samples in a subset of 115 subjects.


RESULTS
Metabolomic profiles distinguished CTD-PAH from IPAH, with CTD-PAH patients demonstrating aberrant lipid metabolism, with lower circulating levels of sex steroid hormones and higher free fatty acids (FA) and FA intermediates in CTD-PAH. Acylcholines were taken up by the right ventricular-pulmonary vascular circulation, particularly in CTD-PAH, while free FAs and acylcarnitines were released. In both PAH subtypes, dysregulated lipid metabolites, among others, were associated with hemodynamic and right ventricular measurements and with transplant-free survival.


CONCLUSIONS
CTD-PAH is characterized by aberrant lipid metabolism that may signal shifted metabolic substrate utilization. Abnormalities in RV-pulmonary vascular FA metabolism may imply reduced capacity for mitochondrial beta oxidation within the diseased pulmonary circulation.

Metabolomic differences in connective tissue disease-associated versus idiopathic pulmonary arterial hypertension in the PVDOMICS cohort.

To the Editor: We read with great interest the article by Christie et al (1) published in a recent issue of Critical Care Medicine. A major hurdle in designing clinical trials of pharmaceutical therapies for patients with the acute respiratory distress syndrome (ARDS) is the difficulty in translating promising findings from preclinical animal experiments to human clinical trials. This is likely due in part to tremendous heterogeneity in ARDS. Recent work has identified discrete molecular patterns of injury based on the etiology of ARDS, where direct causes of lung injury lead to significantly more epithelial damage, whereas indirect causes lead to more endothelial damage (2). The clinical importance of this distinction was highlighted in the recent clinical trials of β-agonists for the treatment of ARDS. The β-Agonist Lung Injury Trial (BALTI) demonstrated a significant reduction in lung water with salbutamol treatment compared with placebo (3). However, these promising results could not be replicated in the BALTI-2 trial. In fact, patient recruitment was interrupted early due to safety concerns (4). Interestingly, 64% of patients in BALTI-2 trial, compared with only 15.8% in BALTI trial, randomized to the salbutamol group had a direct cause of injury identified as the source of ARDS. As β-agonists rely on functioning epithelial cells to promote adequate clearance of extravascular lung water, it is reasonable to think that when there is a significant epithelial injury, as is the case with direct lung injury, β-agonists may be ineffective. is questionable as many major public hospitals have highly developed intensive care services in the absence of such arrangements. Autonomy and political power are the key words in their letter. It swings back toward the original premise that professional independence and strength are the predominant driving factors in creating ICM as a single, dynamic, committed medical specialty and that training issues follow in their wake (3). The author has disclosed that he does not have any potential conflicts of interest.

Paul M. Hassoun

Papers

Metabolic profiling of in vivo right ventricular function and exercise performance in pulmonary arterial hypertension

Metabolomic differences in connective tissue disease-associated versus idiopathic pulmonary arterial hypertension in the PVDOMICS cohort.

Trials and Tribulations of Therapies for the Acute Respiratory Distress Syndrome.

Relationship Between Hepatic Fat Content and Pulmonary Vascular Disease Severity in a Subset of the Pvdomics Cohort

MiRNA-133-a as a Mediator of Endostatin-dependent Gene Expression in Pulmonary Arterial Hypertension