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

Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is a catastrophic complication of one of the most common and devastating autoimmune diseases. Once diagnosed, it becomes the leading cause of mortality among this patient population. Screening modalities and risk assessments have been designed and validated by various organizations and societies in order to identify patients early in their disease course and promptly refer them to expert centers for a hemodynamic assessment and formal diagnosis. Moreover, several large multicenter clinical trials have now included patients with SSc-PAH to assess their response to therapy. Despite an improved understanding of the condition and significant advances in supportive and targeted therapy, outcomes have remained far from optimal. Therefore, rigorous phenotyping and search for novel therapies are desperately needed for this devastating condition.

https://www.mdpi.com/2075-4418/11/5/911/pdf

Systemic Sclerosis-Associated Pulmonary Hypertension: Spectrum and Impact

To the Editor Pulmonary hypertension (PH) is a pathophysiological disorder that complicates various clinical conditions including cardiovascular and respiratory diseases. The current classification of PH identifies five distinct groups sharing similar clinical presentation, pathological findings, and hemodynamic characteristics. Phenotyping patients and assigning the correct type of PH is essential during diagnostic work-up in order to adopt the most appropriate therapeutic strategy. In this context, invasive assessment of pulmonary hemodynamics and cardiac output by means of right heart catheterization (RHC) plays a crucial role. In particular, from a hemodynamic standpoint, beyond the presence of mean pulmonary arterial pressure 25mmHg (definition of PH), mean pulmonary arterial wedge pressure (PAWP) value 15mmHg further distinguishes pre-capillary from postcapillary PH (typically group 2 due to left heart disease), which is characterized by a PAWP >15mmHg. The latter type of PH may present with isolated post-capillary PH or combined PH with a pre-capillary component, defined by an elevated diastolic pressure gradient and/or an increase in pulmonary vascular resistance, both calculations taking into account PAWP value. Thus, the importance of PAWP measurement is evident in identifying the correct PH group for individual patients, although it is based on a rather arbitrary cut-off. Unfortunately, PAWP measurements are often affected by calibration issues, respiratory variation, and are dependent on the operator’s skills as suggested by poor interobserver reliability. In addition, PAWP measurement can significantly be affected by methodological issues, and may vary from individual patients’ measurements of left ventricular end-diastolic pressure. Furthermore, a one-time measurement of PAWP could be affected by volume status or diuretic intake at a specific moment in time (e.g. patients with group 2 PH may have a normal PAWP in conditions of optimal medical treatment and volume depletion with diuretics), thus not necessary allowing distinction between preand post-capillary PH. To further complicate the situation, in spite of the clarity and apparent simplicity of this classification, PH categories may actually co-exist in several clinical settings (i.e. the socalled atypical idiopathic pulmonary arterial hypertension [PAH]: hemodynamic features of pre-capillary PH, but associated with multiple risk factors for left-sided heart failure such as systemic hypertension, obesity, diabetes, and atrial fibrillation). Such a complex situation is typified by PH associated with connective tissue disease, particularly systemic sclerosis (scleroderma [SSc]). Patients with SSc are indeed a unique group of patients since they are prone to developing PH that may fit in any of the first four groups of PH, whether they have a predominantly vascular disease with little evidence of parenchymal disease (group 1), or PH associated with left heart disease (group 2), chronic lung diseases (group 3 with, for instance, interstitial lung disease but also obstructive sleep apnea), or in the presence of chronic thromboembolic disease (group 4). SSc patients are also generally older than patients with idiopathic PAH and thus more prone to having co-morbid conditions such as left heart disease from systemic hypertension, diastolic dysfunction, and atrial arrhythmias, and to developing lung fibrosis. In this issue of Pulmonary Circulation, Lammi et al. deal with the change of PH classification based on changes in PAWP that occurred over a median time of about 1.4 years in the prospective observational PHAROS (Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma) registry cohort of patients with SSc complicated by PH. The study was limited to patients who had at least two RHC procedures performed. A total of 120 patients were included in the analysis. While there was an overall average change in PAWP from 11 5 to 13 6mmHg, about 30% of patients were found to have a change in PAWP values that eventually required a change in PH hemodynamic classification. Patients initially classified as group 2 PH (post-capillary PH) were most likely to change PAWP class (to pre-capillary PH) over time. The authors conclude that PAWP values commonly change over time in patients with SSc-associated PH and warn about using a single time-point PAWP to define classification groups in this patient population. The results of the current study are not too surprising. Phenotyping these patients, in terms of the cause of

https://journals.sagepub.com/doi/pdf/10.1177/2045894018785247

Phenotyping pulmonary hypertension in systemic sclerosis: a moving target:

Survival of Pulmonary Arterial Hypertension Related to Systemic Sclerosis: A Single Center Longitudinal Study.

Editorial: Tackling the Challenges of Systemic Sclerosis–Associated Pulmonary Hypertension: One Step Forward

Introduction: We assessed the effects of upfront, combination therapy with a phosphodiesterase inhibitor (tadalafil) and endothelin receptor antagonist (ambrisentan) on changes in size and function...

Abstract 14254: Right Ventricular Reverse Remodeling and Improved Systolic Function After Upfront Combination Therapy With Ambrisentan and Tadalafil in the Treatment of Scleroderma-Associated Pulmonary Arterial Hypertension

Paul M. Hassoun

Papers

Systemic Sclerosis-Associated Pulmonary Hypertension: Spectrum and Impact

Phenotyping pulmonary hypertension in systemic sclerosis: a moving target:

Survival of Pulmonary Arterial Hypertension Related to Systemic Sclerosis: A Single Center Longitudinal Study.

Editorial: Tackling the Challenges of Systemic Sclerosis–Associated Pulmonary Hypertension: One Step Forward

Abstract 14254: Right Ventricular Reverse Remodeling and Improved Systolic Function After Upfront Combination Therapy With Ambrisentan and Tadalafil in the Treatment of Scleroderma-Associated Pulmonary Arterial Hypertension