by Victor R. Gordeuk, Oswaldo L. Castro, and Roberto F. Machado

Slides:

Advertisements

Similar presentations

Pulmonary Hypertension

Advertisements

Riociguat directly stimulates the native sGC independently of NO Riociguat increases the sensitivity of native soluble guanylate cyclase (sGC) to NO Both.

Pulmonary Hypertension Sung Chul Hwang, M.D. Dept of Pulmonary and Critical Care Medicine Ajou University School of Medicine.

Prevalence of PAH in SSc ReferenceMethodologyDiagnosisPAH prevalence Mukerjee; 2003, UK n = 722, single center Prospective RHC12%

Picture of my family – love to start talks in the comfort of my living room. Feel passionate about my girls and about this unique population of patients.

PATHOPHYSIOLOGY OF TRANSPOSITION OF THE ARTERIES (TGA)

Effort Dependence of change in 6-Minute Walk Test in Pulmonary Hypertension was improved by Correction with the Change in Heart Rate: The Beat-Yield Pulmonology.

Date of download: 7/9/2016 Copyright © The American College of Cardiology. All rights reserved. From: Pulmonary Arterial Hypertension J Am Coll Cardiol.

Pulmonary hypertension

Hemodynamic classification of pulmonary hypertension (PH)

An overview for patients and caregivers

REVEAL Registry PAH Risk Score Calculator

Guideline for approaching the differential diagnosis of pulmonary hypertension. CHD, congenital heart disease; CO, cardiac output; CT, contrast-enhanced.

Nat. Rev. Cardiol. doi: /nrcardio

An Unusual Cause of Abdominal Pain in Sickle Cell Disease

Physiological changes in pulmonary arterial hypertension (PAH) patients which occur in response to pregnancy. Physiological changes in pulmonary arterial.

Has the 6-Min Walk Distance Run Its Course?

G. Michael Felker et al. JCHF 2014;2:

ECG: electrocardiogram; PFT: pulmonary function testing; Dlco: diffusion capacity of the lung for carbon monoxide; BGA: blood gas analysis; HRCT: high-resolution.

Diagnostic algorithm for pulmonary arterial hypertension (PAH).

Cohort identification and exclusion.

Sildenafil for Improving Outcomes in Patients With Corrected Valvular Heart Disease and Persistent Pulmonary Hypertension: A Multicenter, Double-Blind,

Pulmonary hypertension in valve disease: A beast of the past?

Volume 126, Issue 1, Pages 14S-34S (July 2004)

The effect of sequential addition of sildenafil to first-line epoprostenol on exercise capacity measured using 6-min walk distance (6MWD) in the PACES.

Severe pulmonary hypertension (PH) in interstitial lung disease (ILD) stepwise composite echocardiographic score. Severe pulmonary hypertension (PH) in.

Diagnostic algorithm. Diagnostic algorithm. PH: pulmonary hypertension; group: clinical group; TTE: transthoracic echocardiography; PFT: pulmonary function.

Algorithm for the diagnosis and treatment of portopulmonary hypertension (POPH). Algorithm for the diagnosis and treatment of portopulmonary hypertension.

REVEAL pulmonary arterial hypertension (PAH) risk score.

by Santosh L. Saraf, Xu Zhang, Tamir Kanias, James P. Lash, Robert E

Treatment algorithm for managing chronic thromboembolic pulmonary hypertension (CTEPH), from the European Society of Cardiology/European Respiratory Society.

Inclusion characteristics of a) previously diagnosed and b) newly diagnosed pulmonary arterial hypertension (PAH) patients enrolled in REVEAL. PVR: pulmonary.

An algorithm for the early diagnosis of pulmonary arterial hypertension in systemic sclerosis. An algorithm for the early diagnosis of pulmonary arterial.

An expert proposal for a treat-to-target checklist for pulmonary arterial hypertension (PAH). An expert proposal for a treat-to-target checklist for pulmonary.

A Symptomatic Calcified Pericardial Cyst

Risk assessment and treat-to-target approach for pulmonary arterial hypertension. Risk assessment and treat-to-target approach for pulmonary arterial hypertension.

Selection of patients: 248 consecutive patients with newly diagnosed pre-capillary pulmonary hypertension were included in the study. Selection of patients:

Reverse remodelling of left and right cavities under specific therapy in a patient with severe idiopathic pulmonary arterial hypertension. a) Before specific.

Definition, clinical classification and initial diagnosis of pulmonary hypertension: Updated recommendations from the Cologne Consensus Conference 2018

Micha T. Maeder, MD, PhD, Niklas F. Ehl, MD

Rapid progression of midventricular obstruction in adults with double-chambered right ventricle José María Oliver, MD, Ana Garrido, MD, Ana González,

Simplified REVEAL (Registry to Evaluate Early And Long-term PAH Disease Management) risk score. Simplified REVEAL (Registry to Evaluate Early And Long-term.

Proposed screening algorithm for identification of pulmonary arterial hypertension (PAH) associated with connective tissue disease. Proposed screening.

Predicting Survival in Patients With Pulmonary Arterial Hypertension

Chronic effect of 28-day oral administration of vehicle (n = 6–10/group) or aprocitentan 1, 10, and 100 mg/kg per day (n = 6–9/group) on mean arterial.

Clinical Comparisons in CTEPH

Mean change from baseline in 6MWD in (A) Chinese patients with PAH in PATENT-1 and (B) Chinese patients with CTEPH in CHEST-1. Mean change from baseline.

Volume 133, Issue 4, Pages (April 2008)

Use of a mixed endothelin receptor antagonist in portopulmonary hypertension: A safe and effective therapy? Christian Kuntzen, Veit Gülberg, Alexander.

Haemodynamic response to exercise in a) normal subjects and b) Fontan patients. Haemodynamic response to exercise in a) normal subjects and b) Fontan patients.

Conceptual model of the ageing Fontan circulation

Results of classification and regression tree analysis in patients with pulmonary hypertension-sickle cell disease. Results of classification and regression.

Definition of pulmonary hypertension (PH) associated with left heart disease. mPAP: mean pulmonary arterial pressure; PAWP: pulmonary artery wedge pressure;

Treatment algorithm for management of CRS based on the revised CRS grading system. Treatment algorithm for management of CRS based on the revised CRS grading.

Mean pulmonary arterial pressure (Ppa) as a function of cardiac output (Q) at two different levels of pulmonary vascular resistance (PVR). Mean pulmonary.

An algorithm for investigating pulmonary hypertension (PH) using echocardiography. An algorithm for investigating pulmonary hypertension (PH) using echocardiography.

Algorithm for haemodynamic evaluation in the elderly.

Example of resting haemodynamic characteristics of two different patients with PAH (black dots) and HFpEF–PH (open dots). Example of resting haemodynamic.

Mean Pulmonary Artery Pressure Change Post-Thrombectomy Average pre-procedural mean pulmonary artery pressure (mPAP) in patients with pulmonary hypertension.

Leah M. Wright et al. JIMG 2016;9:

Diagnostic algorithm for chronic thromboembolic pulmonary hypertension

Module 2: evaluation of the REVEAL risk score in the FPHN validation cohort. Module 2: evaluation of the REVEAL risk score in the FPHN validation cohort.

Schematic drawing of PAH crisis and contributing factors.

Schematic representations of pulmonary hypertension.

Reduction in mean pulmonary vascular resistance (PVR) in 37 subjects following acute sildenafil administration to ongoing bosentan therapy in the COMPASS-1.

(A) The normal cardiovascular circulation.

by Victor A. Chow, Mazyar Shadman, and Ajay K. Gopal

Treatment algorithm for chronic thromboembolic pulmonary hypertension.

Schematic diagram of the DETECT study [56].

Effect of placebo (n=88) and bosentan (n=80) on the co-primary end-point pulmonary vascular resistance (PVR) in the EARLY (Endothelial Antagonist Trial.

Presentation transcript:

by Victor R. Gordeuk, Oswaldo L. Castro, and Roberto F. Machado Pathophysiology and treatment of pulmonary hypertension in sickle cell disease by Victor R. Gordeuk, Oswaldo L. Castro, and Roberto F. Machado Blood Volume 127(7):820-828 February 18, 2016 ©2016 by American Society of Hematology

Schematic representations of pulmonary hypertension. Schematic representations of pulmonary hypertension. (A) Schematic cross-sectional representation of a normal pulmonary arteriole and a pulmonary arteriole affected by pulmonary hypertension. (Adapted from Pugliese et al.7) (B) Schematic representation of site of initiation of elevated pulmonary arterial pressure of precapillary pulmonary hypertension, postcapillary pulmonary hypertension, and CTEPH. L.V., left ventricle; PH, pulmonary hypertension; R.A., right atrium; R.V., right ventricle. Victor R. Gordeuk et al. Blood 2016;127:820-828 ©2016 by American Society of Hematology

Proposed algorithm for evaluation of pulmonary hypertension related to sickle cell disease. 6MWD, 6-minute walk distance; ANA, anti-nuclear antibody; CXR, chest X-ray; EKG, electrocardiogram; LFTs, liver function tests; mPAP, mean pulmonary artery pressure;... Proposed algorithm for evaluation of pulmonary hypertension related to sickle cell disease. 6MWD, 6-minute walk distance; ANA, anti-nuclear antibody; CXR, chest X-ray; EKG, electrocardiogram; LFTs, liver function tests; mPAP, mean pulmonary artery pressure; NT-pro-BNP, N-terminal pro–brain natriuretic peptide; PAWP, pulmonary artery wedge pressure; PH, pulmonary hypertension; PVR, pulmonary vascular resistance; SCD, sickle cell disease; TRV, tricuspid regurgitation velocity. Note: Echocardiography should be performed while patients are clinically stable. PAH therapy is to be considered on the basis of a weak recommendation and very low-quality evidence. Reprinted with permission of the American Thoracic Society.84 The American Journal of Respiratory and Critical Care Medicine is an official journal of the American Thoracic Society. Victor R. Gordeuk et al. Blood 2016;127:820-828 ©2016 by American Society of Hematology