A5033758604- Pulmonary Hypertension Research and Treatments
- Systemic Sclerosis and Related Diseases
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Cardiovascular Function and Risk Factors
- Vascular Anomalies and Treatments
- Heart Failure Treatment and Management
- Cardiovascular Issues in Pregnancy
- Medical Imaging and Pathology Studies
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Cardiovascular Effects of Exercise
- Liver Disease and Transplantation
- Congenital Heart Disease Studies
- Sarcoidosis and Beryllium Toxicity Research
- Cardiac Valve Diseases and Treatments
- Respiratory Support and Mechanisms
- Cardiovascular Disease and Adiposity
- Transplantation: Methods and Outcomes
- Connective Tissue Growth Factor Research
- Cardiac Imaging and Diagnostics
- Venous Thromboembolism Diagnosis and Management
- Hemodynamic Monitoring and Therapy
- Eosinophilic Disorders and Syndromes
- Advanced MRI Techniques and Applications
- Tracheal and airway disorders
- Cardiac Arrhythmias and Treatments
Johns Hopkins Medicine
2016-2025
Johns Hopkins University
2016-2025
Johns Hopkins Hospital
2010-2025
Royal Victoria Hospital
2024
University of Ulster
2024
Papworth Hospital
2024
University of Arizona
2023
Pulmonary and Critical Care Associates
2010-2022
American Heart Institute
2021
American Thoracic Society
2018
Right ventricular (RV) function is an important determinant of prognosis in pulmonary hypertension. However, noninvasive assessment the RV often limited by complex geometry and poor endocardial definition.To test whether degree tricuspid annular displacement (tricuspid plane systolic excursion [TAPSE]) a useful echo-derived measure with prognostic significance hypertension.We prospectively studied 63 consecutive patients hypertension who were referred for clinically indicated right heart...
Background— Right ventricular failure from increased pulmonary vascular loading is a major cause of morbidity and mortality, yet its modulation by disease remains poorly understood. We tested the hypotheses that, unlike systemic circulation, resistance (R PA ) compliance (C are consistently inversely related regardless age, hypertension, or interstitial fibrosis that this relation may be changed elevated capillary wedge pressure, augmenting right pulsatile load. Methods Results— Several...
Abstract Objective Pulmonary arterial hypertension related to scleroderma (PAH‐Scl) is associated with high morbidity and mortality as well poorer response therapy worse outcomes compared the idiopathic form of PAH (IPAH). Scleroderma an autoimmune disease that can affect left right heart function directly through inflammation fibrosis indirectly systemic pulmonary hypertension. This study tested hypothesis increased prevalence might explain higher in patients PAH‐Scl IPAH. Methods The was...
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Background— Systemic sclerosis–associated pulmonary artery hypertension (SScPAH) has a worse prognosis compared with idiopathic arterial (IPAH), median survival of 3 years after diagnosis often caused by right ventricular (RV) failure. We tested whether SScPAH or systemic sclerosis–related interstitial lung disease imposes greater vascular load than IPAH and leads to RV contractile function. Methods Results— analyzed pressures mean flow in 282 patients (166 SScPAH, 49 disease, 67 IPAH). An...
Although commonly used as the primary outcome measure of clinical trials in pulmonary arterial hypertension (PAH), minimal important difference (MID) 6-minute walk test (6MWT) has not been well defined for this population patients.To estimate MID 6MWT patients with PAH.Study subjects from trial tadalafil PAH, a 16-week, parallel-group, randomized who were treatment naive or on background therapy an endothelin receptor antagonist, eligible. was performed using standardized protocol....
Pulmonary arterial hypertension (PAH) affects up to 15% of patients with connective tissue diseases (CTDs). Previous recommendations developed as part larger efforts in PAH did not include detailed for CTD-associated PAH. Therefore, we sought develop screening and early detection PAH.We performed a systematic review the literature on diagnosis CTD. Using RAND/University California, Los Angeles consensus methodology, case scenarios followed by 2 stages voting. First, international experts...
Right ventricular (RV) functional reserve affects capacity and prognosis in patients with pulmonary arterial hypertension (PAH). PAH associated systemic sclerosis (SSc-PAH) has a substantially worse than idiopathic (IPAH), even though many measures of resting RV function vascular load are similar. We therefore tested the hypothesis that is depressed SSc-PAH patients.RV pressure-volume relations were prospectively measured IPAH (n=9) (n=15) at rest during incremental atrial pacing or supine...
In pulmonary arterial hypertension (PAH), there are no data comparing initial triple oral therapy with double therapy.TRITON (The Efficacy and Safety of Initial Triple Versus Dual Oral Combination Therapy in Patients With Newly Diagnosed Pulmonary Arterial Hypertension; NCT02558231), a multicenter, double-blind, randomized phase 3b study, evaluated (macitentan, tadalafil, selexipag) versus placebo) newly diagnosed, treatment-naive patients PAH.Efficacy was assessed until the last patient...
Background Current mortality data for pulmonary arterial hypertension (PAH) in the United States are based on registries that enrolled patients prior to 2010. We sought determine PAH modern era using PHAR (Pulmonary Hypertension Association Registry). Methods and Results identified all adult with between September 2015 2020 (N=935). used Kaplan‐Meier survival analysis Cox proportional hazards models assess at 1, 2, 3 years. Patients were stratified disease severity by validated risk scores....
Pulmonary hypertension (PH) is an important cause of mortality in systemic sclerosis (SSc), where it can be isolated (pulmonary arterial [PAH]) or associated with interstitial lung disease (ILD). This study was undertaken to characterize determinants survival among SSc patients either type PH who received PAH-specific therapy.Consecutive PAH ILD-associated confirmed by right heart catheterization were included the study. Kaplan-Meier and Cox proportional hazards models used compare between...
Rationale: Pulmonary arterial hypertension (PAH) related to systemic sclerosis (SSc) has a poorer prognosis compared with other forms of PAH for reasons that remain unexplained.Objectives: To identify risk factors mortality in well-characterized cohort patients (SSc-PAH).Methods: Seventy-six consecutive SSc (64 women and 12 men; mean age 61 ± 11 yr) were diagnosed by heart catheterization single center, starting January 2000, followed over time. Kaplan-Meier estimates calculated...
Combination therapy has been recommended for the treatment of pulmonary arterial hypertension (PAH). However, there is scant information on combination after failure monotherapy, particularly in patients with scleroderma-associated PAH (PAH-SSD). From a group 82 consecutive who received initial bosentan total 13 idiopathic (IPAH) and 12 PAH-SSD requiring additional sildenafil were studied. Sildenafil was added clinical deterioration based upon symptoms, New York Heart Association (NYHA)...
The aim of this study was to examine the causes and outcomes hospitalisation in patients with pulmonary arterial hypertension (PAH). 205 consecutive hospitalisations occurring between 2000 2009 90 PAH were studied. leading for right heart failure (RHF; 56%), infection (16%) bleeding disorders (8%). For RHF, in-hospital mortality 14% overall, 46% receiving inotropes 48% those admitted intensive care unit. predictors presence connective tissue disease (CTD) (OR 4.92), systolic blood pressure...
Pulmonary hypertension (PH) secondary to left heart failure portends a poor prognosis and is relative contraindication transplantation at many centers. We tested the hypothesis that when PH persists after adequate ventricle unloading via recent ventricular assist device (LVAD) therapy, phosphodiesterase type 5A inhibition would decrease in this population.We performed an open-label clinical trial using control patients not receiving therapy. Between 1999 2007, 138 consecutive undergoing...
Hyponatremia is associated with decompensated heart failure and poor prognosis in patients left ventricular systolic dysfunction.We sought to determine if hyponatremia right worse pulmonary arterial hypertension (PAH).We prospectively followed 40 PAH examined the relationship between serum sodium function as well survival.Subjects (Na < or = 136 mEq/L) were more symptomatic (11/13 World Health Organization [WHO] class III/IV vs. 12/27 WHO III/IV; P 0.02), had peripheral edema (69 26%;...
Scleroderma-associated pulmonary arterial hypertension (SSc-PAH) is a rare disease characterized by very dismal response to therapy and poor survival. We assessed the effects of up-front combination PAH in patients with SSc-PAH.In this prospective, multicenter, open-label trial, 24 treatment-naive SSc-PAH received ambrisentan 10 mg tadalafil 40 daily for 36 weeks. Functional, hemodynamic, imaging (cardiac magnetic resonance echocardiography) assessments at baseline weeks included changes...
N-terminal pro-brain natriuretic peptide (NT-proBNP) is a marker of neurohormonal activation that useful in the diagnosis and prognosis various forms pulmonary arterial hypertension (PAH). We sought to characterise compare NT-proBNP cohort PAH related systemic sclerosis (PAH-SSc) idiopathic (IPAH) patients. levels, collected from PAH-SSc IPAH patients followed prospectively, were compared correlated with haemodynamic variables. Cox proportional hazard models created assess predictive value...
Background: Patients with systemic sclerosis (SSc)–associated pulmonary arterial hypertension (PAH) have a far worse prognosis than those idiopathic PAH (IPAH). In the intact heart, SSc-PAH exhibits depressed rest and reserve right ventricular (RV) contractility compared IPAH. We tested whether this disparity involves underlying differences in myofilament function. Methods: Cardiac myocytes were isolated from RV septal endomyocardial biopsies patients SSc-PAH, IPAH, or SSc exertional dyspnea...