Clinically Significant Change in Stroke Volume in Pulmonary Hypertension

Wolferen, S A van; Veerdonk, Mariëlle C. van de; Mauritz, Gert-Jan; Jacobs, Wouter; Marcus, J. Tim; Marques, Koen M.; Bronzwaer, Jean G.F.; Heymans, Martijn W.; Boonstra, Anco; Postmus, Pieter E.; Westerhof, Nico; Noordegraaf, Anton Vonk

doi:10.1378/chest.10-1066

Cited by 104 publications

(78 citation statements)

References 33 publications

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“…94 Another study by the same authors suggested that compared with 6MWD, changes in SV of at least 10 mL were clinically important after 1 year of PAH therapy. 95 These results have not been evaluated in SSc-PAH. Members of our group demonstrated significant decreases in myocardial perfusion reserve affect ing the right ventricle as well as the left ventricle in a population of patients with PAH largely represented by SSc-PAH.…”

Section: Role Of Sponsorsmentioning

confidence: 99%

Systemic Sclerosis-associated Pulmonary Arterial Hypertension

Pavec

Humbert

Mouthon

et al. 2010

Am J Respir Crit Care Med

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Systemic sclerosis (SSc) is a complex, multisystem disease characterized by fi brosis and excessive collagen deposition within the skin and internal organs, chronic infl ammation, autoimmune dysregulation, and microvascular endothelial dysfunction. There are two forms of SSc that are characterized by the extent of skin involvement. Limited cutaneous SSc predominantly involves the skin of the hands, arms, and face, and diffuse SSc involves large areas of skin and multiple organs. 1 Both forms are systemic diseases associated with signifi cant morbidity and mortality. Historically, mortality was largely caused by renal disease. With the advent of angiotensin-converting enzyme inhibitors to treat SSc renal crisis, SSc-associated pulmonary arterial hypertension (SSc-PAH) has emerged as a leading cause of morbidity and mortality, accounting for up to 30% of premature deaths. 2 Although SSc-PAH represents a signifi cant proportion of the pulmonary arterial hypertension (PAH) population (15%-30%), 3,4 much of what we know about PAH is derived from studies of idiopathic PAH (IPAH).Pulmonary arterial hypertension (PAH) is the leading cause of death in systemic sclerosis (SSc ) and affects up to 12% of all patients with SSc, with a 50% mortality rate within 3 years of PAH diagnosis. Compared with the idiopathic form of PAH (IPAH), patients with SSc-associated PAH (SSc-PAH) have a threefold increased risk of death and may receive a diagnosis late in the course of disease because of insidious onset and the high prevalence of cardiac, musculoskeletal, and pulmonary parenchymal comorbidities. Treatment with conventional forms of PAH therapy often yield poor results compared with IPAH cohorts; unfortunately, the exact reasons behind this remain poorly understood but likely include variations in the pathologic mechanisms, differences in cardiovascular response to increasing afterload, and inadequate strategies to detect and treat SSc-PAH early in its course. Current methods for screening and longitudinal evaluation of SSc-PAH, such as the 6-min walk test, transthoracic echocardiography, and MRI, each have notable advantages and disadvantages. We provide an up-to-date, focused review of SSc-PAH and how it differs from IPAH, including pathogenesis, appropriate screening for disease onset, and new approaches to treatment and longitudinal assessment of this disease. CHEST 2013; 144(4):1346-1356Abbreviations: 6MWD 5 6-min walk distance; CCB 5 calcium channel blocker; CTD 5 connective tissue disease; D lco 5 diffusing capacity of lung for carbon monoxide; EIPAH 5 exercise-induced pulmonary arterial hypertension; ERA 5 endothelin receptor antagonist; FC 5 functional class; ILD 5 interstitial lung disease; IPAH 5 idiopathic pulmonary arterial hypertension; LV 5 left ventricular; mPAP 5 mean pulmonary artery pressure; PAH 5 pulmonary arterial hypertension; PASP 5 pulmonary artery systolic pressure; PDE-5I 5 phosphodiesterase type 5 inhibitor; PFT 5 pulmonary function test; PH 5 pulmonary hypertension; pro-BNP 5 pro-brain natriuretic pe...

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Section: Role Of Sponsorsmentioning

confidence: 99%

Systemic Sclerosis-associated Pulmonary Arterial Hypertension

Pavec

Humbert

Mouthon

et al. 2010

Am J Respir Crit Care Med

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“…More recent prediction equations have used a combination of invasive measures and noninvasive measures, such as 6-minute-walk distance (6MWD) and World Health Organization (WHO) functional class, along with serologic markers of renal function and right ventricular strain (4)(5)(6). Similarly, other characteristics, such as age, sex, disease type (idiopathic PAH [IPAH] vs. connective tissue disease-associated PAH [CTD-PAH]), and right ventricular function assessed by cardiac magnetic resonance imaging or echocardiography, among others, have been shown to predict outcome in PAH (7)(8)(9)(10)(11)(12)(13). However, there are few data examining the potential role of patient-reported outcomes (PRO) in the prognosis of PAH.…”

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confidence: 99%

Health-related Quality of Life and Survival in Pulmonary Arterial Hypertension

et al. 2016

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Rationale: Pulmonary arterial hypertension is a progressive disease with high morbidity and mortality despite advances in medical therapy. The relationship between patient-related outcomes, such as healthrelated quality of life (HRQOL), and survival is not well described.Objective: To assess the relationship between HRQOL and outcomes in patients with pulmonary arterial hypertension.Methods: Consecutive patients with right heart catheterization-proven pulmonary arterial hypertension who completed the Medical Outcomes Survey Short Form-36 survey (SF-36) were included. Demographic, clinical, physiological, and hemodynamic data were collected at baseline. Survival was assessed from the time of diagnosis of pulmonary arterial hypertension. Cox proportional hazard models were constructed to assess the relationship between HRQOL and transplant-free survival.Measurements and Main Results: Eighty-seven patients with pulmonary arterial hypertension were enrolled and followed prospectively for a median of 3.8 years. At baseline, HRQOL was significantly worse than U.S. normal values for six of eight domains of the SF-36. Several domains demonstrated moderate correlation (r value > 0.40) with 6-minute-walk distance and World Health Organization functional class; there were no significant associations with hemodynamics. In univariable Cox proportional hazard models, six of eight domains and both summary scores were significantly associated with survival. In multivariable models, adjusted for age, disease type, and cardiac function, these relationships largely persisted. Conclusions:In this cohort of patients with pulmonary arterial hypertension, HRQOL, as assessed by the SF-36, was strongly associated with transplant-free survival. These relationships persisted when controlling for potential confounders such as disease type and disease severity. These findings suggest that HRQOL may be an important predictor of outcomes in pulmonary arterial hypertension and therefore a target for future therapeutic interventions.

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“…Examples of parameters obtained from imaging are stroke volume (SV) as a measure of global cardiovascular performance, right ventricular ejection fraction, i.e. SV over end-diastolic volume, and tricuspid annular plane systolic excursion, as measures of right heart function [4][5][6]. More recent advances allow measurement of regional myocardial shortening by means of speckle echocardiography or strain analysis by magnetic resonance imaging (MRI) through tagging [7,8] and calculation of wall stress to get more insight into (changes in) muscle properties per se.…”

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confidence: 99%

Describing right ventricular function

Vonk‐Noordegraaf

Westerhof

2013

Eur Respir J

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In pulmonary hypertension and other conditions, such as acute respiratory distress syndrome and left heart failure, right ventricular dysfunction is a prognostic determinant. Therefore, functional parameters pertaining to the right heart alone, i.e. independent of its loading conditions, should be used to quantify right ventricular disease progression and the results of treatment. Two methods to quantitatively describe right ventricular function are reviewed, the pressure-volume relationship and the pump function graph; examples of each method are given. How to estimate wall stress and how to describe diastolic ventricular properties are also discussed.

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Clinically Significant Change in Stroke Volume in Pulmonary Hypertension

Cited by 104 publications

References 33 publications

Systemic Sclerosis-associated Pulmonary Arterial Hypertension

Systemic Sclerosis-associated Pulmonary Arterial Hypertension

Health-related Quality of Life and Survival in Pulmonary Arterial Hypertension

Describing right ventricular function

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