Relationship of Pulmonary Arterial Capacitance and Mortality in Idiopathic Pulmonary Arterial Hypertension

Mahapatra, Srijoy; Nishimura, Rick A.; Sorajja, Paul; Cha, Stephen; McGoon, Michael D.

doi:10.1016/j.jacc.2005.09.054

Cited by 360 publications

(318 citation statements)

References 18 publications

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“…Miyamoto et al found a direct association between 6MWD and survival, independent of other noninvasive measures [90]. Almost all other studies of 6MWD in PAH have confirmed this association [19,35,38,[40][41][42]44,49,50,91]. Sitbon et al found inverse associations between 6MWD at baseline and after three months of epoprostenol therapy with the risk of death [19].…”

Section: Exercise Testing Six Minute Walk Test (6mwt)mentioning

confidence: 94%

“…While these findings were published in an era before effective therapy was available, they have generally (but not universally) been validated in more recent studies of patients at diagnosis or initiation of therapy [19,[33][34][35][36][37][38][39][40][41][42][43][44][45][46][47][48][49][50]. Importantly, PAH patients with more severe cardiac dysfunction or history of heart failure at baseline continue to have a higher risk of death than those with less severe heart failure despite treatment with new therapies [33,34,36,38,41,45,46,48,49]. Improvement in these characteristic hemodynamic abnormalities after therapy has been associated with better survival [19,34,49].…”

Section: Potential Surrogate and Intermediate End Points In Pah Hemodmentioning

confidence: 96%

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Surrogate End Points in Pulmonary Arterial Hypertension: Assessing the Response to Therapy

Snow¹,

Kawut²

2007

Clinics in Chest Medicine

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Pulmonary arterial hypertension (PAH) is a rare disease which is characterized by increased pulmonary vascular resistance and right heart failure. Recent discoveries in disease pathophysiology have been translated into effective therapies tested in clinical trials. The studies which have led to the regulatory board approval of therapies for PAH have focused on surrogate and intermediate end points, thought to reflect quantity and quality of life, respectively. However, validation of many of these surrogates is incomplete. It is also unknown which indicators of function or long-term survival should be used to formulate decisions regarding addition, discontinuation, or combination of therapies. Identification of suitable end points would therefore not only help investigators design appropriate clinical trials, but also assist clinicians in caring for patients with PAH. Hemodynamic, cardiac imaging, plasma biomarkers, and exercise testing hold some promise as potential surrogate end points for PAH. Functional status and quality of life assessments may also have important roles. Future studies should validate the most promising surrogate markers, so that patients, clinicians, subjects, and investigators may benefit from the advantages they confer on clinical care and on clinical trials.The past several years have yielded an explosion of randomized clinical trials (RCTs) of several new medical therapies for pulmonary arterial hypertension (PAH). The hopes for these and all treatments for potentially fatal diseases are 1) to improve how a patient feels or functions and/ or 2) to prolong survival. While simply stated, the assessment of progress towards these goals may be quite complex [1][2][3][4][5][6].New PAH therapies have gained regulatory board approval based on studies with intermediate and surrogate end points as primary outcomes, believed to reflect the impact of these drugs on quality of life (QOL) and survival, respectively. While hemodynamic, plasma, cardiac imaging, exercise, functional, and QOL parameters are promising candidates, the reliability and validity of these measures are not clearly established. In addition, there are no RCTs addressing the use of such end points in guiding management decisions in PAH, such as changing, intensifying, escalating, or combining therapies. Despite this lack of evidence, clinicians who

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Section: Exercise Testing Six Minute Walk Test (6mwt)mentioning

confidence: 94%

Section: Potential Surrogate and Intermediate End Points In Pah Hemodmentioning

confidence: 96%

Surrogate End Points in Pulmonary Arterial Hypertension: Assessing the Response to Therapy

Snow¹,

Kawut²

2007

Clinics in Chest Medicine

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“…1 PAC is a strong predictor of mortality in patients with idiopathic PAH as well as in patients with PAH related to systemic sclerosis. 2,3 However, the prognostic ability of PAC has not been demonstrated in other subtypes of PAH nor has it been compared with the prognostic ability of other hemodynamic parameters in patients with different subtypes of PAH. This report aims to address these objectives in a diverse, well-characterized cohort of patients with PAH.…”

mentioning

confidence: 99%

The Prognostic Significance of Pulmonary Arterial Capacitance in Pulmonary Arterial Hypertension: Single‐Center Experience

et al. 2016

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This study explores the prognostic utility of pulmonary arterial capacitance (PAC) in a diverse cohort of patients with pulmonary arterial hypertension (PAH) from a tertiary referral center and compares it with the prognostic utility of other hemodynamic parameters. PAC is a strong independent predictor of mortality in patients with PAH.Keywords: pulmonary arterial hypertension, pulmonary arterial capacitance, prognosis.Pulm Circ 2016;6(4):608-610. DOI: 10.1086/688900.The importance of pulmonary arterial capacitance (PAC) in the pathogenesis and prognostication of pulmonary arterial hypertension (PAH) is increasingly recognized. 1 PAC is a strong predictor of mortality in patients with idiopathic PAH as well as in patients with PAH related to systemic sclerosis. 2,3 However, the prognostic ability of PAC has not been demonstrated in other subtypes of PAH nor has it been compared with the prognostic ability of other hemodynamic parameters in patients with different subtypes of PAH. This report aims to address these objectives in a diverse, well-characterized cohort of patients with PAH. METHODSWe identified all consecutive, adult patients with World Health Organization (WHO) group 1 PAH (both incident and prevalent disease) from the Tufts Medical Center Pulmonary Hypertension (PH) Registry who underwent a right heart catheterization (RHC) between July 1, 2001, and December 31, 2012. Patients who had WHO group 2, 3, 4, or 5 PAH were excluded from this analysis. Parameters measured during the RHC included heart rate (HR), mean right atrial pressure (RAP), mean pulmonary artery pressure (mPAP), pulmonary capillary wedge pressure (PCWP), and cardiac output (CO) by thermodilution method. These parameters were determined by manual review of the tracings. The pulmonary vascular resistance (PVR) was calculated as (mPAP − PCWP)/CO (Wood units), cardiac index (CI) as CO/body surface area (L/min/m 2 ), pulmonary artery pulse pressure as PA systolic − PA diastolic, and PAC as stroke volume/pulmonary artery pulse pressure (mL/mmHg). Patients were followed up until death or October 1, 2015. None of the patients was lost to follow-up, and all patients who were still alive were censored at the end of follow-up.Categorical variables were summarized as numbers and percentages, whereas continuous variables were summarized as mean value ± standard deviation (SD) or median (interquartile range [IQR]), as appropriate. Receiver-operating characteristic (ROC) curves were used to evaluate the ability of PAC, mRAP, mPAP, CI, and PVR to discriminate between patients who had died within the first year after RHC and those who were still alive. The area under the ROC curve (AUC) was compared using the DeLong method. Cox proportional hazards models were used to estimate hazard ratios for these hemodynamic parameters and mortality. These models were then adjusted for age, sex, and etiology of PAH. All analyses were conducted in R version 3.2.1. RESULTSA total of 105 patients were identified in the database and included in the analysis...

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“…These are worse in iPAH, leading to increased RV load. 1,28 Secondly, the RV response to its given afterload might differ. In PS the RV faces increased pressure load from fetal or early postnatal life.…”

Section: Pathophysiologymentioning

confidence: 99%

Regional right ventricular remodeling and function in children with idiopathic pulmonary arterial hypertension vs those with pulmonary valve stenosis: Insights into mechanics of right ventricular dysfunction

et al. 2017

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Background Right ventricular (RV) pressure overload in the context of pulmonary stenosis (PS) has a much better prognosis than in the context of idiopathic pulmonary arterial hypertension (iPAH), which may be related to differences in global and regional RV remodeling and systolic function. We compared RV mechanics in children with PS to those with iPAH, aiming to identify mechanisms and markers of RV dysfunction. Methods Eighteen controls, 18 iPAH and 16 PS patients were retrospectively studied. Age, BSA, and sex distribution were comparable. Two‐dimensional echocardiography, blood flow and tissue Doppler, and longitudinal RV deformation were analyzed. ANCOVA—including RV systolic pressure (RVSP) and length as covariates—was used to compare patient groups. Results RV systolic pressure was higher in iPAH vs PS (96.8±25.4 vs 75.4±18.9 mm Hg, P=.011). Compared to controls, PS patients showed mild dilation (P<.01) and decreased longitudinal deformation (P<.001) at the RV apex. Compared to both PS and controls, iPAH patients showed marked spherical RV dilation (P<.001), reduced global, RV free wall and septal longitudinal deformation (iPAH −22.07%±4.35% vs controls −28.18%±1.69%; −9.98%±4.30% vs −17.45%±2.52%; P<.001) and RV postsystolic shortening (P<.001). RV transverse shortening (radial performance) was increased in PS (31.75%±10.35%; P<.001) but reduced in iPAH (−1.62%±11.11% vs controls 12.00%±7.74%; P<.001). Conclusion Children with iPAH demonstrate adverse global and regional RV remodeling and mechanics compared to those with PS. Mechanisms of RV systolic dysfunction in iPAH include decreased longitudinal deformation, decreased or absent transverse shortening, and postsystolic shortening. These markers may be useful to identify children at risk of RV failure.

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Relationship of Pulmonary Arterial Capacitance and Mortality in Idiopathic Pulmonary Arterial Hypertension

Abstract: The capacitance index (SV/PP) is a strong independent predictor of mortality in patients with IPAH.

Cited by 360 publications

References 18 publications

Surrogate End Points in Pulmonary Arterial Hypertension: Assessing the Response to Therapy

Surrogate End Points in Pulmonary Arterial Hypertension: Assessing the Response to Therapy

The Prognostic Significance of Pulmonary Arterial Capacitance in Pulmonary Arterial Hypertension: Single‐Center Experience

Regional right ventricular remodeling and function in children with idiopathic pulmonary arterial hypertension vs those with pulmonary valve stenosis: Insights into mechanics of right ventricular dysfunction

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