Right Ventricular Dysfunction in Systemic Sclerosis–Associated Pulmonary Arterial Hypertension

Tedford, Ryan J.; Mudd, James O.; Girgis, Reda E.; Mathai, Stephen C.; Zaiman, Ari; Housten-Harris, Traci; Boyce, Danielle; Kelemen, Benjamin W.; Bacher, Anita C.; Shah, Ami A.; Hummers, Laura K.; Wigley, Fredrick M.; Russell, Stuart D.; Saggar, Rajeev; Saggar, Rajan; Maughan, W L; Hassoun, Paul M.; Kass, David A.

doi:10.1161/circheartfailure.112.000008

Cited by 242 publications

(245 citation statements)

References 43 publications

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“…In post-capillary PH, RC-time decreases linearly with increasing left atrial pressure [22][23][24]. Finally, in precapillary PH, it has been suggested that at high values of PVR, compliance reaches a minimum and that further increases in PVR would result in an increase in RC-time [17]. The above observations have led to concerns and cast doubt regarding the RC-time constancy paradigm even among specific disease subgroups.…”

Section: Rc-time: a Critical Evaluation And New Proposalmentioning

confidence: 99%

“…It has been proposed that a unique feature of the pulmonary circulation is that PVR and total arterial compliance are tightly coupled through an inverse hyperbolic relationship, resulting in a constant RC-time product that prevails in both health and disease [8][9][10][11][12][13][14][15][16][17][18][19][20][21]. A constant RC-time product has important implications for the assessment of RV afterload, namely that PVR and total arterial compliance are redundant measurements and knowledge of one enables the derivation of the other.…”

Section: Introductionmentioning

confidence: 99%

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Pulmonary vascular resistance and compliance relationship in pulmonary hypertension

et al. 2015

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Right ventricular adaptation to the increased pulmonary arterial load is a key determinant of outcomes in pulmonary hypertension (PH). Pulmonary vascular resistance (PVR) and total arterial compliance (C) quantify resistive and elastic properties of pulmonary arteries that modulate the steady and pulsatile components of pulmonary arterial load, respectively. PVR is commonly calculated as transpulmonary pressure gradient over pulmonary flow and total arterial compliance as stroke volume over pulmonary arterial pulse pressure (SV/PApp). Assuming that there is an inverse, hyperbolic relationship between PVR and C, recent studies have popularised the concept that their product (RC-time of the pulmonary circulation, in seconds) is "constant" in health and diseases. However, emerging evidence suggests that this concept should be challenged, with shortened RC-times documented in post-capillary PH and normotensive subjects. Furthermore, reported RC-times in the literature have consistently demonstrated significant scatter around the mean. In precapillary PH, the true PVR can be overestimated if one uses the standard PVR equation because the zero-flow pressure may be significantly higher than pulmonary arterial wedge pressure. Furthermore, SV/PApp may also overestimate true C. Further studies are needed to clarify some of the inconsistencies of pulmonary RC-time, as this has major implications for our understanding of the arterial load in diseases of the pulmonary circulation. @ERSpublications Empiric estimates of pulmonary arterial load are prone to errors and may result in overestimation of RC-time

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Section: Rc-time: a Critical Evaluation And New Proposalmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Pulmonary vascular resistance and compliance relationship in pulmonary hypertension

et al. 2015

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“…При ССД повышение СДЛА связано с поражением легоч-ных артерий, а также сердца и лег-ких. В связи с этим интересно отме-тить, что при сравнении больных ле-гочной артериальной гипертензией (ЛАГ), ассоциированной с ССД, и пациентов с идиопатической ЛАГ дисфункция правого желудочка была более выражена при ССД [24].…”

Section: параметр врс число больных N (%)unclassified

Structural and Functional Changes Evaluated by Echocardiography in Patients With Systemic Sclerosis and Heart Rate Variability

Saad¹,

Ананьева²,

Алекперов³

et al. 2017

rsp

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“…41 In patients with idiopathic PAH, RV-arterial coupling may be preserved with no increase in RV volume for a while, but the presence of systemic disease, such as systemic sclerosis, may be a cause of earlier RV failure. 18 The determinants of long-term preservation of RV-arterial coupling in patients with severe pulmonary hypertension or a systemic RV are not known. The pathobiologic events leading to RV-arterial uncoupling and increased RV volumes remain to be identified.…”

Section: Coupling Of Systolic Function To Afterloadmentioning

confidence: 99%

“…The E es is best determined by interpolation of pressure-volume coordinates, 54 with further tightening by a correction for EDV. 9,18 Further uncer- Figure 5. Decreased right ventricular (RV) maximum elastance/ arterial elastance (E max /E a ) ratio correlated with the Bax/Bcl2 ratio indicating activation of apoptosis as a universal mechanism in models of acute or chronic RV failure.…”

Section: Pressure Measurementsmentioning

confidence: 99%

Biomechanics of the Right Ventricle in Health and Disease (2013 Grover Conference Series)

Naeije¹,

Brimioulle²,

Dewachter³

2014

Pulm. circ.

107

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Right ventricular (RV) function is a major determinant of the symptomatology and outcome in pulmonary hypertension. The normal RV is a thin-walled flow generator able to accommodate large changes in venous return but unable to maintain flow output in the presence of a brisk increase in pulmonary artery pressure. The RV chronically exposed to pulmonary hypertension undergoes hypertrophic changes and an increase in contractility, allowing for preserved flow output in response to peripheral demand. Failure of systolic function adaptation (homeometric adaptation, described by Anrep's law of the heart) results in increased dimensions (heterometric adaptation; Starling's law of the heart), with a negative effect on diastolic ventricular interactions, limitation of exercise capacity, and vascular congestion. Ventricular function is described by pressure-volume relationships. The gold standard of systolic function is maximum elastance (E max ), or the maximal value of the ratio of pressure to volume. This value is not immediately sensitive to changes in loading conditions. The gold standard of afterload is arterial elastance (E a ), defined by the ratio of pressure at E max to stroke volume. The optimal coupling of ventricular function to the arterial circulation occurs at an E max /E a ratio between 1.5 and 2. Patients with severe pulmonary hypertension present with an increased E max , a trend toward decreased E max /E a , and increased RV dimensions, along with progression of the pulmonary vascular disease, systemic factors, and left ventricular function. The molecular mechanisms of RV systolic failure are currently being investigated. It is important to refer biological findings to sound measurements of function. Surrogates for E max and E a are being developed through bedside imaging techniques.Keywords: right ventricle, pulmonary hypertension, preload, afterload, maximum elastance, end-systolic elastance, arterial elastance. One must inquire how increasing pulmonary vascular resistance results in impaired right ventricular function.-J. T. Reeves, 1989 1 In 1988, Jack Reeves and his colleagues noted that pulmonary hypertension is a common complication of cardiac and pulmonary diseases, that associated alterations in right ventricular (RV) function cause symptoms and limit survival, and that, paradoxically, research in this area had been relatively scarce. 1 Accordingly, they called for more studies to improve the understanding of RV failure in health and disease. Twenty-five years later, there has been significant progress, but our present knowledge remains incomplete, and calls for more research have been repeated. 2,3 The right ventricle (RV) in mammals and birds is a thin-walled flow generator, designed to accommodate the entire systemic venous return undergoing gas exchange in the pulmonary circulation, which is a separate high-flow, low-pressure system. 4 The pulmonary vascular pressures at rest and at mild levels of exercise are so low that a mean systemic filling pressure in the range of 10-15 m...

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Right Ventricular Dysfunction in Systemic Sclerosis–Associated Pulmonary Arterial Hypertension

Cited by 242 publications

References 43 publications

Pulmonary vascular resistance and compliance relationship in pulmonary hypertension

Pulmonary vascular resistance and compliance relationship in pulmonary hypertension

Structural and Functional Changes Evaluated by Echocardiography in Patients With Systemic Sclerosis and Heart Rate Variability

Biomechanics of the Right Ventricle in Health and Disease (2013 Grover Conference Series)

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