Abnormal pulmonary vascular responses in patients registered with a systemic autoimmunity database: Pulmonary Hypertension Assessment and Screening Evaluation using stress echocardiography (PHASE-I)

Collins, Nicholas; Bastian, B.; Quiqueree, L.; Jones, Carol; Morgan, Renae; Reeves, Glenn

doi:10.1016/j.euje.2005.12.002

Cited by 72 publications

(48 citation statements)

References 36 publications

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“…It is difficult to treat symptomatic advanced PAH; therefore, early detection of PAH by a sensitive method is important. Annual cardiac echocardiography or exercise echocardiography is recommended for the detection of early-stage PAH (12,13). The present case showed that 6-minute walk stress echocardiography was a sensitive procedure for detection of PAH; therefore, patients at high risk for PAH such as those with SSc should undergo 6-minute walk stress echocardiography not only for evaluation of exercise capacity but also for detection of early-stage PAH, even if their resting pulmonary arterial pressure is normal.…”

Section: Discussionmentioning

confidence: 99%

Bosentan Ameliorated Exercise-Induced Pulmonary Arterial Hypertension Complicated with Systemic Sclerosis

et al. 2010

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Pulmonary arterial hypertension (PAH) is a frequent complication in patients with systemic sclerosis. Bosentan is used in patients with symptomatic PAH; however, it has not been established whether or not bosentan ameliorates the progression of PAH in patients with no PAH-related symptoms.We present a case of systemic sclerosis with no PAH-related symptoms in which bosentan ameliorated exercise-induced PAH evaluated by 6-minute walk stress echocardiography, brachial flow-mediated dilation, and skin temperature of hands and feet. The results suggest that administration of bosentan in patients with early-stage PAH ameliorates pulmonary arterial vasodilatation through improvement of endothelial function.

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Section: Discussionmentioning

confidence: 99%

Bosentan Ameliorated Exercise-Induced Pulmonary Arterial Hypertension Complicated with Systemic Sclerosis

et al. 2010

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“…Abnormal exercise physiology has been recognized in SSc, idiopathic PAH, idiopathic pulmonary fibrosis (IPF), and chronic obstructive pulmonary disease (21,28,(34)(35)(36)(37)(38)(39)(40). Studies have described exerciseinduced PH in 46-59% of patients with scleroderma spectrum disorders, characterized by Doppler echocardiogram pulmonary artery systolic pressure Ͼ40 mm Hg during exercise (23,41,42). Collectively, these studies suggest an abnormal phenotype in "at-risk" populations after assessing the response of the pulmonary vasculature to exercise.…”

mentioning

confidence: 99%

Exercise‐induced pulmonary hypertension associated with systemic sclerosis: Four distinct entities

Saggar

Khanna

Fürst

et al. 2010

Arthritis & Rheumatism

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Objective. Exercise-induced pulmonary hypertension (PH) may represent an early but clinically relevant phase in the spectrum of pulmonary vascular disease. There are limited data on the prevalence of exerciseinduced PH determined by right heart catheterization in scleroderma spectrum disorders. We undertook this study to describe the hemodynamic response to exercise in a homogeneous population of patients with scleroderma spectrum disorders at risk of developing pulmonary vascular disease. Methods.Patients with normal resting hemodynamics underwent supine lower extremity exercise testing. A classification and regression tree (CART) analysis was used to assess combinations of variables collected during resting right heart catheterization that best predicted abnormal exercise physiology, applicable to each individual subject.Results. Fifty-seven patients who had normal resting hemodynamics underwent subsequent exercise right heart catheterization. Four distinct hemodynamic groups were identified during exercise: a normal group, an exercise-induced pulmonary venous hypertension (ePVH) group, an exercise out of proportion PH (eoPH) group, and an exercise-induced PH (ePH) group. The eoPH and ePVH groups had higher pulmonary capillary wedge pressure (PCWP) than the ePH group (P < 0.05). The normal and ePH groups had exercise PCWP <18 mm Hg, which was lower than that in the ePVH and eoPH groups (P < 0.05). During submaximal exercise, the transpulmonary gradient and pulmonary vascular resistance (PVR) were elevated in the ePH and eoPH groups as compared with the normal and ePVH groups (P < 0.05). CART analysis suggested that resting mean pulmonary artery pressure (mPAP) >14 mm Hg and PVR >160 dynes/seconds/cm ؊5 were associated with eoPH and ePH (positive predictive value 89% for mPAP 14-20 mm Hg and 100% for mPAP >20 mm Hg).Conclusion. We characterized the exercise hemodynamic response in at-risk patients with scleroderma spectrum disorders who did not have resting PH. Four distinct hemodynamic groups were identified during exercise. These groups may have potentially different prognoses and treatment options.

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“…18,19 It has also been used as a screening tool for exerciseinduced pulmonary hypertension in scleroderma 20,21 and in relatives of patients with PAH. 22,23 In addition, Ex-echo has been used in cases chronic thrombo-embolic pulmonary hypertension.…”

Section: Clinical Application Of Exercise Echocardiography In Pulmonamentioning

confidence: 99%

Exercise echocardiography in pulmonary hypertension

Vachiéry

Pavelescu

2007

European Heart Journal Supplements

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Doppler echocardiography plays a key role in the detection of pulmonary hypertension, the leading cause of right ventricular (RV) dysfunction. Reliable estimates of pulmonary pressures and cardiac output (CO) can be obtained at rest, allowing for screening and diagnosis of pulmonary arterial hypertension (PAH), a group of diseases characterized by increased pulmonary vascular resistance progressing to RV failure and death. However, these resting indices poorly correlate with symptoms, exercise response, and have modest prognosis significance. This is partially explained by the limited information obtained from pressure measurements on the severity of RV dysfunction, the major determinant of exercise response and prognosis in PAH. Exercise echocardiography (Ex-echo) may provide significant additional information in the understanding and the management of the disease. In asymptomatic subjects at risk of PAH, it may uncover abnormal increases in pulmonary pressure and CO during exercise, which may be reflecting early stage disease. In addition, the technique may be useful to better understand the physiology of exercise limitation, a typical manifestation of PAH. Several limitations need to be considered when using Ex-echo in the management of PAH, including the absence of a clear consistent definition of normal values, an unknown threshold for abnormal response and the limited quantitative information on RV function provided by simple pressure analysis. Newer echocardiographic techniques such as Tissue-Doppler imaging, and strain and strain rate analysis may provide further insights in the understanding of RV dysfunction. However, technical limitations currently prevent their application to Ex-echo. Clinical studies are underway to better explore the potential role of Ex-echo in the management of PAH.

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Abnormal pulmonary vascular responses in patients registered with a systemic autoimmunity database: Pulmonary Hypertension Assessment and Screening Evaluation using stress echocardiography (PHASE-I)

Cited by 72 publications

References 36 publications

Bosentan Ameliorated Exercise-Induced Pulmonary Arterial Hypertension Complicated with Systemic Sclerosis

Bosentan Ameliorated Exercise-Induced Pulmonary Arterial Hypertension Complicated with Systemic Sclerosis

Exercise‐induced pulmonary hypertension associated with systemic sclerosis: Four distinct entities

Exercise echocardiography in pulmonary hypertension

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