Borderline Pulmonary Arterial Pressure Is Associated with Decreased Exercise Capacity in Scleroderma

Kovàcs, Gabór; Maier, Robert; Aberer, Elisabeth; Brodmann, Marianne; Scheidl, Stefan; Tröster, Natascha; Hesse, Christina; Salmhofer, Wolfgang; Graninger, Winfried; Gruenig, Ekkehard; Rubin, Lewis J.; Olschewski, Horst

doi:10.1164/rccm.200904-0563oc

Cited by 136 publications

(117 citation statements)

References 15 publications

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“…Patients with baseline FVC Յ60% predicted and PCWP Ͼ15 mm Hg were excluded, representing an important difference from our study. A median mPAP Ͼ17 mm Hg at rest and a median mPAP Ͼ23 mm Hg during slight exercise were associated with both a significant decrease in 6MW and peak V O 2 (38). The PVR from baseline to slight exercise was relatively unchanged, however, similar to our experience, suggesting that a PVR Ն160 dynes/seconds/cm Ϫ5 at rest was potentially abnormal.…”

supporting

confidence: 83%

“…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).…”

mentioning

confidence: 99%

“…Few studies have used right heart catheterization to address whether an mPAP Ͼ30 mm Hg during exercise represents an aberrant pulmonary vascular system in "at-risk" populations (19,21,28,34,38,39,43). Recently, Tolle and coworkers evaluated a large cohort of non-SSc symptomatic patients who underwent simultaneous cardiopulmonary exercise testing and right heart catheterization (21).…”

mentioning

confidence: 99%

“…More recently, Kovacs and coworkers described the association of resting and exercise mPAP with exercise capacity (6-minute walk [6MW] and peak V O 2 ) in a well-characterized SSc cohort (38). Patients with baseline FVC Յ60% predicted and PCWP Ͼ15 mm Hg were excluded, representing an important difference from our study.…”

mentioning

confidence: 99%

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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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confidence: 83%

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confidence: 99%

mentioning

confidence: 99%

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confidence: 99%

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

Saggar

Khanna

Fürst

et al. 2010

Arthritis & Rheumatism

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“…Пред-полагается, что параметры гемодинамики, в том числе показатель нормального СрДЛА, меняются с возрастом. В систематическом обзоре доступной литературы [3] не обнаружено существенных различий по СрДЛА в покое в разных возрастных группах, однако во время ФН СрДЛА оказалось значительно выше у пациентов старше 50 лет. Основываясь на этих данных, рабочая группа 4-го Симпо-зиума в Dana Point пришла к заключению, что определе-ние универсального пограничного уровня СрДЛА при ФН недостаточно и рекомендовала не использовать этот кри-терий в клинической практике как недостаточно изучен-ный [4].…”

Section: терапевтический архив 1 2015unclassified

Impact of bosentan therapy on stress-induced pulmonary hypertension in patients with scleroderma systematica

et al. 2015

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Diagnosis, Treatment, and Clinical Management of Pulmonary Arterial Hypertension in the Contemporary Era

2016

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Importance Pulmonary arterial hypertension (PAH) is characterized by severe remodeling of distal pulmonary arteries, increased pulmonary vascular resistance and right ventricular dysfunction that promotes heart failure. Once regarded as largely untreatable, evidence-based decision-making now guides clinical management of patients and improves PAH outcome. Yet, misconceptions regarding the approach to PAH in the modern era are common and associated with substandard clinical care. Observations The clinical profile of PAH has changed substantially since its original description: the age at diagnosis is older than previously reported, disease severity appears greater in men compared to women, and PAH in association with connective tissue disease is identified as a particularly high risk patient subgroup. Risk stratification scales for PAH are now available at point of care, which inform treatment goals including: 6-minute walk distance >440 m, peak VO2 >15 ml/min/kg, right atrial area <18 cm2, cardiac index >2.5 l/min/m2, and absent/low symptom burden with routine physical activity. Currently, 14 therapies targeting six PAH-specific molecular intermediaries are in use clinically. Recent landmark trial data have demonstrated the critical importance of initial combination therapy in treatment-naïve patients. Taken together, these findings underscore a global shift in PAH coupling early disease detection with aggressive pharmacotherapy. Indeed, recent longitudinal data from combination therapy patients shows that the 3-year survival rate in PAH may be as high as 84% compared with 48% from the original National Institutes of Health registry on idiopathic PAH (1980-1985). Despite these gains, incomplete clinical evaluation and misdiagnosis by referring practitioners is common and associated with inappropriate therapy. Conclusions and Relevance Compared to the original clinical experience, PAH has evolved into a contemporary and treatable disease characterized by improved survival and a high standard for defining therapeutic success. However, under-awareness among clinicians regarding the importance of early and accurate PAH diagnosis persists and is a potentially reversible cause of adverse outcome in this disease.

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Borderline Pulmonary Arterial Pressure Is Associated with Decreased Exercise Capacity in Scleroderma

Cited by 136 publications

References 15 publications

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

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

Impact of bosentan therapy on stress-induced pulmonary hypertension in patients with scleroderma systematica

Diagnosis, Treatment, and Clinical Management of Pulmonary Arterial Hypertension in the Contemporary Era

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