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

Abstract

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.

Contribution of Speckle Tracking to Estimation of Pulmonary Hypertension by Standard Doppler Echocardiography in Patients with Systemic Sclerosis and MCTD

“…Therefore, numerous authors have searched for both non-invasive and invasive predictors of pulmonary hypertension such as stress Doppler echocardiography [10] or invasively assessed exercise-induced pulmonary hypertension [11]. The latter could reveal more types of pulmonary hypertension including pulmonary venous hypertension and out-of-proportion PAH [12] and distinguish between exercise-induced PAH and diastolic dysfunction [13] but d invasive character and remaining problems, it DETECT study algorithm [14] provided clinicians with a helpful and evidence-based tool for identification of patients with a higher risk for PAH and indicated for RHC. However, there are some limitations to the use of the DETECT algorithm.…”

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

“…Мы предполагаем, что исходный отбор паци-ентов по этим признакам позволяет выделить группу пациентов, предрасположенных к развитию ЛАГ, с СИ-ЛГ, возможно, являющейся ее доклинической стадией. Поэтому дополнительного разделения на клинические группы, как в ряде работ [7] зарубежных авторов, мы не проводили. Предварительная оценка частоты СИ-ЛГ в группе пациентов с ССД составила чуть более 7% (9 из 124 подвергнутых скрининговому обследованию боль-ных, см.…”

“…В исследовании R. Saggar и со-авт. [7] описаны 4 гемодинамических фенотипа ответа на ФН у пациентов с ССД по сравнению с нормальными по-казателями гемодинамики. G. Kovacs и соавт.…”

“…[7] включены 10 пациентов с СИ-ЛГ. Перед началом терапии после оцен-ки исходных параметров, в том числе гемодинамических, все пациенты наблюдались в течение года.…”

“…R. Saggar и соавт. [7] применили амбризентан в дозе 5 или 10 мг/сут в течение 24 нед у 12 больных ССД с СИ-ЛГ. Отмечалось статистически значимое снижение ЛСС при нагрузке, увеличение расстояния в ТШХ, СрДЛА при ФН.…”