Pulmonary artery pressure variation in patients with connective tissue disease: 24 hour ambulatory pulmonary artery pressure monitoring

Abstract

Background-The specific contribution of secondary pulmonary hypertension to the morbidity and mortality of patients with underlying lung disease can be diYcult to assess from single measurements of pulmonary artery pressure. We have studied patients with secondary pulmonary hypertension using an ambulatory system for measuring continuous pulmonary artery pressure (PAP). We chose to study patients with connective tissue disease because they represent a group at high risk of pulmonary vascular disease, but with little disturbance of lung function. Methods-Six patients (five with progressive systemic sclerosis and one with systemic lupus erythematosis) were studied. They underwent preliminary cardiopulmonary investigations followed by Doppler echocardiography, right heart catheterisation, and ambulatory pulmonary artery pressure monitoring to measure changes in pressure over a 24 hour period including during a formal exercise test. Results-All patients had pulmonary hypertension as measured by Doppler echocardiography with estimated pulmonary artery systolic pressures of 40-100 mm Hg. Pulmonary function testing revealed virtually normal spirometric values (mean FEV 1 86.9% predicted) but marked reduction in CO gas transfer factor (KCO 57.8% predicted). Exercise responses were impaired with mean VO 2 max 50.6% predicted. Ambulatory PAP monitoring indicated significant changes in pressures with variation in posture and activity throughout 24 hours. Resting PAP did not predict the change in PAP seen on exercise. Conclusion-Conventional methods of assessment of the pulmonary circulation based on single measurements in the supine position may underestimate the stresses faced by the right side of the circulation. This ambulatory system allows monitoring of pulmonary haemodynamics continuously over 24 hours during normal activities of daily living. These measurements may increase our understanding of the contribution made by secondary pulmonary hypertension to the morbidity and mortality of the underlying lung disease. (Thorax 1998;53:857-862)

Pulmonary artery pressure measurement during exercise testing in patients with suspected pulmonary hypertension

“…Again this is not surprising and has been shown previously with fluid filled, pulmonary artery catheters [19] though it has been found that this is not always the case in patients with milder degrees of pulmonary hypertension [9]. Pulmonary artery occlusion pressure was not remeasured after exercise if the resting measurement was normal for the reasons previously discussed.…”

“…The recorder is worn over the patient's shoulder and is lightweight and compact (weighing 650 g and measuring 1883137345 mm) and is described fully elsewhere [9].…”

“…Previously a micro-manometer tipped pulmonary artery catheter has been used to measure PAP in fully ambulant patients, including during a formal cardiopulmonary exercise test [9]. This technique avoids some of the disadvantages mentioned previously and permits accurate pressure recording in exercising patients.…”

VEGF-C mediates cyclic pressure-induced endothelial cell proliferation

“…Moreover, pulsatile pressures in the range of 60/20 mmHg occur at the level of the human pulmonary artery during primary pulmonary hypertension (14) as well as pulmonary hypertension secondary to connective tissue disease (20); these pathological conditions are associated with pulmonary vessel remodeling (10), intimal hyperplasia (21), as well as dysregulated endothelial growth (3) and gene expression (27) associated with formation of plexiform lesions (resulting from either neoplastic or angiogenic endothelial growth) on the vascular wall.…”

Clinical end-points and surrogate markers of pulmonary arterial hypertension in the light of evidence-based treatment

“…Hemodinamik belirteçlerin klinik sonlanımı öngörebileceğini bildiren çalışmalar olduğu gibi, bunun aksini öne sürenler de az değildir. Kawut ve ark.ları IPAH ve bağ dokusu ile ilişkili PAH hastalarında, sağkalımın bağ dokusu ile ilişkili PAH hastalarında daha kötü olmasına rağmen her iki grupta hemodinamik verilerin benzer olduğunu belirtmiştir (35). Miyamoto ve ark.larının yaptığı çalışmada 6DYM ve oPAB arasın-da çok zayıf bir ilişkinin olduğu belirtilmiştir (9).…”