Assessment of Survival in Patients With Primary Pulmonary Hypertension Importance of Cardiopulmonary Exercise Testing (original) (raw)

Assessment of Survival in Patients With Primary Pulmonary Hypertension

Circulation, 2002

Background — Primary pulmonary hypertension (PPH) is a life-threatening disease. Prognostic assessment is an important factor in determining medical treatment and lung transplantation. Whether cardiopulmonary exercise testing data predict survival has not been reported previously. Methods and Results — We studied 86 patients with PPH (58 female, age 46±2 years, median NYHA class III) between 1996 and 2001 who were followed up in a tertiary referral center. Right heart catheterization was performed and serum uric acid levels were measured in all patients. Seventy patients were able to undergo exercise testing. At the start of the study, the average pulmonary artery pressure was 60±2 mm Hg, average pulmonary vascular resistance was 1664±81 dyne · s · cm −5 , average serum uric acid level was 7.5±0.35 mg/dL, and average peak oxygen uptake during exercise (peak V̇ o 2 ) was 11.2±0.5 mL · kg −1 · min −1 . During follow-up (mean: 567±48 days), 28 patients died and 16 underwent lung transp...

Exercise Testing to Estimate Survival in Pulmonary Hypertension

Medicine & Science in Sports & Exercise, 2008

Background: The 6-min walk distance (6MWD) predicts survival in pulmonary hypertension (PH). The peak oxygen consumption (V O 2peak ) measured during a cardiopulmonary exercise test (CPET) also relates to survival in PH, and it is unknown how the prognostic information from measurements of ventilatory responses and gas exchange during CPET compares to the prognostic information obtained by the 6MWD alone. The aims of our study were to compare prognostic values of different exercise parameters in PH and to assess whether CPET adds prognostic value to the information from the 6MWD. Methods: After baseline right-heart catheterization and exercise testing, survival was assessed in a cohort of 115 PH patients. Results: During the 4 yr of follow-up, 18 patients died. At baseline, pulmonary arterial pressure was 49 T 17 mm Hg, the slope relating minute ventilation to carbon dioxide output (V E /V CO 2slope ) = 45 T 11, V O 2peak = 15 T 6 mLIkg j1 Imin j1 , increase in O 2 pulse from rest to peak exercise ($O 2 pulse) = 5 T 2 mLIbeat j1 , and 6MWD = 445 T 128 m. For the prediction of mortality, the areas under the receiver operating curves were very similar for the different parameters and ranged from 0.69 to 0.74. Patients with a V E /V CO 2slope G 48, V O 2peak 9 13.2 mLIkg j1 Imin j1 , O2pulse93.3mLIbeatj1,ora6MWD9399mhadahighercumulativesurvival(PG0.05).MultivariableCoxregressionwithaforwardselectionprocedureshowedthatonlyO 2 pulse 9 3.3 mLIbeat j1 , or a 6MWD 9 399 m had a higher cumulative survival (P G 0.05). Multivariable Cox regression with a forward selection procedure showed that only O2pulse93.3mLIbeatj1,ora6MWD9399mhadahighercumulativesurvival(PG0.05).MultivariableCoxregressionwithaforwardselectionprocedureshowedthatonlyO 2 pulse improved the univariate 6MWD prediction model significantly (P G 0.05). Conclusion: CPET parameters predict survival in PH patients and add marginally to the prognostic value of the 6MWD.

Prognostic Relevance of Changes in Exercise Test Variables in Pulmonary Arterial Hypertension

PLoS ONE, 2013

Introduction: Exercise variables determined in patients with pulmonary arterial hypertension (PAH) at the time of diagnosis, predict survival. It is unknown whether upon treatment, subsequent changes in these exercise variables reflect improvements in survival. The aim of this study was to determine changes in exercise variables in PAH patients and to relate these changes to survival. Methods: Baseline cardiopulmonary exercise test (CPET) variables and six-minute-walk-distance (6MWD) were available from 65 idiopathic PAH patients (50 females; mean age 45±2yrs). The same variables were determined after treatment (13months) in a sub group of 43 patients. To estimate the association between changes in exercise variables and changes in cardiac function, right-ventricle ejection fraction (RVEF) was measured by cardiac MRI at baseline and after treatment in 34 patients. Mean follow-up time after the second CPET was 53 (range: 4-111) months. Kaplan-Meier analysis was used to relate survival to baseline and treatment-associated changes in exercise variables. Results: Survivors showed a significantly greater change in maximal oxygen uptake than non-survivors and this change in aerobic capacity was significantly related to changes in RVEF. From baseline until the end of the study period, two patients underwent a lung transplantation and 19 patients died. Survival analysis showed that baseline 6MWD (p<0.0001), maximal heart rate (p<0.0001) and the slope relating ventilation with carbon dioxide production (p≤0.05) were significant predictors of survival, whereas baseline oxygen uptake and oxygen pulse held no predictive value. Treatment associated changes in 6MWD (p<0.01), maximal heart rate (p<0.05), oxygen uptake (p<0.001) and oxygen pulse predicted survival (p<0.05), whereas changes in the slope relating ventilation with carbon dioxide production did not. Conclusion: Exercise variables with prognostic significance when determined at baseline, retain their prognostic relevance after treatment. However, when changes in exercise variables upon treatment are considered, a different set of variables provides prognostic information.

Prognostic value of exercise pulmonary haemodynamics in pulmonary arterial hypertension

European Respiratory Journal, 2014

The aim of the study was to investigate the prognostic value of right heart catheterisation variables measured during exercise. 55 incident patients with idiopathic, familial or anorexigen-associated pulmonary arterial hypertension (PAH) underwent right heart catheterisation at rest and during exercise and 6-min walk testing before PAH treatment initiation. Patients were treated according to recommendations within the next 2 weeks. Right heart catheterisation was repeated 3-5 months into the PAH treatment in 20 patients. Exercise cardiac index decreased gradually as New York Heart Association (NYHA) functional class increased whereas cardiac index at rest was not significantly different across NYHA groups. Baseline 6-min walk distance correlated significantly with exercise and change in cardiac index from rest to exercise (r50.414 and r50.481, respectively; p,0.01). Change in 6-min walk distance from baseline to 3-5 months under PAH treatment was highly correlated with change in exercise cardiac index (r50.746, p,0.001). The most significant baseline covariates associated with survival were change in systolic pulmonary artery pressure from rest to exercise and exercise cardiac index (hazard ratio 0.56 (95% CI 0.37-0.86) and 0.14 (95% CI 0.05-0.43), respectively). Change in pulmonary haemodynamics during exercise is an important tool for assessing disease severity and may help devise optimal treat-to-target strategies.

Survival in Primary Pulmonary Hypertension

Circulation, 2002

Background-Primary pulmonary hypertension (PPH) is a severe and progressive disease. Without treatment, the median survival is 2.8 years, with survival rates of 68%, 48%, and 34% at 1, 3, and 5 years, respectively. Intravenous epoprostenol was the first Food and Drug Administration-approved therapy for PPH. The long-term impact that epoprostenol has made on PPH remains to be defined. Methods and Results-One hundred sixty-two consecutive patients diagnosed with PPH and treated with epoprostenol were followed for a mean of 36.3 months (median, 31 months). Data including functional class, exercise tolerance, and hemodynamics were recorded in a customized database. Vital status was verified in each patient. Observed survival with epoprostenol therapy at 1, 2, and 3 years was 87.8%, 76.3%, and 62.8% and was significantly greater than the expected survival of 58.9%, 46.3%, and 35.4% based on historical data. Baseline predictors of survival included exercise tolerance, functional class, right atrial pressure, and vasodilator response to adenosine. Predictors of survival after the first year of therapy included functional class and improvement in exercise tolerance, cardiac index, and mean pulmonary artery pressure.

Haemodynamics, exercise capacity and clinical events in pulmonary arterial hypertension

European Respiratory Journal, 2012

The purpose of this study was to clarify whether changes in cardiopulmonary haemodynamics induced by pharmacological therapy correlate with exercise capacity and clinical events in patients with pulmonary arterial hypertension. 16 randomised trials including 2353 patients, followed up for 16.4¡10.6 weeks, measuring cardiopulmonary haemodynamics by right heart catheterisation and reporting clinical events were included. Meta-analysis and meta-regression analysis were performed to assess the effects of treatments on clinical events and the relationship between haemodynamic changes (pulmonary artery pressure, pulmonary vascular resistance, cardiac index and right atrial pressure) and clinical events. Treatments significantly reduced all-cause death (OR 0.5, 95% CI 0.3-0.7; p,0.01), hospitalisation for pulmonary arterial hypertension (OR 0.4, 95% CI 0.2-0.7; p,0.01), initiation of rescue therapy (OR 0.3, 95% CI 0.2-0.6; p,0.01) and the composite outcome (OR 0.3, 95% CI 0.3-0.5; p,0.01). No relationship was found between changes of haemodynamic parameters and clinical events, whereas changes of cardiac index and pulmonary vascular resistance significantly correlated with changes in the 6-min walking distance (r50.64, p50.03; r5-0.55, p50.04, respectively). In patients with pulmonary arterial hypertension, improvements of cardiopulmonary haemodynamics observed in randomised clinical trials correlate with exercise capacity changes but do not predict clinical events in a short-term follow-up. @ERSpublications In PAH, cardiopulmonary haemodynamic improvements correlate with exercise capacity changes but not with clinical events http://ow.ly/lzTdC This article has supplementary material available from www.erj.ersjournals.com

Right Atrial Pressure During Exercise Predicts Survival in Patients With Pulmonary Hypertension

Journal of the American Heart Association, 2020

Background We investigated changes in right atrial pressure (RAP) during exercise and their prognostic significance in patients assessed for pulmonary hypertension (PH). Methods and Results Consecutive right heart catheterization data, including RAP recorded during supine, stepwise cycle exercise in 270 patients evaluated for PH, were analyzed retrospectively and compared among groups of patients with PH (mean pulmonary artery pressure [mPAP] 25 mm Hg), exercise-induced PH (exPH; resting mPAP <25 mm Hg, exercise mPAP >30 mm Hg, and mPAP/cardiac output >3 Wood Units (WU)), and without PH (noPH). We investigated RAP changes during exercise and survival over a median (quartiles) observation period of 3.7 (2.8-5.6) years. In 152 patients with PH, 58 with exPH, and 60 with noPH, median (quartiles) resting RAP was 8 (6-11), 6 (4-8), and 6 (4-8) mm Hg (P<0.005 for noPH and exPH versus PH). Corresponding peak changes (95% CI) in RAP during exercise were 5 (4-6), 3 (2-4), and-1 (-2 to 0) mm Hg (noPH versus PH P<0.001, noPH versus exPH P=0.027). RAP increase during exercise correlated with mPAP/cardiac output increase (r=0.528, P<0.001). The risk of death or lung transplantation was higher in patients with exercise-induced RAP increase (hazard ratio, 4.24; 95% CI, 1.69-10.64; P=0.002) compared with patients with unaltered or decreasing RAP during exercise. Conclusions In patients evaluated for PH, RAP during exercise should not be assumed as constant. RAP increase during exercise, as observed in exPH and PH, reflects hemodynamic impairment and poor prognosis. Therefore, our data suggest that changes in RAP during exercise right heart catheterization are clinically important indexes of the cardiovascular function.

Prognostic factors in pulmonary hypertension

Mædica, 2012

Pulmonary hypertension is a hemodynamic and pathophysiological condition defined as an increase in mean pulmonary pressure more than or equal to 25 mm Hg. Evaluation of pulmonary hypertension severity and prognosis plays a central role in the management of these patients, between diagnosis and therapeutic decision making. The aim of our study was to identify the adverse prognostic factors in patients with pulmonary hypertension and their impact on mortality, quality of life, need for hospitalization and complications during hospitalization. We performed a prospective study that included 553 patients diagnosed with pulmonary hypertension in the Institute of Cardiovascular Diseases of Iasi between 1st November 2008 and 1st July 2011. We analyzed a series of demographic and clinical data, echocardiographic and hemodynamic parameters, which correlated with mortality, WHO functional class, complications and need for hospitalization. We identified as main negative prognostic factors: WHO ...