Pulmonary arterial hypertension

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1 doi: /j x O RIGINAL P APER REVEAL Registry: Correlation of Right Heart Catheterization and Echocardiography in Patients With Pulmonary Arterial Hypertension Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vasculature that leads to increased pulmonary vascular resistance (PVR), right heart failure, and death. Currently, PAH is diagnosed by right heart catheterization (RHC); however, because RHC is invasive and often inconvenient for following patient status, noninvasive alternatives are often used to monitor disease progression and response to therapy. 1 Based on reports of a high correlation with invasive measurements, 2 4 Doppler echocardiography (ECHO) is recommended as a tool for early screening and assessment of patients suspected of having PAH. 5,6 However, the lack of correlation between cardiopulmonary hemodynamics estimated by ECHO and measured by RHC in patients with pulmonary hypertension may limit its utility in the definitive diagnosis and follow-up of patients with PAH Hemodynamic parameters estimated by ECHO may be imprecise and highly variable, so that both underestimates and overestimates of pulmonary artery systolic pressure (PASP) are common. 7 9 In the current study, we investigated the correlation between ECHO-estimated right ventricular systolic pressure (RVSP), which corresponds to PASP in the absence of pulmonary valve stenosis, and RHC-measured PASP in patients enrolled in the Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL), the largest registry of patients with PAH assembled to date. Since the calculation of PASP from ECHO data relies on the estimation of mean right atrial Cardiopulmonary hemodynamics are estimated by Doppler echocardiogram (ECHO) and measured by right heart catheterization (RHC) in patients with pulmonary arterial hypertension (PAH). Whether there is a correlation between these measurements is controversial. The authors investigated ECHOandRHCinpatientsenrolledintheRegistry to Evaluate Early and Long-Term PAH Disease Management (REVEAL), a multicenter, observational, US-based study designed to provide current information about patients with PAH. Patients with PAH who had an ECHO and RHC within 12 months of each other were included. Correlation between subsequent ECHO and RHC was also investigated. Of 2967 patients, 2838 were 18 years and older at enrollment and 1883 had an RHCwithin12 monthsofanecho.correlations between ECHO-estimated and RHC-measured pulmonary artery systolic pressures (PASPs) and mean right atrial pressures did not change based on temporal proximity of the two baseline studies, whether they occurred on the same day or were separated by up to 12 months. In contrast, there was little correlation of serial measurements between ECHO and RHC. Although there is good correlation in PASP between ECHO and RHC at baseline, repeat ECHO measurements alone are not sufficient to monitor change in PASP or progression of PAH. Congest Heart Fail. 2011;17: Ó 2011 Wiley Periodicals, Inc. Harrison W. Farber, MD; 1 Aimee J. Foreman, MA; 2 Dave P. Miller, MS; 2 Michael D. McGoon, MD 3 From the Boston University School of Medicine, Boston, MA; 1 ICON Clinical Research, San Francisco, CA; 2 and the Mayo Clinic, Rochester, MN 3 Address for correspondence: Harrison W. Farber, MD, Department of Medicine, Boston University School of Medicine, 715 Albany Street, R-304, Boston, MA hfarber@bu.edu Manuscript received August 9, 2010; accepted October 27, 2010 pressure (mrap), 12 we also investigated the correlation of ECHO-estimated mrap and RHC-measured mrap in patients enrolled in REVEAL. The correlation of RHC and ECHO measurements was estimated for the overall study population during a 12-month period including specific different time intervals between measurements to investigate whether the proximity of the tests was associated with a stronger relationship. Methods Study Design. The design and baseline characteristics of patients enrolled in the REVEAL Registry have been described previously. 13,14 Briefly, REVEAL is a multicenter (54 sites, universityaffiliated and community hospitals), observational, US-based study designed to provide current information about demographics, disease course, and management of approximately 3500 consecutively enrolled patients with World 56 right heart catheterization vs echocardiography in PAH march april 2011

2 Health Organization group I PAH. Patients are followed for 5 years from the time of enrollment, allowing longitudinal comparison of ECHO and RHC data. Study Population. Patients in the REVEAL Registry included newly diagnosed (diagnosis confirmed by RHC within 3 months before enrollment in REVEAL) and previously diagnosed patients (diagnosis confirmed by RHC 3 months before enrollment in REVEAL) with PAH (group I pulmonary hypertension) at the time of enrollment. Eligible patients were required to meet the following hemodynamic criteria: mpap >25 mm Hg at rest or mpap >30 mm Hg with exercise contemporaneous with pulmonary capillary wedge pressure (PCWP) or left ventricular end-diastolic pressure 18 mm Hg; and pulmonary vascular resistance (PVR) 240 dynes sec cm 5. The minimum age at diagnosis was 3 months. Time of diagnosis was definedasthetimeatwhichrhcfirst confirmed the presence of PAH prior to study entry. REVEAL also used a less restrictive PCWP criterion of 18 mm Hg to be inclusive of patients seen in clinical practice. Specific inclusion criteria for this analysis included patients aged 18 years and older at enrollment with an initial ECHO and RHC performed no more than 12 months apart. The analysis of serial assessments included the subset of patients with subsequent ECHO and RHC studies performed within 3 months of each other. Patients with ECHO-estimated mrap >30 mm Hg (n=7) were excluded from the mrap correlation analyses because of concern regarding the accuracy of the data and inability to confirm the value upon querying the source documentation. ECHO and RHC Protocol and Interpretation. ECHOs and RHCs were performed and interpreted according to the practice and protocols of each individual site. As such, the results of REVEAL are based on the clinical reports from each contributing center. Since ECHO-estimated RVSP is equivalent to PASP, the term ECHO-estimated PASP will be used throughout the paper to denote this measure. Statistical Analyses. Correlations between ECHO and RHC measurements were reported for tests performed on the same day, 1 day to <1 month apart, 1 to <3 months apart, 3 to <6 months apart, and 6 to 12 months apart. Due to the skewed nature of the data, the Spearman rank-order correlation was used. The Spearman rank-order correlation is a nonparametric measure of association that does not assume a normal distribution of the data and uses the ranks of the test values to estimate correlation. Correlations closer to 1 and )1 reflect a stronger association between the ECHO and RHC values. P values for the null hypothesis of no correlation were reported, and values <.05 indicate a correlation significantly different from 0. The accuracy of the ECHO measurement in relation to the RHC value was also assessed based on clinically relevant ranges, where ECHOestimated PASP values within 10 mm Hg of RHC-measured PASP were considered accurate and ECHO-estimated mrap values within 5 mm Hg of RHC-measured mrap were considered accurate. Serial changes in PASP and mrap were investigated in patients with follow-up RHC and ECHO assessments performed within 3 months of each other. The relationship between serial changes in RHC-measured PASP and mrap and changes in the corresponding ECHO-estimated PASP and mrap was tested using the Spearman correlation. Sensitivity variants of all analyses were conducted in which obese patients (body mass index [BMI] >34 kg m 2 ) and patients with poor pulmonary function (percent predicted forced expiratory volume in 1 second [% FEV 1 ] <60%) were excluded because of concerns that ECHO data from these individuals may have been suboptimal. Results Patient Characteristics. Atthetimeof this analysis, 2967 patients with PAH were consecutively enrolled in REVEAL. A total of 1883 patients met the analysis criteria of 18 years and older at enrollment and RHC performed within 12 months of an ECHO. Baseline characteristics are detailed in Table I. A total of 420 patients had at least one subsequent RHC performed (median time to next RHC: 13.2 months), and 1201 patients had at least one subsequent ECHO performed (median time to next ECHO: 8.7 months). In the analysis cohort, 307 patients had both a subsequent RHC and ECHO (median time to subsequent RHC, 14.4 months; median time to subsequent ECHO, 13.8 months). Of these, 167 patients had a subsequent RHC within 3 months of an ECHO and were included in the serial analyses (median time to subsequent RHC, 12 months; median time to subsequent ECHO, 12 months). All of these patients also had their initial RHC and ECHO tests within 3 months of each other (Figure 1). Comparison of Initial ECHO and RHC Hemodynamic Parameters. Hemodynamic data from initial RHC and ECHO assessments conducted within 12 months of one another are presented in Table I. RHC-measured PASP and ECHO-estimated PASP were similar for this group ( mm Hg vs mm Hg, respectively) as were RHC-measured mrap and ECHO-estimated mrap ( mm Hg vs mm Hg, respectively). For initial RHC and ECHO assessments separated by no more than 12 months, the Spearman rank correlations were.56 for PASP (P<.001) (Figure 2A) and 0.36 for mrap (P<.001) (Figure 2B). When the accuracy of ECHO was estimated by using RHC as the reference standard, ECHO was considered inaccurate in 57.4% of PASP estimates (>10 mm Hg higher or lower than RHC) (Figure 2A) and in 36.5% of mrap estimates (>5 mm Hg higher or lower than RHC) (Figure 2B). right heart catheterization vs echocardiography in PAH march april

3 Table I. REVEAL Patient Characteristics PARAMETER There was no effect of time on the strength of the relationship between the initial RHC and the initial ECHO. The correlation between initial RHC and initial ECHO parameters was similar whether the tests were performed on the same day, within the same month, or within1to3months,3to6months,or 6 to 12 months (Table II). TOTAL COHORT (N=1883) a Age at PAH diagnosis, meansd, y Age at enrollment, meansd, y Women, No. (%) 1482 (78.7) Newly diagnosed, No. (%) 348 (18.5) Race, No. (%) White 1394 (75.1) Black 238 (12.8) Hispanic 139 (7.5) Other 85 (4.6) Group I PH subgroup, No. (%) Idiopathic PAH 867 (46.0) Familial PAH 63 (3.4) Associated PAH 947 (50.3) Congenital heart disease 174 (9.2) Connective tissue disorder 472 (25.1) Drugs and toxins 97 (5.2) Human immunodeficiency virus 39 (2.1) Portopulmonary hypertension 117 (6.2) Other 48 (2.6) Other b 6 (0.3) NYHA class at enrollment, c No. (%) I 122 (7.1) II 604 (35.0) III 893 (51.8) IV 106 (6.1) NYHA class at diagnostic RHC, No. (%) I 50 (3.6) II 299 (21.7) III 854 (62.0) IV 175 (12.7) Hemodynamics, No. d (meansd) RHC PASP, mm Hg 1841 ( ) mrap, mm Hg 1770 (9.25.7) PVR index, Wood units m ( ) Cardiac index, L min m (2.50.8) mpap, mm Hg 702 ( ) ECHO, No. (meansd) Estimated PASP, mm Hg 1394 ( ) Estimated right atrial pressure, e mm Hg 763 ( ) Abbreviations: ECHO, echocardiography; mpap, mean pulmonary artery pressure; mrap, mean right atrial pressure; NYHA, New York Heart Association; PAH, pulmonary arterial hypertension; PASP, pulmonary artery systolic pressure: PH, pulmonary hypertension; PVR, pulmonary vascular resistance; REVEAL, Registry to Evaluate Early and Long-Term PAH Disease Management; RHC, right heart catheterization; SD, standard deviation. a Includes the subset of patients in REVEAL with RHC within 12 months of ECHO. b Other category includes patients with World Health Organization group I diagnoses of pulmonary capillary hemangiomatosis and pulmonary veno-occlusive disease. c Not all patients had functional class coded at enrollment. No. represents the number of patients for whom data was available. d Hemodynamic data are from the initial RHC and ECHO within 12 months of each other. e Estimated mrap values from ECHO >30 mm Hg were excluded from analysis. Sensitivity Analysis. The sensitivity analysis included 826 patients with a BMI 34 kg m 2 and % FEV 1 60% (Table III). Spearman rank correlations over the full 12-month period were similar to the full analysis population (PASP, 0.60 for sensitivity population vs 0.56 for all patients; mrap, 0.33 for sensitivity population vs 0.36 for all patients). Same-day measurements of RHC-measured and ECHO-estimated PASP were more highly correlated (0.76 vs 0.57) in the sensitivity analysis population (P<.001), while the sameday measurements of RHC-measured mrap vs ECHO-estimated mrap were less correlated (.12 vs.40) in the sensitivity population (P=.62). Correlation of Serial Changes in RHC vs ECHO Hemodynamic Parameters. Of 167 patients in the serial analysis, a total of 61 had both RHCmeasured and ECHO-estimated PASP obtained 3 months apart at the initial and follow-up assessments. The Spearman rank correlation for the change between the two assessments was.43 (P=.001) (Figure 3A). Fewer patients had RHC-measured and ECHO-estimated mrap data obtained 3 months apart available at both the initial and follow-up assessments (n=31). In this group, the change from initial to follow-up assessment was not as well correlated (Spearman correlation, ).34; P=.059) (Figure 3B). Lack of a statistically significant correlation between changes in ECHO-estimated mrap and changes in RHC-measured mrap may be due to the small number of patients. Correlations were even less robust if the time between follow-up ECHO and follow-up RHC was >3 months (data not shown). Sensitivity Analysis. The sensitivity analysis of changes in PASP from initial to follow-up assessment included 30 patients with BMI 34 kg m 2 and % FEV 1 60% in whom RHC and ECHO data were available at both time points. The Spearman rank correlations were similar to the overall sensitivity population (.45; P=.014) (Figure 3C). There were only 18 patients in the analysis population for mrap who had data available from both tests at both time points, and the correlation was impacted by outliers (Spearman correlation, ).51; P=.031) (Figure 3D). Discussion In the REVEAL Registry population of patients with proven PAH, there was 58 right heart catheterization vs echocardiography in PAH march april 2011

4 Figure 1. CONSORT diagram. ECHO indicates echocardiography; RHC, right heart catheterization. correlation between ECHO and RHC parameters regardless of the time interval between them. This suggests a relationship between RHC measurements and ECHO estimations of PASP regardless of the time between assessments in patients with known PAH. A combination of hemodynamic variability and the imprecision of the ECHO introduces a level of random noise that remains constant at all intervals and may obscure any actual hemodynamics changes. Although RHC is considered the standard by which other assessments are calibrated, it is clear that spontaneous physiologic variability may lead to substantial changes in hemodynamic measurements over short periods ECHO inaccuracy may be due to poor Doppler signals, selection of measurement points, and other interpretative factors. Thus, differences between the two in nonsimultaneous measurements cannot be confidently attributed entirely to inaccuracy of one test (ie, ECHO). ECHO-estimated PASP is calculated using the modified Bernoulli equation RVSP = 4V 2 +mrap, in which V = tricuspid regurgitant jet velocity (a reflection of the right ventricular to-right atrial systolic pressure gradient) and mrap is estimated from the diameter and the respirophasic variability of the inferior vena cava during normal respiration. 12 Thus, the PASP estimated by ECHO can be affected by biological fluctuations of the tricuspid regurgitant jet velocity or by incorrect calculations of mrap. This raises the question of whether isolated single measurements of a continuously fluctuating variable, such as PASP, can provide either a truly representative picture of the general hemodynamic status of a patient or an accurate assessment of changes due to disease progression or impact of therapy. In general, other studies comparing ECHO-estimated PASP with RHCdetermined PASP agree with the results of the present analysis. A recent study in patients with connective tissue disease found no significant difference between PASP measured by RHC and that estimated by ECHO during rest or in response to exercise during screening for PAH. 18 The levels of correlation reported here are similar to those previously reported for ECHO-estimated PASP and RHC-measured PASP conducted within 1 hour (correlation=.66; P<.001) and 3 days (correlation=.69; P<.0001). 7,9 Recently, Testani and coworkers 19 reported a large study correlating RHC-measured PASP with ECHO-estimated PASP in 618 patients who had the two assessments within 48 hours of each other. The RHC-measured PASP was set as the reference standard per PAH guidelines. 6 The Pearson correlation coefficient between RHC-measured PASP and ECHO-estimated PASP was 0.52 (P<.001). 19 The correlation of RHC-measured mrap and ECHO-estimated mrap was 0.32 (P<.001), although it was overestimated by a mean of 2.7 mm Hg. When patients whose mrap could not be estimated (originally estimated to be 14 mm Hg) were removed, the correlation increased to.40 (P<.001), which is identical to patients in the REVEAL cohort who had RHC and ECHO performed on the same day. The higher correlation of PASP on the same day among the sensitivity population and the fact that later measurements have a lower level of correlation right heart catheterization vs echocardiography in PAH march april

5 Figure 2. Plot of echocardiographic (ECHO) vs right heart catheterization (RHC) measurements. (A) Plot of ECHO-estimated systolic right ventricular systolic pressure vs RHC-measured pulmonary artery systolic pressure (PASP). Includes patients who had available RHC data within 12 months of ECHO (1360 of 1883). Spearman correlation,.56; P<.001. (B) Plot of ECHO vs RHC mean right atrial pressure (mrap) measurements. Includes patients who had available RHC data within 12 months of ECHO (721 of 1883). Spearman correlation,.36; P<.001. Similar results occurred when comparisons were restricted to ECHO and RHC on the same day, within 1 month of each other, andwithin1to3months,3to6months,or6to12 months of each other (data not shown). than those taken the same day suggests that variations in measurement and real changes over time have an impact on the results obtained. In the REVEAL Registry, ECHO overestimation (5 mm Hg) of mrap is more prevalent, whereas overestimation or underestimation ( or 10 mm Hg) of PASP is equally distributed. Compared with previously reported discrepancies in measurement of PASP between ECHO and RHCs conducted within 1 hour (48%) and 3 days (52%) of each other, 7,9 the proportions of discrepant PASP from same-day ECHO and RHCs are higher (60%) in the REVEAL population. In the Testani and coworkers investigation, 75.4% were discrepant by at least 10% (65% overestimation and 35% underestimation), and 54.4% were discrepant by at least 20% (72% overestimation and 28% underestimation), 19 while in the exercise study performed in patients with connective tissue disease, ECHO underestimated PASP in 37% of patients during maximum exercise. 18 Among the 1364 patients in the REVEAL cohort who had available data for both RHC-measured PASP and ECHO-measured PASP, 69.8% had ECHO PASP values that were discrepant by at least 10%, and 44.4% were discrepant by at least 20% (in both cases, evenly divided between overestimation and underestimation). Variability in the proportion of overestimation and underestimation in these studies may be due to differences in study populations. A recent multivariable analysis of the REVEAL population has shown that a prognostic equation comprising PAH subtype, functional class, age, sex, renal 60 right heart catheterization vs echocardiography in PAH march april 2011

6 Table II. Correlation of Time Between Initial RHC and ECHO a SAME DAY 1DAY TO 1MONTH 1 TO 3MONTHS 3 TO 6MONTHS 6 TO 12 MONTHS PASP vs estimated PASP, mm Hg n=98 n=481 n=280 n=238 n=267 Spearman correlation P value <.001 <.001 <.001 <.001 <.001 Percent of ECHOs accurate within 10 mm Hg, No. (%) ECHO underestimates 28 (28.6) 121 (25.2) 84 (30.0) 84 (35.3) 93 (34.8) ECHO within 10 mm Hg of RHC 39 (39.8) 209 (43.5) 124 (44.3) 94 (39.5) 114 (42.7) ECHO overestimates 31 (31.6) 151 (31.4) 72 (25.7) 60 (25.2) 60 (22.5) mrap vs estimated mrap, b mm Hg, No Spearman s correlation P value.018 <.001 <.001 < Percent of ECHOs accurate within 5 mm Hg, No. (%) ECHO underestimates 4 (11.4) 33 (11.5) 13 (9.4) 11 (8.9) 17 (12.4) ECHO within 5 mm Hg of RHC 22 (62.9) 185 (64.2) 91 (65.9) 76 (61.8) 84 (61.3) ECHO overestimates 9 (25.7) 70 (24.3) 34 (24.6) 36 (29.3) 36 (26.3) Abbreviations: ECHO, echocardiography; mrap, mean right atrial pressure; PASP, pulmonary artery systolic pressure; RHC, right heart catheterization. a Among a total of 1883 patients with RHC within 12 months of ECHO. b Estimated mrap values >30 mm Hg have been excluded from this analysis. Table III. Correlation of Time Between Initial RHC and ECHO in the Subset of Patients With BMI 34 kg m 2 and Percent Predicted FEV 1 60% a SAME DAY 1DAY TO 1MONTH 1 TO 3MONTHS 3 TO 6MONTHS 6 TO 12 MONTHS PASP vs estimated PASP, mm Hg, No Spearman correlation P value <.001 <.001 <.001 <.001 <.001 Percent of ECHOs accurate within 10 mm Hg, No. (%) ECHO underestimates 12 (23.5) 46 (20.8) 31 (24.2) 38 (32.2) 33 (28.7) ECHO within 10 mm Hg of RHC 24 (47.1) 97 (43.9) 67 (52.3) 50 (42.4) 55 (47.8) ECHO overestimates 15 (29.4) 78 (35.3) 30 (23.4) 30 (25.4) 27 (23.5) mrap vs estimated mrap, b mm Hg, No Spearman s correlation P value.62 < < Percent of ECHOs accurate within 5 mm Hg, No. (%) ECHO underestimates 0 (0.0) 17 (12.8) 3 (5.0) 4 (6.6) 5 (8.9) ECHO within 5 mm Hg of RHC 14 (73.7) 75 (56.4) 40 (66.7) 38 (62.3) 36 (64.3) ECHO overestimates 5 (26.3) 41 (30.8) 17 (28.3) 19 (31.2) 15 (26.8) Abbreviations: BMI, body mass index; ECHO, echocardiography; % FEV 1, percent of forced expiratory volume in 1 second; PASP, pulmonary artery systolic pressure; RHC, right heart catheterization. a Among a total of 826 patients who are neither obese (BMI >34 kg m 2 ) nor have poor pulmonary function (% FEV 1 <60%), with RHC within 12 months of ECHO. b Estimated mrap values >30 mm Hg have been excluded from this analysis. status, 6-minute walk test, brain natriuretic peptide, percent predicted carbon monoxide diffusing capacity, and pericardial effusion status can predict mortality in patients with PAH. 20 This suggests that an accurate assessment of PAH disease progression and prognosis must take into account the multitude of assessments available to the clinician. The lack of correlation of serial ECHO and RHC parameters in this report supports this, as well as underlines the importance of not relying on a single test when assessing patients over time, particularly when using that test to monitor a clinically relevant change such as clinical status or disease progression. Limitations The majority of patients in REVEAL take PAH medications that may be altered at any time at the discretion of the physician. Not all patients in REVEAL routinely undergo an ECHO, and the number of patients with both ECHO and RHC values for comparison is small. For those patients who do have available data, not all ECHOs were conducted or reviewed at a centralized specialty center. Although PASP is estimated from ECHO by a standard technique (modified Bernoulli equation), 4 estimated mrap from ECHO may vary, dependent on the operator and the method used to estimate it. Nevertheless, when methodology is more rigorously standardized, similar discrepancies are observed, 19 suggesting that use of a specialized center is not always required to obtain accurate ECHOderived hemodynamic measurements. There is considerable debate about the amount of variability to allow before considering a reading unreliable, and lack of right heart catheterization vs echocardiography in PAH march april

7 Figure 3. Correlation of changes in right heart catheterization (RHC) and changes in echocardiography (ECHO). (A) Correlation of changes in RHC-measured pulmonary artery systolic pressure (PASP) vs changes in ECHO-estimated PASP. Spearman correlation,.43; P=.001. Includes patients with initial RHC 12 months from initial ECHO and follow-up RHC within 3 months of follow-up ECHO and available pulmonary artery pressure (PAP) data (N=61). (B) Correlation of changes in RHC-measured mean right atrial pressure (mrap) vs changes in ECHO-estimated mrap. Spearman correlation, ).34; P=.059. Includes patients with initial RHC 12 months from initial ECHO and follow-up RHC within 3 months of follow-up ECHO and available mrap data (N=31). (C) Correlation of changes in RHC-measured PASP vs changes in ECHO-estimated PASP, sensitivity analysis cohort (body mass index [BMI] 34 kg m 2 and percent predicted forced expiratory volume in 1 second [FEV 1 ] 60%). Spearman correlation,.45; P=.014. Includes patients with initial RHC 12 months from initial ECHO and follow-up RHC within 3 months of follow-up ECHO and available PAP data (N=30). (D) Correlation of changes in RHCmeasured mrap vs changes in ECHO-estimated mrap, sensitivity analysis cohort (BMI 34 kg m 2 and percent predicted FEV 1 60%). Spearman correlation, ).51; P=.031. Includes patients with initial RHC 12 months from initial ECHO and follow-up RHC within 3 months of follow-up ECHO and available mrap data (N=18). correlation may be attributed to temporal, biological, or technical variations, including reproducibility of ECHO and RHC and technician variability. Conclusions The REVEAL Registry was undertaken to evaluate the modern population of patients with PAH and to assess current clinical practices. While RHC remains the gold standard test for determining PASP, the invasive nature of the procedure is a disadvantage. ECHO is a noninvasive test capable of determining cardiopulmonary hemodynamics, but doubt remains as to its accuracy when compared with RHC. Our results suggest that ECHO is a reasonable proxy for RHC at a single time point, but may not sufficiently correspond with RHC data to permit precise measurement of serial changes in pulmonary hemodynamics. The different results from serial assessment of the two tests, however, may offer complementary findings that are needed to develop a full clinical picture. Thus, the use of these tests in follow-up assessments depends on the clinical context and type of information that is needed. If a definitive 62 right heart catheterization vs echocardiography in PAH march april 2011

8 measurement of pulmonary hemodynamics at a specific moment in time is needed, then RHC is warranted. On the other hand, if a global impression of pulmonary hemodynamics, right and left ventricular function and morphology, and valvular function and morphology is required, then a carefully performed and interpreted ECHO may be appropriate. Disclosures: The REVEAL Registry is supported by Actelion Pharmaceuticals US, Inc. Harrison W. Farber, MD, serves as a consultant for Actelion, Gilead, and United Therapeutics and is on the speakers bureau for Actelion and Gilead. Dr Farber has received a research grant from United Therapeutics and has received honoraria for his service on the REVEAL steering committee, which is supported by Actelion. Aimee J. Foreman, MA, is employed by ICON Clinical Research, a company that receives research support from Actelion and other pharmaceutical companies. Michael D. McGoon, MD, serves as a consultant with Actelion, Gilead, Lung Rx, and Medtronic. Dr McGoon has received research grants from Gilead and Medtronic. Dr McGoon has received honoraria for his service on the REVEAL steering committee, which is supported by Actelion. Dave P. Miller, MS, is employed by ICON Clinical Research, a company that receives research support from Actelion and other pharmaceutical companies. The authors wish to thank Jennifer M. Kulak, PhD, of in Science Communications, a Wolters Kluwer business, for editorial support in the development of this manuscript. This support was funded by Actelion Pharmaceuticals US, Inc. REFERENCES 1 Schannwell CM, Steiner S, Strauer BE. Diagnostics in pulmonary hypertension. J Physiol Pharmacol. 2007;58suppl 5 (pt 2): Currie PJ, Seward JB, Chan KL, et al. Continuous wave Doppler determination of right ventricular pressure: a simultaneous Dopplercatheterization study in 127 patients. JAm Coll Cardiol. 1985;6(4): Kitabatake A, Inoue M, Asao M, et al. 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Any queries (other than missing material) should be directed to the corresponding author for the article. right heart catheterization vs echocardiography in PAH march april