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Welcome to the Q4 2010 Quarterly Update,
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Scleroderma and Pulmonary Arterial Hypertension (PAH):
Focus on Early Diagnosis
About MD Primer Inc:
MD Primer Inc. (MDP) was created by physicians for physicians with a mission to provide our colleagues with timely and practical access to key information, knowledge and clinical tools for optimal patient management.
MDP is a fully integrated academic research organization with a primary focus on conducting evidence-based research and promoting guideline-mandated approaches to patient management. MDP is actively involved in the design and execution of Phase IIB, III and IV clinical trials as well as outcomes-based patient Registries.
MDP has a significant interest in the area of Pulmonary Hypertension and Pulmonary Arterial Hypertension (PAH) in particular.
Our fourth quarter installment entitled “Scleroderma and Pulmonary Arterial Hypertension (PAH): Focus on Early Diagnosis” is intended not only for clinicians managing patients with cardio-pulmonary disease but also those managing patients at risk for PAH, for example patients with scleroderma or other connective tissue diseases.
We hope that the Q4 Update is of interest and value to you.
Strategies are needed for early recognition of pulmonary arterial hypertension (PAH) in
patients with systemic sclerosis (SSc, scleroderma). Current modalities include history and physical exam, pulmonary function testing, biomarkers of myocardial stress (NTproBNP) and echocardiography with Doppler for an estimate of right ventricular systolic pressure, size, and function. None of these modalities have been fully validated and few assessments have been made of a potential “care gap” between specialty centers and primary care settings.
The need for early recognition of PAH in scleroderma patients relates in part to the poor prognosis observed once the diagnosis is made and therefore the need for as early as possible an intervention (1).
The poor outcome in scleroderma patients has recently been confirmed in the PAH QuERI and reported at the American College of Rheumatology Meeting (ACR) (2).
These results are very similar to those recently published from the REVEAL registry where the mortality in the IPAH group was 93% and in the CTD-PAH group 86% (3).
The UNCOVER study (4) evaluated a total of 909 patients with either scleroderma
or mixed connective tissue disease (MCTD) among the 50 community rheumatology practices. Of 909 screened patients, 791 were evaluable and completed the study; and 669 have not been previously studied for PAH and thus represented an interesting opportunity for the early detection study. Of these 669 patients, 89 (13.3%) were found by Doppler echocardiography to have an ERVSP of >40 mm Hg. Interestingly, among the 178 patients in the Scleroderma QuERI reported this month at the ACR Meeting (5), the baseline echocardiography revealed a median RVSP of 30 mm Hg and 12% had RVSP >40 mm Hg. Therefore; our more contemporary results support the original findings of the UNCOVER.
Importantly, in UNCOVER the total prevalence of PAH was 26.7% (211 of 791 patients, including 122 with known PAH and 89 newly diagnosed as having PAH). Doppler echocardiographic data showed 20 of 89 patients (22.5%) with ERVSP of >50
mm Hg, 20 of 89 patients (22.5%) with increased RV dimension, and 25 of 89 patients (28.1%) with right atrial enlargement. Patients with ERVSP >40 mm Hg had decreased exercise tolerance compared with those with ERVSP <40 mm Hg (27% compared with 9.5%, respectively, were severely symptomatic).
These findings are supported by our observations that doppler echocardiography is thought to be a robust tool for screening for PAH in patients with systemic sclerosis in a research setting and in specialized programs. However, our data derived from community practices also suggests that RVSP/PASP and/or tricuspid jet velocity measures are inconsistently reported. This creates an opportunity to improve early diagnosis and therefore earlier treatment in patients at risk.
The important issue of echocardiography based early diagnosis was the subject of another abstract presented at the American College of Chest Physicians (ACCP) meeting (6). The abstract focused on a retrospective study in which the authors looked at reports from 3960 2-D echocardiograms performed at the
Cincinnati VAMC in 2005 and 2006. All patients without known PH, an estimated pulmonary artery (PA) pressure > 40 mm Hg and an ejection fraction > 50% were included. Interestingly, 5.8% of patients met the inclusion criteria for PH based on elevated PA pressures. The authors went on to show that only patients with the more severely elevated pulmonary pressures and those with evidence of right heart pressure overload were being referred.
In our evaluation of the scleroderma patients without prior diagnosis of PAH and of whom 178 underwent echocardiography, 31 patients (17.4%) were thought to have findings consistent with PAH (5). This number is larger than the 12% mentioned above (i.e. only those with RVSP >40 mm Hg) and is based on additional considerations such as RV enlargement, right ventricular hypertrophy, evidence of septal flattening, and presence of the pericardial effusion.
Finally and most importantly, of the total of 207 patients enrolled in the Scleroderma QuERI, 190 patients (92%) had at least one follow-up echocardiogram conducted during the observation period of three years. However, only 155 (82%) of these 190 patients had an RVSP or PASP value recorded (either at baseline, n = 124 or follow-up, n = 31) and 35 had no values reported for either visit.
In those 155 patients with RVSP or PASP recorded at any time through the duration of the study, the estimated RVSP/PASP value was greater than the a priori threshold of 40 mm Hg in 36 patients (23%), and of those patients 10 had RHC, 10 had only echocardiography repeated, and 16 patients had neither test performed through the duration of the study.
During the follow up period, the mortality/hospitalization rate was 36% among patients with a RVSP/PASP >40 mm Hg versus 13% in patients with a RVSP/PASP ≤40 mm Hg and 14% in patients in whom RVSP/PASP was not reported (P = 0.007).
These findings highlight the importance of echocardiography as a screening tool and a prognostic indicator when the evidence of pulmonary hypertension is detected.
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