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Stress Doppler echocardiography for early detection of systemic sclerosis-associated pulmonary arterial hypertension.

Nagel C, Henn P, Ehlken N, D'Andrea A, Blank N, Bossone E, Böttger A, Fiehn C, Fischer C, Lorenz HM, Stöckl F, Grünig E, Egenlauf B - Arthritis Res. Ther. (2015)

Bottom Line: In patients with systemic sclerosis (SSc), associated pulmonary arterial hypertension (SSc-APAH) is the leading cause of death.Investigators of RHC were blinded to the results of non-invasive measurements.When a cutoff value for PASP was more than 45 mm Hg during low-dose exercise, SDE missed PH diagnosis in one of the 22 patients with PH and improved sensitivity to 95.2 % (95 % CI 0.81-1.0) but reduced specificity to 84.9 % (95 % CI 0.74-0.93).

View Article: PubMed Central - PubMed

Affiliation: Centre for Pulmonary Hypertension Thoraxclinic, University Hospital Heidelberg, Amalienstr. 5, 69126, Heidelberg, Germany. c.nagel@klinikum-mittelbaden.de.

ABSTRACT

Introduction: In patients with systemic sclerosis (SSc), associated pulmonary arterial hypertension (SSc-APAH) is the leading cause of death. The objective of this prospective screening study was to analyse sensitivity and specificity of stress Doppler echocardiography (SDE) in detecting pulmonary hypertension (PH).

Methods: Pulmonary artery pressures and further parameters of PH were assessed by echocardiography and right heart catheterisation (RHC) at rest and during exercise in patients with SSc. Investigators of RHC were blinded to the results of non-invasive measurements.

Results: Of 76 patients with SSc (64 were female and mean age was 58±14 years), 22 (29 %) had manifest PH confirmed by RHC: four had concomitant left heart diseases, three had lung diseases, and 15 had SSc-APAH. Echocardiography at rest missed PH diagnosis in five of 22 patients with PH when a cutoff value for systolic pulmonary arterial pressure (PASP) was more than 40 mm Hg at rest. The sensitivity of echocardiography at rest was 72.7 % (95 % confidence interval (CI) 0.52-0.88), and specificity was 88.2 % (95 % CI 0.78-0.95). When a cutoff value for PASP was more than 45 mm Hg during low-dose exercise, SDE missed PH diagnosis in one of the 22 patients with PH and improved sensitivity to 95.2 % (95 % CI 0.81-1.0) but reduced specificity to 84.9 % (95 % CI 0.74-0.93). Reduction of specificity was partly due to concomitant left heart disease.

Conclusions: The results of this prospective cross-sectional study using RHC as gold standard in all patients showed that SDE markedly improved sensitivity in detecting manifest PH to 95.2 % compared with 72.7 % using echocardiography at rest only. Thus, for PH screening in patients with SSc, echocardiography should be performed at rest and during exercise.

Trial registration: ClinicalTrials.gov NCT01387035. Registered 29 June 2011.

No MeSH data available.


Related in: MedlinePlus

Results of the screening of 76 patients with systemic sclerosis (SSc) by right heart catheterisation (RHC). This figure shows that within the screening assessment 29 % of the 76 patients were newly diagnosed with a manifest pulmonary hypertension (PH) and 43 % had no signs of PH. CHD coronary heart disease, HFpEF heart failure with preserved ejection fraction, mPAP mean pulmonary arterial pressure, PH-ILD pulmonary hypertension-interstitial lung disease
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Fig1: Results of the screening of 76 patients with systemic sclerosis (SSc) by right heart catheterisation (RHC). This figure shows that within the screening assessment 29 % of the 76 patients were newly diagnosed with a manifest pulmonary hypertension (PH) and 43 % had no signs of PH. CHD coronary heart disease, HFpEF heart failure with preserved ejection fraction, mPAP mean pulmonary arterial pressure, PH-ILD pulmonary hypertension-interstitial lung disease

Mentions: Twenty-two (29 %) of 76 patients with SSc had an elevated mPAP of at least 25 mm Hg and therefore a manifest PH (Fig. 1). Four of these 22 patients with PH showed an elevated PAWP of more than 15 mm Hg. Further diagnostic work-up by left heart catheter revealed a coronary heart disease or left heart failure with preserved ejection fraction (HFpEF) (or both) in two patients. Signs of significant pulmonary fibrosis in a CT scan and an FVC of less than 70 % were found in four patients with PH. Three patients were classified as having PH-interstitial lung disease. One patient presented with a PAWP of more than 15 mm Hg and was classified as having PH with left heart disease after further diagnostic work-up, including left heart catheterisation.Fig. 1


Stress Doppler echocardiography for early detection of systemic sclerosis-associated pulmonary arterial hypertension.

Nagel C, Henn P, Ehlken N, D'Andrea A, Blank N, Bossone E, Böttger A, Fiehn C, Fischer C, Lorenz HM, Stöckl F, Grünig E, Egenlauf B - Arthritis Res. Ther. (2015)

Results of the screening of 76 patients with systemic sclerosis (SSc) by right heart catheterisation (RHC). This figure shows that within the screening assessment 29 % of the 76 patients were newly diagnosed with a manifest pulmonary hypertension (PH) and 43 % had no signs of PH. CHD coronary heart disease, HFpEF heart failure with preserved ejection fraction, mPAP mean pulmonary arterial pressure, PH-ILD pulmonary hypertension-interstitial lung disease
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4504224&req=5

Fig1: Results of the screening of 76 patients with systemic sclerosis (SSc) by right heart catheterisation (RHC). This figure shows that within the screening assessment 29 % of the 76 patients were newly diagnosed with a manifest pulmonary hypertension (PH) and 43 % had no signs of PH. CHD coronary heart disease, HFpEF heart failure with preserved ejection fraction, mPAP mean pulmonary arterial pressure, PH-ILD pulmonary hypertension-interstitial lung disease
Mentions: Twenty-two (29 %) of 76 patients with SSc had an elevated mPAP of at least 25 mm Hg and therefore a manifest PH (Fig. 1). Four of these 22 patients with PH showed an elevated PAWP of more than 15 mm Hg. Further diagnostic work-up by left heart catheter revealed a coronary heart disease or left heart failure with preserved ejection fraction (HFpEF) (or both) in two patients. Signs of significant pulmonary fibrosis in a CT scan and an FVC of less than 70 % were found in four patients with PH. Three patients were classified as having PH-interstitial lung disease. One patient presented with a PAWP of more than 15 mm Hg and was classified as having PH with left heart disease after further diagnostic work-up, including left heart catheterisation.Fig. 1

Bottom Line: In patients with systemic sclerosis (SSc), associated pulmonary arterial hypertension (SSc-APAH) is the leading cause of death.Investigators of RHC were blinded to the results of non-invasive measurements.When a cutoff value for PASP was more than 45 mm Hg during low-dose exercise, SDE missed PH diagnosis in one of the 22 patients with PH and improved sensitivity to 95.2 % (95 % CI 0.81-1.0) but reduced specificity to 84.9 % (95 % CI 0.74-0.93).

View Article: PubMed Central - PubMed

Affiliation: Centre for Pulmonary Hypertension Thoraxclinic, University Hospital Heidelberg, Amalienstr. 5, 69126, Heidelberg, Germany. c.nagel@klinikum-mittelbaden.de.

ABSTRACT

Introduction: In patients with systemic sclerosis (SSc), associated pulmonary arterial hypertension (SSc-APAH) is the leading cause of death. The objective of this prospective screening study was to analyse sensitivity and specificity of stress Doppler echocardiography (SDE) in detecting pulmonary hypertension (PH).

Methods: Pulmonary artery pressures and further parameters of PH were assessed by echocardiography and right heart catheterisation (RHC) at rest and during exercise in patients with SSc. Investigators of RHC were blinded to the results of non-invasive measurements.

Results: Of 76 patients with SSc (64 were female and mean age was 58±14 years), 22 (29 %) had manifest PH confirmed by RHC: four had concomitant left heart diseases, three had lung diseases, and 15 had SSc-APAH. Echocardiography at rest missed PH diagnosis in five of 22 patients with PH when a cutoff value for systolic pulmonary arterial pressure (PASP) was more than 40 mm Hg at rest. The sensitivity of echocardiography at rest was 72.7 % (95 % confidence interval (CI) 0.52-0.88), and specificity was 88.2 % (95 % CI 0.78-0.95). When a cutoff value for PASP was more than 45 mm Hg during low-dose exercise, SDE missed PH diagnosis in one of the 22 patients with PH and improved sensitivity to 95.2 % (95 % CI 0.81-1.0) but reduced specificity to 84.9 % (95 % CI 0.74-0.93). Reduction of specificity was partly due to concomitant left heart disease.

Conclusions: The results of this prospective cross-sectional study using RHC as gold standard in all patients showed that SDE markedly improved sensitivity in detecting manifest PH to 95.2 % compared with 72.7 % using echocardiography at rest only. Thus, for PH screening in patients with SSc, echocardiography should be performed at rest and during exercise.

Trial registration: ClinicalTrials.gov NCT01387035. Registered 29 June 2011.

No MeSH data available.


Related in: MedlinePlus