Progressive Right Ventricular Dysfunction in Patients With Pulmonary Arterial Hypertension Responding to Therapy

Veerdonk, Mariëlle C. van de; Kind, Taco; Marcus, J. Tim; Mauritz, Gert-Jan; Heymans, Martijn W.; Bogaard, Harm Jan; Boonstra, Anco; Marques, Koen M.; Westerhof, Nico; Vonk‐Noordegraaf, Anton

doi:10.1016/j.jacc.2011.06.068

Cited by 710 publications

(557 citation statements)

References 35 publications

Supporting

Mentioning

534

Contrasting

Unclassified

Order By: Relevance

“…Adaptation of the RV to the increase in right load (evaluated by PVR) may be a determinant for long-term outcome. However, discrepant trends can be observed between RV function and PVR, despite PAH-specific therapy [5], thereby suggesting that tools which specifically evaluate RV function may be useful [6].…”

Section: Introductionmentioning

confidence: 99%

“…Cardiac MRI can be used to assess RV ejection fraction (RVEF) [7]. A decreased stroke volume, increased RV end-diastolic volume and decreased RVEF at MRI predict a poor outcome in patients with PAH [5,8,9]. However, cardiac MRI is not widely available in all centres, and its use may be limited by intensive and time-consuming data acquisition and analysis, with significant technical support and expertise needed.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Prognostic value of right ventricular ejection fraction in pulmonary arterial hypertension

et al. 2014

View full text Add to dashboard Cite

Right ventricle ejection fraction (RVEF) evaluated with magnetic resonance imaging is a strong determinant of patient outcomes in pulmonary arterial hypertension. We evaluated the prognostic value of RVEF assessed with conventional planar equilibrium radionuclide angiography at baseline and change 3-6 months after initiating pulmonary arterial hypertension-specific therapy.In a prospective cohort of newly diagnosed patients with idiopathic, heritable or anorexigen-associated pulmonary arterial hypertension, RVEF was measured at baseline (n=100) and 3-6 months after initiation of therapy (n=78). After a median follow-up of 4.1 years, 41 deaths occurred, including 35 from cardiovascular causes. Patients with a (median) baseline RVEF >25% had better survival than those with a RVEF <25% using Kaplan-Meier analysis ( p=0.010). RVEF at baseline was an independent predictor of all-cause and cardiovascular mortality in adjusted Cox regression model ( p=0.002 and p=0.007, respectively; HR 0.93 for both). Patients with stable or increased RVEF at 3-6 months had a trend for improved all-cause survival (HR 2.43, p=0.086) and had less cardiovascular mortality (HR 3.25, p=0.034) than those in whom RVEF decreased despite therapy.RVEF assessed with conventional planar equilibrium radionuclide angiography at baseline and change in RVEF 3-6 months after therapy initiation independently predict outcomes in patients with pulmonary arterial hypertension. @ERSpublications RVEF assessed with CPERA at baseline, and its changes on therapy, independently predict outcome in patients with PAH http://ow.ly/DsCS4

show abstract

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Prognostic value of right ventricular ejection fraction in pulmonary arterial hypertension

et al. 2014

View full text Add to dashboard Cite

show abstract

“…Elevation of PAP increases right ventricular (RV) pressure-overload, leading to right heart failure and, potentially, premature death. As right heart failure is the direct cause of death in most patients with PH, adequate evaluation of RV function is critical in the management of PH [2,3].…”

Section: Introductionmentioning

confidence: 99%

Simple prediction of right ventricular ejection fraction using tricuspid annular plane systolic excursion in pulmonary hypertension

Sato

Tsujino

Oyama‐Manabe

et al. 2013

Int J Cardiovasc Imaging

View full text Add to dashboard Cite

Purpose: The present study examined whether tricuspid annular plane systolic excursion (TAPSE) can simply predict right ventricular ejection fraction (RVEF) in patients with pulmonary hypertension (PH). The TAPSE cut-off value to predict reduced RV EF was also evaluated. Methods and results:The association between TAPSE and cardiac magnetic resonance imaging (CMRI)-derived RVEF was examined in 53 PH patients. The accuracy of the prediction equation to calculate RVEF using TAPSE was also evaluated. In PH patients, TAPSE was strongly correlated with CMRI-derived RVEF in PH patients (r=0.86, p<0.0001). We then examined the accuracy of the two equations: the original regression equation (RVEF = 2.01 x TAPSE + 0.6) and the simplified prediction equation (RV EF = 2 x TAPSE). Bland-Altman plot showed that the mean difference ± limits of agreement was 0.0 ± 10.6 for the original equation and −0.6 ± 10.6 for the simplified equation. Intraclass correlation coefficient was 0.84 for the original and 0.82 for the simplified equation. Normal RV EF was considered to be ≥ 40% based on the data from 53 matched controls, and the best TAPSE cut-off value to determine reduced RV EF (< 40%) was calculated to be 19.7 mm (sensitivity 88.9%, specificity 84.6%). Conclusion:A simple equation of RV EF = 2 x TAPSE enables easy prediction of RV 3 EF using TAPSE, an easily measurable M-mode index of echocardiography. TAPSE of 19.7 mm predicts reduced RV EF in PH patients with clinically acceptable sensitivity and specificity.4

show abstract

“…Perfusion MRI can be performed in the same sitting as high‐resolution pulmonary MR angiography and cardiac MRI scan, and has the potential for a “one‐stop‐shop” analysis of pulmonary perfusion and assessment of right heart and pulmonary vascular characteristics. Cardiac MR assessment of baseline and progression of right ventricular characteristics over time in idiopathic pulmonary artery hypertension (IPAH) has been previously shown to be a predictor of outcome 16, 17…”

Section: Discussionmentioning

confidence: 99%

Lung perfusion: MRI vs. SPECT for screening in suspected chronic thromboembolic pulmonary hypertension

Johns

Swift

Rajaram

et al. 2017

Magnetic Resonance Imaging

View full text Add to dashboard Cite

PurposeTo assess the diagnostic accuracy of magnetic resonance imaging (MRI) perfusion against perfusion single photon emission tomography (SPECT) screening for chronic thromboembolic pulmonary hypertension (CTEPH). Ventilation/perfusion (V/Q) scintigraphy is recommended to screen for suspected CTEPH. It has previously been shown that 3D dynamic contrast‐enhanced (DCE) lung perfusion MRI has a similar sensitivity for diagnosing CTEPH in comparison to planar perfusion scintigraphy; however, planar scintigraphy has now been largely replaced by SPECT, due to higher spatial resolution and sensitivity.Materials and MethodsConsecutive patients with suspected CTEPH or unexplained pulmonary hypertension attending a referral center, who underwent lung DCE perfusion MRI at 1.5T, perfusion SPECT, and computed tomography pulmonary angiography (CTPA) within 14 days of right heart catheterization, from April 2013 to April 2014, were included. DCE‐MR, SPECT, and CTPA were independently analyzed by two blinded radiologists. Disagreements were corrected by consensus. The gold standard reference for the diagnosis of chronic thromboemboli was based on a review of multimodality imaging and clinical findings.ResultsIn all, 74 patients with suspected CTEPH underwent all three modalities. Forty‐six were diagnosed with CTEPH (36) or chronic thromboembolic disease (CTED) (10). 3D DCE perfusion MRI correctly identified all patients (sensitivity of 100%), compared with a 97% sensitivity for SPECT.ConclusionDCE lung perfusion MRI has increased sensitivity when compared with perfusion scintigraphy in screening for CTEPH. As MRI does not use ionizing radiation, it should be considered as a first‐line imaging modality in suspected CTEPH. Level of Evidence: 3 Technical Efficacy: Stage 3J. Magn. Reson. Imaging 2017;46:1693–1697.

show abstract

Progressive Right Ventricular Dysfunction in Patients With Pulmonary Arterial Hypertension Responding to Therapy

Abstract: After PAH-targeted therapy, RV function can deteriorate despite a reduction in PVR. Loss of RV function is associated with a poor outcome, irrespective of any changes in PVR.

Cited by 710 publications

References 35 publications

Prognostic value of right ventricular ejection fraction in pulmonary arterial hypertension

Prognostic value of right ventricular ejection fraction in pulmonary arterial hypertension

Simple prediction of right ventricular ejection fraction using tricuspid annular plane systolic excursion in pulmonary hypertension

Lung perfusion: MRI vs. SPECT for screening in suspected chronic thromboembolic pulmonary hypertension

Contact Info

Product

Resources

About