Survival and prognostic factors in patients with connective tissue disease‐associated pulmonary hypertension diagnosed by echocardiography: results from a Korean nationwide registry

Kang, Kwi Young; Jeon, Chan Hong; Choi, Sung Jae; Yoon, Bo Young; Choi, Chan Bum; Lee, Chang Hoon; Suh, Chang Hee; Lee, Choong Won; Cho, Chul Soo; Nam, Eon Jeong; Koh, Eunmi; Kim, Ho Youn; Choi, Hyo Jin; Kim, Hyoun‐Ah; Jun, Jae Bum; Lee, Jaejoon; Kim, Jinseok; Ji, Jong Dae; Min, Jun Ki; Kim, Ki Jo; Shin, Kichul; So, Min Wook; Kwon, Seong Ryul; Kim, Seong-Kyu; Nah, Seong Su; Kwok, Seung Ki; Lee, Soo Kon; Lee, Sung Won; Park, Sung Hwan; Park, Won; Park, Yong Bum; Lee, Young Ho; Lee, Shin Seok; Yoo, Dae Hyun

doi:10.1111/1756-185x.12645

Cited by 30 publications

(32 citation statements)

References 28 publications

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“…Our findings are in accordance with a cohort study in China which suggested that the most prevalent autoantibody was the anti‐U1RNP antibody in PAH associated with three major CTDs . The results from a Korean nationwide registry also revealed that anti‐RNP antibody was significantly associated with higher mortality …”

Section: Discussionsupporting

confidence: 91%

Pulmonary arterial hypertension in systemic lupus erythematosus based on a CSTAR‐PAH study: Baseline characteristics and risk factors

Zhang

Qian

et al. 2019

Int J of Rheum Dis

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Aim: Pulmonary arterial hypertension (PAH) is a complex and devastating complication of systemic lupus erythematosus (SLE). We sought to describe the baseline characteristics of right heart catheterization (RHC)-confirmed SLE-associated PAH and identify risk factors for PAH in SLE patients. Methods: A multicenter, cross-sectional study was conducted using the Chinese SLE Treatment and Research group (CSTAR) registry. Baseline data for patients with SLEassociated PAH and SLE patients without PAH were collected and compared. Risk factors for PAH among patients with SLE were identified.

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Section: Discussionsupporting

confidence: 91%

Pulmonary arterial hypertension in systemic lupus erythematosus based on a CSTAR‐PAH study: Baseline characteristics and risk factors

Zhang

Qian

et al. 2019

Int J of Rheum Dis

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“…Furthermore, the presence of exercise induced pulmonary hypertension, assessed by exercise echocardiography, is independently associated with reduced survival in patients with valvular disease and could be useful to identify a high-risk subset of asymptomatic patients 35,36 . While RHC is the gold standard to assess pulmonary pressures and is required to confirm the diagnosis of PH, there is a need for safe and practical noninvasive clinical tests to screen at risk patients for PH.…”

Section: Discussionmentioning

confidence: 99%

Accuracy of Echocardiography to Estimate Pulmonary Artery Pressures With Exercise

Riel

Opotowsky

Santos

et al. 2017

Circ: Cardiovascular Imaging

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Background Exercise echocardiography is often applied as a noninvasive strategy to screen for abnormal pulmonary hemodynamic response, but it is technically challenging and limited data exist regarding its accuracy to estimate pulmonary arterial pressure (PAP) during exercise. Methods and Results Among 65 patients with exertional intolerance undergoing upright invasive exercise testing, tricuspid regurgitation (TR) Doppler estimates and invasive measurement of PAP at rest and peak exercise were simultaneously obtained. TR Doppler envelopes were assessed for quality. Correlation, Bland-Altman, and receiver-operating characteristic curve analyses were performed to evaluate agreement and diagnostic accuracy. Mean age was 62±13 years and 31% were male. High quality (grade A) TR Doppler was present in 68% at rest and 34% at peak exercise. For grade A TR signals, echocardiographic measures of systolic PAP correlated reasonably well with invasive measurement at rest (r=0.72, p<0.001; bias=−2.9±8.0 mmHg) and peak exercise (r=0.75, p<0.001; bias=−1.9±15.6). Lower quality TR signals (grade B and C) correlated poorly with invasive measurements overall. In patients with grade A TR signals, mean PAP-to-workload ratio at a threshold of 1.4mmHg/10W, was able to identify abnormal pulmonary hemodynamic response during exercise (>3.0 mmHg/L/min increase), with 91% sensitivity and 82% specificity (AUC 0.90 [95% CI 0.77–1.0], p=0.001). Conclusions Agreement between echocardiographic and invasive measures of pulmonary pressures during upright exercise is good among the subset of patients with high quality TR Doppler signal. While the limits of agreement are broad, our results suggest that in those patients sensitivity is adequate to screen for abnormal pulmonary hemodynamic response during exercise.

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“…Among the CTDs group, however, SLE patients had the best rates for 1-year survival (94% vs 82% for scleroderma and 88% for MCTD), although all patients had comparable hemodynamic characteristics at PAH diagnosis. Data from the Korean CTD-PAH registry, with 174 patients enroled, demonstrated that the 1- and 3-year survival were 90.7% and 87.3%, respectively 59. In that study, low D LCO , diabetes, and pleural effusion were poor prognostic factors, while anti-U1-RNP antibodies seemed to be protective.…”

Section: Prognosismentioning

confidence: 67%

Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies

Tselios¹,

Gladman²,

Urowitz³

2016

OARRR

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Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (≥25 mmHg) with normal pulmonary capillary wedge pressure (≤15 mmHg) and increased pulmonary vascular resistance. Estimated prevalence ranges from 0.5% to 17.5% depending on the diagnostic method used and the threshold of right ventricular systolic pressure in studies using transthoracic echocardiogram. Its pathogenesis is multifactorial with vasoconstriction, due to imbalance of vasoactive mediators, leading to hypoxia and impaired vascular remodeling, collagen deposition, and thrombosis of the pulmonary circulation. Multiple predictive factors have been recognized, such as Raynaud’s phenomenon, pleuritis, pericarditis, anti-ribonuclear protein, and antiphospholipid antibodies. Secure diagnosis is based on right heart catheterization, although transthoracic echocardiogram has been shown to be reliable for patient screening and follow-up. Data on treatment mostly come from uncontrolled observational studies and consist of immunosuppressive drugs, mainly corticosteroids and cyclophosphamide, as well as PAH-targeted approaches with endothelin receptor antagonists (bosentan), phosphodiesterase type 5 inhibitors (sildenafil), and vasodilators (epoprostenol). Prognosis is significantly affected, with 1- and 5-year survival estimated at 88% and 68%, respectively.

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Survival and prognostic factors in patients with connective tissue disease‐associated pulmonary hypertension diagnosed by echocardiography: results from a Korean nationwide registry

Cited by 30 publications

References 28 publications

Pulmonary arterial hypertension in systemic lupus erythematosus based on a CSTAR‐PAH study: Baseline characteristics and risk factors

Pulmonary arterial hypertension in systemic lupus erythematosus based on a CSTAR‐PAH study: Baseline characteristics and risk factors

Accuracy of Echocardiography to Estimate Pulmonary Artery Pressures With Exercise

Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies

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