Six-Minute Walk Test as a Predictor of Diagnosis, Disease Severity, and Clinical Outcomes in Scleroderma-Associated Pulmonary Hypertension: The DIBOSA Study

Gadre, Abhishek; Ghattas, Christian; Xiao, Han; Minai, Omar A.; Highland, Kristin B.

doi:10.1007/s00408-017-0034-1

Cited by 15 publications

(9 citation statements)

References 33 publications

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“…This suggests that the 6MWD still provides useful prognostic information, consistent with the meta-analysis by LEFÈVRE et al [4]. Another recent study by GADRE et al [36] derived a clinical score including the 6MWD, Borg dyspnoea score and oxygen saturation during the walk test, which was associated with haemodynamic severity and survival in SSc-PAH. Interestingly, while baseline 6MWD independently predicted long-term outcomes, haemodynamics and NYHA-FC at baseline did not.…”

Section: Discussionsupporting

confidence: 71%

Haemodynamics and serial risk assessment in systemic sclerosis associated pulmonary arterial hypertension

et al. 2018

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The prognostic importance of follow-up haemodynamics and the validity of multidimensional risk assessment are not well established for systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH).We assessed incident SSc-PAH patients to determine the association between clinical and haemodynamic variables at baseline and first follow-up right heart catheterisation (RHC) with transplant-free survival. RHC variables included cardiac index, stroke volume index (SVI), pulmonary arterial compliance and pulmonary vascular resistance. Risk assessment was performed according to the number of low-risk criteria: functional class I or II, 6-min walking distance (6MWD) >440 m, right atrial pressure <8 mmHg and cardiac index ≥2.5 L·min·mTransplant-free survival from diagnosis (n=513) was 87%, 55% and 35% at 1, 3 and 5 years, respectively. At baseline, 6MWD was the only independent predictor. A follow-up RHC was available for 353 patients (median interval 4.6 months, interquartile range 3.9-6.4 months). The 6MWD, functional class, cardiac index, SVI, pulmonary arterial compliance and pulmonary vascular resistance were independently associated with transplant-free survival at follow-up, with SVI performing better than other haemodynamic variables. 1-year outcomes were better with increasing number of low-risk criteria at baseline (area under the curve (AUC) 0.63, 95% CI 0.56-0.69) and at first follow-up (AUC 0.71, 95% CI 0.64-0.78).Follow-up haemodynamics and multidimensional risk assessment had greater prognostic significance than at baseline in SSc-PAH.

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Section: Discussionsupporting

confidence: 71%

Haemodynamics and serial risk assessment in systemic sclerosis associated pulmonary arterial hypertension

et al. 2018

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“…In CTD-PAH, the most investigated underlying disease is SSc. Cohort studies and meta-analyses reported that the parameters classically associated with the severity of IPAH, including 6MWD, mPAP, cardiac index, and RAP, were also prognostic factors in SSc-PAH [22][23][24][25]. For pSS-PAH, however, it is still difficult to identify the patients who may benefit from the treatment more than others due to a paucity of data.…”

Section: Discussionmentioning

confidence: 99%

Is it possible to apply the treat-to-target strategy in primary Sjögren’s syndrome-associated pulmonary arterial hypertension?

Liu¹,

Wang²,

Lai

et al. 2018

Clin Rheumatol

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The treat-to-target (T2T) strategy improved long-term survival of patients with pulmonary arterial hypertension (PAH). Little was known about applying the T2T strategy in primary Sjogren's syndrome-associated PAH (pSS-PAH). We investigated how to identify patients who are more likely to reach the treatment goals in a cohort of pSS-PAH. In this way, we explored the possibility of implementing T2T in pSS-PAH. Data were retrospectively collected from patients visiting our center between 2007 and 2017. PAH was confirmed by right heart catheterization (RHC). Patients were treated following the T2T strategy. PAH treatment goals were defined by the 5th World Symposium on Pulmonary Hypertension. The primary end point was reaching the PAH treatment goals. Of the 62 patients enrolled, 98.4% were female, with a mean age of 38.9 ± 9.1 years at the diagnostic RHC. The median disease duration of pSS was 46 months (0-365), while the median disease duration of PAH was 12 months (0-149). Following the T2T strategy, 32 (50%) patients achieved the treatment goals. The 1-, 3-, and 5- year cumulative rates of reaching the goals were 40.6, 67.4, and 73.9%, respectively. Predictive factors included using immunosuppressants (HR 4.715, 95% CI 1.101-20.192, p = 0.037) and right ventricular anterior-posterior diameter (RV-APD) > 30 mm at baseline (HR 0.426, 95% CI 0.188-0.968, p = 0.042). The results provide strong evidence that patients who received immunosuppressants are more likely to reach the treatment goals. In contrast, impaired RV structure correlates to worse treatment response. The T2T strategy is effective in pSS-PAH.

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“…Recently, a combination of features obtained during the 6MWT, ie, distance walked, degree of oxygenation and Borg dyspnea index was found useful in identifying patients who need further cardiopulmonary evaluation. 44 Compared with echocardiography, cardiac MRI (CMR) may provide information on the underpinnings of cardiac involvement in patients with SSc, including inflammatory, microvascular and fibrotic mechanisms. 45 Furthermore, CMR provides a more comprehensive assessment of the right ventricular function.…”

Section: Screening For Pulmonary Hypertension In Systemic Sclerosismentioning

confidence: 99%

<p>Management of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis</p>

Almaaitah¹,

Highland²,

Tonelli³

2020

IBPC

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Systemic sclerosis (SSc) is a rare and complex immune-mediated connective tissue disease characterized by multi-organ fibrosis and dysfunction. Systemic sclerosisassociated pulmonary arterial hypertension (SSc-PAH) is a leading cause of death in this population. Pulmonary arterial hypertension (PAH) can coexist with other forms of pulmonary hypertension in SSc, including pulmonary hypertension related to left heart disease, interstitial lung disease, chronic thromboembolism and pulmonary venous occlusive disease, which further complicates diagnosis and management. Available pulmonary arterial hypertension therapies target the nitric oxide, endothelin and prostacyclin pathways. These therapies have been studied in SSc-PAH in addition to idiopathic PAH, often with different treatment responses. In this article, we discuss the management as well as the treatment options for patients with SSc-PAH.

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Six-Minute Walk Test as a Predictor of Diagnosis, Disease Severity, and Clinical Outcomes in Scleroderma-Associated Pulmonary Hypertension: The DIBOSA Study

Abstract: DIBOSA score is a sensitive tool for the prediction of SSc-PH. The DIBOSA score is a direct predictor of mortality in SSc-PH and strongly correlates with pulmonary pressures. 6MWT can be used to predict clinical outcomes in SSc-PH.

Cited by 15 publications

References 33 publications

Haemodynamics and serial risk assessment in systemic sclerosis associated pulmonary arterial hypertension

Haemodynamics and serial risk assessment in systemic sclerosis associated pulmonary arterial hypertension

Is it possible to apply the treat-to-target strategy in primary Sjögren’s syndrome-associated pulmonary arterial hypertension?

<p>Management of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis</p>

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