Diagnosis of pulmonary hypertension

Frost, Adaani E.; Badesch, David B.; Gibbs, J. Simon R.; Gopalan, Deepa; Khanna, Dinesh; Manes, Alessandra; Oudiz, Ronald J.; Satoh, Tsuyoshi; Torres, Fernando; Torbicki, Adam

doi:10.1183/13993003.01904-2018

Cited by 357 publications

(373 citation statements)

References 80 publications

(77 reference statements)

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“…The presence of PH early on in patients with SSc‐associated ILD is a key factor we must recognize when designing clinical trials for SSc‐associated ILD, since PH may be confounding patient‐reported outcomes and cardiopulmonary physiology in these patients, which may affect the outcome of clinical trials. Future prospective studies in SSc‐associated ILD should confirm our findings and also explore the impact of the new hemodynamic definition of PH, which was recently proposed at the 6th World Symposium on Pulmonary Hypertension .…”

Section: Discussionsupporting

confidence: 70%

Prevalence, Treatment, and Outcomes of Coexistent Pulmonary Hypertension and Interstitial Lung Disease in Systemic Sclerosis

Young

Vummidi

Visovatti

et al. 2019

Arthritis & Rheumatology

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Objective Systemic sclerosis (SSc) is associated with interstitial lung disease (ILD) and pulmonary hypertension (PH). This study was undertaken to determine the prevalence, characteristics, treatment, and outcomes of PH in a cohort of patients with SSc‐associated ILD. Methods Patients with SSc‐associated ILD on high‐resolution computed tomography (HRCT) were included in a prospective observational cohort. Patients were screened for PH based on a standardized screening algorithm and underwent right‐sided heart catheterization (RHC) if indicated. PH classification was based on hemodynamic findings and the extent of ILD on HRCT. Summary statistics and survival using the Kaplan‐Meier method were calculated. Results Of the 93 patients with SSc‐associated ILD included in the study, 76% were women and 65.6% had diffuse cutaneous SSc. The mean age was 54.9 years, and the mean SSc disease duration was 8 years. Twenty‐nine patients (31.2%) had RHC‐proven PH; of those 29 patients, 24.1% had PAH, 55.2% had World Health Organization (WHO) Group III PH, 34.5% had WHO Group III PH with pulmonary vascular resistance >3.0 Wood units, 48.3% had a PH diagnosis within 7 years of SSc onset, 82.8% received therapy for ILD, and 82.8% received therapy for PAH. The survival rate 3 years after SSc‐associated ILD diagnosis for all patients was 97%. The survival rate 3 years after PH diagnosis for those with SSc‐associated ILD and PH was 91%. Conclusion In a large cohort of patients with SSc‐associated ILD, a significant proportion of patients had coexisting PH, which often occurs early after SSc diagnosis. Most patients were treated with ILD and PAH therapies, and survival was good. Patients with SSc‐associated ILD should be evaluated for coexisting PH.

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Section: Discussionsupporting

confidence: 70%

Prevalence, Treatment, and Outcomes of Coexistent Pulmonary Hypertension and Interstitial Lung Disease in Systemic Sclerosis

Young

Vummidi

Visovatti

et al. 2019

Arthritis & Rheumatology

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“…38,40 A cardiopulmonary exercise test (CPET) can suggest pulmonary vascular disease in cases of low end-tidal partial pressure of carbon dioxide (EtPCO 2 ), high ventilator equivalents for carbon dioxide (VE/VCO 2 ), low oxygen pulse (VO 2 /HR) and low peak oxygen uptake (VO 2 ). 20,41 The six-minute walk test (6MWT) is a non-invasive submaximal functional test that correlates with maximum exercise capacity as measured by CPET. 42 In addition, heart rate recovery (HRR, as measured by the difference of heart rate at the end of 6MWT and after 1 min of resting) may predict clinical worsening in patients with connective tissue disease associated PAH (CTD-PAH), including SSc.…”

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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“…Therefore, it is recommended that all patients with SSc receive HRCT at baseline . Additionally, all patients should be evaluated for evidence of cardiac involvement including assessment for pulmonary hypertension at the initial visit …”

Section: Risk Factors and Screeningmentioning

confidence: 99%

Management of systemic sclerosis‐associated interstitial lung disease in the current era

Amjadi

Roofeh

Namas³

et al. 2020

Int J of Rheum Dis

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Diagnosis of pulmonary hypertension

Cited by 357 publications

References 80 publications

Prevalence, Treatment, and Outcomes of Coexistent Pulmonary Hypertension and Interstitial Lung Disease in Systemic Sclerosis

Prevalence, Treatment, and Outcomes of Coexistent Pulmonary Hypertension and Interstitial Lung Disease in Systemic Sclerosis

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

Management of systemic sclerosis‐associated interstitial lung disease in the current era

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