Idiopathic and Systemic Sclerosis-Associated Pulmonary Arterial Hypertension

Ramjug, Sheila; Hussain, Nehal; Hurdman, Judith; Billings, Catherine; Charalampopoulos, Athanasios; Elliot, Charlie; Kiely, David G.; Sabroe, Ian; Rajaram, Smitha; Swift, Andrew J.; Condliffe, Robin

doi:10.1016/j.chest.2017.02.010

Cited by 65 publications

(29 citation statements)

References 38 publications

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“…In patients where there is a high prevalence of pulmonary hypertension, such as systemic sclerosis, interrogation of the CT can be helpful where an enlarged PA may be the first indicator of the presence of pulmonary hypertension. Dilatation of the PA in this setting, in particular a pulmonary to aortic ratio of >1.0, can be highly predictive [34,35]. CTPA is particularly useful in CTEPH, where the distribution of vascular webs, stenosis and mural thrombus allows for treatment planning and assessment of suitability for pulmonary endarterectomy.…”

Section: Computed Tomography Pulmonary Angiography (Ctpa)mentioning

confidence: 99%

Current and emerging imaging techniques in the diagnosis and assessment of pulmonary hypertension

Johns

Wild

Rajaram

et al. 2018

Expert Review of Respiratory Medicine

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Pulmonary hypertension (PH) is a challenging condition to diagnose and treat. Over the last two decades, there have been significant advances in therapeutic approaches and imaging technologies. Current guidelines emphasize the importance of cardiac catheterization; however, the increasing availability of non-invasive imaging has the potential to improve diagnostic rates, whilst providing additional information on patient phenotypes. Areas covered: This review discusses the role of imaging in the diagnosis, prognostic assessment and follow-up of patients with PH. Imaging methods, ranging from established investigations (chest radiography, echocardiography, nuclear medicine and computerized tomography (CT)), to emerging modalities (dual energy CT, magnetic resonance imaging (MRI), optical coherence tomography and positron emission tomography (PET)) are reviewed. The value and limitations of the clinical utility of these imaging modalities and their potential clinical application are reviewed. Expert commentary: Imaging plays a key role in the diagnosis and classification of pulmonary hypertension. It also provides valuable prognostic information and emerging evidence supports a role for serial assessments. The authors anticipate an increasing role for imaging in the pulmonary hypertension clinic. This will reduce the need for invasive investigations, whilst providing valuable insights that will improve our understanding of disease facilitate a more targeted approach to treatment.

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Section: Computed Tomography Pulmonary Angiography (Ctpa)mentioning

confidence: 99%

Current and emerging imaging techniques in the diagnosis and assessment of pulmonary hypertension

Johns

Wild

Rajaram

et al. 2018

Expert Review of Respiratory Medicine

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“…In a recently published article Ramjug et al did not confirm the aforementioned findings, as no significant differences of RV mass were recorded between SSc-PAH and IPAH patients at increased RV load. Nevertheless, they found a notable correlation between the VMI and PVR in SSc-PAH through the entire range of PVR, which may be a predictor for survival, as earlier studies suggested [ 48 , 49 ].…”

Section: Right Ventricular Involvement In Scleroderma: a Distinctimentioning

confidence: 70%

Right Ventricle Remodeling and Function in Scleroderma Patients

Cucuruzac

Muntean

Benedek

et al. 2018

BioMed Research International

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Scleroderma, known also as systemic sclerosis (SSc), is a severe disease associated with high mortality rates, and right ventricular (RV) remodeling and dysfunction, along with pulmonary artery hypertension (PAH), are among the most important internal organ manifestations of this disease. PAH has a higher prevalence in patients with SSc compared to the general population and represents a significant predictor of mortality in SSc. In patients with SSc, the morphological remodeling and alteration of RV function begin even before the setting of PAH and lead to development of a specific adaptive pattern of the RV which is different from the one recorded in patients with IAPH. These alterations cause worse outcomes and increased mortality rates in SSc patients. Early detection of RV dysfunction and remodeling is possible using modern imaging tools currently available and can indicate the initiation of specific therapeutic measures before installation of PAH. The aim of this review is to summarize the current knowledge related to mechanisms involved in the remodeling and functional alteration of the RV in SSc patients.

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“…[713] However, we have found a younger mean age than SSc-PAH Caucasian patients (60.9 years) and patients with idiopathic PAH (IPAH). [91213]…”

Section: Discussionmentioning

confidence: 99%

Pulmonary arterial hypertension in Saudi patients with systemic sclerosis: Clinical and hemodynamic characteristics and mortality

et al. 2019

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BACKGROUND:Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality in patients with systemic sclerosis (SSc). The objective of this study is to describe the clinical characteristics, mortality, and predictors of SSc-PAH in Saudi patients.METHODS:Retrospective chart review study of SSc patients who were followed for at least 1 year in three tertiary care centers in Saudi Arabia was conducted. Clinical information, echocardiographic findings, and right heart catheterization (RHC) results were collected. Descriptive statistics were used for demographic and disease characteristics.RESULTS:Fifty-seven patients with SSc were reviewed. PAH was confirmed by RHC in 40 patients (87.5%, females). Their mean age was 45.43 ± 13.48 years. The mean pulmonary artery pressure was 42.9 ± 12.7 mmHg, the pulmonary vascular resistance index was 19.4 ± 7.7 woods unit, and cardiac index was 2.43 ± 0.68 min/m2. The median time from symptoms to first assessment was 42.8 ± 115.62 months. Most patients (77.5%) presented with functional Class III or IV and more than half (22.55%) were on dual combination therapy. Ten patients (25%) SSc PAH died over a follow up period of 37 ± 7 months. Compared to SSc patients without PAH, SSc-PAH patients had shorter 6-min walk distance (6MWD) (296.1 ± 116.5 vs. 399.59 ± 40.60 m, P < 0.0001), higher pro-brain natriuretic peptide (1755.8 ± 2123.4 vs. 69.8 ± 44.3 pg/ml P = 0.004), and more frequent Raynaud's phenomenon (RP) (90% vs. 35%, P < 0.0001). Logistic regression showed RP (odds ratio [OR] =48.58, 95% confidence interval [CI]; 3.73–633.10) and 6MWD (OR 1.02: 95% CI; 1.01–1.03) were associated with the development of PAH.CONCLUSION:Our cohort of Saudi SSc-PAH patients has a younger disease onset and a lower mortality than what is described worldwide despite late presentation and requirement of combination therapy. The presence of RP and lower were associated with the development of SSc-PAH.

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Idiopathic and Systemic Sclerosis-Associated Pulmonary Arterial Hypertension

Cited by 65 publications

References 38 publications

Current and emerging imaging techniques in the diagnosis and assessment of pulmonary hypertension

Current and emerging imaging techniques in the diagnosis and assessment of pulmonary hypertension

Right Ventricle Remodeling and Function in Scleroderma Patients

Pulmonary arterial hypertension in Saudi patients with systemic sclerosis: Clinical and hemodynamic characteristics and mortality

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