Screening for pulmonary arterial hypertension in systemic sclerosis

Weatherald, Jason; Montani, David; Jevnikar, Mitja; Jaïs, Xavier; Savale, Laurent; Humbert, Marc

doi:10.1183/16000617.0023-2019

Cited by 75 publications

(82 citation statements)

References 102 publications

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“…Screening programs have been proposed in other patient populations at risk for PH such as systemic sclerosis (scleroderma) and sickle cell disease. In systemic sclerosis, incorporating TTE with pulmonary function testing and biomarkers may have a higher predictive value in asymptomatic patients than echocardiography alone 27,28 . This may be important because even those patients with relatively mild PH require more frequent hospitalization and suffer shortened survival 29,30 .…”

Section: Discussionmentioning

confidence: 99%

“…In systemic sclerosis, incorporating TTE with pulmonary function testing and biomarkers may have a higher predictive value in asymptomatic patients than echocardiography alone. 27,28 This may be important because even those patients with relatively mild PH require more frequent hospitalization and suffer shortened survival. 29,30 Future studies of PH in MPN should clearly define prospective criteria for screening, risk stratification, and criteria for RHC confirmation of PH.…”

Section: Ph Prevalence and Risk Factorsmentioning

confidence: 99%

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Prevalence and risk factors for Pulmonary Hypertension associated with chronic Myeloproliferative Neoplasms

Ferrari

Scandura

Masciulli

et al. 2020

European J of Haematology

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Objectives Pulmonary hypertension (PH) is commonly reported in Philadelphia‐chromosome negative myeloproliferative neoplasms (MPNs) including polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF). PH may be diagnosed directly by right heart catheterization (RHC) or estimated by transthoracic echocardiography (TTE). Survival is shortened by PH but despite the potential significance of PH to management and prognosis of MPN, estimates of its prevalence in MPNs vary and risk factors for the condition are poorly established. We performed a systematic review and meta‐analysis of available studies to fill this void. Methods We searched EMBASE, MEDLINE, and Clinicaltrials.gov for the terms “pulmonary hypertension,” “myeloproliferative disorders,” “polycythemia vera,” “essential thrombocythemia,” and “myelofibrosis.” We restricted analysis to the 1999‐2019 window to improve uniformity of MPN diagnostic criteria. We retrieved 221 records and, after abstract and full‐text screening, identified 17 papers meeting criteria for inclusion in our meta‐analysis. A modified Newcastle‐Ottawa scale was used to assess quality. Results Results for 935 patients were available, 309 of these having PH (33%). Using logistic mixed‐effect regression, we found that diagnosis mode (RHC vs TTE) and MPN duration influenced PH prevalence. Studies employing predominantly TTE yielded prevalence estimates ~5‐fold higher than those using RHC (35% vs 7.2%). We identified MF and duration of MPN as significant risk factors for development of PH. Conclusions Prevalence of PH in MPNs is poorly understood with estimates ranging from 3.8% to 58%. Patients with MF and longer duration of disease seem at particularly high risk and should be carefully monitored for PH.

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Section: Discussionmentioning

confidence: 99%

Section: Ph Prevalence and Risk Factorsmentioning

confidence: 99%

Prevalence and risk factors for Pulmonary Hypertension associated with chronic Myeloproliferative Neoplasms

Ferrari

Scandura

Masciulli

et al. 2020

European J of Haematology

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“…Cardiopulmonary exercise testing (CPET) allows discrimination between the metabolic, cardiovascular, and pulmonary components of exercise intolerance 50 . However, it requires expertise and specialist facilities, currently limiting its use, and as such it is not currently recommended as a screening tool for PAH 1 , 2 , 51 , 52 . The typical CPET findings in patients with PAH include reductions in peak rate of oxygen consumption (VO 2 ), ratio of oxygen consumption to heart rate (O 2 pulse), work rate, anaerobic threshold, and an increase in the ratio of minute ventilation to CO 2 production (V E /V CO2 slope) and the ratio of dead space to tidal volume (V D /V T ) 53–55 .…”

Section: Screening Strategies For Pulmonary Arterial Hypertensionmentioning

confidence: 99%

Screening strategies for pulmonary arterial hypertension

Kiely

Lawrie

Humbert

2019

European Heart Journal Supplements

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Pulmonary arterial hypertension (PAH) is rare and, if untreated, has a median survival of 2–3 years. Pulmonary arterial hypertension may be idiopathic (IPAH) but is frequently associated with other conditions. Despite increased awareness, therapeutic advances, and improved outcomes, the time from symptom onset to diagnosis remains unchanged. The commonest symptoms of PAH (breathlessness and fatigue) are non-specific and clinical signs are usually subtle, frequently preventing early diagnosis where therapies may be more effective. The failure to improve the time to diagnosis largely reflects an inability to identify patients at increased risk of PAH using current approaches. To date, strategies to improve the time to diagnosis have focused on screening patients with a high prevalence [systemic sclerosis (10%), patients with portal hypertension assessed for liver transplantation (2–6%), carriers of mutations of the gene encoding bone morphogenetic protein receptor type II, and first-degree relatives of patients with heritable PAH]. In systemic sclerosis, screening algorithms have demonstrated that patients can be identified earlier, however, current approaches are resource intensive. Until, recently, it has not been considered possible to screen populations for rare conditions such as IPAH (prevalence 5–15/million/year). However, there is interest in the use of artificial intelligence approaches in medicine and the application of diagnostic algorithms to large healthcare data sets, to identify patients at risk of rare conditions. In this article, we review current approaches and challenges in screening for PAH and explore novel population-based approaches to improve detection.

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“…[9][10][11] However, in SSc, mildly elevated pulmonary vascular resistance (PVR) also seems to portend a poor prognosis, but this has not yet been incorporated into diagnostic guidelines. 12 Screening programs that include different measures and algorithms have been established in many centers worldwide caring for patients with SSc, with the goal of detecting PAH earlier 6,7,13 ; however, none has been designed to detect mildly elevated PAP (mPAP > 20 mm Hg) or PVR (PVR > 2 Wood units), which have important clinical impact. A potentially valuable tool to detect pulmonary vascular disease earlier in patients at risk for PAH, such as SSc, is to assess pulmonary hemodynamics during exercise.…”

mentioning

confidence: 99%