Prostacyclin-analog therapy in sickle cell pulmonary hypertension

Weir, Nargues; Saiyed, Rehan; Alam, Shoaib; Conrey, Anna; Desai, Himanshu; George, M. Patricia; Keeley, Jennifer; Klings, Elizabeth S.; Mehari, Alem; Taylor, VI James G.; Minniti, Caterina P.; Kato, Gregory J.

doi:10.3324/haematol.2015.131227

Cited by 17 publications

(16 citation statements)

References 14 publications

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“…The effect of PAH-specific therapy (total n 5 71) on 6MWD was also examined in 5 observational studies and 1 additional RCT, although in this RCT and 3 of the observational studies, PH was not confirmed by right-heart catheterization. [50][51][52][53][54][55] It is important to note that the diagnosis of PH and PAH by right-heart catheterization in these studies does not reflect the decision made at the recent 6th World Symposium of Pulmonary Hypertension to lower the mean pulmonary arterial pressure threshold from $25 mm Hg to .20 mm Hg. 46 Improvements in 6MWD were variable in degree and significance across these studies of PAH-specific therapies (ie, sildenafil, arginine, bosentan, and prostacyclin).…”

Section: Good Practice Statementmentioning

confidence: 99%

“…Treatment side effects associated with PAH-specific therapies were reported in 1 RCT and 4 observation studies (total n 5 88), [50][51][52]54,55 and mortality was reported in 3 studies (total n 5 111), in which there were 4 deaths. 49,54,55 For all other outcomes, blood pressure changes, NYHA classification, and health-related quality of life were each reported in 1 study of PAH-specific therapies, 51,52 and oxygen requirement was reported in 3 studies (2 for PAH-specific therapies and 1 for regular automated red blood cell exchange). 52,53,56 However, there were no major treatment effects across studies for these outcomes.…”

Section: Good Practice Statementmentioning

confidence: 99%

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American Society of Hematology 2019 guidelines for sickle cell disease: cardiopulmonary and kidney disease

et al. 2019

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Background: Prevention and management of end-organ disease represent major challenges facing providers of children and adults with sickle cell disease (SCD). Uncertainty and variability in the screening, diagnosis, and management of cardiopulmonary and renal complications in SCD lead to varying outcomes for affected individuals. Objective: These evidence-based guidelines of the American Society of Hematology (ASH) are intended to support patients, clinicians, and other health care professionals in their decisions about screening, diagnosis, and management of cardiopulmonary and renal complications of SCD. Methods: ASH formed a multidisciplinary guideline panel that included 2 patient representatives and was balanced to minimize potential bias from conflicts of interest. The Mayo Evidence-Based Practice Research Program supported the guideline development process, including performing systematic evidence reviews up to September 2017. The panel prioritized clinical questions and outcomes according to their importance for clinicians and patients. The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including GRADE evidence-to-decision frameworks, to assess evidence and make recommendations, which were subject to public comment. Results: The panel agreed on 10 recommendations for screening, diagnosis, and management of cardiopulmonary and renal complications of SCD. Recommendations related to anticoagulation duration for adults with SCD and venous thromboembolism were also developed. Conclusions: Most recommendations were conditional due to a paucity of direct, high-quality evidence for outcomes of interest. Future research was identified, including the need for prospective studies to better understand the natural history of cardiopulmonary and renal disease, their relationship to patient-important outcomes, and optimal management.

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Section: Good Practice Statementmentioning

confidence: 99%

Section: Good Practice Statementmentioning

confidence: 99%

American Society of Hematology 2019 guidelines for sickle cell disease: cardiopulmonary and kidney disease

et al. 2019

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“…49 Prostacyclin analogs have not been widely used in SCD due to concerns about line thrombosis but retrospective data on 11 patients with SCD and pulmonary arterial hypertension treated for at least 100 days showed that prostacyclin analogs were well tolerated with evidence of functional improvement and no evidence of increased thrombotic events. 50 This needs further investigation in prospective trials.…”

Section: Comments On Patients 1 Andmentioning

confidence: 98%

How I treat the older adult with sickle cell disease

Thein

Howard

2018

Blood

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With increasing survival, cumulative complications of sickle cell disease (SCD), which develop insidiously over time, are becoming more apparent and common in older patients, particularly those in their fifth decade and beyond. The older patient is also more likely to develop other age-related nonsickle conditions that interact and add to the disease morbidity. A common misconception is that any symptom in a SCD patient is attributable to their SCD and this may lead to delays in diagnosis and appropriate intervention. We recommend regular comprehensive reviews and monitoring for early signs of organ damage and a low threshold for the use of hydroxyurea and blood transfusions as preventative measures for end-organ disease. Treatable comorbidities and acute deterioration should be managed aggressively. Although the primary goal in management of the older adult with SCD is improving anemia and minimizing organ damage, the time has come for us to be more proactive in considering curative therapies previously offered to the younger patient. Curative or experimental interventions should be discussed early, before complications render the patients ineligible for these treatments.

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“…These 19 patients received therapy with sildenafil (n = 15), bosentan (n = 1), sildenafil and bosentan (n = 2) or a combination of tadalafil, treprostinil and bosentan (n = 1). Responses to PH therapies have been reported previously (Machado et al, 2011;Weir et al, 2017).…”

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confidence: 71%