2021
DOI: 10.1111/ejh.13612
|View full text |Cite
|
Sign up to set email alerts
|

Long‐term tolerability of phosphodiesterase‐5 inhibitors in pulmonary hypertension of sickle cell disease

Abstract: Objectives Sickle cell disease‐related pulmonary hypertension (SCD‐PH) is a complex disorder with multifactorial contributory mechanisms. Previous trials have evaluated the efficacy of pulmonary arterial hypertension (PAH) therapies in SCD‐PH with mixed results. We hypothesized that a subset of patients with right heart catheterization (RHC) confirmed disease may benefit from PAH therapy. Methods We performed a retrospective chart review of patients with SCD‐PH diagnosed by RHC who were treated with phosphodie… Show more

Help me understand this report

Search citation statements

Order By: Relevance

Paper Sections

Select...
1
1
1
1

Citation Types

0
4
0

Year Published

2021
2021
2023
2023

Publication Types

Select...
5
1

Relationship

0
6

Authors

Journals

citations
Cited by 8 publications
(4 citation statements)
references
References 25 publications
0
4
0
Order By: Relevance
“…PDE5 inhib i tors would pre vent priapism epi sodes or improve exer cise tol er ance in SCD. 24,28 In a recent ret ro spec tive obser va tional study, regimented dos ing of either sildenafil or tadalafil (median dura tion 3 months, interquartile range 27 months) showed that 92% (22 of 24) of the par tic i pants reported improve ment on a PDE5 inhib i tor, with an observed reduc tion in the emer gency visit by 4.4fold com pared with the period before the inter ven tion. 25 However, when the par tic i pants stopped the drug, their priapism recurred with sig nifi cantly increased fre quency (P < .001) and dura tion (P < .001).…”
Section: Secondary Priapism Pre Ven Tion Is the Key To Reduc Ing Pain...mentioning
confidence: 99%
“…PDE5 inhib i tors would pre vent priapism epi sodes or improve exer cise tol er ance in SCD. 24,28 In a recent ret ro spec tive obser va tional study, regimented dos ing of either sildenafil or tadalafil (median dura tion 3 months, interquartile range 27 months) showed that 92% (22 of 24) of the par tic i pants reported improve ment on a PDE5 inhib i tor, with an observed reduc tion in the emer gency visit by 4.4fold com pared with the period before the inter ven tion. 25 However, when the par tic i pants stopped the drug, their priapism recurred with sig nifi cantly increased fre quency (P < .001) and dura tion (P < .001).…”
Section: Secondary Priapism Pre Ven Tion Is the Key To Reduc Ing Pain...mentioning
confidence: 99%
“…Clinical trials using hemopexin, a scavenger molecule that removes heme from circulation, are underway in humans and have shown promising results in murine models [63]. Unfortunately, trials with ETR antagonists [64], Riociguat [65], and Sildenafil [66,67] were either limited by small sample size or adverse side effects, underscoring the need to better understand the pathology and the need for larger clinical trials researching PH in SCD.…”
Section: Inflammation and Pulmonary Hypertensionmentioning
confidence: 99%
“…Indeed, these cellular and molecular mechanisms have informed the current therapeutics usually used in patients with pulmonary hypertension such as endothelin receptor (ETR) antagonists (Bosentan, Ambrisetan), those which prevents the degradation of cyclic guanosine monophosphate (cGMP) (Riociguat and Sildenafil), vasodilators (Epoprostenol) and anticoagulant (warfarin) among others. Clinical trials using hemopexin, a scavenger molecule that removes heme from circulation are underway in humans and have shown promising results in murine models 65 Unfortunately, trials with ETR antagonists 66 , Riociguat 67 , and Sildenafil 68,69 were either limited by small sample size or adverse side effects respectively underscoring the need to better understand the pathology and larger clinical trials researching PH in SCD.…”
Section: Inflammation and Acute Chest Syndromementioning
confidence: 99%