Recent Advances in the Treatment of Pulmonary Arterial Hypertension

Otani, Naoyuki; Tomoe, Takashi; Kawabe, Atsuhiko; Sugiyama, Takushi; Horie, Yasuto; Sugimura, Hiroyuki; Yasu, Takanori; Nakamoto, Takaaki

doi:10.3390/ph15101277

Cited by 9 publications

(15 citation statements)

References 86 publications

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“…The clinical and pathological similarities between CTEPH and PAH suggest that targeting PAH may be effective. Currently, the three targets of PAH treatment are the endothelin, nitric oxide, and prostacyclin pathways [ 91 ].…”

Section: Drug Therapymentioning

confidence: 99%

Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary Hypertension

Otani

Watanabe

Tomoe

et al. 2023

IJMS

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Chronic thromboembolic pulmonary hypertension (CTEPH) is a condition in which an organic thrombus remains in the pulmonary artery (PA) even after receiving anticoagulation therapy for more than 3 months and is complicated by pulmonary hypertension (PH), leading to right-sided heart failure and death. CTEPH is a progressive pulmonary vascular disease with a poor prognosis if left untreated. The standard treatment for CTEPH is pulmonary endarterectomy (PEA), which is usually performed only in specialized centers. In recent years, balloon pulmonary angioplasty (BPA) and drug therapy for CTEPH have also shown good results. This review discusses the complex pathogenesis of CTEPH and presents the standard of care, PEA, as well as a new device called BPA, which is showing remarkable progress in efficacy and safety. Additionally, several drugs are now demonstrating established evidence of efficacy in treating CTEPH.

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Section: Drug Therapymentioning

confidence: 99%

Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary Hypertension

Otani

Watanabe

Tomoe

et al. 2023

IJMS

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“…It is metabolized hepatically and converted into the active metabolite hydroxyfasudil [ 101 ]. It has been clinically used to prevent vasospasm after a subarachnoid hemorrhage [ 102 ]. In addition to fasudil, three other ROCK inhibitors have been evaluated in the treatment of PH.…”

Section: Novel Therapiesmentioning

confidence: 99%

New Drugs and Therapies in Pulmonary Arterial Hypertension

Shah

Beckmann

Vorla

2023

IJMS

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Pulmonary arterial hypertension is a chronic, progressive disorder of the pulmonary vasculature with associated pulmonary and cardiac remodeling. PAH was a uniformly fatal disease until the late 1970s, but with the advent of targeted therapies, the life expectancy of patients with PAH has now considerably improved. Despite these advances, PAH inevitably remains a progressive disease with significant morbidity and mortality. Thus, there is still an unmet need for the development of new drugs and other interventional therapies for the treatment of PAH. One shortcoming of currently approved vasodilator therapies is that they do not target or reverse the underlying pathogenesis of the disease process itself. A large body of evidence has evolved in the past two decades clarifying the role of genetics, dysregulation of growth factors, inflammatory pathways, mitochondrial dysfunction, DNA damage, sex hormones, neurohormonal pathways, and iron deficiency in the pathogenesis of PAH. This review focuses on newer targets and drugs that modify these pathways as well as novel interventional therapies in PAH.

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“…BMPR2 mutations, the transforming growth factor-β (TGF-β) superfamily, are crucial in heritable PAH and acquired PAH [49]. Thus, sotatercept a recombinant fusion protein consisting of the extracellular part of the human activin receptor type IIA, which is linked to the Fc piece of human IgG1 restores balance between growth-promoting and growthinhibiting signaling.…”

Section: Sotaterceptmentioning

confidence: 99%

Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases

Smukowska-Gorynia,

Gościniak,

Woźniak

et al. 2023

Preprint

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Pulmonary hypertension (PH) is a severe vascular complication of connective tissue diseases (CTD). Patients with CTD may develop PH belonging to different groups: 1) pulmonary arterial hypertension (PAH), 2) PH secondary to lung disease and/or hypoxia, 3) PH due to left heart disease, and 4) chronic thromboembolic pulmonary hypertension (CTEPH). PAH most often develops in systemic scleroderma (SSc), mostly in its limited variant. PAH-CTD is a progressive disease characterized by poor prognosis, therefore the early diagnosis should be established A specific treatment for PAH-CTD is currently available and recommended: prostacyclin derivative (treprostinil, epoprostenol, iloprost, selexipag), nitric oxide and natriuretic pathway: stimulators of soluble guanylate cyclase (sGC: riociguat) and phosphodiesterase 5 inhibitors (PDE5i: sildenafil, tadalafil), endothelin receptor antagonists (ERA: bosentan, macitentan, ambrisentan). Moreover, novel drugs e.g. sotatercept are intensively investigated in the clinical trials. The aim of this paper is to review the literature on the recent advances in treatment strategy and prognosis of patients with PAH-CTD.

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Recent Advances in the Treatment of Pulmonary Arterial Hypertension

Cited by 9 publications

References 86 publications

Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary Hypertension

Pathophysiology and Treatment of Chronic Thromboembolic Pulmonary Hypertension

New Drugs and Therapies in Pulmonary Arterial Hypertension

Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases

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