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Bilateral nonsimultaneous visual loss associated with idiopathic arterial pulmonary hypertension.

PURPOSE: To report ischemic central retinal vein occlusion in one eye and recurrent serous retinal detachment in the other eye of a patient with idiopathic arterial pulmonary hypertension. METHODS: A 33-year-old woman was referred to our institution 2 months after an episode of central retinal vein occlusion in her right eye. She had arterial pulmonary hypertension for 2 years and was enrolled in a double-blind clinical trial of a novel vasodilator (bosentan) for arterial pulmonary hypertension. At presentation, corrected visual acuity was hand movements in the right eye and 20/20 in the left…

Current management of primary pulmonary hypertension.

Primary pulmonary hypertension (PPH) is a rare disorder with an annual incidence of 1 to 2 per million people. The aetiology of this disorde … g transplant appears to be the procedure of choice. Newer agents, such as sildenafil, beraprost and bosentan, are presently being evaluated for the treatment of this disorder. Future study should include elucidation of the pathogenic mechanisms in the development of this vasculopathy, which will hopefully lead to the development of improved treatment optio …

Pulmonary artery denervation versus conventional therapies for PAH: a systematic review and updated network meta-analysis.

AIMS: A recent guideline presented by the ESC Congress in 2022 had indicated a novel therapy targeted at pulmonary artery hypertension, know … Placebo (-77.76 m, 95% CI: -102.04 to -54.34 m), Macitentan (-65.32 m, 95% CI: -95.34 to -36.1 m), Bosentan (-64.5 m, 95% CI: -94.7 to -35.07 m), Iloprost (-62.66 m, 95% CI: -99.48 to -27.13 m), Oxygen (-62.42 m, 95% CI: -100.01 to -25.78 m), Treprostinil (-62.01 m, 95% CI: -89.04 to -35.61 m), Riociguat (-60.59 m, 95% CI: -86.11 to -35.98 m), Selexipag ( …

Ambrisentan for the management of pulmonary arterial hypertension.

BACKGROUND: Approved by the US Food and Drug Administration in 2007, ambrisentan is the second oral endothelin A-receptor antagonist availab … r up to 1 year. Patients who had elevations in serum aminotransferases during previous therapy with bosentan or sitaxsentan therapy were able to make a successful transition to ambrisentan without further abnormalities in liver function. Ambrisentan was generally well tolerated. The most common adverse effects associated with ambrisentan in clinical trials …

The extracellular matrix protein type I collagen and fibronectin are regulated by β-arrestin-1/endothelin axis in human ovarian fibroblasts.

BACKGROUND: The invasive and metastatic spread of serous ovarian cancer (SOC) results from the cooperative interactions between cancer and s … plicated, evident from inhibitory effects after ETAR or ETBR antagonist treatments and notably with bosentan, a dual antagonist, in vitro and in vivo. At the molecular level, ET-1 triggers the activation of COL1A1 promoter activity and its enhanced expression via β-arr1 nuclear function. Transcriptome analysis of β-arr1-silenced HOFs confirms the nuclear r …

The safety and persistence of intravenous iloprost in systemic sclerosis.

INTRODUCTION: Vasculopathy is a crucial feature of systemic sclerosis (SSc). It occurs in almost every patient with SSc, with Raynaud's phen … t with the generalised erythroderma reaction, who suspended the perfusion and was later switched to bosentan. After a mean follow-up time of 6.9 (+/-) 4.0 years of treatment (range 0.06-22), 24 patients (40%) stopped the therapy, 14 (58.3%) of whom due to clinical improvement. The overall 5-, and 10-year survival rates of IV iloprost were 68.2% and 55.6%, …

Case Report: Post-Total anomalous pulmonary venous connection pulmonary hypertension - novel treatment using sirolimus and atrial flow regulator implantation.

OBJECTIVE: In patients who have undergone surgical repair of TAPVC and who develop treatment-resistant pulmonary vein stenosis and postcapil … rmed. Hemodynamic and oximetric measurements were consistent with mixed PH. Sirolimus, losartan and bosentan therapy were initiated. Systemic infections complicated the in-hospital course. Due to prolonged dependence on invasive mechanical ventilation, tracheostomy was performed. Invasive haemodynamic assessment in the 3rd year of life revealed severe prec …

[Pulmonary hypertension in Russia: the first results of the national register].

AIM: To investigate the demographic and clinical characteristics of patients with pulmonary hypertension (PH) and chronic thromboembolic pul … (16.9%), and obesity (16.5%). 66% of the patients received PH-specific therapy: sildenafil (51.6%), bosentan (20.9%), or iloprost (7%); 7.4% of the patients were observed within the framework of clinical trials, 17.7% received combined therapy for PH: 16.3 and 1.4% had dual- or triple-component therapy, respectively; 55.8% of the patients took calcium anta …

Pulmonary arterial hypertension in pregnancy.

Pulmonary hypertension is a medical condition characterized by elevated pulmonary arterial pressure and secondary right heart failure. Pulmonary arterial hypertension is a subset of pulmonary hypertension, which is characterized by an underlying disorder of the pulmonary arterial vasculature. Pulmonary hypertension can also occur secondarily to structural cardiac disease, autoimmune disorders, and toxic exposures. Although pregnancies affected by pulmonary hypertension and pulmonary arterial hypertension are rare, the pathophysiology exacerbated by pregnancy confers both high maternal and feta

Idiopathic pulmonary arterial hypertension.

Idiopathic pulmonary arterial hypertension (IPAH), formerly called primary pulmonary hypertension, is a rare disease (incidence and prevalence rates of approximately one and six cases per million inhabitants, respectively) with different clinical phenotypes. A group of diverse conditions manifest pulmonary arterial hypertension (PAH) and share similar pathological and/or clinical findings with IPAH. By definition, IPAH is diagnosed only after alternative diagnoses have been ruled out. Extensive investigation is needed to determine if PAH is associated with thyroid diseases, infectious diseases

Cutaneous adverse drug reactions in patients with congenital heart disease: a systematic review with focus on perioperative outcomes.

BACKGROUND: Cutaneous adverse drug reactions (CADRs) are uncommon but clinically significant complications among patients with congenital he … s (n = 3, 14%), typically in postoperative or intensive care settings. Additional reports described bosentan-associated indurated erythema in an adult with repaired tetralogy of Fallot and amoxicillin-associated morbilliform exanthem in pediatric patients after surgical repair. Overall, prostaglandin analogs accounted for the majority of implicated drugs ( …

Doppler ultrasound, a noninvasive tool for the study of mesenteric arterial flow in systemic sclerosis: a cross-sectional study of a patient cohort with review and meta-analysis of the literature.

Gastrointestinal involvement (GI) is a frequent and troublesome complication of systemic sclerosis (SSc), whose etiology is poorly understoo … tween SMA RI and SMA PI and mRSS (β 0.248, p = 0.030 and β 2.995, p = 0.004, respectively) and with bosentan (β 0.400, p = 0.003 and β 3.508, p = 0.001, respectively), but not with anticentromere antibody (ACA). No significant differences were found between the weighted median values of SMA RI and SMA PI of SSc patients compared to those of HS that were de …

Targeted therapy is required for management of pulmonary arterial hypertension after defect closure in adult patients with atrial septal defect and associated pulmonary arterial hypertension.

BACKGROUND: Therapeutic strategies for pulmonary arterial hypertension (PAH) associated with atrial septal defect (ASD) remain a matter of debate. METHODS AND RESULTS: We identified 5 outpatients who had been diagnosed with ASD-PAH and undergone ASD closure in combination with targeted therapy with certified PAH drugs. We assessed changes in hemodynamic parameters and exercise capacity. The combination of ASD closure and targeted therapy significantly increased systemic blood flow (Qs) from the baseline (from 3.3 ± 0.6 L/minute to 4.2 ± 1.0 L/minute, P < 0.05) with a significant improvement i

Opportunities and challenges of pharmacotherapy for pulmonary arterial hypertension in children.

Pediatric pulmonary hypertension (PAH) is a rare disease that carries a poor prognosis if left untreated. Although there are published guidelines for the treatment of children with pulmonary hypertension, due to the limited number of robust pediatric clinical trials, recommendations are often based on limited data or clinical experience. Furthermore, many practical aspects of care, particularly for the pediatric patient, are learned through experience and best navigated with a multidisciplinary team. While newer PAH therapies have been approved for adults, there is still limited but expanding

Raynaud, digital ulcers and calcinosis in scleroderma.

Raynaud, digital ulcers and calcinosis are frequent manifestations of patients with systemic sclerosis. Digital ulcers are seen in more than half of the patients with scleroderma. Hospitalizations, ischemic complications and impairment of hand function are frequently observed in patients with digital ulcers, especially if treatment is delayed. Rapid and intensive treatment escalation in patients with scleroderma and refractory Raynaud's phenomenon is one of the most effective preventive action available in order to avoid the development of digital ulcers and tissue loss.

Availability of and access to orphan drugs: an international comparison of pharmaceutical treatments for pulmonary arterial hypertension, Fabry disease, hereditary angioedema and chronic myeloid leukaemia.

BACKGROUND: Market authorization does not guarantee patient access to any given drug. This is particularly true for costly orphan drugs beca … rovided by healthcare payers; and (iii) the price of the drugs in each country. For PAH we analysed bosentan, iloprost, sildenafil, treprostinil (intravenous and inhaled) as well as sitaxentan and ambrisentan; for FD we analysed agalsidase alfa and agalsidase beta; for HAE we analysed icatibant, ecallantide and two complement C1s inhibitors; for CML we ana …

A review of sitaxsentan sodium in patients with pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a life threatening, progressive condition which eventually leads to fatal right heart failure. Endothelin-1 (ET-1), a potent vasoconstrictor peptide, is increased in the pulmonary arteries of patients with pulmonary hypertension. Endothelin-1 acts through the stimulation of 2 subtypes of receptors (endothelin receptor subtypes A [ET(A)] and B [ET(B)]). In PAH patients, ETRAs block the deleterious vasoconstrictor effects of ET-1, and ETRA treatment in PAH patients has been shown to be safe and efficacious. Sitaxsentan is an orally active, highly ET(A) se

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… Only 3% of patients in the sitaxsentan group discontinued due to adverse events compared with 10% for placebo and 10% for bosentan. … Warfarin management in pulmonary arterial hypertension is similar between bosentan, placebo, and sitaxsentan. Chest. 2005;128:366S. Coyne TC, Garces PC, Dixon RA. Warfarin Management and Bleeding with Sitaxsentan and Bosentan. …