Tratamiento con sildenafilo y/o bosentán en niños y jóvenes con hipertensión arterial pulmonar idiopática y síndrome de Eisenmenger

Raposo-Sonnenfeld, Inés; Otero-González, Isabel; Blanco-Aparicio, Marina; Ferrer-Barba, Ángela; Medrano-López, Constancio

doi:10.1157/13101640

Cited by 17 publications

(1 citation statement)

References 26 publications

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“…Several studies in adult patients with ES have reported a persistent beneficial effect of bosentan (an oral dual endothelin receptor antagonist) on exercise capacity and clinical status without any worsening of pulmonary-to-systemic shunt 14–17. In an open-label study, 24 patients with DS-ES were treated with bosentan with a median follow-up period of 11.5 months 18.…”

Section: Introductionmentioning

confidence: 99%

Effect of Bosentan on Exercise Capacity and Clinical Worsening in Patients with Dual down and Eisenmenger Syndrome

Serino

Guazzi

Micheletti

et al. 2013

Clin Med Insights Cardiol

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This single-center, retrospective analysis evaluated long-term bosentan treatment in adult patients (n = 7) with both Down and Eisenmenger syndromes (DS-ES). Laboratory tests, 6-minute walk distance (6MWD), functional class, and Doppler echocardiography were assessed at baseline and during 2 years’ follow-up. Improvements or maintenance of 6MWD were observed (68 m improvement from baseline at month 12) after bosentan initiation. 6MWD was maintained up to year 2. Overall, 6 patients experienced a significant improvement in functional class during 2 years’ therapy (P = 0.01). There were no significant changes in parameters measured by Doppler echocardiography. None of the patients required either hospitalization or additional pulmonary arterial hypertension (PAH) therapy because of PAH progression. Bosentan treatment was generally well tolerated; no liver function abnormalities or serious adverse drug reactions were noted. In this DS-ES cohort, bosentan seemed to be well tolerated and clinically effective.

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Section: Introductionmentioning

confidence: 99%

Effect of Bosentan on Exercise Capacity and Clinical Worsening in Patients with Dual down and Eisenmenger Syndrome

Serino

Guazzi

Micheletti

et al. 2013

Clin Med Insights Cardiol

View full text Add to dashboard Cite

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Pediatric Pulmonary Hypertension: Definitions, Mechanisms, Diagnosis, and Treatment

Mukherjee

Konduri

2021

Comprehensive Physiology

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Pediatric pulmonary hypertension (PPH) is a multifactorial disease with diverse etiologies and presenting features. Pulmonary hypertension (PH), defined as elevated pulmonary artery pressure, is the presenting feature for several pulmonary vascular diseases. It is often a hidden component of other lung diseases, such as cystic fibrosis and bronchopulmonary dysplasia. Alterations in lung development and genetic conditions are an important contributor to pediatric pulmonary hypertensive disease, which is a distinct entity from adult PH. Many of the causes of pediatric PH have prenatal onset with altered lung development due to maternal and fetal conditions. Since lung growth is altered in several conditions that lead to PPH, therapy for PPH includes both pulmonary vasodilators and strategies to restore lung growth. These strategies include optimal alveolar recruitment, maintaining physiologic blood gas tension, nutritional support, and addressing contributing factors, such as airway disease and gastroesophageal reflux. The outcome for infants and children with PH is highly variable and largely dependent on the underlying cause. The best outcomes are for neonates with persistent pulmonary hypertension (PPHN) and reversible lung diseases, while some genetic conditions such as alveolar capillary dysplasia are lethal.

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Treatment of pediatric pulmonary arterial hypertension: A focus on the NO‐sGC‐cGMP pathway

Beghetti¹,

Gorenflo

Ivy

et al. 2019

Pediatric Pulmonology

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Objective While pulmonary arterial hypertension (PAH) is rare in infants and children, it results in substantial morbidity and mortality. In recent years, prognosis has improved, coinciding with the introduction of new PAH‐targeted therapies, although much of their use in children is off‐label. Evidence to guide the treatment of children with PAH is less extensive than for adults. The goal of this review is to discuss the treatment recommendations for children with PAH, as well as the evidence supporting the use of prostanoids, endothelin receptor antagonists (ERAs), and phosphodiesterase type 5 inhibitors (PDE5i) in this setting. Data Sources Nonsystematic PubMed literature search and authors’ expertise. Study Selection Articles were selected concentrating on the nitric oxide (NO)‐soluble guanylate cyclase (sGC)‐cyclic guanosine monophosphate (cGMP) pathway in PAH. The methodology of an ongoing study evaluating the sGC stimulator riociguat in children with PAH is also described. Results Despite recent medical advances, improved therapeutic strategies for pediatric PAH are needed. The efficacy and tolerability of riociguat in adults with PAH have been well trialed. Conclusion The pooling of data across trials, supplemented by registry data, will help to confirm the safety and tolerability of prostanoids, ERAs, and PDE5i in children. Ongoing studies will clarify the place of sGC stimulators in the treatment strategy for pediatric PAH.

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Tratamiento con sildenafilo y/o bosentán en niños y jóvenes con hipertensión arterial pulmonar idiopática y síndrome de Eisenmenger

Cited by 17 publications

References 26 publications

Effect of Bosentan on Exercise Capacity and Clinical Worsening in Patients with Dual down and Eisenmenger Syndrome

Effect of Bosentan on Exercise Capacity and Clinical Worsening in Patients with Dual down and Eisenmenger Syndrome

Pediatric Pulmonary Hypertension: Definitions, Mechanisms, Diagnosis, and Treatment

Treatment of pediatric pulmonary arterial hypertension: A focus on the NO‐sGC‐cGMP pathway

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