ArticlePulmonary circulation
First-in-child use of the oral soluble guanylate cyclase stimulator riociguat in pulmonary arterial hypertension.
Article in Pulmonary circulation. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 35 citations in OpenAlex.
- Advances in targeted therapy for pulmonary arterial hypertension in children.European journal of pediatrics · 2023Review
- Pulmonary vasodilator strategies in neonates with acute hypoxemic respiratory failure and pulmonary hypertension.Seminars in fetal & neonatal medicine · 2022Review
- Riociguat in children with pulmonary arterial hypertension: The PATENT-CHILD study.Pulmonary circulation · 2022Article
- Early diagnosis and targeted approaches to pulmonary vascular disease in bronchopulmonary dysplasia.Pediatric research · 2022Review
- Evaluating Riociguat in the Treatment of Pulmonary Arterial Hypertension: A Real-World Perspective.Vascular health and risk management · 2022Review
- Novel use of riociguat in infants with severe pulmonary arterial hypertension unable to wean from inhaled nitric oxide.Frontiers in pediatrics · 2022Article
- Successful Management of an Infant with Atypical Presentation of Alveolar Capillary Dysplasia with Misalignment of the Pulmonary Veins.Journal of pediatric intensive care · 2021Article
- Treatment of pulmonary arterial hypertension in children.Cardiovascular diagnosis and therapy · 2021Review
- Pediatric Pulmonary Hypertension: Definitions, Mechanisms, Diagnosis, and Treatment.Comprehensive Physiology · 2021Article
- Drug Treatment of Pulmonary Hypertension in Children.Paediatric drugs · 2020Review
- Therapies that enhance pulmonary vascular NO-signaling in the neonate.Nitric oxide : biology and chemistry · 2020Review
- Diagnosis, Evaluation and Treatment of Pulmonary Arterial Hypertension in Children.Children (Basel, Switzerland) · 2018Review
Corrections and comments
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Riociguat has been approved for use in adults with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension. No clinical data on its therapeutic use in children with PAH are currently available. We report the case of a now four-year-old boy who initially presented at the age of 10 months with suprasystemic pulmonary hypertension (PH) and right ventricular (RV) failure, vomiting, peripheral cyanosis, and failure to thrive. Cardiac catheterization revealed severe PAH. At radiologic suspicion of interstitial lung disease, repeated CT scan and an open lung biopsy were performed but could not clarify the entity of PAH. Given the demonstrated vasoreactivity, the boy was started on the calcium channel blocker amlodipine, in combination with the endothelin-1 receptor antagonist bosentan. Two years later, based on persistently systemic PAH with lost vasoreactivity, PAH therapy was changed to bosentan and phosphodiesterase-5 inhibitor sildenafil. No significant improvement on the aforementioned therapy was seen, so that the patient was referred to our institution. Invasive hemodynamic evaluation showed suprasystemic PAH and marked acute vasoreactivity (PAP 127/103/83 mmHg, PVRi 23.48 WU·m
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