Children with Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension Treated with Pulmonary Vasodilators—The Pediatric Cardiologist Point of View

Migdal, Anna; Sadel-Wieczorek, Anna; Ryciak, Edyta; Mirecka-Rola, Alicja; Brzezińska‐Rajszys, Grażyna; Żuk, Małgorzata

doi:10.3390/children8050326

Cited by 2 publications

(2 citation statements)

References 29 publications

(63 reference statements)

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“…Bosentan is mostly used in combination with sildenafil in infants with severe PH, in whom sildenafil does not provide control of symptoms. 24 It is frequently associated with side effects, including liver toxicity.…”

Section: Traditional Vasodilator Therapies Regulation Of the Vascular...mentioning

confidence: 99%

The vascular phenotype of BPD: new basic science insights—new precision medicine approaches

Durlak

Thébaud

2022

Pediatr Res

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Bronchopulmonary dysplasia (BPD) is the most common complication of preterm birth. Up to 1/3 of children with BPD develop pulmonary hypertension (PH). PH increases mortality, the risk of adverse neurodevelopmental outcome and lacks effective treatment. Current vasodilator therapies address symptoms, but not the underlying arrested vascular development. Recent insights into placental biology and novel technological advances enabling the study of normal and impaired lung development at the single cell level support the concept of a vascular phenotype of BPD. Dysregulation of growth factor pathways results in depletion and dysfunction of putative distal pulmonary endothelial progenitor cells including Cap1, Cap2, and endothelial colony-forming cells (ECFCs), a subset of vascular progenitor cells with self-renewal and de novo angiogenic capacity. Preclinical data demonstrate effectiveness of ECFCs and ECFC-derived particles including extracellular vesicles (EVs) in promoting lung vascular growth and reversing PH, but the mechanism is unknown. The lack of engraftment suggests a paracrine mode of action mediated by EVs that contain miRNA. Aberrant miRNA signaling contributes to arrested pulmonary vascular development, hence using EV-and miRNAbased therapies is a promising strategy to prevent the development of BPD-PH. More needs to be learned about disrupted pathways, timing of intervention, and mode of delivery.

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Section: Traditional Vasodilator Therapies Regulation Of the Vascular...mentioning

confidence: 99%

The vascular phenotype of BPD: new basic science insights—new precision medicine approaches

Durlak

Thébaud

2022

Pediatr Res

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“…Pulmonary hypertension is a rare disease in children that is characterized by a series of vascular changes that result in increased pulmonary vascular resistance and eventually right heart failure [1] [2] [3]. Despite varying etiologies, pulmonary hypertension in the pediatric age group carries a poor prognosis if left untreated [2] [4] [5] [6] [7]. Significant therapeutic advances have been made over the past several decades; this is due in part to an improved understanding of the mechanisms involved in the pathogenesis of this disease.…”

Section: Introductionmentioning

confidence: 99%

Bosentan Is Associated with a Reduction in Right Ventricular Systolic Pressure N-Terminal Pro-Hormone B-Type Natriuretic Peptide Levels in Young Patients with Pulmonary Hypertension

Landry¹,

Burks²,

Osakwe³

et al. 2023

OJPed

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Pulmonary hypertension is a rare and potentially fatal disease in children if left untreated. Emerging therapies, including Bosentan, a dual endothelin receptor antagonist, have shown significant benefits in the adult pulmonary hypertension population; however, few studies have assessed the efficacy and safety of endothelin receptor antagonists in infants and young children. Our study was a single-center retrospective analysis of patients less than two years of age with a confirmed diagnosis of pulmonary hypertension and initiated on Bosentan therapy between 2017 and 2020. Twelve cases met eligibility criteria. Demographic data, laboratory data, echocardiographic, and cardiac catheterization data were analyzed. With treatment, there was a statistically significant decrease in mean right ventricular systolic pressure estimated by the tricuspid regurgitation jet (79 ± 23 mmHg reduced to 52 ± 25 mmHg; p < 0.001) N-terminal pro-hormone B-type natriuretic peptide levels (21,071 reduced to 2,037; p < 0.001). Additionally, improvement and eventual normalization of right ventricular function and septal geometry was seen within the first four months of therapy. Patients who underwent cardiac catheterization after therapy initiation (n = 4) demonstrated hemodynamic improvements; however, only the decrease in diastolic pulmonary artery pressure was statistically significant (p = 0.018). No significant differences in hemoglobin, platelet count, or liver function tests were observed between groups.

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Children with Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension Treated with Pulmonary Vasodilators—The Pediatric Cardiologist Point of View

Cited by 2 publications

References 29 publications

The vascular phenotype of BPD: new basic science insights—new precision medicine approaches

The vascular phenotype of BPD: new basic science insights—new precision medicine approaches

Bosentan Is Associated with a Reduction in Right Ventricular Systolic Pressure N-Terminal Pro-Hormone B-Type Natriuretic Peptide Levels in Young Patients with Pulmonary Hypertension

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