Risk factors for pulmonary arterial hypertension in children and young adults

Naumburg, Estelle; Söderström, Lars; Huber, Daniel; Axelsson, Inge

doi:10.1002/ppul.23633

Cited by 39 publications

(39 citation statements)

References 34 publications

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“…Long term, preterm birth is associated with an 8.5‐fold increased risk for developing pulmonary hypertension (defined as a mean pulmonary artery pressure ≥25 mmHg) in childhood and adolescence, and a 3.1‐fold increased risk in adulthood, even after adjusting for confounding factors such as acute pulmonary disorders, congenital heart defects, congenital diaphragmatic hernia and chromosomal disorders (Naumburg et al . , ). At rest, otherwise healthy young adults born preterm exhibit increased vascular stiffness with elevations in resting mean pulmonary arterial pressure (Goss et al .…”

Section: Impaired Pulmonary Vascularizationmentioning

confidence: 99%

Long‐term pulmonary vascular consequences of perinatal insults

Goss

2018

The Journal of Physiology

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Development of the pulmonary circulation is a critical component of fetal lung development, and continues throughout infancy and childhood, marking an extended window of susceptibility to vascular maldevelopment and maladaptation. Perinatal vascular insults may result in abnormal vascular structure or function, including decreased angiogenic signaling and vascular endowment, impaired vasoreactivity through increased pulmonary artery endothelial dysfunction and remodeling, or enhanced genetic susceptibility to pulmonary vascular disease through epigenetic modifications or germline mutations. Although some infants develop early onset pulmonary hypertension, due to the unique adaptive capabilities of the immature host many do not have clinically evident early pulmonary vascular dysfunction. These individuals remain at increased risk for development of late-onset pulmonary hypertension, and may be particularly susceptible to secondary insults. This review will address the role of perinatal vascular insults in the development of late pulmonary vascular dysfunction with an effort to highlight areas of critical research need.

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Section: Impaired Pulmonary Vascularizationmentioning

confidence: 99%

Long‐term pulmonary vascular consequences of perinatal insults

Goss

2018

The Journal of Physiology

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“…The incidence of PH in very low birth weight infants is estimated to range between 16 and 43% [5–8], and infants with bronchopulmonary dysplasia and PH have been suggested to have up to a 4.6-fold higher odds of mortality than those infants with bronchopulmonary dysplasia without PH [5–7, 9]. Further, infants who were born prematurely continue to have an increased risk for PH that persists into childhood and adolescence [10]. …”

Section: Introductionmentioning

confidence: 99%

Early characteristics of infants with pulmonary hypertension in a referral neonatal intensive care unit

et al. 2017

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BackgroundApproximately 8–23% of premature infants develop pulmonary hypertension (PH), and this diagnosis confers a higher possibility of mortality. As a result, professional societies recommend PH screening in premature infants. However, the risk factors for and the outcomes of PH may differ depending on the timing of its diagnosis, and little evidence is available to determine at-risk infants in the referral neonatal population. The objective of this study was to define clinical and echocardiographic characteristics of infants with pulmonary hypertension during the neonatal hospital course and at or near-term.MethodsInfants who had the following billing codes: < 32 weeks, birth weight < 1500 g, neonatal unit, and echocardiograph had records abstracted from a data warehouse at Children’s Healthcare of Atlanta. The outcome was defined as late PH on the final echocardiogram for all patients, and, separately, for patients with multiple studies. Descriptive statistics, univariable, and multivariable models were evaluated, and odds ratios and 95% confidence intervals are expressed below as (OR, CI).Results556 infants were included in the overall study, 59 had PH on their final echocardiogram (11%). In multivariable analyses, atrial septal defect (2.9, 1.4–6.1), and intrauterine growth restriction (2.7, 1.2–6.3) increased the odds of late PH, whereas caffeine therapy decreased PH (0.4, 0.2–0.8). When the analyses were restricted to 32 infants who had multiple echocardiograms during their hospitalization, the association between atrial septal defect (5.9, 2.0–16.5) and growth restriction (3.7, 1.3–10.7) and late PH was strengthened, but the effect of caffeine therapy was no longer significant. In this smaller subgroup, infants with late PH had their final echocardiogram at a median of 116 days of life, and 42–74% of them had right ventricular pathology.ConclusionsEarly clinical variables are associated with PH persistence in a referral neonatal population. Identification of early clinical factors may help guide the ascertainment of infant risk for late PH, and may aid in targeting sub-groups that are most likely to benefit from PH screening.

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“…Results from studies among children born within the old and new BPD eras suggest that they have an increased risk of subclinical pulmonary hypertensive vascular disease, exercise induced pulmonary hypertension, right ventricular dysfunction and autonomic dysfunction [82,[87][88][89][90]. Protocols for screening and diagnosing these adverse health outcomes, specifically pulmonary hypertensive vascular diseases, have been suggested [81,83,91].…”

Section: General Considerationsmentioning

confidence: 99%

European Respiratory Society guideline on long-term management of children with bronchopulmonary dysplasia

et al. 2019

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This document provides recommendations for monitoring and treatment of children in whom bronchopulmonary dysplasia (BPD) has been established and who have been discharged from the hospital, or who were >36 weeks of postmenstrual age. The guideline was based on predefined Population, Intervention, Comparison and Outcomes (PICO) questions relevant for clinical care, a systematic review of the literature and assessment of the evidence using the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) approach. After considering the balance of desirable (benefits) and undesirable (burden, adverse effects) consequences of the intervention, the certainty of the evidence, and values, the task force made conditional recommendations for monitoring and treatment of BPD based on very low to low quality of evidence. We suggest monitoring with lung imaging using ionising radiation in a subgroup only, for example severe BPD or recurrent hospitalisations, and monitoring with lung function in all children. We suggest to give individual advice to parents regarding daycare attendance. With regards to treatment, we suggest the use of bronchodilators in a subgroup only, for example asthma-like symptoms, or reversibility in lung function; no treatment with inhaled or systemic corticosteroids; natural weaning of diuretics by the relative decrease in dose with increasing weight gain if diuretics are started in the neonatal period; and treatment with supplemental oxygen with a saturation target range of 90–95%. A multidisciplinary approach for children with established severe BPD after the neonatal period into adulthood is preferable. These recommendations should be considered until new and urgently needed evidence becomes available.

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Risk factors for pulmonary arterial hypertension in children and young adults

Cited by 39 publications

References 34 publications

Long‐term pulmonary vascular consequences of perinatal insults

Long‐term pulmonary vascular consequences of perinatal insults

Early characteristics of infants with pulmonary hypertension in a referral neonatal intensive care unit

European Respiratory Society guideline on long-term management of children with bronchopulmonary dysplasia

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