Clinical Characteristics and Risk Factors for Developing Pulmonary Hypertension in Children with Down Syndrome

Bush, Douglas; Galambos, Csaba; Ivy, D. Dunbar; Abman, Steven H.; Wolter‐Warmerdam, Kristine; Hickey, Francis

doi:10.1016/j.jpeds.2018.06.031

Cited by 94 publications

(128 citation statements)

References 31 publications

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“…In a large observational study, Yang and colleagues found that the risk of death from aspiration, pneumonia, or influenza for individuals with DS was more than seven times that of the general population . Multiple factors contribute to this risk including the high prevalence of congenital airway and lung abnormalities, obstructive sleep apnea, pulmonary hypertension, and dysphagia . Bronchiectasis, structural lung disease, and airway inflammation can be seen in any child with dysphagia and chronic aspiration from a young age .…”

Section: Discussionmentioning

confidence: 99%

“…Pulmonary hypertension specifically has been reported to occur in over 25% of children with DS. Comorbidities that result in intermittent hypoxia including chronic aspiration increase the likelihood of identifying pulmonary hypertension . Further study of the relationship between chronic aspiration and pulmonary hypertension and their contributions to morbidity and mortality in DS is warranted.…”

Section: Discussionmentioning

confidence: 99%

“…Comorbidities that result in intermittent hypoxia including chronic aspiration increase the likelihood of identifying pulmonary hypertension. 25 Further study of the relationship between chronic aspiration and pulmonary hypertension and their contributions to morbidity and mortality in DS is warranted. Improved understanding of these risks for pulmonary morbidity may allow earlier focused screening for comorbid conditions, proper intervention, and improved outcome.…”

Section: Discussionmentioning

confidence: 99%

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Associations between age, respiratory comorbidities, and dysphagia in infants with down syndrome

Jackson

Maybee

Wolter‐Warmerdam

et al. 2019

Pediatric Pulmonology

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Objectives: Children with Down syndrome (DS) have a high risk of dysphagia and the pediatric pulmonologist may be involved in diagnosis and management. The objective of this study is to evaluate the associations between age, dysphagia, and medical comorbidities in young children with DS. We hypothesized that swallow study findings are more likely to change in younger infants and that medical comorbidities may be associated with dysphagia. Study design: Results of videofluoroscopic swallow studies (VFSS) and fiberoptic endoscopic evaluation of swallowing (FEES) from 2010 to 2016 were collected retrospectively in children with DS with initial swallow study at less than 12 months of age. Results were analyzed for findings and change based on age at initial study, reason for referral, and medical comorbidities. Results: One hundred eleven infants with 247 VFSS and 14 FEES were included. Deep laryngeal penetration and/or aspiration were found in 31.9% of infants less than 6 months and 51.3% of infants 6 to 12 months. Children with initial swallow study performed at greater than or equal to 6 months of age were more likely (80.0%) to have unchanged findings on follow‐up study compared to children imaged at less than 6 months (35.3%). Laryngomalacia, pulmonary hypertension, pneumonia, and congenital cardiac disease were associated with dysphagia. Conclusion: We confirmed that dysphagia is common in infants with DS and comorbidities and provided preliminary evidence that swallow study findings may be more likely to change in children tested under 6 months of age. Providers should consider that results for instrumental swallow studies may change, particularly if the test was completed on a young infant.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Associations between age, respiratory comorbidities, and dysphagia in infants with down syndrome

Jackson

Maybee

Wolter‐Warmerdam

et al. 2019

Pediatric Pulmonology

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“…The natural history and outcome of intervention for CHDs in patients with Down syndrome varies. The presence of a CHD in Down syndrome increases the chance of pulmonary hypertension (Bush et al, 2018) and increases neonatal mortality (Cua, Haque, Santoro, Nicholson, & Backes, 2017). Patients with Down syndrome and single ventricle palliation are at high risk for procedural and long-term mortality (Peterson et al, 2019).…”

Section: Down Syndromementioning

confidence: 99%

Congenital heart defects associated with aneuploidy syndromes: New insights into familiar associations

Lin

Santoro

High

et al. 2019

American J of Med Genetics Pt C

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The frequent occurrence of congenital heart defects (CHDs) in chromosome abnormality syndromes is well‐known, and among aneuploidy syndromes, distinctive patterns have been delineated. We update the type and frequency of CHDs in the aneuploidy syndromes involving trisomy 13, 18, 21, and 22, and in several sex chromosome abnormalities (Turner syndrome, trisomy X, Klinefelter syndrome, 47,XYY, and 48,XXYY). We also discuss the impact of noninvasive prenatal screening (mainly, cell‐free DNA analysis), critical CHD screening, and the growth of parental advocacy on their surgical management and natural history. We encourage clinicians to view the cardiac diagnosis as a “phenotype” which supplements the external dysmorphology examination. When detected prenatally, severe CHDs may influence decision‐making, and postnatally, they are often the major determinants of survival. This review should be useful to geneticists, cardiologists, neonatologists, perinatal specialists, other pediatric specialists, and general pediatricians. As patients survive (and thrive) into adulthood, internists and related adult specialists will also need to be informed about their natural history and management.

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“…2 Most importantly, pediatric PH is intrinsically linked to issues of lung growth and development, including many prenatal and early postnatal influences. 7,8,[12][13][14][15][16] Pediatric PH often presents in the immediate neonatal period, which led to its own specific disease classification in Group 1 disease as PPHN. 2 The Pediatric Task Force further emphasized that PPHN represents a syndrome that is composed of specific diseases, ranging from its most common form as a transient disease after birth of term or near-term infants to more severe forms that include diverse developmental lung diseases and specific genetic disorders (Tables 1 and 2).…”

Section: Developmental Lung Diseasesmentioning

confidence: 99%

Clinical Characteristics and Risk Factors for Developing Pulmonary Hypertension in Children with Down Syndrome

Cited by 94 publications

References 31 publications

Associations between age, respiratory comorbidities, and dysphagia in infants with down syndrome

Associations between age, respiratory comorbidities, and dysphagia in infants with down syndrome

Congenital heart defects associated with aneuploidy syndromes: New insights into familiar associations

Pediatric Pulmonary Hypertension on the World Stage: Do We Need Separate Neonatal Guidelines?

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