3/12/2019 Background, Classification, & Incidence Background, - - PDF document

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3/12/2019 Background, Classification, & Incidence Background, - - PDF document

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3/12/2019 Comprehensive Evaluation and Ongoing Approach to Children With Disclosures: Both presenters have no Down Syndrome and Pulmonary relevant financial relationships with any Hypertension companies related to the content of this 12 th

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Comprehensive Evaluation and Ongoing Approach to Children With Down Syndrome and Pulmonary Hypertension

12th International Conference on Neonatal & Childhood Pulmonary Vascular Disease Advanced Nursing Workshop Emma Olson Jackson, PNP & Anne Davis, RN March 7, 2019

Disclosures: Both presenters have no

relevant financial relationships with any companies related to the content of this course

Objectives

  • Discuss risk factors that increase the chance of

developing PH for patients with Down Syndrome

  • Understand common comorbidities for patients

with Down Syndrome and PH

  • Discuss guidelines for screening Down

Syndrome patients for specific comorbidities

Introduction

  • Down syndrome affects ~600-800 live births
  • Up to ~25% of patients with Down syndrome have PH
  • Specific risk factors and comorbidities increase chance of PH (Bush, et al.)
  • PH can occur:
  • Transiently
  • PPHN or resolution after repair of CHD
  • Persistent and Progressive
  • no resolution of PH
  • Recurrent
  • when PH returns after a previous resolution
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Background, Classification, & Incidence

  • WHO Group I: Most frequent classification for

children with PH and DS

  • CHD, PPHN, or combo of the two

CHD ~50% of all patients with DS (AAP) PPHN ~5% (Netherlands data)

  • significantly higher than 0.1% in general

population

  • Most common cardiac malformations are

AVC, PDA, ASD, VSD

  • AAP recommends echo in the first month of life
  • Most diagnosed and repaired in infancy

Background, Classification, & Incidence

  • Large cohort of Down syndrome patients

followed at Denver Children’s Hospital (n=1,252) (Bush, et al.)

  • 28% identified as having PH
  • 82% had associated CHD
  • 45% had PPHN
  • Vast majority presented with PH in infancy
  • Age at first diagnosis for entire cohort = 5 days
  • Non PPHN patients average age at first

diagnosis = 96 days

Background, Classification, & Incidence

WHO Group III: most common etiology of PH after infancy or with recurrence

  • 87% of children experiencing a second

episode of PH (after previous resolution) classified as WHO Group III (Bush, et al.)

Inadequate alveolarization of terminal lung units in patients with DS

  • 58-83% of predicted alveoli present

(autopsy studies)

PH develops more rapidly from hypoxia Common respiratory co-morbidities include:

  • Obstructive Sleep Apnea (OSA)
  • Intermittent hypoxia
  • Recurrent pneumonia
  • Chronic aspiration

Pulmonary Management

  • Identify primary pediatric pulmonologist for

Down syndrome patients with PH

  • co-follow with PH team indefinitely
  • Regular screening for comorbid

respiratory conditions (despite presence

  • r absence of symptoms)
  • Goal is to prevent the recurrence of PH in this high

risk population

  • Avoid screening only after PH becomes apparent
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Chronic Aspiration

  • Frequently overlooked cause of PH and other respiratory symptoms in DS
  • DS children are at significant risk due to:
  • Delayed oral development
  • Structural abnormalities
  • Hypotonia
  • UK patients followed in Sleep Clinic:
  • 16/17 CT scans showed signs of aspiration
  • Denver cohort:
  • 35% of all Down syndrome patients with PH had aspiration
  • 48% of patients with recurrent PH had aspiration

Chronic Aspiration

  • AAP recommends:
  • Swallow study (VFSS) for any DS child with marked hypotonia, slow

feeding, choking with feeds, recurrent respiratory symptoms, FTT

  • No recommendations for routine screening
  • We recommend VFSS for all DS children (despite presence or

absence of symptoms):

  • Initial comprehensive evaluation for new PH diagnosis
  • Recurrence of previously resolved PH
  • No improvement or worsening PH
  • Periodically for those with history of diagnosed aspiration
  • All patients with presumed or confirmed aspiration should be

regularly followed by feeding therapist and pulmonologist for

  • ngoing assessment and monitoring

Obstructive Sleep Apnea

  • 30-79% of patients with DS
  • compared with 2% of general peds population
  • Denver cohort
  • 78% of those with PH were also diagnosed

with OSA

  • Parental report is problematic
  • Parents consistently underestimate sleep

disordered breathing

  • 69% denied sleep problems yet 54% of those

same children had abnormal sleep study

(Trucco, et al.)

Obstructive Sleep Apnea

  • AAP recommends:
  • Sleep study by age 4 years for all children with DS
  • Sooner if symptomatic
  • We recommend:
  • Standardized approach rather than depending on parental report
  • New PH diagnosis or new recurrence
  • sleep study with initial evaluation
  • Resolved or controlled PH
  • Annual Sleep Clinic evaluation
  • Sleep specialists help determine frequency of sleep study need
  • No improvement or worsening PH
  • Sleep study every 1-2 years (sooner if highly suspicious of poorly treated OSA)
  • Reassessment imperative after any airway surgery (adenotonsillectomy or other)
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Parenchymal and Structural Airway Disease

  • Common structural airway abnormalities:
  • Macroglossia
  • Tonsil and adenoid hypertrophy
  • Laryngomalacia
  • Tracheobronchomalacia
  • Subglottic stenosis
  • Tracheal stenosis
  • Airway abnormalities predispose patients to intermittent hypoxia which can

lead to PH

  • 50% of patients with upper airway obstruction had PH with 91% resolution
  • f PH following surgery (Jacobs, et al.)

Parenchymal and Structural Airway Disease

  • DS children can have primary parenchymal lung

disease, but more likely to have diffuse parenchymal disease from secondary causes such as:

  • Post-infectious changes
  • Chronic lung disease of prematurity
  • Chronic aspiration
  • Screening for pulmonary abnormalities with advanced

imaging:

  • High resolution chest CT at the time of PH diagnosis or reoccurrence

Parenchymal and Structural Airway Disease

  • DS patients may not respond to pulmonary

vasodilators in the same way as patients without DS

(Beghetti, et al.)

  • Potentially indicates that ongoing pulmonary (or other) insults

have not been properly identified or treated correctly prior to initiation of PH specific therapy

  • Pediatric PH Guidelines from the AHA and ATS (2015)
  • Highlight importance of identifying and treating primary or

secondary respiratory disease PRIOR TO initiation of long term pulmonary vasodilator therapy

PH Center at Seattle Children’s Hospital Guidelines

  • Both inpatient and outpatient PH referrals too often come

after months to years of unrecognized pulmonary insults

  • Our hope is that comorbidities are recognized and

treated prior to the development of or worsening of PH

  • Ideally prior to referral to the PH Center
  • Also as part of the ongoing, comprehensive care of the child with

Down syndrome and PH

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Questions & Discussion References

  • Abman, S. H., Hansmann, G., Archer, S. L., Ivy, D. D., Adatia, I., Chung, W. K., . . . the American Thoracic, S. (2015). Pediatric Pulmonary Hypertension:

Guidelines From the American Heart Association and American Thoracic Society. Circulation, 132(21), 2037-2099. doi:10.1161/CIR.0000000000000329

  • Beghetti, M., Rudzinski, A., & Zhang, M. (2017). Efficacy and safety of oral sildenafil in children with Down syndrome and pulmonary hypertension. BMC

Cardiovasc Disord, 17(1), 177. doi:10.1186/s12872-017-0569-3

  • Bull, M. J., & Committee on, G. (2011). Health supervision for children with Down syndrome. Pediatrics, 128(2), 393-406. doi:10.1542/peds.2011-1605
  • Bush, D., Galambos, C., Ivy, D. D., Abman, S. H., Wolter-Warmerdam, K., & Hickey, F. (2018). Clinical Characteristics and Risk Factors for Developing

Pulmonary Hypertension in Children with Down Syndrome. J Pediatr, 202, 212-219 e212. doi:10.1016/j.jpeds.2018.06.031

  • Espinola-Zavaleta, N., Soto, M. E., Romero-Gonzalez, A., Gomez-Puente Ldel, C., Munoz-Castellanos, L., Gopal, A. S., . . . Lupi-Herrera, E. (2015). Prevalence
  • f Congenital Heart Disease and Pulmonary Hypertension in Down's Syndrome: An Echocardiographic Study. J Cardiovasc Ultrasound, 23(2), 72-77.

doi:10.4250/jcu.2015.23.2.72

  • Fitzgerald, D. A., Paul, A., & Richmond, C. (2007). Severity of obstructive apnoea in children with Down syndrome who snore. Arch Dis Child, 92(5), 423-425.

doi:10.1136/adc.2006.111591

  • Jacobs, I. N., Gray, R. F., & Todd, N. (1996). Upper airway obstruction in children with down syndrome. Archives of Otolaryngology–Head & Neck Surgery,

122(9), 945-950. doi:10.1001/archotol.1996.01890210025007

  • McDowell, K. M., & Craven, D. I. (2011). Pulmonary complications of Down syndrome during childhood. J Pediatr, 158(2), 319-325.

doi:10.1016/j.jpeds.2010.07.023

  • Trucco, F., Chatwin, M., Semple, T., Rosenthal, M., Bush, A., & Tan, H. L. (2018). Sleep disordered breathing and ventilatory support in children with Down
  • syndrome. Pediatr Pulmonol, 53(10), 1414-1421. doi:10.1002/ppul.24122
  • Weijerman, M. E., van Furth, A. M., van der Mooren, M. D., van Weissenbruch, M. M., Rammeloo, L., Broers, C. J., & Gemke, R. J. (2010). Prevalence of

congenital heart defects and persistent pulmonary hypertension of the neonate with Down syndrome. Eur J Pediatr, 169(10), 1195-1199. doi:10.1007/s00431- 010-1200-0