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

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 International Conference on Neonatal & course Childhood Pulmonary Vascular Disease Advanced Nursing Workshop Emma Olson Jackson, PNP & Anne Davis, RN March 7, 2019 Objectives Introduction • Down syndrome affects ~600-800 live births • Discuss risk factors that increase the chance of • Up to ~25% of patients with Down syndrome have PH developing PH for patients with Down Syndrome • Specific risk factors and comorbidities increase chance of PH (Bush, et al.) • PH can occur: • Understand common comorbidities for patients • Transiently with Down Syndrome and PH • PPHN or resolution after repair of CHD • Persistent and Progressive • Discuss guidelines for screening Down • no resolution of PH Syndrome patients for specific comorbidities • Recurrent • when PH returns after a previous resolution 1

3/12/2019 Background, Classification, & Incidence Background, Classification, & Incidence • WHO Group I: Most frequent classification for • Large cohort of Down syndrome patients children with PH and DS followed at Denver Children’s Hospital • CHD, PPHN, or combo of the two (n=1,252) (Bush, et al.) CHD ~50% of all patients with DS (AAP) • 28% identified as having PH PPHN ~5% (Netherlands data) • 82% had associated CHD • significantly higher than 0.1% in general population • 45% had PPHN • Most common cardiac malformations are • Vast majority presented with PH in infancy AVC, PDA, ASD, VSD • Age at first diagnosis for entire cohort = 5 days • AAP recommends echo in the first month of life • Non PPHN patients average age at first • Most diagnosed and repaired in infancy diagnosis = 96 days Background, Classification, & Incidence Pulmonary Management • Identify primary pediatric pulmonologist for WHO Group III: most common etiology of Common respiratory co-morbidities include: Down syndrome patients with PH PH after infancy or with recurrence • Obstructive Sleep Apnea (OSA) • 87% of children experiencing a second • Intermittent hypoxia • co-follow with PH team indefinitely episode of PH (after previous resolution) • Recurrent pneumonia • Regular screening for comorbid classified as WHO Group III (Bush, et al.) • Chronic aspiration respiratory conditions (despite presence Inadequate alveolarization of terminal lung units in patients with DS or absence of symptoms) • 58-83% of predicted alveoli present • Goal is to prevent the recurrence of PH in this high (autopsy studies) risk population • Avoid screening only after PH becomes apparent PH develops more rapidly from hypoxia 2

3/12/2019 Chronic Aspiration Chronic Aspiration • AAP recommends: • Frequently overlooked cause of PH and other respiratory symptoms in DS • Swallow study (VFSS) for any DS child with marked hypotonia, slow • DS children are at significant risk due to: feeding, choking with feeds, recurrent respiratory symptoms, FTT • Delayed oral development • No recommendations for routine screening • Structural abnormalities • We recommend VFSS for all DS children (despite presence or absence of symptoms): • Hypotonia • Initial comprehensive evaluation for new PH diagnosis • UK patients followed in Sleep Clinic: • Recurrence of previously resolved PH • 16/17 CT scans showed signs of aspiration • No improvement or worsening PH • Denver cohort: • Periodically for those with history of diagnosed aspiration • 35% of all Down syndrome patients with PH had aspiration • All patients with presumed or confirmed aspiration should be • 48% of patients with recurrent PH had aspiration regularly followed by feeding therapist and pulmonologist for ongoing assessment and monitoring Obstructive Sleep Apnea Obstructive Sleep Apnea • AAP recommends: • 30-79% of patients with DS • Sleep study by age 4 years for all children with DS • compared with 2% of general peds population • Sooner if symptomatic • Denver cohort • We recommend: • Standardized approach rather than depending on parental report • 78% of those with PH were also diagnosed • New PH diagnosis or new recurrence with OSA • sleep study with initial evaluation • Parental report is problematic • Resolved or controlled PH • Parents consistently underestimate sleep • Annual Sleep Clinic evaluation disordered breathing • Sleep specialists help determine frequency of sleep study need • 69% denied sleep problems yet 54% of those • No improvement or worsening PH same children had abnormal sleep study • Sleep study every 1-2 years (sooner if highly suspicious of poorly treated OSA) (Trucco, et al.) • Reassessment imperative after any airway surgery (adenotonsillectomy or other) 3

3/12/2019 Parenchymal and Structural Airway Disease Parenchymal and Structural Airway Disease • Common structural airway abnormalities: • DS children can have primary parenchymal lung • Macroglossia disease, but more likely to have diffuse parenchymal • Tonsil and adenoid hypertrophy disease from secondary causes such as: • Laryngomalacia • Tracheobronchomalacia • Post-infectious changes • Subglottic stenosis • Chronic lung disease of prematurity • Tracheal stenosis • Chronic aspiration • Airway abnormalities predispose patients to intermittent hypoxia which can • Screening for pulmonary abnormalities with advanced lead to PH imaging: • 50% of patients with upper airway obstruction had PH with 91% resolution of PH following surgery (Jacobs, et al.) • High resolution chest CT at the time of PH diagnosis or reoccurrence PH Center at Seattle Children’s Hospital Parenchymal and Structural Airway Disease Guidelines • Both inpatient and outpatient PH referrals too often come • DS patients may not respond to pulmonary after months to years of unrecognized pulmonary insults 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 • Our hope is that comorbidities are recognized and initiation of PH specific therapy treated prior to the development of or worsening of PH • Pediatric PH Guidelines from the AHA and ATS (2015) • Ideally prior to referral to the PH Center • Highlight importance of identifying and treating primary or • Also as part of the ongoing, comprehensive care of the child with secondary respiratory disease PRIOR TO initiation of long term Down syndrome and PH pulmonary vasodilator therapy 4

3/12/2019 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 of 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 5