Abstract Introduction The link between sleep disordered breathing (SDB) and pulmonary hypertension (PH) in children is not well understood. Management of these patients with SDB and comorbid PH usually begins with treatment of the SDB with positive airway pressure therapy. In patients with SDB and severe PH, additional therapies might be necessary. We present a case of a child with severe obstructive sleep apnea (OSA)-associated PH, who underwent a multimodal treatment approach for stabilization. Report of case(s) A 10-year-old male with obesity, prediabetes and hypertriglyceridemia presented with extreme somnolence and desaturation to 80% on room air while seated in otolaryngology clinic. History elicited in Sleep Clinic the day prior included six-month history of snoring, frequent apnea and cyanosis. He was urgently admitted to the pediatric intensive care unit and empirically started and titrated on bilevel positive airway pressure to 20/10 cmH2O. His labs showed respiratory acidosis with metabolic compensation suggestive of chronic hypoventilation. An echocardiogram was obtained which showed systolic bowing of the interventricular septum with right ventricular hypertrophy (RVH) and low normal systolic function suggestive of severe PH. Inhaled nitric oxide was started for stabilization and transitioned to tadalafil. Polysomnogram showed a baseline apnea hypopnea index (AHI) of 178.3/hr. During the titration portion of the study, his OSA was only partially treated with a max pressure of 20/16 cmH2O so he was started on assured pressure support for targeted volume by non-invasive positive pressure ventilation (NIPPV). Surgical intervention was deferred until PH was improved. Over the next four months, parents reported less daytime sleepiness and absence of snoring. He had improved clinical assessment by six-minute walk test and successfully started a weight management program. Echocardiogram demonstrated normal septal motion and resolved RVH, so he underwent tonsillectomy adenoidectomy with a drug-induced sleep endoscopy by the otolaryngology service. He was maintained on tadalafil and NIPPV through surgery and post-operative period for continued rehabilitation. Conclusion This case highlights the need for a multi-disciplinary approach to the management and evaluation of patients with severe OSA-associated PH. Use of PH medications may be needed to afford clinical stabilization while SDB work up and management continues. Support (if any)
Wang et al. (Fri,) studied this question.