0838 Cheyne-Stokes Breathing in a Pediatric Patient with Dilated Cardiomyopathy and Muscular Dystrophy Prior to Heart Transplant

Abstract

Introduction Cheyne-Stokes breathing (CSB) has rarely been identified in the pediatric population. Neuromuscular diseases (NMD) such as Duchene Muscular Dystrophy (DMD) can predispose patients to sleep-disordered breathing including central sleep apnea (CSA) and CSB. Sleep-disordered breathing in children with NMD may not have symptoms; thus, treatment can be delayed. Currently, there is limited data to support resolution of CSB in DMD with dilated cardiomyopathy post-transplant. Report of Cases: We present a 15-year old female with a significant history of both dilated cardiomyopathy and DMD who presented with acute on chronic heart failure. Due to her disease progression, she was listed for heart transplant. Prior to her transplant, she completed an inpatient polysomnography (PSG) to rule out sleep-disordered breathing due to concerns of snoring and dyspnea during sleep. Her Pediatric Daytime Sleepiness Scale score (PDSS) was 8. The polysomnogram recorded moderate obstructive sleep apnea (OSA) and central sleep apnea (CSA) consistent with Cheyne-Stokes breathing along with rare premature ventricular contractions (PVCs). Patient was started on BPAP of 13/8 cm H2O with a back-up rate of 12 breaths per minute after titration study. The patient subsequently received a heart transplant in which the patient’s dyspnea and snoring resolved. Post-transplant PSG pending to reassess the severity of sleep-disordered breathing. Conclusion Though CSA can be seen in children, CSB is rarely seen in children with either heart failure or muscular dystrophy. When CSB is observed, the cornerstone of treatment is correcting the underlying cause. This patient demonstrated CSB with symptoms that improved with BPAP and now post-heart transplant. When both heart failure and neuromuscular disease are involved, close monitoring for clinical symptoms along with screening for CSB is important and may affect overall quality of life and recovery. Support (If Any)