Sleep disordered breathing is well described in adults with heart failure but not in pediatric population. We describe a 13-year-old Caucasian male with severe heart failure related to dilated cardiomyopathy who demonstrated polysomnographic features of Cheyne-Stokes respiration, which completely resolved following cardiac transplantation. Cheyne-Stokes respiration in children with advanced heart failure and its resolution after heart transplant can be observed similar to adults.
In adults with heart failure (HF), Cheyne-Stokes respiration (CSR) is found to be a common polysomnographic (PSG) finding [
The patient, a 13-year-old Caucasian male, presented with DCM and, after progressive deterioration of his ejection fraction, eventually required admission to the hospital for progressive HF. His symptoms were increasing daytime tiredness and lethargy as well as history of syncopal episodes and increased dyspnea on effort which all were attributed to the severe heart failure. His symptoms were New York Heart Association (NYHA) class III. Specifically, echocardiography showed deterioration of his cardiac function (decreased left ventricular ejection fraction [LVEF] from 30% to 9% over a two-month period). Medical therapy had included Digoxin, Carvedilol, Ramipril, and Spironolactone. He underwent a standard PSG according to international guidelines at the time of admission that was part of a prospective observational study to describe sleep disordered breathing (SDB) in children with known cardiomyopathy, the group data of which have been published elsewhere [
Cheyne-Stokes respiration in the child before heart transplant.
Four weeks after the baseline PSG, and before any treatment was initiated specifically for CSR, the patient underwent an orthotropic heart transplant. Following this, he was able to recover to normal NYHA status, with no symptoms, and normal cardiac function (LVEF was 61%). A follow-up baseline PSG was done 3 months after heart transplantation to reevaluate his sleep related breathing. At this time point, he was clinically stable. His medications at this time included Prednisone, Mycophenolate Mofetil, and Tacrolimus. His repeat PSG showed that his breathing pattern had normalized with no further evidence of CSR. Table
PSG results of the patient before and after heart transplant.
Results | Pretransplant | Posttransplant |
---|---|---|
TST, min | 358.5 | 389.5 |
Sleep latency, min | 10.8 | 1.8 |
Sleep efficiency, % | 96.5 | 91.5 |
REM latency, min | 159 | 167 |
Stage 1% TST | 0.6 | 1.4 |
Stage 2% TST | 59.6 | 54 |
Slow wave sleep % TST | 21.2 | 24.9 |
REM % TST | 18.7 | 19.6 |
Arousals, total index | 32.8 | 10 |
Mean sleep SaO2 (%) | 93 | 98 |
Minimum SaO2 (%) | 85 | 94 |
Highest TcCO2/etCO2 (mmHg) | 35 | 45 |
OAHI (events/hour) | 1.2 | 0.5 |
CAHI (events/hour) | 17.6 | 0.9 |
LVEF% (Simpsons) | 9 | 61 |
CAHI: central apnea-hypopnea index, etCO2: end tidal carbon dioxide, LVEDd: left ventricular diastolic dimension, LVEF: left ventricular ejection fraction, OAHI: obstructive apnea-hypopnea index, REM: rapid eye movements, SaO2: oxygen saturation, TcCO2: transcutaneous carbon dioxide, and TST: total sleep time.
In adults, CSR has been described in detail in patients with HF [
Central apneas during sleep occur when PaCO2 falls below the apnea threshold. CSR is present when central apneas occur in cyclical fashion. The phenomenon is thought to be related to respiratory control system instability as well as a delayed circulatory time in association with low cardiac output in HF [
To date, there are two cases of children in HF with description of CSR [
In children, cardiomyopathy is a rare but serious and life-threatening condition with an annual incidence of 1.1–1.2/100,000. Dilated cardiomyopathy is the dominant phenotype, with two-thirds of all cases remaining idiopathic after diagnostic evaluation. The prognosis of DCM in children is guarded with increased morbidity and mortality, and currently 32–46% of patients with DCM will either receive a heart transplant or die within five years of diagnosis. Our patient had severe DCM with severely impaired cardiac function and he received an urgent heart transplant because of his deteriorating condition.
Several adult case reports and series have shown resolution and improvement of CSR in patients with HF after heart transplant [
In conclusion CSR can be present in children with advanced heart failure. We speculate that it may contribute to the symptom progression in these patients and may be a risk factor for death for that reason, although there are insufficient cases to determine this at present. Resolution of CSR after pediatric heart transplant would be expected assuming no other contributory factors are identified. Prospective evaluation of patients for the presence of sleep related disordered breathing appears justified, even in the pediatric age group, when advanced heart failure is identified.
Central sleep apnea
Central apnea index
Cheyne-Stokes respiration
Dilated cardiomyopathy
Heart failure
Left ventricular ejection fraction
Obstructive sleep apnea
Obstructive apnea-hypopnea index
Polysomnography
Sleep disordered breathing.
The authors declare that they have no competing interests.
All authors were involved in the writing and editing of the paper. All authors read and approved the final paper.