Sudden unexpected death 12 years after “near-miss” sudden infant death syndrome in infancy

Sudden infant death syndrome (SIDS) is recognized as the largest single cause of death in infants between 1 week and 1 year of age in the U.S., accounting for the unexpected deaths of approximately 10,000 previously well infants annually.¹ Infants who survive episodes of respiratory or cardiorespiratory arrest have been described as having aborted, or “near-miss,” SIDS events, which are generally presumed to represent a variant of true SIDS.² Although “near-miss” occurrences may be repetitive, death rarely occurs in infancy.^2–3 However, the long-term significance of aborted SIDS events and the risk for subsequent catastrophe in these children is not known. The following case of an apparently healthy young girl with a history of “nearmiss” SIDS at age 6 weeks who died suddenly and unexpectedly in her sleep 12 years later may reflect an important and unappreciated aspect of the natural history of SIDS.     

Incomplete arousal processes in infants who were victims of sudden death

Infants who became victims of sudden infant death syndrome (SIDS) aroused less from sleep than control infants. This study was conducted to determine the characteristics of arousal from sleep of infants who eventually died of SIDS. Sixteen infants were monitored some days or weeks before they died of SIDS. Their polygraphic sleep recordings were compared with those of matched control infants. Arousals were scored as subcortical activation (incomplete arousals) or cortical arousal (complete arousals). Cortical arousals were significantly less frequent in the victims who would succumb to SIDS in the future than in the control infants during both REM and non-REM sleep (p = 0.039). The frequency (p = 0.017) and duration (p = 0.005) of subcortical activation were significantly greater in the infants who died of SIDS than in the control infants during REM sleep. Compared with the control infants, the infants who later died of SIDS had more frequent subcortical activation in the first part of the night, between 9:00 P.M. and 12:00 A.M. (p = 0.038), and fewer cortical arousals during the latter part of the night, between 3:00 and 6:00 A.M. (p = 0.011). The present data are suggestive of incomplete arousal processes in infants who eventually died at a time they were presumed to have been asleep.     

Cardiorespiratory adaptation during sleep in infants and children

The cardiorespiratory control system undergoes functional maturation after birth. Until this process is completed, the cardiorespiratory system is unstable, placing infants at risk for cardiorespiratory disturbances, especially during sleep. The profound influence of states of alertness on respiratory and cardiac control has been the focus of intense scrutiny during the last decade. The effects of rapid-eye movement (REM) sleep on various mechanisms involved in cardiorespiratory control are of particular significance during the postnatal period since newborns spend much of their time in this sleep state. In fullterm newborns, REM sleep occupies more than 50% of total sleep time, and this percentage is even greater in preterm newborns. From term to six months of age, the proportion of REM sleep decreases.² Since respiratory and cardiac disturbances are known to occur selectively during REM sleep, the predominance of REM sleep may be a risk factor for abnormal sleep-related events during early infancy.³ Awareness of these developmental changes in sleep patterns is important for clinicians dealing with problems such as apparent life-threatening events (ALTE), sudden infant death syndrome (SIDS), and/or cardiorespiratory responses to respiratory disorders. Our current understanding of respiratory and cardiac control rests mainly on studies conducted during the first months of life. There is a paucity of data on late infancy and early childhood. The present paper will review available data on how sleep affects 1) ventilatory mechanics, in particular of the upper airways and the chest wall; ventilation and apnea; gas exchange; chemoreceptor function; and arousal responses; 2) changes in heart rate and heart rate variability, and the occurrence and mechanisms of bradycardia. © 1995 Wiley-Liss, Inc.     

Hypercarbic ventilatory responses of infants at risk for SIDS

We examined the hypercarbic ventilatory responses (HVR) of 143 infants at risk for sudden infant death syndrome (SIDS) and 34 normal control infants. Sixty-five of the at-risk infants had experienced apparent life-threatening events (ALTE), and 78 were siblings of SIDS victims. Twenty-three (35%) of the ALTE infants experienced subsequent apnea; one died of SIDS. Seven (9%) of the SIDS siblings experienced subsequent apnea; two ultimately died of SIDS. In the HVR studies, we measured tidal volume (VT), minute ventilation (VE), frequency of breathing (f), and end-tidal PCO2 (PETCO2) at rest and while breathing 2% and 4% CO2. Mean HVR vales for the ALTE, sibling, and control groups were all similar. The mean HVR values for those at-risk infants who experienced subsequent apnea were not different from those who did not experience subsequent apnea. However, those infants experiencing subsequent apnea had higher mean VT/kg values (P less than 0.01) and lower mean PETCO2 values (P less than 0.001) than those who did not. The SIDS siblings had significantly lower resting VT/kg values than either the near-miss infants or normal controls (P less than 0.01). We did not find depressed HVR values in infants at risk for SIDS. On the contrary, those infants who experienced subsequent apnea had evidence suggesting relative hyperventilation. SIDS siblings had evidence suggesting relative hypoventilation. These findings are interesting and thought-provoking. However, HVR studies do not appear to be sensitive, specific, or appropriate for the general screening of infants at risk for SIDS.     

Higher SaO2 in chronic neonatal lung disease: Does it improve sleep?

Sleep fragmentation, decreased rapid eye movement (REM) sleep time, and REM sleep hypoxemia have been reported in infants with chronic neonatal lung disease (CNLD) in early infancy despite an awake hemoglobin oxygen saturation (SaO₂) >93%. Interestingly, higher inspired O₂ concentrations have been demonstrated to reduce REM sleep fragmentation in CNLD patients in middle infancy. However, the effect of increased SaO₂ on sleep architecture in infants with CNLD near the time of discharge from neonatal intensive care has not been reported. We performed paired overnight polysomnography in a sleep laboratory on 16 infants with CNLD (4 weeks median corrected age) in air or their usual inspired oxygen (SaO₂ >93%) and again when receiving 0.25 L/min higher than baseline inspired oxygen via nasal catheters (SaO₂ >97%). A control group of seven healthy preterm infants was similarly studied. For CNLD infants on supplemented O₂, sleep duration decreased by 15% (422 ± 66 min vs. 359 ± 89 min; P < 0.005), and sleep efficiency decreased by 7% (73.2 ± 10.6% vs. 66.4 ± 14.0%; P < 0.005) but percentage of time in REM sleep (REM%) (31.5 ± 8.9% vs. 29.8 ± 8.6%; P = 0.560), REM epoch duration (12.4 ± 2.8 min vs. 13.4 ± 4.3 min; P = 0.420), and REM arousal index (18.6 ± 6.5 vs. 18.8 ± 7.2; P = 0.990) were not significantly affected. Conversely, higher O₂ did not alter sleep architecture in the control group. The mean non-REM (NREM) respiratory rate decreased (CNLD: P = 0.003; controls: P = 0.02), NREM SaO₂ increased (P < 0.05), although the mean transcutaneous CO₂ was unaltered in both CNLD and control groups. This study confirmed low REM% in CNLD infants in early infancy and demonstrated that a higher SaO₂ adversely affected sleep time but did not influence REM sleep duration or arousal frequency. A target SaO₂ >93% is, therefore, as efficacious as an SaO₂ >97% in optimizing sleep architecture in CNLD infants. Pediatr Pulmonol. 1998; 26:235–240. © 1998 Wiley-Liss, Inc.     

Impaired objective daytime vigilance in obesity-hypoventilation syndrome: impact of noninvasive ventilation

BACKGROUND: Obesity-hypoventilation syndrome (OHS) is efficiently treated by noninvasive ventilation (NIV). Sleep respiratory disturbances, reduced ventilatory drive, and excessive daytime sleepiness (EDS) are commonly reported, but their relationships remain unclear. OBJECTIVES: To characterize sleep breathing disorders encountered in patients with OHS, to compare low and normal CO(2) responders in terms of sleep abnormalities, subjective and objective measures of EDS, and to measure the changes induced by NIV on these parameters. METHODS: At baseline and after 5 nights of NIV, 15 consecutive patients (mean [+/- SD] age, 55 +/- 9 years; mean body mass index, 38.7 +/- 6.1 kg/m(2); Paco(2), 47.3 +/- 2.3 mm Hg) prospectively underwent polysomnography, CO(2) ventilatory response testing, Epworth sleepiness scale scoring, and the Oxford Sleep Resistance (OSLER) test, which is an objective vigilance test. RESULTS: OHS patients exhibited obstructive sleep apnea syndrome (mean apnea-hypopnea index, 62 +/- 32 events per hour) and rapid eye movement (REM) sleep hypoventilation (mean REM sleep time, 35 +/- 33%). Baseline CO(2) sensitivity was significantly related to the proportion of hypoventilation during REM sleep (r = 0.54; p = 0.037). Six patients showed abnormal sleep latencies during the OSLER test (71% of the low CO(2) responders vs 14% of the normal CO(2) responders). Low CO(2) responders exhibited significantly shorter sleep latencies during the OSLER test (23 +/- 14 vs 37 +/- 8 min, respectively; p = 0.05). Using NIV, diurnal blood gas levels were improved and REM sleep hypoventilation were suppressed. Objective sleepiness was improved in low CO(2) responders (p = 0.04). CONCLUSION: In OHS patients, the lower the daytime CO(2) response, the higher the proportion of REM sleep hypoventilation and daytime sleepiness. Short-term therapy with NIV improves all of these parameters.     

Effect of triazolam on sleep and arterial oxygen saturation in patients with chronic obstructive pulmonary disease

We compared the effects of a placebo with 0.125 and 0.25 mg of triazolam (Halcion) on sleep quality, oximetry, and respiratory events during sleep in ten stable outpatients with chronic obstructive pulmonary disease. The subjects had a forced expiratory volume in 1 s ranging from 17% to 76% of the predicted value (mean +/- SD, 38.1% +/- 19%) and a waking arterial oxygen pressure from 46 to 84 mm Hg (mean +/- SD, 67 +/- 12 mm Hg). Polysomnography was done on three nights within a two-week period after the patients received on a "blinded" basis either placebo or 0.125 or 0.25 mg of triazolam. Triazolam produced improvements in total sleep duration, time spent in stage 2 nonrapid eye movement (NREM) sleep, and subjective of sleep quality. For most patients, there was a nighttime drop in arterial oxygen percentage of saturation (SaO2) in the placebo condition, but triazolam did not cause a significant worsening, of the mean SaO2, minimum SaO2, or the number of apneic and hypopneic events. Across all experimental conditions, we documented little desaturation during wakefulness (mean low, 87.2% +/- 10.2%), more during NREM sleep (mean low, 83.2% +/- 12.6%), and most desaturation in REM sleep (mean low, 80.1% +/- 15.7%). We conclude that single-night use of triazolam improved the quality and duration of sleep in patients with chronic obstructive pulmonary disease. In patients without severe waking hypoxemia and without carbon dioxide retention, triazolam did not increase either nocturnal hypoxemia or respiratory events during sleep.     

Respiration during sleep in children with COPD

Seventeen children (mean age, nine years) with chronic obstructive pulmonary disease (COPD) were studied during sleep. Electroencephalography, electrooculography, and electromyography were all recorded. Airflow was measured by nasal and oral thermistors, and abdominal and thoracic anteroposterior diameters by magnetometers. Transcutaneous partial pressure of O2 (tcPO2) and of CO2 (tcPCO2) were monitored. The average total sleep time was 283 min +/- 36 (1 SD). Breathing pauses (BP) five seconds or longer were measured. The mean time of BP expressed as a percentage of TST was 1.3 percent +/- 0.8 (1 SD). The BP occurred most frequently during REM sleep. Forty-six percent of BP were obstructive (OBP). The percentage of OBP was significantly related to the degree of lung resistance during wakefulness. Periodic breathing was observed with a mean frequency of 2.2 times per night (range: 0 to 7). Episodes with paradoxic inward rib cage motion were seen one to 29 times (mean 6.6). Drops in tcPCO2 greater than 5 mm Hg occurred one to eight times and 67 percent were observed during REM sleep. Compared to tcPCO2 during W the mean maximal decrease in tcPCO2 was 14 mm Hg (range 8 to 29). tcPCO2 rose with a mean maximal of 9.1 mm Hg (range 6 to 13). It was concluded that children with COPD had worsened gas exchange during sleep.     

The effect of rebreathing CO2 on ventilation and diaphragmatic electromyography in newborn infants

We tested the hypothesis of whether the reduced ventilatory response to CO2 in preterm as compared to term infants is related to primary central unresponsiveness, or to mechanical impairment of the respiratory pump. Eleven preterm (n = 19; gestational age 32 +/- 0.4 wk) and 14 term (n = 24; GA 40 +/- 0.3 wk) infants were studied. Minute integrated diaphragmatic activity EMGDi X f), and mean inspiratory diaphragmatic activity (EMGDi/TI), were used as indices of central output. After 3 min breathing 21% O2 (control), infants rebreathed from a bag containing 5% CO2 in 40% O2 for 2 to 3 minutes. We measured VE, VT, f, VT/TI. Sleep states were monitored. Preterm infants had a decreased ventilatory response to CO2 both in quiet sleep (QS) (0.0379 +/- 0.067 vs 0.505 +/- 0.032 L . (min . kg . kPa PACO2)-1; P less than 0.04) and in active sleep (AS) (0.210 +/- 0.032 vs 0.331 +/- 0.048 L . (min . kg . kPa PACO2)-1; P less than 0.04). The decrease in response primarily was a function of a lack of increase in tidal volume with CO2 in QS and a lack of increase in f in AS. Parallel to these changes there were significant correlations between the increases in EMGDi X f and VE with inhaled CO2 (r = 0.75; P less than 0.001); VT and EMGDi (r = 0.63; P less than 0.01); and between the increases in EMGDi/TI and VT/TI with inhaled CO2 (r = 0.64; P less than 0.001). The results suggest that ventilatory response to CO2 is (1) correlated highly with diaphragmatic indices of central output; (2) less in active than in quiet sleep; (3) less in preterm than in term infants. We conclude that despite their increased chest wall compliance, preterm infant respond less to CO2 because of central unresponsiveness.     

Hypoxemia during sleep in Duchenne muscular dystrophy

Overnight polysomnography after acclimatization was performed on 14 patients with Duchenne muscular dystrophy (mean age, 18.3 yr; mean VC, 1.24 L). Despite their lack of sleep-related symptoms and normal daytime blood gas tensions, periods of hypopnea and/or apnea (H/A) were observed in all patients (mean frequency 9.6/h; range, 3.7 to 17.0; mean duration 23.1; range of means, 16 to 36 s). In 9 patients, between 0.5 and 12.3 oxygen desaturations of greater than 5% occurred per hour, with falls from a mean SaO2 baseline of 95.4 +/- 0.6% (SEM) to a mean nadir of 74.2 +/- 3.9% (range, 58 to 90). This desaturating group (n = 9) showed longer and more frequent H/A than did the 5 nondesaturators; the proportion of REM sleep occupied by H/A was 37.7 +/- 3.8% in the desaturating group compared with only 15.1 +/- 5.1% in the remainder (p less than 0.01). The severity of sleep-disordered breathing could not be reliably predicted from daytime pulmonary function test results, and only maximal static expiratory pressure appeared significantly lower in the desaturating group. Hypopneas were associated with reduced chest wall movement in all subjects, and with chest wall paradox in one; continued submental "inspiratory" EMG activity throughout "central" apneas in 2 subjects suggested that these episodes were not truly central in origin. Sleep hypoxemia is imputed in the progression of several chronic respiratory diseases, and its prevention in Duchenne and related neuromuscular diseases may influence morbidity and mortality.     

Hypoxemia and hypercapnia during exercise and sleep in patients with cystic fibrosis.

BACKGROUND: In patients with cystic fibrosis (CF), it has been proposed that hypoxemia and hypercapnia occur during episodes of stress, such as exercise and sleep, and that respiratory muscle weakness because of malnutrition may be responsible. METHODS: Pulmonary function, respiratory muscle strength, and nutrition were assessed and correlated with the degree of hypoxemia and hypercapnia during exercise and sleep in 14 patients with CF and 8 control subjects. RESULTS: Despite no differences in maximum static inspiratory pressure (PImax) between the two groups, the CF group developed more severe hypoxemia (minimum oxyhemoglobin saturation [SpO2], 89 +/- 5% vs 96 +/- 2%; p < 0.001) and hypercapnia (maximum transcutaneous CO2 tension [PtcCO2], 43 +/- 6 vs 33 +/- 7 mm Hg; p < 0.01) during exercise. Similarly, during sleep, the CF group developed greater hypoxemia (minimum SpO2, 82 +/- 8% vs 91 +/- 2%; p < 0.005), although CO2 levels were not significantly different (maximum PtcCO2, 48 +/- 7 vs 50 +/- 2 mm Hg). Within the CF group, exercise-related hypoxemia and hypercapnia did not correlate with FEV1, residual volume/total lung capacity ratio (RV/TLC), PImax, or body mass index (BMI). Hypoxemia and hypercapnia during sleep correlated with markers of gas trapping (RV vs minimum arterial oxygen saturation [r = -0.654; p < 0.05]), RV vs maximum PtcCO2 (r = 0.878; p < 0.001), and RV/TLC vs maximum PtcCO2 (r = 0.790; p < 0.01) but not with PImax or BMI. CONCLUSION: Patients with moderately severe CF develop hypoxemia and hypercapnia during exercise and sleep to a greater extent than healthy subjects with similar respiratory muscle strength and nutritional status. Neither respiratory muscle weakness nor malnutrition are necessary to develop hypoxemia or hypercapnia during exercise or sleep.     

Treatment of respiratory failure during sleep in patients with neuromuscular disease. Positive-pressure ventilation through a nose mask

Severe nocturnal hypoxemia may occur in patients with respiratory muscle weakness caused by neuromuscular disorders. Negative pressure ventilators may be partially effective in these patients but can cause upper airway obstructive apneas. We examined the effectiveness of positive pressure ventilation through a nose mask in preventing nocturnal hypoxemia and compared it with negative pressure systems. We reasoned that nasal positive pressure would provide stability for the upper airway. Five patients with neuromuscular disorders underwent a series of all-night sleep studies under control conditions, negative pressure ventilation, and positive pressure ventilation through a comfortable nose mask. Sleep staging and respiratory variables were monitored during all studies. Daytime awake lung function, respiratory muscle strength, and arterial blood gases were also measured. The severe hypoxemia and hypercapnia that occurred under control conditions were prevented by positive pressure ventilation through a nose mask. Negative pressure ventilation improved NREM ventilation in all patients, but did not prevent severe oxyhemoglobin desaturation, which occurred during REM sleep. Negative pressure ventilation appears to contribute to upper airways obstruction during REM sleep as evidenced by cessation of air flow, reduced chest wall movements, falls in arterial oxyhemoglobin saturation, and hypercapnia. With treatment, daytime PaO2 improved from a mean of 70 to 83 mm Hg, and PaCO2 decreased from a mean of 61 to 46 mm Hg. We conclude that nasally applied positive pressure ventilation is a highly effective method of providing nocturnal assisted ventilation because it stabilizes the oropharyngeal airway.     

Polysomnographic characteristics in patients with Prader-Willi syndrome

To evaluate the occurrence of sleep-disordered breathing and to clarify the characteristics of sleep among patients with Prader-Willi syndrome (PWS). Overnight continuous EEG-polysomnographic studies were performed in 30 patients with PWS (16 males and 14 females; mean age, 7.4 +/- 4.1 years; age range, 1-19 years) unselected for sleep disturbance. The baseline arterial oxygen saturation (SpO2) was 96.6 +/- 0.6%, with a nadir of 77.2 +/- 10.2%. The rapid eye movement (REM) latency was 67.4 +/- 30.0 min. The percent of total sleep time spent in sleep stages 1, 2, slow wave, and REM were 13.1 +/- 8.2%, 41.9 +/- 10.5%, 21.5 +/- 9.4%, and 21.1 +/- 5.7%, respectively. The respiratory disturbance index (RDI) was 5.8 +/- 3.7/hr and desaturation index (DI) was 8.1 +/- 7.3/hr, respectively. Age-adjusted BMI was associated with more severe hypoxemia during sleep (baseline SpO2, r = -0.53, P < 0.01; nadir SaO2, r = -0.65, P < 0.01; RDI, r = 0.37, P < 0.05; DI, r = 0.53, P < 0.01) and more sleep disruption (arousal index, r = 0.46, P < 0.01). There were no significant associations between gender or genotype pattern (deletion vs. uniparental disomy) and the results of polysomnography. Sleep hypoxemia and sleep disruption are more prevalent in patients with PWS than in normal children. Obesity in these patients is associated with more severe sleep-disordered breathing.     

A possible mechanism for mixed apnea in obstructive sleep apnea

Hypopneas or pauses in respiratory effort frequently precede episodes of obstructive sleep apnea resulting in mixed apneas. We studied five subjects after chronic tracheostomy for obstructive sleep apnea. During stable non-REM (NREM) sleep, subjects breathed entirely through the tracheostomy. Tracheostomy occlusion caused experimental obstructive apnea which lasted 13.9 +/- 4.7 sec and ended with transient arousal and pharyngeal opening. At the end of the apnea there was marked hyperventilation (inspired minute ventilation rose 21.6 +/- 3.5 L on the first breath) followed by hypocapnia, hypopnea, and pauses in inspiratory effort as the subjects resumed NREM sleep. Hypocapnia was greater before inspiratory pauses lasting at least 5 sec than before shorter pauses (PETco2, 4.2 +/- 1.8 mm Hg below baseline vs 1.2 +/- 2.5 mm Hg below baseline). In three patients, pauses in inspiratory effort following experimental obstructive apnea were prevented by administration of 4 percent CO2 and 40 percent O2 inspired gas. This study suggests that: hyperventilation with hypocapnia occurs at the termination of obstructive apneas, and hypocapnia may be responsible for the attenuation or cessation of respiratory effort initiating the subsequent cycle of obstruction.     

Apnea duration and hypoxemia during REM sleep in patients with obstructive sleep apnea

Nocturnal sleep studies of 12 patients with obstructive sleep apnea and a matched control group of 12 subjects without the sleep apnea syndrome were analyzed to compare arterial oxyhemoglobin saturation (SaO2) during REM and non-REM sleep. Mean percentage of total sleep time spent in REM sleep was not significantly different in patients with obstructive sleep apnea and in subjects without significant apnea (14.2 +/- SEM 2.2 percent in patients vs 12.0 +/- 2.2 percent in nonapnea subjects). Apneas were longer during REM than non-REM sleep in all 12 patients (p less than 0.01). Oxyhemoglobin desaturations were more frequent during REM than non-REM sleep in both apnea patients and the control subjects. In addition, there was a greater mean fall in SaO2 per desaturation episode in both the apnea patients and non-apnea subjects. We conclude: 1) sleep apneas are longer during REM sleep than non-REM sleep in patients with obstructive sleep apnea; 2) hypoxemia is greater during REM sleep than non-REM sleep in subjects with and without the sleep apnea syndrome.     

Influence of Low-Flow Oxygen Supply on Sleep Architecture in Patients with Severe Heart Failure (NYHA III-IV) and Cheyne-Stokes Respiration

Patients suffering from severe heart failure may develop breathing pattern disorders during sleep, especially in the form of Cheyne-Stokes respiration. Results may be severe disturbances in sleep architecture and worsening of hemodynamics and of prognosis of these patients. Causes of the periodic breathing disorders are probably hypocapnia, hypersensitivity of respiratory control centers, hypoxemia, and prolonged blood circulation time. This study examined the influence of different concentrations of continously administered oxygen during the nighttime on breathing pattern disorders, oxygen saturation, and sleep architecture in 65 patients with severe heart failure (NYHA III–IV). Fifty-two of 65 patients showed an improvement of sleep architecture. Total sleeping time increased significantly (p < 0.01). Fragmentations of sleep by arousals decreased (p < 0.01); time of random eye movement (REM) sleep and non–REM sleep III and IV increased significantly.     

Action of the inspiratory muscles of the rib cage during breathing in newborns

To determine whether the rib cage muscles actively contribute to tidal volume change in infancy, we measured tidal volume (VT), using a pneumotachograph, respiratory gastric pressure swings (Pga), using a liquid-filled gastric catheter, and rib cage and abdominal volume, using respiratory inductive plethysmography in 15 newborns, both before and during 2% CO2-induced hyperventilation. Active rib cage expansion produced by phasic contraction of the inspiratory muscles of the rib cage should reduce respiratory abdominal pressure fluctuations by moving the anterior abdominal wall outward and cephalad, thereby having an expanding influence on the abdominal cavity. During quiet sleep (n = 13), CO2-induced hyperventilation was associated with significant increases in VT, Pga, rib cage volume (Vrc), and abdominal volume (Vab). Increments in Pga were small relative to VT, as shown by an increase in the slope of the VT versus Pga respiratory loop (VT/Pga) in all subjects (p less than 0.001, paired t test). CO2 breathing was associated with an increase in the contribution of the rib cage compartment to total volume change (Vrc/Vrc + Vab) in all infants studied (p less than 0.001, paired t test), and the total volume response to hyperventilation was more strongly related to changes in rib cage volume (slope = 0.62, r = 0.90) than to abdominal volume (slope = 0.31, r = 0.60). During REM sleep (n = 6), mean VT/Pga did not change significantly, and the rib cage contribution to tidal breathing decreased in three of six infants.(ABSTRACT TRUNCATED AT 250 WORDS)     

Sudden infant death syndrome (crib death).

Sudden infant death syndrome (SIDS) is diagnosed by the absence of lethal autopsy findings, or in a resuscitatable, "near miss" form with cyanosis, apnea, and bradycardia. The event is unexpected, although a minor respiratory infection is common, and occurs during sleep, between 1 and 6 months of age. There is growing evidence that the victims have had previous hypoxic episodes. Although suffocation is no longer considered a tenable explanation, other forms of airway obstruction are still postulated by many; the evidence, however, favors hypoxia as the common feature. A lethal arrhythmia had been proposed by several groups, based on inappropriate reflex activity, "pathology" of the conduction system, and the long QT syndrome, but the evidence is against arrhythmia as the primary event in most cases of SIDS. Based on the reversible "near miss," apnea is likely as the primary event in SIDS. Several reflexes have the ability to produce apnea, in addition to the relatively common sleep apnea; the crucial aspect, rather, appears to be thefailure of the immature infant to resume respiration. The possibility exists that the infant, who did not have to breather for 9 months of fetal life, literally is not alarmed and aroused by the persistance of apnea. In human and animal studies, respiratory infections and sleep deprivation have been proved to increase the likelihood and duration of sleep apnea. If primary apnea continues for long (45 seconds or more), a dangerous positive feedback develops into hypoxic apnea. Hhis will persist until circulatory failure occurs, or until gasping occurs. The gasp is a highly effective mechanism at birth, but will occur too late for autoresuscitation after the anerobic capacity of fetal life dimineshes; we believe this capacity lasts for approximately 1 month, accounting for the hiatus of crib death, sparing the first month. The "near-miss" infant, after resuscitation, should be monitored at home, if practical, until 6 months of age. A simple cardiac monitor for bradycardia has definite advantage over an apnea monitor alone.     

Chronic ventilatory failure caused by abnormal respiratory pattern generation during sleep

A 67-yr-old man presented with a 4-yr history of chronic ventilatory failure in the absence of any restrictive or obstructive ventilatory defect. Detailed neurologic investigations were unremarkable, and the response of minute volume of ventilation to inhaled CO2 was normal. During sleep there was no evidence of upper airway obstruction, and minute volume of ventilation averaged 6.7 L/min, compared with 7.2 L/min during quiet wakefulness. However, sleep was associated with a rapid and shallow pattern of breathing, resulting in high dead space ventilation, inadequate alveolar ventilation, hypoxemia, and hypercapnia. Correction of the abnormal ventilatory pattern during sleep by diaphragmatic pacing abolished all features of chronic respiratory failure. The findings indicate that a disorder of respiratory pattern during sleep can produce chronic ventilatory failure, despite normal respiratory drive.     

Polysomnographic values in adolescents with ataxia telangiectasia

Most adolescents with ataxia telangiectasia (A-T) develop progressive bulbar muscle weakness and decreased pulmonary reserve. The purpose of this study was to define the patterns of sleep and respiration during sleep, and to identify sleep-related breathing problems in subjects with A-T. To address these issues, overnight polysomnography was performed on 12 adolescents with A-T. Eleven of the 12 subjects completed overnight polysomnography. The median age was 16 years (range, 13-20 years). All subjects in the study were wheelchair-bound and the median forced vital capacity (% predicted of normal) was 44% (range, 16-82%). The mean sleep efficiency was 72.6% with a mean apnea hypopnea index (AHI) of 0.7 events/hr (range, 0-2.2). The majority of apnea/hypopneas were REM related. The mean central apnea index was 0.1 events/hr (range, 0-0.2). The mean oxygen saturation nadir was 92.7% (range, 87-96) and the mean peak end-tidal carbon dioxide ET(CO(2) ) value was 53.8 mm Hg (range, 49-60). Two of 11 subjects had ET(CO(2) ) values >or=50 mm Hg for more than 50% of total sleep time. In this study, the majority of A-T adolescents had infrequent partial or complete upper airway obstructions during sleep and minimal nighttime hypoxemia. They did, however, have decreased sleep efficiency most likely, due in part, to their underlying neurological condition. This decrease in total sleep time may underestimate hypoventilation. Based on these findings, overnight polysomnography should be considered in adolescents with A-T, particularly in those in which there is a clinical suspicion of sleep related breathing abnormalities.