Respiratory and hemodynamic study during wakefulness and sleep in myotonic dystrophy.

Six young male patients with grade I (mild) myotonic dystrophy and a complaint of excessive daytime sleepiness were studied during wakefulness and sleep. Pulmonary function tests during wakefulness showed evidence of mild abnormality related to respiratory muscle weakness. During sleep, some patients developed a sleep apnea syndrome with high sleep Apnea Indices. There was no relation between hypoxic and hypercapnic ventilatory responses during wakefulness and sleep Apnea Indices. But hypoxemia and hypercapnia worsened considerably during REM sleep. Myotonic dystrophy patients with sleep apnea presented increased pulmonary and systemic arterial pressures during sleep. It was also during sleep that arrhythmias were observed.

Role of selected muscles of respiration as influenced by posture and tidal volume.

Studies of the activity of accessory respiratory muscles were conducted in 30 normal subjects (15 male and 15 female subjects, age 5 to 62 years). Electromyograms were recorded over the right serratus anterior muscle and the adjacent sixthe intercostal space, and the results were correlated with tidal volume. Phasic respiratory activity in the serratus anterior muscle was detected in every subject but was markedly affected by the prevailing posture and level of ventilation (appearing at mean tidal volumes equivalent to 60.5 percent of vital capacity when subjects were standing relaxed, and equivalent to 35.2 percent of vital capacity when subjects were seated, leaning forward with elbows supported). The activity was predominantly inspiratory in timing but in some subjects extended into early expiration. No significant differences were noted in subjects of different ages, sex, height, or weight. Similar results were recorded from the inspiratory intercostal muscle. On the basis of these findings, we conclude that, contrary to reports, the serratus anterior muscle should be classified as an accessory muscle of respiration, at least at increased levels of ventilation, and that its activity is most pronounced in postures that place the muscles' origin in an advantageous position for moving the ribs.

Effect of hypoxia on immediate ventilatory load response in dogs.

The effective elastance of the respiratory system (which has been previously shown to provide an index of the ability of the respiratory musculature to compensate rapidly for transient mechanical ventilatory loads) was measured in six hypoxic dogs to determine whether hypoxia hindered immediate load-compensatory mechanisms. The effective elastance value was computed from measurements of control tidal volume and the pressure developed at the airway opening during the first inspiratory effort following airway occlusion at FRC. The mean effective elastance was 197 cmH2O/l while the animals were breathing room air and did not change significantly when the animals were rendered hypoxic by reducing the inspired oxygen concentration, in five dogs, or by controlled hemorrhage, in two dogs. It was concluded that inasmuch as effective elastance measurements remain constant during hypoxia, the stability of ventilation is not significantly impaired in this situation.

Spectral analysis of human inspiratory diaphragmatic electromyograms.

The inspiratory diaphragmatic EMG was recorded via esophageal electrodes in six normal subjects. The EMG and ECG signals were analyzed by power density spectral analysis, before and after band-pass filtering (20--1,600 Hz). The EMG spectrum was concentrated in the bandwidth 25--250 Hz. Electrode motion introduced a significant artifact only at low frequencies. The ECG spectrum was also concentrated at lower frequencies, but substantial power from the cardiac signal spilled over across most of the EMG spectrum. Band-pass filtering was therefore effective in minimizing the former but not the latter. Of the various power and frequency parameters used to quantitate the EMG spectrum, the most stable was the centroid frequency. This was reproducible within and between subjects, and was not affected by changing tidal volume or inspiratory flow rate.

Hypoxemia during hemodialysis using acetate versus bicarbonate dialysate.

To evaluate the extent and cause(s) of dialysis-related hypoxemia, we studied 10 patients, 7 days apart using acetate (AC) and bicarbonate dialysate (HCO3). We measured arterial blood gases, WBC, minute ventilation (VE) and inspired and expired gas concentrations and calculated the respiratory quotient (R) and the alveolar-arterial oxygen difference (A-a)DO2 before and during hemodialysis. 8 patients developed hypoxemia. Arterial PO2 (PaO2) dropped similarly at 30 min from 93 +/- 5 to 78 +/- 6 (p less than 0.05) and 89 +/- 4 to 79 +/- 5 mm Hg (p less than 0.05) with AC and HCO3, respectively. R and VE decreased during AC (p less than 0.05). (A-a)DO2 increased at 30 min and correlated with the drop in PaO2 during both AC (r = 0.68, p less than 0.025) and HCO3 (r = 0.76, p less than 0.025). The fall in PaO2 also correlated with the fall in WBC count for both AC and HCO3 (r = 0.63, p less than 0.005). The increase in arterial pH during HCO3 (up to 7.45 +/- 0.01) was significantly greater than that during AC (up to 7.42 +/- 0.01) (p less than 0.025), and coincided with a relative decrease in VE. We conclude that (1) HCO3 does not prevent hypoxemia, and (2) hypoventilation V/Q abnormalities and increase in arterial pH, contribute variably to dialysis related hypoxemia depending on the type of dialysate and the time during dialysis.