Pulmonary capillary haemangiomatosis - An unusual cause of hypoxia.

We describe the case of a 58-year-old man who presented with progressive dyspnoea on exertion and severe exertional hypoxia. There was a paucity of radiological findings, mild pulmonary hypertension, and no demonstrable anatomical shunt. Post mortem examination of lung tissue suggested a diagnosis of pulmonary capillary haemangiomatosis. The case is unusual in displaying few radiological findings. We postulate that the severe hypoxia was due to shunting through the abnormal capillary proliferations.

Gas exchange responses to constant work-rate exercise in patients with glycogenosis type V and VII.

During constant work-rate exercise above the lactic acidosis threshold, oxygen consumption fails to plateau by 3 minutes, but continues to rise slowly. This slow component correlates closely with the rise in lactate in normal subjects. We investigated if oxygen consumption during constant work-rate exercise could rise after 3 minutes in the absence of a rise in lactate. We studied five patients with McArdle's disease, one patient with phosphofructokinase deficiency and six normal subjects. Subjects performed two 6-minute duration constant work-rate exercise tests at 40 and 70% of peak oxygen consumption. During low-intensity exercise, oxygen consumption reached steady state by 3 minutes in both groups. Lactate rose slightly in control subjects but not in patients. During high-intensity exercise, oxygen consumption rose from the third to the sixth minute by 144 (21-607) ml/minute (median and range) in control subjects and by 142 (73-306) ml/minute in patients (p = not significant, Mann-Whitney U test). Over the same period, lactate (geometric mean and range) rose from 2.68 (1.10-5.00) to 5.39 (2.70-10.00) mmol/L in control subjects, but did not rise in patients (1.20 [0.64-1.60] to 0.70 [0.57-1.20] mmol/L). We conclude that the slow component of oxygen consumption during heavy exercise is not dependent on lactic acidosis.

Ventilatory and gas exchange responses during heavy constant work-rate exercise.

PURPOSE: At constant work-rates below the gas exchange threshold (VO(2 theta)), VO(2) normally achieves steady-state values within 3 min, whereas at heavier work-rates, VO(2) may continue to rise. The VO(2) response to heavy exercise can be described by a three-exponential model with the slow phase usually commencing 2-3 min after the onset of exercise. The aim of our study was to estimate precisely the VO(2), VCO(2), VE and f(C) required for above-VO(2 theta) exercise from the relationship of the specific variable to work-rate below VO(2 theta) and to compare this with the actual value achieved. METHODS: Nine cyclists performed five constant work-rates of 8 min duration, four below VO(2 theta) (40, 80, 120, 160 W) and one midway between VO(2 theta) and VO(2max) (295 +/- 34 W). The VO(2), VCO(2), VE and f(C) were averaged for the final 2 min of each below-VO(2 theta) test and were found to be linear with respect to work-rate (average r2 >0.95). Variables for the above-VO(2 theta) work-rate were predicted by extrapolation and compared with the actual measured values at the end of the exercise bout. RESULTS: VO(2) exceeded the predicted value by 0.48 +/- 0.21 L x min(-1) (12.4 +/- 5.1%), VCO(2) by 0.78 +/- 0.26 L x min(-1) (23.2 +/- 7.2%), VE by 40.3 +/- 16.3 L x min(-1) (51.0 +/- 23.1%), and f(C) by 12.2 +/- 12.5 beats x min(-1) (8.8 +/- 9.3%), all P < 0.0001 except f(C) P < 0.02, paired t-test. The point at which VO(2) during above-VO(2 theta) exercise exceeded the predicted value (145.7 +/- 64.9 s) agreed with the point at which the slow component of VO(2) began, as determined by nonlinear regression analysis (131.5 +/- 44.3 s, P = NS, ANOVA). CONCLUSION: There is an excessive metabolic response to heavy exercise over and above that predicted by extrapolation from light-moderate exercise and this excess VO(2) approximates on average to the slow phase of a three-compartment exponential model.