Cardiopulmonary response to exercise in patients with intrapulmonary vascular shunts

The majority of patients with intrapulmonary right-to-left shunting due to pulmonary arteriovenous malformations exhibit good maximum exercise capacity (>70% predicted) despite profound arterial oxygen desaturation. We studied seven such patients to assess tissue oxygen delivery during steady-state exercise. From rest to exercise [50 ± 7 (SE) W] arterial saturation fell from 80 ± 3 to 74 ± 3%, and mean right-to-left shunt increased slightly from 31 ± 4 to 34 ± 5% (P = NS). Minute ventilation was high for oxygen uptake, and the ventilatory equivalent was raised (174 ± 19% predicted) and was correlated with shunt size (r = 0.93). The majority of the patients maintained pulmonary alveolar blood flow within the predicted range for their power output, but total cardiac output was increased to 142 ± 11% predicted due to flow through the shunt. Consequently, on exercise, oxygen delivery per unit oxygen consumption [2.3-3.3 (normal range 1.6-2.4)] and calculated mixed venous oxygen tension (27.0 ± 0.8 Torr) were preserved. Arterial PCO₂ rose on exercise by 2.8 ± 1.2 Torr, in proportion to the ratio of flow through the shunt to total cardiac output (r = 0.73), but remained low (33.1 ± 1.4 Torr) in absolute terms. The high cardiac output on exercise may be facilitated by a low pulmonary vascular resistance (0.33 ± 0.08 mmHg · l^{- 1} · min, measured at rest), which may explain why exercise performance is better in these patients than in patients with equivalent hypoxemia from other causes.