A STUDY OF CARDIOPULMONARY ALTERATIONS IN PATIENTS WITH SICKLE CELL DISEASE AND ITS VARIANTS1

Abstract
Unsaturation of the arterial blood was demonstrated in 16 out of 21 patients possessing "S" hemoglobin in various combinations. Alveolar to arterial oxygen gradients were measured, employing a Clark polarographic electrode, on 3 levels of oxygenation, and it was determined that the desaturation was principally the result of intrapulmonary shunting of blood, although defects in diffusion also existed. Increased cardiac outputs and blood volumes were accompanied by generally decreased pulmonary vascular resistances, suggesting that cor pulmonale is not common in these states. The administration of very low concentrations of O2 (9 to 11%), while producing increased sickling in both the arterial and venous circulation, resulted in no discernible acute or chronic symptomatology.