HELMHOLZ, HENRY F.

Necropsy studies in cases of pulmonary hypertension of unknown cause show structural changes in the pulmonary vessels that are called irreversible. Acetylcholine chloride was infused at a rate of 2 mg. per minute into the outflow tract of the right ventricle of a 48-year-old woman who had pulmonary hypertension. The mean pulmonary artery pressure decreased from 56 to 25 mm. Hg and the pulmonary flow increased slightly. These findings are consistent with the theory that so-called idiopathic pulmonary hypertension may at some stage be maintained by vasoconstriction.

Survival following surgical correction of congenital cardiovascular defects is unlikely if pulmonary hypertension cannot be decreased significantly. Infusions of acetylcholine decreased pulmonary vascular resistance in 6 of 11 patients having pulmonary hypertension associated with atrial or ventricular septal defects. When the acetylcholine was combined with the breathing of oxygen, resistance decreased further. These findings are consistent with the theory that, at some stage, the pulmonary hypertension in cases of congenital heart disease is maintained at least in part by vasoconstriction and therefore is potentially reversible.

The relative importance of organic changes and vasoconstrictive influences in the production of increased pulmonary vascular resistance in congenital heart disease is problematic. With use of a grading system previously described, a study was made of the relation between each of the grades of hypertensive pulmonary vascular disease associated with ventricular or atrial septal defects and the change of pulmonary artery blood pressure immediately following closure of the abnormal communications. The immediate reversibility of the pulmonary hypertension varies inversely with the severity of the structural changes.