Cyanosis, Cardiomegaly, and Weak Pulses. a Manifestation of Massive Congenital Systemic Arteriovenous Fistula.

LARGE systemic arteriovenous fistulas are usually considered a form of acyanotic heart disease characterized by bounding pulses and cardiomegaly. This report deals with a cyanotic infant whose pulses were so weak that blood pressure was unobtainable either by the auscultatory or flush method. Although the infant died before hemodynamic studies were completed, we believe the cyanosis was due to a large central right-to-left shunt. On the basis of the frequency of cyanosis reported in other cases of gross congenital systemic arteriovenous fistula producing congestive heart failure in early infancy,'-,' and a consideration of the hemodynamic changes caused by large experimental fistulas in animals,9 10 it is suggested that such cyanosis is practically a mandatory hemodynamic and clinical manifestation of the disease. Previous authors have often described cyanosis and the "mistaken diagnosis of cyanotic heart disease' without attempting to explain the mechanism of the cyanosis.