Cardio-respiratory studies in a patient with an absent left pulmonary artery.

Congenital absence of a main pulmonary artery to one lung is a well-documented anomaly (Fraentzel, 1868; Miller, 1937; Blalock, 1948; Findlay and Maier, 1951; Madoff, Gaensler, and Strieder, 1952) and its association with unilateral cystic lung disease was emphasized in particular by Mannix and Haight (1955) and by Steinberg (1958). According to Madoff and others, a preponderance of congenital cardiac defects is apparent in patients with an absence of the left pulmonary artery. In their own report of a patient with a right-sided anomaly, other congenital heart defects were absent. When a lung is deprived of its main pulmonary arterial circulation, there is considerable expansion of a systemic collateral circulation (Bloomer, Harrison, Lindskog, and Liebow, 1949). Haemoptyses, usually stemming from the expanded arterial collateral circulation (Cudkowicz, 1952), are a frequent, early, and most distressing symptom (Findlay and Maier, 1951) and pneumonectomy may be necessary. Such a decision may also be reinforced by the failure of bronchospirometry to demonstrate a significant oxygen consumption by the affected lung. Oxygen uptakes varying from 0 to 7% of the total consumption have been found under such circumstances (Madoff et al., 1952; Smart and Pattinson, 1956; Fishman, Turino, Brandfonbrener, and Himmelstein, 1958; Tabakin, Hanson, Adhikari, and Miller, 1960), suggesting little, if any, participation in gas exchange by the abnormal lung. Fishman and his colleagues (1958) and Tabakin and others (1960), however, added to the study of the function of these lungs during bronchospirometry by the inclusion of such additional measurements as CO2 excretion and 'effective' bronchial blood flows. Here we report