Total anomalous pulmonary venous connection with severe pulmonary venous obstruction. A clinical entity.

FRIEDLOWSKY, in 1868, was the first to report total anomalous pulmonary venous connection.' 2 This entity, with its, multiple varieties,3 is receiving increasing attention with the recent encouraging reports of suecessful surgical therapy.4 The infradiaphragmatic form was first described by Ghon in 1916,5 and a total of 37 cases has since been reported.5 24 Obstruction to the pulmonary venous drainage has been implicated as the basis for the characteristic radiologic features of this form of anomaly, i.e., a relatively small heart associated with diffuse haziness and reticulation of the lung fields. More recently pulmonary venous obstructio-n complicating the supracardiac type has been reported bv Harris et al. (1960)21 and by Hauck et al. (1960) 25 This, study presents clinical, physiologic, and pathologic data, from six male infants with severe pulmonary venous oibstruction in otherwise uncomplicated total anomalous pulmonary venous connection and a review of the literature. The clinical picture is remarkably uniform and characteristic, regardless of the site of the pulmonary venous obstruction.