Inoperable pulmonary vascular disease in infants with congenital heart disease

Background. Among 120 infants less than 12 months of age who had lung biops y and autopsy, 20 were inoperable because of severe irreversible pulmonary vascular disease. Methods. The infants were classified into three groups. Group 1 comprised 6 patients who showed complete obstruction of the small pulmonary arterial l umen and atrophy of the peripheral arterial media and who were considered t o have absolute operative contraindications. Group 2 comprised 6 patients w ho had no pathologic findings of absolute operative contraindication and ha d an index of pulmonary vascular disease of more than 2.2. They were isolat ed as having advanced plexogenic pulmonary arteriopathy. Group 3 comprised 8 patients who had extremely thickened media of small pulmonary arteries, w ith abnormally thickened media extending into the small peripheral arteries characterized by extremely narrow lumina and medial thickness exceeding lu minal diameter. Results. Six of the 9 patients in whom operative repair was abandoned on th e basis of preoperative or intraoperative lung biopsy are still alive. Of t he II patients who underwent operation without biopsy, none survived. Conclusions. Preoperative or intraoperative lung biopsy and assessment of a rteriopathy based on the above criteria are recommended in all patients in whom fatal pulmonary vascular disease is suspected. (C) 1998 by The Society of Thoracic Surgeons.