[Complex pulmonary vessel alterations in an aorto-pulmonary window in adulthood]

An aorto-pulmonary window (aorto-pulmonary septal defect) is considered to be a rare congenital cardiac malformation. We report the case of a patient with an unrepaired congenital aorto-pulmonary window 1.4 cm in size which is the largest aorto-pulmonary septal defect yet reported. This patient died at the age of 58 not from the congenital cardiac malformation but from the after-effects of a pulmonary adenocarcinoma diagnosed 2 years previously. Interestingly, the long standing, persistent pulmonary hypertension correlated well with the varying pathomorphological changes seen in the pulmonary vessels. In addition to the complex morphological signs due to the pulmonary hypertension such as the extensive aneurysms found in the left and right main pulmonary arteries (first report of this size), pulmonary vessels with relatively few pathomorphological changes were also found. Hence this first report of such a long survival of a patient with a comparatively large aorto-pulmonary septal defect demonstrates a surprising variety of pathomorphological alterations seen in the pulmonary arteries in the face of excessive pulmonary hypertension. These alterations could be the result of peripheral aneurysmatic formations and thrombembolic stenoses of the lobular or segmental arteries and might provide an explanation for the long survival of this particular congenital cardiac malformation. All these varying findings in the pulmonary arteries emphasise the fact that the pathomorphological findings in lung biopsies might not be representative of the whole lung.