[Haemodynamics in congenital pulmonary lymphangiectasis (author's transl)]

Respiratory insufficiency is the clinical principal sign of cystic pulmonary lymphangiectasis. Clarification of cardiovascular morphology and haemodynamics is the basic prerequisite for the classification and therapy of the disease pattern. The prognosis is unfavourable if it is found that the pulmonary circulation does not flow through the lungs, reaching instead the systemic circulation via the persisting ductus arteriosus. The histopathological findings as well as the results of dissection of the cardiovascular system clarify the question as to the pathogenesis of this disease and allow subdivision of the disease into a primary and a secondary manifestation of cystic pulmonary lymphangiectasis.