Unexplained plexogenic pulmonary arteriopathy: an unusual rapidly progressive case in childhood

A 4-year-old child died because of unexplained plexogenic arteriopathy only seven months after the onset of the symptoms. Cardiac catheterization revealed a primary pulmonary hypertension sensible to nifedipine, but after the initial favourable results the clinical course rapidly went down and the child died from right sided congestive heart failure. The post-mortem pulmonary histology revealed reversible structural abnormalities. It also demonstrates that the vasodilatator drugs are effective only for short time in primary pulmonary hypertension, and moreover that it is even hazardous to generalize when one talks about reversibility of such lesions in each case.