Primary plexogenic pulmonary hypertension shows imperfect formation of the internal elastic lamina of the pulmonary arteries.

Lung tissue from subjects dying from primary plexogenic pulmonary hypertension (PPH) has shown defects of elastin formation of the lung arteries. Lung vessels from 5 cases of PPH were compared with those of 9 age-matched normal subjects, and 24 individuals having secondary pulmonary hypertension (2 degrees PH). PPH cases and those with 2 degrees PH due to congenital heart disease with left-to-right shunts (2 degrees PH, LRS), showed active proliferation of medial smooth muscle cells (SMC) through defects of the internal elastic lamina (IEL) into the arterial lumen to form typical plexiform lesions. Larger arteries showed accelerated intimal thickening similar to normal aging. Plexiform lesions were not seen in normal subjects or in those developing high pulmonary pressures later in life. The observations showed that the development of discontinuities of the IEL of the pulmonary arteries and intimal thickening is accelerated in normal subjects by high pulmonary artery pressure, especially when this is established at a very young age. They suggest that such discontinuities occur in PPH due to inherent abnormality of the elastin of the arterial walls, with advanced early proliferation of medial SMC and obstruction of the pulmonary arterial circulation.