Thromboembolic pulmonary hypertension due to disseminated fibromuscular dysplasia

Pediatr Cardiol. Sep-Oct 1996;17(5):340-5. doi: 10.1007/s002469900076.


We present two patients with thromboembolic pulmonary hypertension associated with unusual complications probably caused by disseminated fibromuscular dysplasia (FMD) or FMD-like vascular lesions. Intimal fibroplasia, which is typical of the vascular lesions associated with FMD, was observed in both patients. The presence of such intimal lesions suggests that there was a systemic factor that caused the formation of recurrent thrombi in the systemic vessels in these patients. These cases are the first ones reported in which an association between FMD and pulmonary hypertension has been observed. The pathogenesis of the thrombi in our patients was thought to be recurrent pulmonary thromboembolisms resulting from FMD.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Aortography
  • Child
  • Fatal Outcome
  • Female
  • Fibromuscular Dysplasia / complications*
  • Fibromuscular Dysplasia / diagnosis
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Renovascular / complications
  • Male
  • Moyamoya Disease / complications
  • Moyamoya Disease / diagnosis
  • Pulmonary Artery / diagnostic imaging
  • Pulmonary Artery / pathology
  • Radionuclide Imaging
  • Renal Artery / diagnostic imaging
  • Renal Artery / pathology
  • Thromboembolism / diagnosis
  • Thromboembolism / etiology*