Giant, dissecting, high-pressure pulmonary artery aneurysm: case report of a 1-year natural course

Tex Heart Inst J. 2005;32(4):589-94.

Abstract

We report the rare subchronic clinical course of a giant, dissecting pulmonary artery aneurysm in an oligosymptomatic middle-aged woman who had idiopathic pulmonary hypertension. Diagnosis was simple with the use of echocardiography and multislice computed tomography. Conversely, deciding on the treatment was difficult, because prominent surgeons declined to perform surgical repair of the aneurysm and recommended heart-lung transplantation. Therefore, we were forced to treat our patient medically. She survived for 1 year, including 8 months of treatment with sildenafil, and then died suddenly while awaiting transplantation. Our patient, who had a dissecting, high-pressure pulmonary artery aneurysm, had an unexpectedly stable and uneventful clinical course for 1 year, which, under more favorable circumstances, might have provided enough time for heart-lung transplantation to be performed.

Publication types

  • Case Reports

MeSH terms

  • 3',5'-Cyclic-GMP Phosphodiesterases / antagonists & inhibitors
  • Adult
  • Aortic Dissection / complications
  • Aortic Dissection / diagnosis*
  • Aortic Dissection / drug therapy
  • Echocardiography
  • Fatal Outcome
  • Female
  • Follow-Up Studies
  • Humans
  • Hypertension, Pulmonary / complications
  • Hypertension, Pulmonary / physiopathology
  • Piperazines / therapeutic use
  • Pulmonary Artery*
  • Pulmonary Wedge Pressure / physiology*
  • Purines
  • Severity of Illness Index
  • Sildenafil Citrate
  • Sulfones
  • Tomography, X-Ray Computed
  • Vasodilator Agents / therapeutic use

Substances

  • Piperazines
  • Purines
  • Sulfones
  • Vasodilator Agents
  • Sildenafil Citrate
  • 3',5'-Cyclic-GMP Phosphodiesterases