Eisenmenger syn

Eisenmenger syn

high pulmonary blood flow cos of large L to R shunt or common mixing not treated early
1. -> pul aa wall thicken and resistance to flow increases
  1. children who survive gradually become less symptomatic as shunt decreases
    1. at 10-15 years old shunt reverses
      1. teen becomes blue (Eisenmenger syn)
prognosis
1. progressive
  1. adult will die of right heart failure usually in 40s/50s
treatment
1. aimed to prevent ES
  1. early transplantation for high pulmonary blood flow
2. Rx to palliate pulmonary vascular disease
  1. delay transplantation
  2. act on pulmonary vasculature on cGMP pathway
    1. inhaled nitric oxide
    2. iv magnesium sulphate
    3. oral phosphodiesterase inhibitors
      1. sildenafil
  3. act on cAMP pathway
    1. iv prostacyclin
    2. inhaled iloprostt
  4. endothelin receptor antag
    1. oral Bosentan
  5. anticoagulation
    1. heparin
    2. aspirin
    3. warfarin
clinical features
1. no murmur
2. right heart failure (late)

Resource summary

	Created by v.djabatey over 10 years ago