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