Define Eisenmenger syndrome
Most severe form of CHD-PH, triad of:
- large intracardiac shunt with initial L to R shunt (systemic to pulmonary)
- PAH with shunt reversal (R to L) or bidirectional shunting due to increased pulmonary blood flow
- Hypoxemia with cyanosis
Pathogenesis- similar to IPAH where pulmonary vasculature changes (from high blood flow => elevation in PVR eventually reverses the direction of shunt
Hallmark clinical features of Eisenmengers
Cyanosis with consequences of chronic hypoxemia = secondary erythrocytosis
PVR cutoff for consideration of repair for moderate-large congenital defects (predominantly L to R shunt) with mild-moderate PVR elevation
3 groups
(a) CHD still possibility repairable (PVR < 4)
(b) PVR 4-8, consider targeted PH therapy with re-evaluation for operability
(c) PVR > 8, physiology more similar to Eisenmengers, CHD likely not repairable
List the 4 categories of group 1 PAH-congenital heart disease
- Eisenmengers- severe elevation in PVR causes reversal of shunt
- Unrepaired moderate-large defects
- PAH with coincidental CHD- small shunt not causative of degree of PAH
- Persistent PVR elevation after surgical repair w/o residual anatomic lesion
(Jones et al AHA 2023)D
Diastolic pressure gradient and use
DPG = dPAP - PAWP
Similar to PVR can be used to see if component of pre (and not just post) capillary disease in group II
Higher (over 7, aka higher wedge) associated with isolated post-capillary
