CHD-PH

Question 1

Define Eisenmenger syndrome

Answer 1

Most severe form of CHD-PH, triad of:

1. large intracardiac shunt with initial L to R shunt (systemic to pulmonary)
2. PAH with shunt reversal (R to L) or bidirectional shunting due to increased pulmonary blood flow
3. Hypoxemia with cyanosis

Pathogenesis- similar to IPAH where pulmonary vasculature changes (from high blood flow => elevation in PVR eventually reverses the direction of shunt

Question 2

Hallmark clinical features of Eisenmengers

Answer 2

Cyanosis with consequences of chronic hypoxemia = secondary erythrocytosis

Question 3

PVR cutoff for consideration of repair for moderate-large congenital defects (predominantly L to R shunt) with mild-moderate PVR elevation

Answer 3

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

Question 4

List the 4 categories of group 1 PAH-congenital heart disease

Answer 4

1. Eisenmengers- severe elevation in PVR causes reversal of shunt
2. Unrepaired moderate-large defects
3. PAH with coincidental CHD- small shunt not causative of degree of PAH
4. Persistent PVR elevation after surgical repair w/o residual anatomic lesion

(Jones et al AHA 2023)D

Question 5

Diastolic pressure gradient and use

Answer 5

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