Articles

Question 1

Sotatercept

Answer 1

NEJM 2023

Question 2

2 drugs aside from riociguat to have benefit in CTEPH patients

Answer 2

Non-operable CTEPH:

-macitentan: MERIT-1, Lancet 2017
-subQ treprostinil: CTrePH, Lancet 2019

Question 3

Name of riociguat trial

Answer 3

Riociguat trial: Chest-1, 2013 NEJM

Phase 3 RCT, 261 pts with inoperable or persistent/recurrent CTEPH to placebo vs. riociguat

Primary outcome- 6MWT
Secondary outcomes- PVR, WHO FC, pro-BNP
^^all 4 significantly better in riociguat group

Question 4

Key trial describing features of portopulmonary HTN

(a) Big hemodynamic difference from primary PAH

Answer 4

Chest 1997 (Kuo et al)- compared 30 patients with portopulmonary (PPAH) with 30 primary PAH w/o liver disease and 30 liver disease w/o PH

(a) PPAH have higher cardiac index and lower SVR than primary PAH = systemic hemodynamic profile more similar to chronic liver disease

Question 5

Portopulmonary HTN

(a) Big hemodynamic difference from primary PAH
(b) Big respiratory (acid/base) difference from chronic liver disease

Answer 5

Portopulmonary HTN = loosely defined as association of primary PH with portal hypertension

(a) Portopulmonary has higher cardiac output/index and lower SVR than primary PH (reflecting hyperdynamic circulation of chronic liver disease)

(b) Portopulmonary has accentuated respiratory alkalosis (lower pCO2), possible that a pCO2 cutoff > 30 could be helpful screening tool for live transplant candidates to rule out severe portopulmonary HTN