Roxana-isms

Question 1

First line regimen for severe HIV-induced PAH

Answer 1

Ambrisentan-Riociguat
-start ambrisentan 10mg first, then sequentially riociguat 1.5 then uptitrate

-avoid PDE5s in HIV (interaction with protease inhibitors)
-Ambrisentan better than macitentan

Question 2

What dose of IV remodulin can switch to orenitram

Answer 2

Uptitrate IV remodulin to 15 ng/kg/min then start orenitram (generally equivalentis 3mg TID)

• vs if starting without inpatient IV initiation need to start at .125mcg TID of orenitram….

Question 3

Orenitram- when to start orally vs. inpatient for IV initiation

Answer 3

Orally when have time (RV isn’t horrible on echo, symptoms not terrible) clinically to uptitrate oral outpatient

Inpatient IV remodulin initiation, titrate up to 15 ng/kg/min (dose at which start to have some clinical benefit) with

Question 4

Ideal dose to get up to on IV remodulin before switching and discharging on orenitram

Answer 4

IV to oral treprostinil
15 ng/kg/min of IV remodulin typically comparable (but weight dependent) to around 3 mg orenitram TID

Question 5

When c/f high output heart failure and need to come down on remodulin

Answer 5

If CI (cardiac index) > 4.1

Question 6

Brief pathophys description of high output heart failure

Answer 6

High cardiac output with low SVR where elevation in cardiac output exceeds metaolic demand

• low SVR => RAAS activation => fluid overload

Question 7

2 main buckets: non-iatrogenic causes of high output heart failure

Answer 7

1. Mainly vasodilatory effects: obesity, cirrhosis, Av fistula
2. Mainly metabolic effects (increased metabolic demand): hyperthryoidism, myeloprofilerative disorders with extramedullary hematopoeisis

Question 8

Definition of high output heart failure via invasive hemodynamics

Answer 8

No specific cutoff but generally signs/symptoms of heart failure with CO > 8 or CI > 4

Question 9

PFT parameter (ratio) that can be used to predict PH in pts with ILD

Answer 9

FVC / DLCO > 1.4 (per literature > 1.4, per Sulica > 1.6)
Classically used for scleroderma-PH

Question 10

PVOD triad of imaging findings

Answer 10

1. diffuse GGO (particularly centrilobular)
2. septal thickening
3. mediastinal lymphadenopathy
   ^^(signs of post-capillary venous congestion)

PH and pulmonary congestion with no evidence of L. heart dysfunction
-also typically with normal sized pulmonary veins and lef tatrium

Question 11

Clinical vignette for PVOD

Answer 11

Severe PH with signs of pulmonary congestion without any evidence of L heart disease (no diastolic dysfunction, no LA dilation, no LVH etc)
- classic CT chest findings: centrilobular diffuse GGOs with septal thickening and mediastinal lymphadenopathy

Question 12

Orenitram max dose

(a) thought to be equivalent IV dose

Answer 12

Orenitram 10mg TID

(a) Remodulin 50 ng/kg/min