Lecture 3 - Pulmonary Arterial Hypertension Flashcards
What can cause pressure in the Pulmonary Artery to inc?
Changes in the blood vessel
What can cause pressure in the Pulmonary Vein to inc?
Changes in the heart
What is Pulmonary Arterial Hypertension?
High BP in lungs
MAP > 25
Often leads to right sided HF
Median survival rate after diagnosis w/o treatment is 2.8yrs
WHO-E Group I
Idiopathic = cant determine reason for PAH
Associated w/ (APAH)
- collagen vascular disease = lupus
- Portal hypertension secondary to liver failure
- HIV infection
- Drugs/toxins = stimulants, diet pills, PPA
WHO-E Group 2
Pulmonary Hypertension owing to Left Heart Failure
- Systolic Dysfunction
- Diastolic Dysfunction
- Valvular disease
- Most common cause for PAH *
WHO-E Group 3
PH Associated w/ Lung disease or Chronic Hypoxemia
COPD
ILD
ONA
Chronic exposure to high altitudes
WHO-E Group 4
Chronic Thromboembolic Pulmonary Hypertension
Chronic thromboembolic disease
Pulmonary Embolism
WHO-E Group 5
Unclear or multifactorial causes
Symptoms of PAH
- Dizziness and/or fainting
- Shortness of Breath
- Chest pain
- Feeling tired/worn out
- Swollen ankles and legs
WHO-F Functional Classes
F-1 (best) - F-4 (worse)
F-1
No SOB/fatigue present at rest or with usual physical activity
F-2
No SOB/fatigue at rest but one or both present with usual physical activity
F-3
No SOB/fatigue at rest but one or both present with simple activities of daily living
F-4
SOB/Fatigue present at rest, nearly impossible to complete activates of daily living
Gold Standard Diagnostic for PAH
Right Heart Catheterization
Measuring MAP
6 minute walk test…
measures patients functional capacity
Goals of Pharmacotherapy
- increase 6 min walk time
- Reduce WHO functional class
- Avoid drug-associated adverse effects
- Reduce mortality
Conventional Pharmacotherapy for PAH
Oxygen
Loop Diuretics
Digoxin
Anticoagulants…usually warfarin
Positive Response in Acute Vasodilator Trial
Given 5min of NO
Positive response = Decrease in mPAP > 10 mmHG AND to a value of less than 40 mmHG
If positive response, Diltiazem dose is….
120mg/day with weekly titration by 120mg/day up to max of 480 mg/day
if SBP < 90, reduce by 120mg/day
if HR < 50, change to LA nifedipine
Phosphodiesterase Inhib doses
Sildenafil, 20mg TID (Revatio)
Tadalafil, 40mg QD (Adcirca)
Sildenafil PAH info
No difference in 6MWT between doses, don’t inc doses
SE:
Headache, Flushing, Nasal congest, Blurred Vision
- Don’t admin nitrates concomitantly*
Endothelial Receptor Antagonists
Bosenten (Tracleer)
Ambrisenten (Letairas)
Bosenten (Tracleer) info
Mechanism: Dual ET-A/ET-B antagonist
Starting dose: 62.5mg BID
Max Dose: 250mg BID
can titrate up, see benefit in 6MWT w/ inc dose
11% incidence of hepatotoxicity