Pulmonary Hypertension Flashcards
Best First step to screen for Chronic Thromboembolic PH?
VQ Scan and a ECHO
Most Common Gene for Autosomal Dominant Pulmonary Hypertension?
BMPR2 is 75% of HPAH and 25% of IPAH, Other mutations less common are ALK-1, KCNK3, Endoglin, SMAD9, Caveolin-1
What Genes to look at if patient has Hereditary Hemorrhagic Telangiectasia and PAH?
ALK-1, BNPR1B, SMAD9 and Endoglin
What Drugs or toxins are commonly associated with Pulmonary HTN?
Methamphetamine, Fenfluramine, Toxic Rapeseed Oil
Most common Connective tissue disease associated with PAH?
Scleroderma (SSc)
Annual Evaluation for risk of PAH in Connective Tissue Disease recommends what algorithm to identify asymptomatic patients?
DETECT algorithm
Screening for PAH in HIV patients with symptoms or with more than one risk Factor. What Risk factors?
IV drug use, Female, Cocaine use, Hepatitis C infection and origin from high prevalence area
Which of the following best characterizes the hemodynamic of a patient with untreated Porto-pulmonary Hypertension?
A. Increased mPAP, Normal PVR, Increased CO, Normal Wedge
B. Increased mPAP, Decreased PVR, Normal CO, Increased Wedge
C. Increased mPAP, Increased PVR, Variable CO, Normal Wedge
C. Increased mPAP, Increased PVR, Variable CO, Normal Wedge
Porto-Pulmonary Hypertension
- High Risk Mortality and Morbidity
- 6th WSPH Recommendations: Screen patient with portal hypertension. If Tricuspid Regurgitant jet of > 3.4 M.s-1, RAE, RVE or dysfunction, further evaluate with RHC. If positive Refferal to PH center for management.
What Initial Comprehensive Assessment to stratify Risk in Pulmonary HTN patients? and for Follow-up?
ESC/ERS PAH Risk Assessment (3-Strata).
Follow-up used 4-Strata
Endothelin receptor Antagonists for Pulmonary Hypertension?
Bosentan, Ambrisetan, Macitentan
NO-CGMP (Pathway: PDE5I/SGCs) for Pulmonary Hypertension?
Sildenafil, Tadalafil, Riociguat
Prostanoids Prostacyclin Analogues used in pulmonary hypertension?
Epoprostenol (IV), Treprostinil (IV, Inhaled), Ilopresot (Inhaled)
Prostacyclin Agonists used in pulmonary Hypertension?
Selexipag (PO)
Activin Signaling inhibition medication used in pulmonary hypertension?
Sotatercept (SC)
A Fusion Protein acting by balancing signalling between pro and anti-proliferation pathways attempting to reverse vascular remodeling.
Combination Therapy of which two drugs for treatment of pulmonary Hypertension?
Obsynvi (PO) (Combination of Macitentan and Tadalafil)
For Adults with WHO functional Class II-III
Name of Trial that showed the effect of sotatercept in treatment of PAH that showed reduction in time to death and clinical worsening?
STELLAR Trial
Side Effects to worry about in Sotatercept?
- Increased Hemoglobin (Eyrthrocytosis)
- Thrombocytopenia
- Potential Embryo-Fetal Toxicity (Must use contraception while on Med)
Side Effects for Endothelin Receptor Antagonists (Bosentan, Ambrisetan, Macitentan)?
All are Teratogenic and require monthly pregnancy test, anemic ( Q3 Months CBC), Nasal congestion and edema.
For Bosentan monthly LFT checks
Drug Interactions:
Bosentatan: Hormonal Contraception, Cyclosporine, prograf, glyburide
Bosentatan/Macitentan: Ketoconazole, rifampin, Ritinovir
Phosphodiesterase-5 Inhibitor side effects?
Common: Headache, flushing, hypotension, diarrhea, epistaxis,
Require Medical Attention: Visual/Auditory Changes, Priapisma
Drug interactions: Nitrates, Alpha blockers, Ritinovir and CYP3A, Riociquat
Prostacylins and prostacyclin receptor agonist side effects?
Vasodilatory effects: Headache, flushing, jaw pain, limb pain, nausea, diarrhea, dizziness and thrombocytopenia. Rebound PH if interruption of delivery.
Soluble Guanylate Cyclase Stimulator (Riociquat) Side effects
- Teratogenic
- Hypotension, Bleeding, PVOD, Hemoptysis
Drug interactions:
- Nitrates, PDE-5i, Cigarette smoking Maalox
