SM 165a - Pulmonary HTN Flashcards
Describe the hemodynamics of CTEPH (WHO group 4)
MAP @ rest:
PCWP:
WHO Group 4:
MAP @ rest: ≥25 mmHg
PCWP: ≤15 mmHg
Describe the hemodynamics of pulmonary venous hypertension (WHO group 2)
MAP @ rest:
PCWP:
mPAP @ exercise:
PVR:
WHO Group 2:
MAP @ rest: >25 mmHg
PCWP: ≥15 mmHg*
mPAP @ exercise: >30 mmHg
PVR: <3 Wood units*
* = difference between group 1 and group 2
Describe the hemodynamics that define pulmonary arterial hypertension (WHO group 1)
MAP @ rest:
PCWP:
mPAP @ exercise:
PVR:
WHO Group 1:
MAP @ rest: >25 mmHg
PCWP: ≤15 mmHg*
mPAP @ exercise: >30 mmHg
PVR: >3 wood units* - WHO Group 1 is a “high resistance” disease
* = hemodynamic differences between WHO groups 1 and 2.
Describe the hemodynamics of pulmonary hypertension due to lung disease (WHO group 3)
MAP @ rest:
WHO Group 3:
MAP @ rest: >20 mmHg
How would you differentiate between the following classification?
Lung disease without PH: mPAP =
Lung disease with PH: mPAP =
Lung disease with severe PH: mPAP =
Lung disease without PH: mPAP <25 mmHg
Lung disease with PH: mPAP >25 mmHg
Lung disease with severe PH: mPAP ≥35 mmHg OR mPAP >25 mmHG w/ low cardiac index
(Think COPD, ILD, CPFE as far as lung disease goes)
CPFE = combined pulmonary fibrosis & emphysema
In approaching this patient (RAP = 15 mmHg, mPAP = 49 mmHg, PCWP = 23 mmHg) with pulmonary hypertension, what would be the most appropriate first step in management?
- Start diuresis with a loop diuretic for heart failure
- Start IV epoprostenol for pulmonary vasodilation to acutely decrease mean pulmonary artery pressure
- Start the endothelin receptor antagonist, bosentan, to decrease pulmonary vascular resistance and decrease RV strain
- Start full anticoagulation with coumadin
a. Start diuresis with a loop diuretic for heart failure
- PCWP ≥ 25 mmHg => pulmonary venous hypertension
- Due to left heart failure
- Treatment = diuresis + optimize HF medication
In lung disease with severe pulmonary hypertension, what is the driver of reduced exercise capacity?
Circulatory impairment (rather than ventilatory impariment)
Likely due to severe vascular abnormalities
Pulmonary hypertension that is idiopathic, heriable, or drug or toxin induced is classified as WHO group _____
(Caused by __________________)
Pulmonary hypertension that is idiopathic, heriable, or drug or toxin induced is classified as WHO group** **1
(Caused by pulmonary arterial hypertension)
What are some of the causes of WHO group 1 pulmonary hypertension?
- Idiopathic
- Heritable
- Drug/toxin-induced
- Associated with…
- Connective tissue dieseases
- HIV infection
- Portal HTN
- Congenital (pulmonary shunt)
- Schistosomiasis
- Chronic hemolytic anemia
What drugs target the endothelin pathway in the treatment of WHO group 1 PAH?
- Ambrisentan
- Bosentan
- Macitentan
What drugs target the nitric oxide pathway in the treatment of WHO group 1 PAH?
- Phosphodiesterase inhibitors
- Sildenafil
- Tadalafil
- Guanylate cyclase stimulators
- Riociguat
What drugs target the prostacyclin pathway in the treatment of WHO group 1 PAH?
Goal = increase the effects of prostacyclin
- Epoprostenol
- Treprostinil
- Iloprost
What histological changes would you expect to see in WHO Group 1 pulmonary hypertension?
WHO Group 1 = Pulmonary arterial hypertension
- Smooth muscle hypertrophy
- Neointima formation neovascularization
- Endothelial cell proliferation
What histological changes would you expect to see in WHO Group 2 pulmonary hypertension?
WHO Group 2 pulmonary hypertension = Pulmonary Venous Hypertension (due to left heart failure)
- Medial thickening
- Occlusive venopathy
What is the equation for pulmonary vascular resistance?
PVR = (mPAP - Left atrial pressure) / CO
What is the gold standard for diagnosing Pulmonary Hypertension?
Right heart catheterization
Many tests preceed this
What is the treatment for WHO Group 1 PAH?
Pulmonary vasodilator therapy:
Not curative, but helps manage symptoms
- Endothelin receptor antagonists
- Phosphodiesterase inhibitors*
- Inhibits the breakdown of NO
- Soluble guanylate cyclase stimulators*
- Stimulates NO receptors
- Prostacyclins
*Targets the Nitric Oxide pathway
What is the treatment for WHO group 2 PVH?
Optimize heart failure medications
Diuresis
Pulmonary vasodilators are contraindicated
What is the treatment for WHO group 3 pulmonary hypertension?
Optimize lung disease medications
Reverse hypoxia
What is the treatment for WHO group 4 pulmonary hypertension?
WHO group 4 = CTEPH
- Anticoagulation
- Referral for thromboendarterectomy
- Remove the clot
- Vasodilators
- Bridge to surgery, or if the operation is contraindicated
Which WHO group pulmonary hypertension is curable?
WHO group 4 - Chronic thromboembolic pulmonary hypertension (CTEPH)
Which WHO group pulmonary hypertension is driven by disease at location D?
WHO group 2 = Pulmonary Venous Hypertension
(due to left heart failure)
Which WHO group pulmonary hypertension is driven by disease at location C?
WHO group 1 = Pulmonary arterial hypertension
Which WHO group pulmonary hypertension is driven by disease at location B?
WHO Group 3 = Lung Disease
Which WHO group pulmonary hypertension is driven by disease at location A?
WHO group 4 = CTEPH (Chronic thromboembolic pulmonary hypertension)
WHO group pulmonary hypertension is caused by left heart disease
WHO group 2 pulmonary hypertension is caused by left heart disease
WHO group pulmonary hypertension is caused by chronic thromboembolic pulmonary hypertension
WHO group 4 pulmonary hypertension is caused by chronic thromboembolic pulmonary hypertension
WHO group pulmonary hypertension is caused by pulmonary arterial hypertension
WHO group 1 pulmonary hypertension is caused by pulmonary arterial hypertension
WHO group pulmonary hypertension is caused by unclear or multifactorial mechanisms
WHO group 5 pulmonary hypertension is caused by unclear or multifactorial mechanisms
WHO group 1 pulmonary hypertension is caused by
WHO group 1 pulmonary hypertension is caused by pulmonary arterial hypertension
WHO group 2 pulmonary hypertension is caused by
WHO group 2** pulmonary hypertension is caused by **left heart disease
WHO group pulmonary hypertension is caused by lung disease and/or hypoxia
WHO group 3 pulmonary hypertension is caused by lung disease and/or hypoxia
WHO group 3 pulmonary hypertension is caused by
WHO group 3 pulmonary hypertension is caused by lung disease and/or hypoxia
WHO group 4 pulmonary hypertension is caused by
WHO group 4 pulmonary hypertension is caused by chronic thromboembolic pulmonary hypertension
WHO group 5 pulmonary hypertension is caused by
WHO group 5 pulmonary hypertension is caused by unclear or multifactorial mechanisms
WHO group pulmonary hypertension is caused by lung disease and/or hypoxia
WHO group 3 pulmonary hypertension is caused by lung disease and/or hypoxia
Why is it important to correctly categorize pulmonary hypertension?
Therapies differ between groups - Incorrect treatments can worsen clinical status
