20 - Pulmonary Vascular Disease Flashcards
What factors influence pulmonary vascular resistance?
- recruitment and distension
- lung volume
- intrathoracic pressure
- increased viscosity
- decreased vessel radius
How much pulmonary vascular destruction needs to occur before pulmonary hypertension occurs at rest/
50-70%
What is the definition of pulmonary arterial hypertension? What is the definition of pulmonary venous hypertension?
Arterial - mean arterial pressure ≥ 25 mmHg at rest, with a pulmonary capillary wedge pressure ≤ 15 mmHg (pre-capillary)
Venous - pulmonary capillary wedge pressure > 15 mmHg (post-capillary)
How does hypoxia cause pulmonary hypertension?
- vasoconstriction
- remodeling (smooth muscle where it normally doesn’t belong)
What are the clinical findings of pulmonary hypertension?
- dyspnea
- syncope
- lower extremity edema
- JVD
- tricuspid regurgitation murmur
- right ventricular heave
- loud P2 (from pulmonic closure)
Cor pulmonale is [right/left]-sided heart failure from lung disease. What causes it?
Right sided
Due to increased pulmonary vascular resistance and pulmonary hypertension
What are the acute and chronic causes of cor pulmonale? Which can lead to right ventricular failure and shock?
Acute - can lead to RV failure and shock if mean PA > 40
- massive pulmonary embolism
Chronic
- idiopathic pulmonary arterial hypertension
- pulmonary hypertension secondary to COPD or IPF
What are the 5 WHO groups for pulmonary hypertension? What are their causes? Which is the most common? What are their associated PVRs and PCWPs?
1 - Pulmonary Artery Hypertension (PAH) - idopathic - connective tissue disease - HIV - cirrhosis - congenital heart disease - drug induced Precapillary, greatly increased PVR, normal PCWP
2 - Pulmonary Venous Hypertension (most common)
- left heart disease (systolic or diastolic dysfunction)
Postcapillary, normal PVR, increased PCWP
3 - Hypoxia or Lung Issue - sleep apnea - obesity hypoventilation syndrome - high altitude - COPD - IPF precapillary, increased PVR, normal PCWP
4 - Thromboembolic
Precapillary, increased PVR, normal PCWP
5 - Miscellaneous
What is the pathogenesis of pulmonary hypertension?
- imbalance between vasodilators (decreased NO and prostacyclin) and vasoconstrictors (increased thromboxane and endothelin)
- fibrinolytic defects
- increased smooth muscle proliferation in pulmonary arteries/arterioles
NO is a [vasodilator/vasoconstrictor]. It is [increased/decreased] in pulmonary hypertension.
vasodilator; decreased
Prostacyclin is a [vasodilator/vasoconstrictor]. It is [increased/decreased] in pulmonary hypertension.
vasodilator; decreased
Thromboxane is a [vasodilator/vasoconstrictor]. It is [increased/decreased] in pulmonary hypertension.
vasoconstrictor; increased
Endothelin is a [vasodilator/vasoconstrictor]. It is [increased/decreased] in pulmonary hypertension.
vasoconstrictor; increased
What mutation is associated with pulmonary hypertension? What is the result of it?
BMPR2 –> increased TGF-beta induced smooth muscle cell proliferation in pulmonary arteries/arterioles
What is the best screening test for pulmonary hypertension?
cardiac echo
What is the workup required to make a diagnosis of pulmonary hypertension?
- chest x ray
- EKG
- cardiac echo
- PFTs
- ABG
- V/Q scan or CT-PA for pulmonary emboli
- testing for HIV/cirrhosis/connective tissue disease
- right heart catheterization
What is the appropriate treatment for pulmonary hypertension?
Group 1:
- oxygen
- anticoagulation
- vasodilators (calcium channel blockers)
- endothelin antagonists
- prostacyclins
- PDE-5 inhibitor
Group 2-5: treat underlying disease
What effect does prostacyclin treatment have in pulmonary hypertension?
- anti-proliferative effect on smooth muscle and endothelium
- antiplatelet effect
What effect does PDE-5 inhibitor treatment have in pulmonary hypertension?
- increased cGMP
- guanylate cyclase stimulant (increases cGMP)
What is Virchow’s triad?
Risk factors for pulmonary embolsim
- stasis or immobilization
- inflammation or injury
- hypercoaguability (cancer, surgery, stroke, tobacco, OCPs, etc.)
Why is a D-dimer test useful in regards to pulmonary embolism?
It has a high negative predictive value - if clinical suspicion is low and test is negative, you can rule out pulmonary embolism
What is the most common x-ray finding with pulmonary embolism?
atelectasis
What is the origin of most pulmonary emboli?
lower extremity DVT
What are the clinical presentations of pulmonary embolism?
- infarction syndrome or pleuritic chest pain + dyspnea + fever + hemoptysis (65%)
- unexplained dyspnea or hypoxemia (22%)
- massive pulmonary embolism (8%)
What are the signs of an acute massive pulmonary embolism?
- signs of right sided heart failure
- right heart strain on EKG
- hypotension
What is the Wells Criteria used for?
determining pretest probability of PE
What is the treatment for acute/chronic pulmonary embolism? What can be done prophylactically?
Acute PE
- low molecular weight heparin (subcutaneous injection) or heparin (IV)
- fondaparinux (Xa inhibitor)
- oral direct factor Xa inhibitor
Chronic PE
- warfarin (vitamin K antagonist)
- rivaroxaban (Xa inhibitor)
Prophylaxis
- heparin
- venous compression boots
Pulmonary embolism has a [normal/wide] A-a gradient.
Wide