Pulmonary Vascular Disease - Presberg Flashcards
- What is the major cause of pulmonary embolism (PE)?
- What is a major risk factor for thromboembolism formation in the first place?
- DVT: 60-90% of PE originate in proximal deep veins of legs
- Thrombophilias
Name some mutations responsible for the thrombophilias that increase the risk of thrombosis / thromboembolism.
Mutation (result of mutation):
- Prot C or S (defective)
- ATIII (defective)
- Factor V (un-inactivatable by Prot C)
- aka Factor V Ledien
- Prothrombin (activating)
- MTHFR (some enzyme that helps with homocysteine → methionine pathway)
- Factor VIII (activating)
Name some medical risks of venous thromboembolism aside from thrombophilias (think of Virchow’s Triad).
- Hip or knee surgery
- Immobility
- CHF
- Obesity
- Malignancy
What is the prognosis of PE if untreated?
If treated?
Untreated: 30%
Treated: 1-3% (worse with chronic PE dur to underlying risk factors)
Describe some nonspecific symptoms of PE.
- Dyspnea (acute or subacute)
- Dizziness & syncope
- massive / saddle PE
-
Pleuritic chest pain
- infarct / bloody effusion
- Palpitations & Tachycardia
- Hemoptysis
- infarct
What type(s) of imaging are typically used to diagnose:
- DVT?
- PE?
- DVT
- Doppler Ultrasound
- PE
- CT pulmonary angiogram
- Ventilation perfusion scans
- Will see mottled/patchy perfusion pattern instead of a nice solid shape
- Some fuzz around the edges of the perfused area is normal - look for major gaps
What are the five groups of chronic pulmonary HTN disorders according to the WHO classification scheme?
What categorization was recently abolished?
I. Pulmonary Arterial HTN (PAH)
- “Precapillary”
- Idiopathic or caused by things not listed below
- e.g. drugs, infections (HIV, Schistosomiasis), genetic disorder, congenital disorder, etc.
II. Pulm HTN + left heart disease
- Most common form
III. Pulm HTN + lung diseases
- e.g. COPD, interstitial lung diseases
- FVC < 70%
IV. Pulm HTN + chronic thromboembolic disorders
V. Pulm HTM + Misc diseases
- Tumors
- Blood disorders
- Systemic disorders (sarcoidosis, vasculitis)
- Metabolic disorders
Primary vs. Secondary classification recently eliminated
What is the single most common etiology of Pulmonary HTN?
Left heart disease
What is the specific diagnostic criteria for Group I Pulmonary Arterial HTN (PAH)?
- Mean pulmonary arterial pressure (mPAP) > 25mmHg at rest
-
Normal wedge pressure (PCWP)
- Recall: PWCP estimates left heart filling pressure
- Abnormal would likely indicate Group II
- This is important differential as Group II is most common type
- Not Groups II, III, IV, or V
What is the best noninvasive test for diagnosing PH? (Pulmonary HTN)
What findings are sought using this test?
Echcardiogram
Look for:
- R atrial and ventricular changes
- R ventricular overload
- Estimate PA pressure
- Congenital heart disease, PFO, or shunt
- Pericardial effusion
- Impacts prognosis
What are the specific criteria that indicate decreased RV function?
- Right Atrial Pressure (RAP) > 10mmHg
- CI < 2.2 L/min•m2
- What heritable gene mutation is often associated with cases of Group I PAH (aka idiopathic PAH or IPAH)
- What is the inheritance pattern?
- How much of IPAH is due to this?
- BMPR2 mutations
- “Bone morphogenic protein receptor type II” (name likely not important)
- Normal receptor helps inhibit proliferation of vascular smooth muscle - without it, overproliferation leads to PH
-
Auto Dom w/ low penetrance
- Pathoma: Typically seen in young females
- 10% of IPAH is heritable, 55% of heritable IPAH is from BMPR2
- What specific (not directly inherited) disease is also associated with Group 1 PAH?
- From a few slides ago: do you recall other more general causes of Group I PAH?
- Scleroderma
- Other causes:
- Infection (HIV, schistosoma)
- Drugs
- Inherited or congenital disorder
- Portal HTN / liver disease
The 5 WHO PAH Groups describe the causes of PAH.
On the other hand, the 4 WHO/NYHA PAH functional Classes describe the severity of PAH.
Describe these 4 PAH classes.
-
Class I
- Sxs do not limit physical activity
- Ordinary activity does not cause undue comfort
-
Class II
- Slight limitation of activity
- Comfortable at rest
- Experiences sxs with ordinary activity
-
Class III
- Marked limitation of activity
- Comfortable at rest
- Expeiences sxs with minimal activity
-
Class IV
- Inability to carry out any activity
- Sxs may be present at rest
- Discomfort increased by any activity
- Manifest signs of right heart failure
What vascular pathologies can be noted in Group I PAH?
Which is pathognomonic to Group I PAH? Hint: Image
- Large pulm. Artery Thickening
- Medial wall smooth muscle hypertrophy
-
Plexiform Lesion in small vessels
- Pathognomonic
