Pulmonary Vascular Disease - Presberg Flashcards Preview

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Flashcards in Pulmonary Vascular Disease - Presberg Deck (26):

  1. What is the major cause of pulmonary embolism (PE)?
  2. What is a major risk factor for thromboembolism formation in the first place?

  1. DVT: 60-90% of PE originate in proximal deep veins of legs
  2. 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?


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


  1. What heritable gene mutation is often associated with cases of Group I PAH (aka idiopathic PAH or IPAH)
  2. What is the inheritance pattern?
  3. How much of IPAH is due to this?

  1. 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
  2. Auto Dom w/ low penetrance
    • Pathoma: Typically seen in young females
  3. 10% of IPAH is heritable, 55% of heritable IPAH is from BMPR2


  1. What specific (not directly inherited) disease is also associated with Group 1 PAH?
  2. From a few slides ago: do you recall other more general causes of Group I PAH?

  1. Scleroderma
  2. 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

Q image thumb

  • Large pulm. Artery Thickening
  • Medial wall smooth muscle hypertrophy


  • Plexiform Lesion in small vessels
    • Pathognomonic


  1. What 3 major endothelial cell defects have been associated with PAH?
  2. What two core effects on vasculature do all three of these pathways share?
  3. Why are these defects important?

  1. Endothelial cell defects:
    • Decreased prostacyclin
    • Increased endothelins
    • Decreased NO Synthase
  2. All three affect:
    • degree of vascular tone
      • (prost and NO dilate, endothelins constrict)
    • level of SMC proliferation
      • (prost and NO inhibit, endothelins activate)
  3. These three pathways are implicated in PAH pathogenesis and are targets for current and emerging therapies


Name the drug form of prostacyclin given to treat PAH.



Name a endothelin receptor antagonist used to treat PAH.


(Bos-EN-t-AN is an ENdothelin receptor ANtagonoist)


Name two drugs used to treat PAH by increased NO levels. How do they work?

  1. Sildenafil (Viagra!)
    • Increases cGMP
  2. Riociguat
    • soluble guanylate cyclase stimulator
    • very recent


What is the morphology of Pulmonary Vasculitis?

  • Pulmonary & alveolar hemorrhage
  • Lung infiltrates
    • nodular
    • cavitary lesions
      • "gas-filled areas of the lung in the center of a nodule, mass or area of consolidation"
    • Associated with interstitial lung disease


Name 4 types of alveolar hemorrhage syndromes

  1. Goodpasture's Syndome
    • aka anti-basement membrane Ab disease / ABMAb
  2. Wegener's Granulomatosis
    • aka ANCA-associated vasculitis
  3. Idiopathic Pulmonary Hemosiderosis
  4. Collagen vascular diseases
    • e.g. SLE


What is the classic triad of symptoms in diagnosis of alveolar hemorrage syndromes?

  1. Hemoptysis
  2. Pumonary infiltrates
  3. Anemia


What are three life-threatening complications of alveolar hemorrhage syndromes?

  1. Respiratory failure
  2. Acute renal failure
  3. Severe anemia


Name some lab and imaging techniques used to diagnose the underlyign causes of alveolar hemorrage syndromes.

  • sputum / tracheal aspirate
  • bronchoalveolar lavage (BAL)
    • heart failure cells
  • urine (may alert you to other organ problems)
  • serial Hgb
  • Serial CXRs
  • Serology:
    • ABMAb
    • ANCA
    • ANA
  • Biopsy:
    • Lung
    • Skin (when a more systemic cause is suspected)
    • Renal (ditto)


(Sort of review)

What drugs are often used to treated vasculitis diseases?

Long-term immune suppression!

  • Prednisone
  • Cyclophosphamide
  • Rituximab
    • (anti CD20 mAb → anti B-cell, if you forgot)


Which part of the respiratory tract is affected first in Wegener's Granulomatosis?

Upper respiratory tract affected before (and somewhat more often than) Lower tract

Decks in M2 Renal/Respiratory Class (50):