Clinical Part 3 pHTN, PE, OSA, ILD (Pence)

Question 1

What is pulmonary HTN?

Answer 1

mean pulmonary artery pressure > 25mmHg

associated with mortality if untreated

Question 2

What are the symptoms of pHTN?

Answer 2

DOE, fatigue, pleuritic chest pain, pre-syncope, edema, JVP

Question 3

What are the cardinal signs of Right HF?

Answer 3

syncope on exertion

excessive and rapid weight gain

JVP

lower extremiyt swelling

Question 4

How is pHTN diagnosed?

Answer 4

EKG changs: RAD, iBBB, RAE in lead II

Labs: increased BNP

Transthoracis Echocardiogram (TTE): estimates pulm. artery systolic pressure, looks at RV size

Cardiac catheterization (Swan Ganz catheterization)

Question 5

What is the best way to diagnose pHTN?

Answer 5

Swan Ganz catheterization

Question 6

How is pHTN treated?

Answer 6

Treat underlying cause (HF, COPD, ILD, OSA, etc)

Prostacyclin agonist: epoprostenol, iloprost, selexipag

PDE inhibtor: tadalafil, sildenafi

Endothelium antagonist: bosentan, ambrisentan, macicentran

CCB: amolidipine, nifedipine, diltiazem

Question 7

What is a PE?

Answer 7

VTE located in pulmonary vasculature

–> usually arising from a DVT

Question 8

What is virchow’s triad?

Answer 8

hypercoagulability

venous stasis

endothelial injury

Question 9

What are the sx of PE?

Answer 9

chest pain

palpitations

dyspnea

syncope

+/- LE edema

Consider HF and Arrythmia as DSS

Question 10

How is PE diagnosed?

What is the Gold Standard?

Answer 10

Well’s criteria!!! or Geneva Score

LABS

D-Dimer (sensitive, rules out PE if normal)

EKG changes: sinus tach, S1Q3T3

CT Chest with Contrast is gold standard

VQ scan is second line image

Question 11

If a D shaped LV champer is seen on Echo, what does this indicate?

Answer 11

PE

Question 12

What is the treatment for an unstable PE?

(presents with hypotension)

Answer 12

1. resuscitation
2. thrombolytic therapy

if thrombolytic therapy fails, repeat thrombolysis, consider catheter-directed thrombolysis, or proceed to surgery

Question 13

How to treat a stable PE?

Answer 13

Heparin

LMWH

Warfarin

DOACs

Question 14

What is the mechanism of direct oral anticoagulants? (DOACs)

Do they require bridge therapy?

What are the cons?

Answer 14

Xa inhibitors-rovaroxaban, apixaban

approved to be used without LMWH bridge

expensive, may be hard to reverse

Question 15

How long is treatment for PE?

Answer 15

at least three months

Pt’s with provoked DVT from travel, surgery or HRT do not require extended therapy

Question 16

Who requires indefinite anticoagulation after PE?

Answer 16

those with underlying disease with high risk of VTE recurrence

• malignancy
• genetic mutations

Question 17

What is the definition of OSA?

Answer 17

disruption in breathing pattern while sleeping that results inexcessive daytime somnolence despite adequate sleep periods and not explained by other causes

(snoring, gasping for air, breathing pause)

Question 18

What is the definition of apnea?

Answer 18

reduction in breathing for at least 10 seconds with a noted drop in SpO2 by >3%

Question 19

How is the Apnea-Hypopnea index calculated?

Answer 19

number of apnea episodes/hour

determines OSA severity

Question 20

If OSA is untreated, what can occur?

Answer 20

death

Sleep related breathing disorders are associated with increased morbidity and mortality if left untreated

Question 21

What is the number 1 cause/predictor of OSA?

What facial malformation can cause OSa?

Answer 21

obesity

micrognathia (small mandible)

Question 22

What is the diagnostic criteria for OSA?

Answer 22

STOP-BANG

Question 23

What is the Gold standard for diagnosing OSA?

Answer 23

PSG

occurs in sleep lab or at home, records sleep activity for 6-7 hrs

Monitors EEG, ECG, ocular movement, airflow, and O2sat

allows clinician to generate AHI and diagnose the severity of sleep apnea

Question 24

What are the treatment options for OSA?

Answer 24

CPAP

oral appliances

Question 25

What is interstitial lung disease?

Answer 25

group of pulmonary disorders with a variety of different causes that present with the same characteristics

Question 26

what are the general characteristics of ILD?

Answer 26

restrictive pattern on PFTs Decreased DLCO DOE absence of infection or malignancy

Question 27

What part of the lung is affected with ILD?

Answer 27

the CT that supports the alveoli and capillaries to allow gas exchange

Question 28

What are the main findings associated with idiopathic pulmonary fibrosis?

Answer 28

Velcro Lung Honeycombing treat with supportive care, steroids, imunimodulators, anti-fibrotics 50% survival at 3-5 years; early diagnosis=better prognosis

Question 29

What are the main findings of Sarcoidosis?

Answer 29

non-caseating granulomas, lungs commonly affected More common in African Americans, females, family members Associated with Lofgren's syndrome and Heerfordt's syndrome

Question 30

What is Lofgren's syndrome What is Heerfordt's syndrome?

Answer 30

Erythema nodosum, hilar lymphadenopathy, fever, arthritis Anterior uveitis, parotitis, CN VII palsy, fever

Question 31

What is the treatment for sarcoidosis?

Answer 31

supportive care steroids: 1st line therapy immunosupression, biologics

Question 32

What are the main findings in granulomatosis with polyangitis

Answer 32

small vessel vasculitis affecting sinuses, lungs, kidneys +C-ANCA Treat with steroids and cyclophosphamide

Question 33

What are the main findings in Goodpasture's syndrome?

Answer 33

autoimmune condition with Anti-GBM against the basement membrane in lungs and kidneys +Anti-GBM treat with plasmaphoresis

Question 34

What are some ILD assicated with CT disease?

Answer 34

Sclerosis RA Dermatomyosistis/Polymyositis

Question 35

What are some causes of hypersensitivity pneumonitis

Answer 35

repeated expsures to specific antigens that cause an extrinsic allergic alveolitis - farmer's lung - Bird fancier's lung - woodworker's lung - baker's lung

Question 36

What are some common symptoms of HSN pneumonitis? What is seen on histology?

Answer 36

cough, dyspnea sx improve on vacation PLasma cells on histology

Question 37

What are some findings associated with Silicosis?

Answer 37

seen in miners, stone cutters, quarry workers nodular lung disease and calcified hilar LN-simple silicosis large nodules with fibrosis-complicated silicosis increased risk for infection and TB

Question 38

What are some findings associated with asbestosis?

Answer 38

insulation workers, construction, demolition nodular opacities, effusions, fibrosis, increased risk for mesothelioma esp if smoker

Question 39

What are some findings associated with coal-workers pneumoconiosis?

Answer 39

miners who inhale coal dust may be asymptomatic or have small nodules can have cough, dysnpea, restrictive pattern, and fibrosis with large nodules (Complicated CWP)

Question 40

What are some findings associated with Berylliosis?

Answer 40

manufacturers of electronics similar to HSN pneumonitis in acute setting chronically, can have hilar LAD, infiltrates, restrictive pattern, obstruction due to LAD enlargement increased risk for lung cancer **treat with steroids**

Question 41

What kind of biopsy is needed to diagnose asbestosis?

Answer 41

pleural biopsy