Vascular Flashcards

1
Q

Clinical classification of GPA

A

Nasal involvement
cartilaginous involvement (hearing loss, hoarseness, endobronchial, saddle nose)

+C ANCA or PR3
pulm nodules, mass or cavitation
granulomas or giant cells on biopsy
pauci-immune glomerulonephritis

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2
Q

Maintenance therapy for GPA

A

RTX or MTX or AZA

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3
Q

Treatment of severe GPA/MPA

A

RTX (preferred over CYC)
and reduced dose GC

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4
Q

Indications for plasma exchange in DAH

A

Anti GBM + ANCA
salvage therapy
Trying to prevent dialysis

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5
Q

Treatment for non-severe GPA

A

GC + MTX

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6
Q

When to avoid rivaroxaban

A

Hepatic impairment
CrCl<30
CYP3A4 inducers or inhibitors

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7
Q

When to avoid dabigatran

A

CrCl <30

Better for liver

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8
Q

When to avoid apixaban

A

severe hepatic impairment

Better for kidneys because ok until CrCl <15

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9
Q

When to avoid Edoxaban

A

Hepatic impairment
use of rifampin

better for bad kidneys b/c ok until CrCl <15

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10
Q

Treatment of VTE in malignancy

A

1st line - LMWH (enoxaparin)
2nd line - oral factor Xa agents (edoxaban, apixaban, rivaroxaban)

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11
Q

YEARS criteria for work up of PE in pregnancy

A

3 criteria: 1. signs of DVT 2. hemoptysis 3. PE as most likely diagnosis

If none of criteria met and D dimer <1000 PE ruled out

If one or more of 3 criteria met and D dimer <500 PE ruled out

Most effective during 1st trimester
D-dimer should not be used alone

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12
Q

Treatment of PAH in pt with sickle cell disease

A

endothelin-1 receptor antagonists

(ambrisentan, bosentan, macitentan

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13
Q

Why avoid sildenafil in pt with PAH and ssd

A

increased pain crisis

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14
Q

When to use calcium channel antagonist in PAH?

A

If patient has vasoreactivity in reponses to inhaled NO (ie pulm vaso dilator) – reduction of PAP by >10 and reaching absolute mean PAP <40

Never use for pts with RHF

preferred agents - amlodipine, dilt, nifedipine

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15
Q

Endothelin receptor antagonist
Names and side effects

A

Anemia
Teratogenic
Bosentan– Liver injury
ambrisentan
Macitentan

DDI - cyclosporine
Bosentan - hormonal contraception, ritinovir

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16
Q

Phosodiesteras type 5 inhibitor
Names and side effects

A

Sildenafil
Tadalafil

Hypotension
visual changes, priapism

DDI- nitrates, riociguat, alpha blockers

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17
Q

Soluble guanylate cyclase stimulator

Name, effect, side effects

A

Riociguat
vasodilation

SE: teratogenic
hypotension, bleeding, PVOD, hemoptysis

DDI: Nitrate, PDE-5i, cigarette smoking, Maalox

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18
Q

Prostacyclin analog

Name, admin route, SE

A

Epoprostenol – IV
Iloprost - Inhaled
Treprostinil - oral, Inhaled, IV and SC

SE: vasodilatory effects – headache, flushing, jaw pain, limb pain, n, diarrhea, dizziness
thrombocytopenia
rebound PH

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19
Q

Prostacyclin receptor agonist

A

Selexipag

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20
Q

Causes of pulmonary artery pseudoaneurysm

A

Infection (septic emboli, TB, syphilis pyogenic bactria and fungi (mucor and aspergillosis), most common)
Iatrogenic
trauma
Bronchogenic squamous cell carcinoma
sarcoma and metastatic sarcoma

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21
Q

Treatment of pulmonary artery Pseudoaneurysm

A

Embolization direct coil or of the feeding vessel
Stent

22
Q

First line treatment of PE in pregnancy

A

Low molecular weight heparin (enoxaparin, dalteparin, tinzaparin)

Duration of therapy - at least 3-6 mo and continues for at least 6 weeks postpartum

23
Q

Plt threshold for VTE ppx in pt with ITP

A

> 30 (usually >50 for pts w/o ITP)

24
Q

Markers of Heritable PAH

A

BMPR2
ALK-1

25
Q

Markers of HHT and PAH

A

Alk 1
BNPR1B

26
Q

Mutation in PVOD and PCH

A

EIF2AK4

27
Q

Characteristics of pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis

A

PAH + venous involvement
Low DLCO, out of proportion hypoxemia
No left sided heart disease
Mutation EIF2AK4
Lack of response or development of pulmonary edema with PAH-specific therapy

CT with diffuse interlobular septal thickening, ggo mosaic, pleural effusion, enlarged PA

28
Q

disease with endoglin mutation (ENG gene)

A

Hereditary hemorrhagic telangiectasia

29
Q

Disease with FLCN gene mutation

A

Birt-Hogg-Dube syndrome

(cystic lung disease, fibrofolliculomas, kidney tumor)

30
Q

Drugs that cause PAH

A

Fenfen
stimulants – meth, cocaine
chemo
interferon alpha and beta
hep c treatment

31
Q

Primary treatment for CTD PH

A

Ambisartan and tadalafil

32
Q

Risk factors for PH in HIV pt

A

IVDU
Female
cocaine use
Hep C

33
Q

PE characteristics that increase risk of CTEPH

A

unprovoked
Increased BNP
RH strain
initial PASP on TTE >50
Delayed diagnosis of PE

History of VTE or recurrent VTE

34
Q

Treatment for CTEPH

A

Riociguat
Macitentan
SQ trprostinil

NOT sildenafil or bosentan

35
Q

Group 5 PH causes

A

Heme dz (SSD)
Metabolic disorders
Chronic renal failure
fibrosing mediastinitis
pulmonary tumor thrombotic microangiopathy

36
Q

RHS on EKG

A

ST depression, TWI in V1-3
R axis deviation
dominant R wave in V1
Dominant S wave V5 or V6

37
Q

CO calculation

A

SV x HR

38
Q

PVR calculation

A

(mPAP-PAWP)/CO

39
Q

SVR calculation

A

SVR= (mSAP-RAP)/CO

40
Q

PAH risk assessment tool REVEAL lite

A

eGFR<60
NYAH functional class
Systolic BP
HR
6MWT
proBNP

Low risk </= 6
intermed 7-8
high >/= 9

used for monitoring treatment

41
Q

PAH risk assessment 4 STRATA (or 3 Strata)

A

Estimate 1-yr mortality

WHO-FC
6MWT
BNP

Low risk 0-3%
inter-low 2-7%
Inter-high 9-19%
High >20%

42
Q

initial tx for pt w/o cardiopulm comorbid and low/intermediate risk PAH

A

Endothelia receptor antagonist + PDE5i

43
Q

initial tx for pt w/o cardiopulm comorbid and High risk PAH

A

ERA + PDE5i +/- IV/SQ Prostacyclin analog

44
Q

initial tx for pt with cardiopulm comorbid and low/intermediate risk PAH

A

Monotherapy with PDE5i or ERA

45
Q

If low intermediate on strata 4 what changes to make to PAH meds

A

Add PRA OR switch from PDE5i to sGCs

46
Q

If High intermediate or high on strata 4 what changes to make to PAH meds

A

Add IV/SQ PCA and/or eval for lung transplant

47
Q

Who needs indefinite AC for VTE?

A

Men - unprovoked
Women with risk factors
- hyperpigmentation, edema or redness of leg
- D-dimer >250 while on AC
- Obesity (BMI>30)
-Older age (>65)

If low risk (0-1 risk factor) and unprovoked in women can stop after 3 mo

48
Q

Predictors of respiratory failure in ANCA vasculitis DAH

A

SpO2/FiO2 <450
Elevated CRP >25
Elevated neutrophils BAL >30%

49
Q

What % of hemosiderine laden mac on BAL suggestive of DAH

A

> 20%

50
Q

DAH in HF

A

Capillary stress failure (bland hemorrhage)

NOT treated with steroids