Vascular Flashcards
Clinical classification of GPA
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
Maintenance therapy for GPA
RTX or MTX or AZA
Treatment of severe GPA/MPA
RTX (preferred over CYC)
and reduced dose GC
Indications for plasma exchange in DAH
Anti GBM + ANCA
salvage therapy
Trying to prevent dialysis
Treatment for non-severe GPA
GC + MTX
When to avoid rivaroxaban
Hepatic impairment
CrCl<30
CYP3A4 inducers or inhibitors
When to avoid dabigatran
CrCl <30
Better for liver
When to avoid apixaban
severe hepatic impairment
Better for kidneys because ok until CrCl <15
When to avoid Edoxaban
Hepatic impairment
use of rifampin
better for bad kidneys b/c ok until CrCl <15
Treatment of VTE in malignancy
1st line - LMWH (enoxaparin)
2nd line - oral factor Xa agents (edoxaban, apixaban, rivaroxaban)
YEARS criteria for work up of PE in pregnancy
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
Treatment of PAH in pt with sickle cell disease
endothelin-1 receptor antagonists
(ambrisentan, bosentan, macitentan
Why avoid sildenafil in pt with PAH and ssd
increased pain crisis
When to use calcium channel antagonist in PAH?
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
Endothelin receptor antagonist
Names and side effects
Anemia
Teratogenic
Bosentan– Liver injury
ambrisentan
Macitentan
DDI - cyclosporine
Bosentan - hormonal contraception, ritinovir
Phosodiesteras type 5 inhibitor
Names and side effects
Sildenafil
Tadalafil
Hypotension
visual changes, priapism
DDI- nitrates, riociguat, alpha blockers
Soluble guanylate cyclase stimulator
Name, effect, side effects
Riociguat
vasodilation
SE: teratogenic
hypotension, bleeding, PVOD, hemoptysis
DDI: Nitrate, PDE-5i, cigarette smoking, Maalox
Prostacyclin analog
Name, admin route, SE
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
Prostacyclin receptor agonist
Selexipag
Causes of pulmonary artery pseudoaneurysm
Infection (septic emboli, TB, syphilis pyogenic bactria and fungi (mucor and aspergillosis), most common)
Iatrogenic
trauma
Bronchogenic squamous cell carcinoma
sarcoma and metastatic sarcoma
Treatment of pulmonary artery Pseudoaneurysm
Embolization direct coil or of the feeding vessel
Stent
First line treatment of PE in pregnancy
Low molecular weight heparin (enoxaparin, dalteparin, tinzaparin)
Duration of therapy - at least 3-6 mo and continues for at least 6 weeks postpartum
Plt threshold for VTE ppx in pt with ITP
> 30 (usually >50 for pts w/o ITP)
Markers of Heritable PAH
BMPR2
ALK-1
Markers of HHT and PAH
Alk 1
BNPR1B
Mutation in PVOD and PCH
EIF2AK4
Characteristics of pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis
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
disease with endoglin mutation (ENG gene)
Hereditary hemorrhagic telangiectasia
Disease with FLCN gene mutation
Birt-Hogg-Dube syndrome
(cystic lung disease, fibrofolliculomas, kidney tumor)
Drugs that cause PAH
Fenfen
stimulants – meth, cocaine
chemo
interferon alpha and beta
hep c treatment
Primary treatment for CTD PH
Ambisartan and tadalafil
Risk factors for PH in HIV pt
IVDU
Female
cocaine use
Hep C
PE characteristics that increase risk of CTEPH
unprovoked
Increased BNP
RH strain
initial PASP on TTE >50
Delayed diagnosis of PE
History of VTE or recurrent VTE
Treatment for CTEPH
Riociguat
Macitentan
SQ trprostinil
NOT sildenafil or bosentan
Group 5 PH causes
Heme dz (SSD)
Metabolic disorders
Chronic renal failure
fibrosing mediastinitis
pulmonary tumor thrombotic microangiopathy
RHS on EKG
ST depression, TWI in V1-3
R axis deviation
dominant R wave in V1
Dominant S wave V5 or V6
CO calculation
SV x HR
PVR calculation
(mPAP-PAWP)/CO
SVR calculation
SVR= (mSAP-RAP)/CO
PAH risk assessment tool REVEAL lite
eGFR<60
NYAH functional class
Systolic BP
HR
6MWT
proBNP
Low risk </= 6
intermed 7-8
high >/= 9
used for monitoring treatment
PAH risk assessment 4 STRATA (or 3 Strata)
Estimate 1-yr mortality
WHO-FC
6MWT
BNP
Low risk 0-3%
inter-low 2-7%
Inter-high 9-19%
High >20%
initial tx for pt w/o cardiopulm comorbid and low/intermediate risk PAH
Endothelia receptor antagonist + PDE5i
initial tx for pt w/o cardiopulm comorbid and High risk PAH
ERA + PDE5i +/- IV/SQ Prostacyclin analog
initial tx for pt with cardiopulm comorbid and low/intermediate risk PAH
Monotherapy with PDE5i or ERA
If low intermediate on strata 4 what changes to make to PAH meds
Add PRA OR switch from PDE5i to sGCs
If High intermediate or high on strata 4 what changes to make to PAH meds
Add IV/SQ PCA and/or eval for lung transplant
Who needs indefinite AC for VTE?
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
Predictors of respiratory failure in ANCA vasculitis DAH
SpO2/FiO2 <450
Elevated CRP >25
Elevated neutrophils BAL >30%
What % of hemosiderine laden mac on BAL suggestive of DAH
> 20%
DAH in HF
Capillary stress failure (bland hemorrhage)
NOT treated with steroids