Pulmonary Htn Flashcards
Definition phtn
Normal value
Increase in mean pap> 25mmhg at rest as assessed by rh catheterization
14+_3 upper limit-20
What is PAH
subgroup of ph with
Pawp<15 with pvr>3wood units in absence of other precapillary htn su h as lung diseases ,pte
Group 1 pah
Idiopathic Familial - bmpr 2, alk-1 Ass with ctd/hiv/chd/portal htn/ parasites/ drugs Pulm veno occlusive diaease Persistent htn of new born
Group 2
Phtn due to left heart failure
Group 3
Due to limg disease/ hypoxemia
Group 4
Cteph Other pulmonary artery obstruction- angio sarcoma Intravascular tumor Areteritis Cong.pa stenosis Parasitic(hydatidosis)
Group 5
Unclear or multifactorial mechansims Hematological Systemic disorder- sarcoid , lch, lam , nf Metabolic- storage disorders. Other- segmental htn Crf Fibrosing mediastinitis
Who functional classification
1- no limitation of physical activity
2- slight limitation
3- marked
4@ inability to carryout any physical activity without symptoms
Pathogenesis of group 1
Genetic +risk factors- endothelial cell injury and imbalance b/w vd and vc , apoptotic and mitogenic factors, thrombotic and antithromobtic
Causes vc, remodelling , thrombosis
Inc pa resistance ane pressure
Pathogenesis of group 2
Valvular disease - inc la pressures - interstitial edema + pa vc- vascular changes- pah
Pathogenesis of group 3
Hypoxia- inc circuoatory bdnf (brain derived neurotrophic factor)
Dec no- dec vd
Smooth muscle proliferation
Inc vasoconstriction
Pathogenesis group 4
Pe- incomplete resolution/organisation of thrombus- occlusion/ shear stress in non obstructed vessel - inc pa pressure and resistance
Diagnosis Ecg Cxr Echo V/q
Rvh , cor pulmonale
Enlargement of central pulmonary arteries ,rvh
Pasp>40
For embolism
Angio Pft Oximetry Usg 6mwt
For embolism Undrlying lung disease Osa Portal htn For severity
Right heart catheterization
To confirm diagnosis At rest 25 Exercise 30 Wedge pressure <15 Pvr >240 dines cm sec
Vasoreactive test Why Recommended in whom Agents Interpretation Use
To detect residual properties of vadodilation of small pulmonary arteries and arterioles
In group 1
Inhaled no , enoprostenol iv. Adenosine
Pos- dec in 10 and value less than 40 with an increased or unchanged co and minimally reduced or unchanged bp
Use- in positive pts ,ccb high dose candidates
Group 1 drug therapy
Ccb- nifedipine- 90-180mg /day
Diltiazem 240-720
Amlodepine 20
S/e- Hypotension, edema , syncope , rv failure
Endothelial receptor antagonist
Ambrisentan 5-10
Bosentan 125be
Macitentan 3-10 /day
S/e: leg swelling , fluid retention, hepato Toxicity, teratogenic
Pde5
Sildenafil 20 tds
Tadalafil
S/e - headache , chanegs in vision, not usedl with nitrates ,hypotensikn
Guanylate cyclase inhibitor
Riociguat- 2.5 tds
Syncope , headache, edema , diarrhoea
C/I in pregnancy. Pde5 inh
Ip receptor
Selexipag-
Prostacyclin analouges
Epoprostenol-iv
Iloprost- inqhled iv
Treprostinil
Beraprsot- oral
Jawpain, diarrhoeaz achesvz
Combination therapy
Macitentan plus sildenafil
Riociguat plus bosentan
Selecipag +era/pde5 inhibitors
Algorithm
Vr test pos- ccb
Negative- who 2, 3- single or combination
4- initiak combination
Inadequate response- double/ triple-rx
Sx
Balloon arterial septostomy
Benefit in fc-4 with rhf refractory to medical therapy
No mortality benefit
Ix of lung tx
Nyh 3, 4 desoite 3 minths combination theraoy including prostanoids
Cardiac index less than 2 lit /m2
Mrap >15
6mwt- <35
Group 2
Diuretics
Prostanoids. Era, pde5- some benefit
3
Ltot
Ccb x
Group 4
Life long anti coagulation
Diuretics o2
Riociguat
Ivf filter
