Pulmonary Hypertension Flashcards
WHO clinical classifiation of PH
group 1 - pulmonary artery hypertension
group 2 - PH owing to left heart disease
group 3 - PH owing to lung diseases or hypoxia
group 4 - chronic thromboembolic PH
group 5 - PH with unclear multifactorial mechanisms
drivers of group 1 PAH rise in PVR
vasoconstriction
remodeling of pulmonary vessel wall
in situ thrombosis
inflammation
drivers of WHO group 2 PH
passive oxygen congestion of pulmonary vasculature by back up of blood from left heart disease
out of proportion PH or persistent PH even after diuresis and decreasing left heart filling pressures - fixed vascular remodeling
drivers of WHO group 3 PH
hypoxic vasoconstriction
endothelial cell dysfunction with imbalance of vasodilators and vasoconstrictors
destruction of capillary bed
drivers of WHO group 4 CTEPH
chronic clot in pulmonary arterial bed
common pathologic findings of WHO group 1 PAH
smooth muscle hypertrophy
neointima formation and neovascularization
in situ thrombosis inflammation
distal extension of smooth muscle into non-muscular PA
endothelial cell proliferation (monoclonality)
common pathologic findings of WHO group 2 and 3
pulmonary arterial side medial thickening of muscular arteries
no endothelial proliferation
smooth muscle hypertrophy
also see changes of the venous side
occlusive venopathy with fibrous intimal thickening
lymphatic dilation and congested alveolar capillaries
clinical signs of PAH
accentuated second heart sound
notable heart murmur - tricuspid valve regurgitation
RV lift
RV failure - right sided third heart sound, JVD, hepatomegaly, peripheral edema, ascites
hemodynamic and clinical course of PAH
constant incrase in PVR
PAP increases and then falls off with CO
CO falls off first
BNP starts being released as CO begins to fall
What is the best method of diagnosis for evaluating Chronic Thromboembolic Pulmonary Hypertension?
V/Q scan
normal scan makes CTEPH unlikely
greater than one segmental sized or larger mismatched perfusion defect sween with CTEPH
What is the gold standard test for pulmonary hypertension?
right heart catheterization
definiteive diagnosis - direct measurement of RAP, PAP, PCWP, CO
calculation of PVR, CI
Why is characterizing the type of PH important?
therapies differ drastically between groups
incorrect treatment can worsen a patient’s clinical status
PAH treatments are costly
treatment of group 1 PAH
endothlin blockers
nitric oxide promoters
prostacyclin pathway blockers
endothelin pathway blockers
ambrisentan
bosentan
macitentan
nitroc oxide promoters
sildenafil
tadalafil
riociguat
