Vascular Lung Disease (PH)- Bootcamp Flashcards
The difference between pulmonary venous hypertension and pulmonary arterial hypertension is that PVH originates with a problem in the _ while PAH is a problem with the _ themselves
The difference between pulmonary venous hypertension and pulmonary arterial hypertension is that PVH originates with a problem in the heart while PAH is a problem with the pulmonary arteries themselves
The most common causes of PVH include _ and _
The most common causes of PVH include left heart failure and valvular disease (mitral stenosis, mitral regurgitation)
* Both of these cause fluid to back up to the left atrium –> pulmonary veins
(PVH/ PAH) tends to present with pulmonary edema
PVH tends to present with pulmonary edema
* Veins –> Capillaries –> edema
Pulmonary arterial hypertension can be defined as _
Pulmonary arterial hypertension can be defined as BP >= 20 mmHg originating in the pulmonary arteries
The primary cause of PAH is generally an intrinsic vascular change that involves _ endothelin and _ NO
The primary cause of PAH is generally an intrinsic vascular change that involves increased endothelin and decreased NO
* This imbalance will lead to vasoconstriction
PAH is associated with extensive vascular remodeling and thickening of the intima, media, and adventitia; this is called _
PAH is associated with extensive vascular remodeling and thickening of the intima, media, and adventitia; this is called arteriolosclerosis
What histologic changes are seen in PAH?
Intima:
Media:
Adventitia:
What histologic changes are seen in PAH?
Intima: endothelial hyperproliferation
Media: smooth muscle hyperproliferation
Adventitia: inflammatory cell recruitment & fibrosis
_ are “slit-like “channels” of endothelial cells within the occluded vessel lumen that are surrounded by smooth muscle cells, myofibroblasts, connective tissue matrix, platelet aggregates, and inflammatory cells” that are characteristic of PAH
Plexiform lesions are “slit-like “channels” of endothelial cells within the occluded vessel lumen that are surrounded by smooth muscle cells, myofibroblasts, connective tissue matrix, platelet aggregates, and inflammatory cells” that are characteristic of PAH
In response to injury, we may see proliferation and migration of vascular smooth muscle cells to the tunica intima in PAH; this forms what we call _
In response to injury, we may see proliferation and migration of vascular smooth muscle cells to the tunica intima in PAH; This forms what we call neointima lesions
Primary PAH in young adult females may be associated with a gene mutation in _
Primary PAH in young adult females may be associated with a gene mutation in BMPR2
* BMPR2 normally functions to keep the smooth muscle in the pulmonary vasculature in check
* Primary PAH can also be associated with scleroderma
Secondary PAH is usually caused by _
Secondary PAH is usually caused by chronic hypoxic pulmonary vasoconstriction
* COPD, obesity
Over time, the consequence of PAH is increased stress on the (right/left) heart
Over time, the consequence of PAH is increased stress on the right heart –> enlargement and dysfunction
Capillary wedge pressure will be _ in PVH
Capillary wedge pressure will be increased in PVH
* We will see pulmonary edema
Capillary wedge pressure will be _ in PAH
Capillary wedge pressure will be normal in PAH (we will not see pulmonary edema)
We can manage pulmonary arterial hypertension with _ , a drug that blocks endothelin
We can manage pulmonary arterial hypertension with bosentan , a drug that blocks endothelin
* We can also give a prostacyclin or sildenafil –> vasodilators
