Flashcards in 33. Pulmonary hypertension Deck (39):
normal mean pulmonary pressure
pulmonary hyperternsion: ( value)
more than 25 mmHg at rest
pulmonary hypertension results in .... (histology)
2. medial hypertrophy
3. intimal fibrosis of PuLMONARY ARTERIES
pulmonary hypertension - course
severe respiratory distress --> cyanosis and RVH --> death from decompensated cor pulmonale
classification group of pulmonary hypertension - number
classiffication group of pulmonary hypertension based on
cause and treatment options
which are the classiffication group of pulmonary hypertension
1. pulmonary arterual hypertension ( PAH)
2. PH due to left heart disease
3. PH due to lung disease or hypoxia
4. chronic thromboembolic PH
5. multifactorial PH
multifactorial pulmonary hypertensions - causes
1. hematologic disorders
2. systemic disorders
3 metabolic disorders
chronic thromboembolic pulmonary hypertension - pathophysiology
recurrent microthrombi --> decreased sectional area of pulmonary vascular bed
pulmonary hypertension due to lung disease or hypoxia - pathophysiology
1. destrcution of lung parenchyma
2. hypoxemic vasoconstriction
pulmonary hypertension due to lug disease or hypoxia - destruction of lung parenchyma example
pulmonary hypertension due to lung disease or hypoxia - hypoxemic vasoconstriction
1. obstructive sleep apnea
2. living in high altitude
pulmonary hypertension due to left heart disease - is often due to
1. systolic dysfuntion
2. diastolic dysfunction
3. valvular disease such as mitral stenosis
pulmonary arterial hypertension (PAH) - idiopathic - prognosis
pulmonary arterial hypertension - causes
3. conncective tissue disease
4. HIV infenction
5. portal hypertension'6. congenital heart disease
pulmonary arterial hypertension - diopathic - pathiohysiology
often due to inactivation mutation in BMPR2 gebe
BMPR2 gene - normal action
inhibits vascular SMCs proliferation
pulmonary arterial hypertension (PAH) also inludes
1. pulmonary venous occlusive disease
2. persisten PH of newborn
idiopathic pulmonary arterial hypertension (PAH) - special feature
pulmonary arterial hypertension (PAH) - drugs that cause it
• What is the normal pulmonary arterial pressure?
• What is the cutoff value for pulmonary hypertension?
≥25 mmHg during rest
• What three pathologic changes in the vasculature are caused by pulmonary hypertension?
Medial hypertrophy, arteriosclerosis, and intimal fibrosis of the pulmonary arteries
• A man is diagnosed with pulmonary hypertension. What undesirable outcome is possible when he finds himself in severe respiratory distress?
Cyanosis and RVH, followed by death from cor pulmonale that is decompensated
• A patient with pulmonary hypertension is lost to follow up. Is he at risk for sequelae from his untreated pulmonary hypertension?
Yes (severe respiratory distress causing right ventricular hypertrophy and cyanosis, resulting in decompensated cor pulmonale and death)
• Name the five classification groups of pulmonary hypertension.
Pulmonary arterial hypertension, chronic thromboembolic PH, PH from left heart disease, PH from lung disease/hypoxia, and multifactorial
• Hereditary pulmonary arterial hypertension is caused by what?
An inactivating mutation in the BMPR2 gene, which normally inhibits vascular smooth muscle proliferation
• What is the prognosis for a patient diagnosed with pulmonary arterial hypertension?
• Name conditions that cause pulmonary arterial hypertension.
Persistent newborn HTN, pulmonic vein occlusion, HIV, schistosomiasis, drugs, connective tissue disease, portal HTN, congenital heart issues
• A patient with narcolepsy is prescribed amphetamines. What pulmonary side effect would you watch out for? Which other drug has this effect?
Pulmonary arterial hypertension; cocaine
• What are some of the potential causes of secondary pulmonary hypertension?
COPD, systolic/diastolic issues, mitral stenosis, recurrent microthrombi, sleep apnea, high altitudes, hematologic/metabolic/systemic illness
• Name conditions of the heart that can lead to pulmonary hypertension.
Systolic or diastolic dysfunction, valvular disease (e.g., mitral stenosis)
• A patient has mitral stenosis. How might this pathology ultimately cause pulmonary hypertension?
Increased resistance to flow in the left heart causes backup of pressure from the left atrium into the pulmonary vasculature
• Name primary conditions of the lungs that lead to pulmonary hypertension.
Lung parenchyma destruction (e.g., COPD), hypoxemic vasoconstriction (e.g., living in high altitudes, sleep apnea)
• A patient has chronic obstructive pulmonary disease (COPD). How might this pathology ultimately lead to pulmonary hypertension?
By the destruction of lung parenchyma, and subsequent vasoconstriction due to hypoxia
• How does obstructive sleep apnea or living at high altitudes cause pulmonary hypertension?
Obstructive sleep apnea and living at high altitudes cause hypoxia, which in turn causes pulmonary vasoconstriction
• A cancer patient has recurrent microthrombi and is repeatedly in the hospital. Could this cause him to develop pulmonary hypertension?
Yes (pressures increase because the emboli decrease the total cross-sectional area of the pulmonary vascular bed)
• Hematologic, metabolic, and systemic disorders cause what type of pulmonary hypertension?