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Clin Med 2: Pulmonary Hypertension

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What is pulmonary hypertension?
when pulmonary artery pressure reaches a level that is inappropriate to cardiac output
What characterizes pulmonary HTN?
high flow circulation, low flow circulation(low pressure, low resistance) and adjusts to increase BP during exercise(blood vessels distends and uses more oxygen)
What is idiopathic (primary) pulmonary hypertension also known?
plexogenic pulmonary arteriopathy
Who does primary pulmonary hypertension affect the most?
young and middle age female
What are the main characteristics of primary pulmonary hypertension?
progressive dyspnea, rapid downward course, plexiform lesion in muscular pulmonary arteries
What medical conditions lead to secondary pulmonary hypertension?
congenital heart diseases, LV failure, pulmonary emboli, chronic hypoxemia, sleep apnea-obstructive, COPD
What is the pathophysiology?
hyperthrophy of pulmonary artery endothelium, loss of elasticity, narrowing of pulmonary arterioles
What causes primary pulmonary hypertension?
increase in pulmonary artery pressure to meet cardiac output, produces structural abnormality-hypertrophy of smooth muscles in the pulmonary artery, causes proliferation of intimal, stimulates atheromatous changes, thrombosis forms
What are some physiological causes of secondary pulmonary HTN?
decrease pulmonary artery cross sectional area, increase pulmonary artery pressure, increase pulmonary artery blood floow
What are examples sof diseases that decrease pulmonary artery cross sectional area?
emphysema, vasculitis, PE, interstitial
What are some disease that increase pulmonary artery pressure?
pericarditis, LVF, mitral stenosis
What are soem of the diseases that cause increased pulmonary artery blood flow?
congential cardiac shunts
What is pulmonary arteriolar vasoconstriction due to?
hypoxia
What are some syptoms of pulmonary hypertension?
initially mainly due to underlying disease- dyspnea, chest discomofrt, fatigue, syncope, edema
What do you see on the physical exam of pulmonary hypertension?
elevated jugular venous pressure(distended neck vein), right ventricular lift(heave), peripheral cyanosis and edema, narrow split of S2, systolic ejection click, hypoxia (need to know this)
What are the lab findings of pulmonary hypertension?
polycythemia secondary to chronic hypoxia
What is are the gold standard test for Pulmonary hypertension?
pulmonary angiography
What do you see in an EKG of pulmonary hypertension?
right ventricular strain, later changes of right ventricular hypertrophy and right atrial enlargement
What do u see in an CXR of pulmonary hypertension?
enlarged central pulmonary vasculature, clear lung fields, RA and RV enlargement
What do you see on an echocardiogram of pulmonary hypertension?
enlarged right ventricle, abnormal septal motion
What can a right heart catherization of primary pulmonary hypertension measure?
pulmonary artery pressure
What do pulmonary function tests of primary pulmonary hypertension show?
hypoxemia, low diffusing capacity, mild restriction
What findings are more characteristic of secondary pulmonary hypertension than primary pulmonary hypertension?
radiographic findings usually those of the underlying process, echocardiographic findings may help distinguish primary from secondary pulmonary hypertension
What is the treatment for primary pulmonary hypertension?
avoid physical exertion, diuretic therapy relieves dyspnea, prostacyclin, anticoagluation,bosentan, vasodilation,
What is the treatment for end stage primary pulmonary hypertension?
single or bilateral lung transplant
How do you treat secondary pulmonary hypertension?
treat underlying cause, O2 for 15 hrs per day if COPD, Phlebotomy for HCT>60%, single or double lung transplant, treat cor pulmonale