#22 - Pulmonary arterial hypertension Flashcards
What lab is required for diagnosis of pulmonary arterial hypertension (PAH)
right heart catheterization
Define pulmonary hypertension (PH), and pulmonary arterial hypertension (PAH)
increase in resting mean pulmonary arterial pressure >25 mmHg by right heart catheterization
For pulmonary arterial hypertension (PAH) the definition includes the above and the following:
normal pulmonary capillary wedge or left atrial pressure (
Symptoms of pulmonary arterial hypertension (PAH)
- unexplained dyspnea
- fatigue/ exercise intolerance
- sometimes syncope
Physical exam signs in the early stage of pulmonary arterial hypertension (PAH)
- accentuated P2
- RV heave (left parasternal lift)
Diagnosis of pulmonary arter
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What are the 5 classifications of pulmonary hypertension
1 - pulmonary arterial hypertension (PAH)
NOTE: many patients may fall into 2 or 3 groups
Most common cause of pulmonary hypertension
Pulmonary venous hypertension due to left heart disease (constitutes 65% of pulmonary hypertension)
What is chronic thromboembolic pulmonary hypertension
occurs in 2-4% of patients after a pulmonary embolism.
if you have pulmonary hypertension 6 months after a PE, you are diagnosed.
Treatment for chronic thromboembolic pulmonary hypertension
surgical - remove the thrombus in the lungs - only a few centers do this, but it can dramatically improve the condition..
3 mechanisms that contribute to pulmonary arterial hypertension (PAH)
- vasoconstriction (increased endothelin, decreased prostacyclin, decreased NO) - THERAPY TARGETS THIS CATEGORY
- smooth muscle proliferation
- thrombosis
Physical exam signs in the advanced stage of pulmonary arterial hypertension (PAH)
lots of heart sounds.
- diastolic murmur of pulmonary regurgitation
- holosystolic mumur of tricuspid valve (right AV valve)
- right ventricular gallop
- elevated JVD
- pulsatile liver
- peripheral edema/ascites
CXR findings in pulmonary arterial hypertension (PAH)
prominent vasculature in the hylum
Classic EKG findings in pulmonary arterial hypertension (PAH)
RV hypertrophy,
right atrial enlargement,
right axis deviation
Best test for initial evaluation of PAH: EKG, CXR, or echo?
echocardiogram (but it is far from perfect)
Echocardiogram: utility and limitations in diagnosis of PAH
- provides estimate of pulmonary arterial systolic pressure.
- tells you about the function/morphology of right heart
- identifies conditions that contribute to PH (valvular disease, LV systolic dysfunction, impaired diastolic function, R to L shunt.
However, it is not very accurate in estimation of pulmonary systolic pressure - only within 10mmHg 50% of the time. It commonly overestimates AND underestimates it.
It is commonly used as a screening test for PAH.
This is why right heart cath remains the gold standard and is needed for diagnosis.
Utility of right heart cardiac catheterization for diagnosis of pulmonary arterial hypertension (PAH)
- differentiates between arterial and venous hypertension
- needed for diagnosis
- determines etiology (assesses L to R shunt, L heart disease, etc)
- establishes the severity and prognosis
- evaluate vasoreactivity (evaluate response to drugs while catheter is in place)
You’ve made a diagnosis of pulmonary arterial hypertension (PAH). What is the NEXT step?
- figure out where it is coming from.
- test serology (ANA, HIV, Liver function tests)
- sleep study (group 3)
- pulmonary function tests, chest CT, arterial blood gas (group 3)
What predicts poor prognosis / survival in PAH patients?
- poor exercise endurance (6 minute walk test
- syncope (represents advanced disease)
- signs of R heart failure (pericardial effusion, high R atrial pressure, high BNP, etc.)
PAH is a relatively benign condition, without treatment.
False. Deadly without treatment. Treatment improves survival though
Goals of therapy for PAH
- improve hemodynamics
- improve exercise capacity/ quality of life
- improve survival
Treatment for PAH
- supportive therapy (oxygen, diuretics, anticoagulants)
- Ca channel blockers (if vasoreactivity in r heart cath - this is very good prognosis!)
- PAH specific therapies
lifestyle changes in PAH
- avoid overexertion
- avoid pregnancy during therapy
- avoid high altitude
- low sodium diet, fluid restriction
who does PAH commonly occur in ?
Young women
What happens to the Right ventricle systolic pressure in PAH?
it goes way up (has to overcome pulm hypertension)
-normally, it is
