Pulmonary Hypertension Flashcards
CLinical features
Exertional dyspnea
Fatigue
Weakness
Advanced - severe dyspnea with exertional dizziness/light-headedness
Exertional syncoe
Honeymoon period following acute PE**
If you are worried about pulmonary HTN, the next step is
Good screening tool
May over or underestimate the PAP
Echo findings
RV much larger than the left
May compromise left ventricle filling
Can help you dx underlying left heart dz
PAH (precapillary)
Elevated mPAP, normal PCWP
Elevated TPP
Elevated PVR
Post-cap or pumonary venous hypertension
Elevated mPAP
Elevated PCWP
Normal TPP
Normal PVR though total resistance is elevated
Both PAH and post-capillary hypertension cause
Increased workload of the right ventricle leading to RV failure
PH vs. PAH
PH - means mean lumonary vasculary pressure is elevated
PAH - must be due to pre-cap
PH - mPAP over 25
PAH - mPAP over 25 plus PAWP less than 15 plus PVR over 3 wood units
PAH dz
Elevated mPAP and PVR leading to RV failure and premature death
Confirmatory dx
Right heart catheterization
MUST be done in all pts to establish dx
This can exlude congenital, differentiate between PAH and venous
Severity and prognosis
Tests vasodilator therapy
Vasodilator testing
SHould be evaled in every pt prior to tx
Done with iNO, prostacylin or adenosine
Decrease in mean PAP of at least 10 mmHg to a pressure of less than 40 mmHg WITH an increased or changed cardiac output is considered positive
Groups of pulm HTN
1 - PAH 2 - due to left heart dz (common venous cause) 3 - due to lung dz or hypoxia 4 - chronic thrombomboli 5 - other unclear mechs
Radiography
Peripheral markings
Enlarged PA shaodws
Loss of retrosternal
CT - more sensitive
PFTs
Usually normal
DLCO - may be normal, mild to moderate reduction common**
Oxygenation may be normal but dest with exercise
increased dead space (increased V/Q)
Exercise testing
6 minute walk test
Decrease in VO2 max, AT, O2 pulse, rate of increase, etc.
Correlates with functional class
CTEPH
Symptomatic PH (mPAP over 25) Persistent perfusion defects After adequate anticoagulation for 3-6 months
Screening for CTEPH
V/Q scan
Dx of CTEPH
Look for hx of PE or pulm HTN of unclear cause (detected by echo)
Then get chest CTA or V/Q lung scan
Pathogenesis
Unbalance between vasoconstrictors and dilators
LEads to dysfunction and injury
Leads to PAH
Disease pathways
Endothelin
Nitric oxide pathway
Prostacyclin pathway
Histopathology
Intimal fibrosis
increased medai lthickness
Pulmonary arteriolar occlusion
Plexiform lesions
Acute to chornic trnasition
Resistance ot lysis
Persistence of ligands
Abnormal clot structure
Acute to CTEPH progresion
PE specific factors
Chonirc medical conditions
Genetic factors
Thrombotic factors
Pathophysiology of CTEPH
2 ways
unaffected part of the lung (away from the blockage) gets increased amount of cardiac outflow…increased flow and pressure leads to stretch which leads to secondary changes
1) large mechanical compoennt
2) downstream changes due to the blockage
Funcational assemement
1 - wihout limitation
2 - slight
3 - marked
4 - inability
Txs
Diuretics
Anticoagulants
Digoxin
Supplemental O2
Calcium channel blockers
Only a small subset benefit from CCB
WHo show resonse ot vasoreactivity
Prostacyclin analogues
Vasodilators
Indicated for all functional
Pulmonary endarterectomy
Surgical tx of choice
EVERY pt should be evaluated for PEA at expert center
