Pulmonary HTN and cor pulmonale Flashcards
What do these symptoms indicate?
Progressive or unexplained dyspnea, fatigue, angina, weakness, syncope, peripheral edema, abdominal distention, orthopnea, dry cough, exercise induced N/V, hypoxia
Rarely – hemoptysis, hoarseness, palpitations
pulmonary HTN
What are risk factors for pulmonary HTN?
Women>men
30s-60s
CAD, COPD
BMPR2 gene defect
What is defined by Increased mean pulmonary arterial pressure >20 or 25 on resting cardiac cath from diseases, drugs, toxins, genetic abnormalities, idiopathic
pulmonary HTN
elevation in pulmonary artery system alone
pre-capillary pulmonary HTN
pressure elevation within the pulmonary venous + capillary systems
post-capillary pulmonary HTn
PE: prominent pulmonary component of S2
- pansystolic tricuspid regurg murmur
- diastolic pulmonary regurg murmur (Graham-Steel)
- third heart sound (RV dysfunction)
pulmonary HTN
What’s your initial test of choice when suspecting pulmonary HTN?
TTE
What’s your gold standard for confirming pulmonary HTN?
right heart cath - can also separate group 1 from others
When can you diagnose pulmonary HTN?
mPAP>/ 25 at rest. 20 per current
What do these indicate:
EKG - RVH, RAE, peaked P wave, could be normal!
CXR - RV and RAE
PFT: Group 1 = decreased DLCO
Labs – BNP elevated
pulmonary HTN
Systolic PAP of 35-40 or higher on echo
pulmonary HTN
Oxygenation studies and/or high resolution CT to diagnose
group 2 or group 3
What test do you do after right heart cath?
Vasodilation testing w/ NO or CCBs after→ significant acute vasodilation response = MPP >10/20% to <40 (helping with treatment)
Group 1
pulmonary arterial HTN (PAH)
Group 2
pulmonary HTN due to left heart disease
Group 3
pulmonary HTN due to lung diseases and/or hypoxia
Group 4
pulmonary HTN due to pulmonary artery obstructions (mainly chronic thromboembolic pulmonary HTN (CTEPH))
Group 5
pulmonary HTN with unclear and/or multifactorial mechanisms
How do you manage pulmonary HTN first?
REFER to pulmonologist (1st) and cardiologist (2nd)
- Nitric oxide pathway w/ phosphodiesterase inhibitors (sildenafil, tadalafil)
- soluble guanylate cyclase stimulators (riociguat)
- Endothelin pathway: endothelin receptor antagonists (bosentan, ambrisentan, macitentan)
- Prostacyclin pathway: IV epoprostenol, treprostinil, inhaled iloprost, selexipag
vasodilators for treating Group 1 that is unable to be treated with CCBs or NO
Tx: group 1 (responsive)
CCBs
tx: group 2 and 3
Treat underlying conditions
NO PAH-specific therapies
tx: group 4
Treat w/ lifelong anticoagulation
Pulmonary endarterectomy (PEA) w/ surgically accessible CTEPH when possible
OR balloon pulmonary artery angioplasty in ineligible
Avoid CCBs
tx: group 5
Treat underlying disorder
What is cor pulmonale?
RHF NOT due to LHF – from pulm HTN, OSA, restrictive lung disease, pulmonary embolism, pneumoconiosis, ARDS, CF, neuromuscular diseases
(group 3!)
What are initial symptoms of cor pulmonale?
Coughing, wheezing, weakness, fatigue, dyspnea
What are later symptoms of cor pulmonale?
Edema of lower extremities, liver enlargement, JVD, weakness, chest discomfort
Cor pulmonale is often associated with
COPD
What’s gold standard in Dx of cor pulmonale?
right heart cath
What does this indicate
Elevated pro-BNP >33
CXR = possible enlargement of RV and PA
EKG = increased P wave, RAD, incomplete RBBB, PACs, PVCs
Echo = ventricular hypertrophy, decreased contractility, valve disorders
MRI
cor pulmonale
increased PAP w/ signs of RHF
acute cor pulmonale
presence of pulmonary HTN w/ normal CO and right ventricular end diastolic pressure + presence of disease affecting structure/lungs
chronic cor pulmonale
How do you treat cor pulmonale?
Treat COPD - oxygen therapy
Vasodilators – may not help w/ COPD-related, + inhalations of NO to O2 therapy to decrease PAP
Exclude other causes, try clinical trials
Diuretics (furosemide)
