Pulmonary HTN and cor pulmonale

Question 1

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

Answer 1

pulmonary HTN

Question 2

What are risk factors for pulmonary HTN?

Answer 2

Women>men
30s-60s

CAD, COPD

BMPR2 gene defect

Question 3

What is defined by Increased mean pulmonary arterial pressure >20 or 25 on resting cardiac cath from diseases, drugs, toxins, genetic abnormalities, idiopathic

Answer 3

pulmonary HTN

Question 4

elevation in pulmonary artery system alone

Answer 4

pre-capillary pulmonary HTN

Question 5

pressure elevation within the pulmonary venous + capillary systems

Answer 5

post-capillary pulmonary HTn

Question 6

PE: prominent pulmonary component of S2
- pansystolic tricuspid regurg murmur
- diastolic pulmonary regurg murmur (Graham-Steel)
- third heart sound (RV dysfunction)

Answer 6

pulmonary HTN

Question 7

What’s your initial test of choice when suspecting pulmonary HTN?

Answer 7

TTE

Question 8

What’s your gold standard for confirming pulmonary HTN?

Answer 8

right heart cath - can also separate group 1 from others

Question 9

When can you diagnose pulmonary HTN?

Answer 9

mPAP>/ 25 at rest. 20 per current

Question 10

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

Answer 10

pulmonary HTN

Question 11

Systolic PAP of 35-40 or higher on echo

Answer 11

pulmonary HTN

Question 12

Oxygenation studies and/or high resolution CT to diagnose

Answer 12

group 2 or group 3

Question 13

What test do you do after right heart cath?

Answer 13

Vasodilation testing w/ NO or CCBs after→ significant acute vasodilation response = MPP >10/20% to <40 (helping with treatment)

Question 14

Group 1

Answer 14

pulmonary arterial HTN (PAH)

Question 15

Group 2

Answer 15

pulmonary HTN due to left heart disease

Question 16

Group 3

Answer 16

pulmonary HTN due to lung diseases and/or hypoxia

Question 17

Group 4

Answer 17

pulmonary HTN due to pulmonary artery obstructions (mainly chronic thromboembolic pulmonary HTN (CTEPH))

Question 18

Group 5

Answer 18

pulmonary HTN with unclear and/or multifactorial mechanisms

Question 19

How do you manage pulmonary HTN first?

Answer 19

REFER to pulmonologist (1st) and cardiologist (2nd)

Question 20

• 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

Answer 20

vasodilators for treating Group 1 that is unable to be treated with CCBs or NO

Question 21

Tx: group 1 (responsive)

Answer 21

CCBs

Question 22

tx: group 2 and 3

Answer 22

Treat underlying conditions
NO PAH-specific therapies

Question 23

tx: group 4

Answer 23

Treat w/ lifelong anticoagulation
Pulmonary endarterectomy (PEA) w/ surgically accessible CTEPH when possible
OR balloon pulmonary artery angioplasty in ineligible
Avoid CCBs

Question 24

tx: group 5

Answer 24

Treat underlying disorder

Question 25

What is cor pulmonale?

Answer 25

RHF NOT due to LHF -- from pulm HTN, OSA, restrictive lung disease, pulmonary embolism, pneumoconiosis, ARDS, CF, neuromuscular diseases (group 3!)

Question 26

What are initial symptoms of cor pulmonale?

Answer 26

Coughing, wheezing, weakness, fatigue, dyspnea

Question 27

What are later symptoms of cor pulmonale?

Answer 27

Edema of lower extremities, liver enlargement, JVD, weakness, chest discomfort

Question 28

Cor pulmonale is often associated with

Answer 28

COPD

Question 29

What's gold standard in Dx of cor pulmonale?

Answer 29

right heart cath

Question 30

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

Answer 30

cor pulmonale

Question 31

increased PAP w/ signs of RHF

Answer 31

acute cor pulmonale

Question 32

presence of pulmonary HTN w/ normal CO and right ventricular end diastolic pressure + presence of disease affecting structure/lungs

Answer 32

chronic cor pulmonale

Question 33

How do you treat cor pulmonale?

Answer 33

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)