Pulmonary HTN and cor pulmonale Flashcards

1
Q

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

A

pulmonary HTN

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2
Q

What are risk factors for pulmonary HTN?

A

Women>men
30s-60s

CAD, COPD

BMPR2 gene defect

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3
Q

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

A

pulmonary HTN

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4
Q

elevation in pulmonary artery system alone

A

pre-capillary pulmonary HTN

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5
Q

pressure elevation within the pulmonary venous + capillary systems

A

post-capillary pulmonary HTn

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6
Q

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

A

pulmonary HTN

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7
Q

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

A

TTE

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8
Q

What’s your gold standard for confirming pulmonary HTN?

A

right heart cath - can also separate group 1 from others

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9
Q

When can you diagnose pulmonary HTN?

A

mPAP>/ 25 at rest

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10
Q

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

A

pulmonary HTN

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11
Q

Systolic PAP of 35-40 or higher on echo

A

pulmonary HTN

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12
Q

Oxygenation studies and/or high resolution CT to diagnose

A

group 2 or group 3

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13
Q

What test do you do after right heart cath?

A

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

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14
Q

Group 1

A

pulmonary arterial HTN (PAH)

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15
Q

Group 2

A

pulmonary HTN due to left heart disease

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16
Q

Group 3

A

pulmonary HTN due to lung diseases and/or hypoxia

17
Q

Group 4

A

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

18
Q

Group 5

A

pulmonary HTN with unclear and/or multifactorial mechanisms

19
Q

How do you manage pulmonary HTN first?

A

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

20
Q
  • 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
A

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

21
Q

Tx: group 1 (responsive)

A

CCBs

22
Q

tx: group 2 and 3

A

Treat underlying conditions
NO PAH-specific therapies

23
Q

tx: group 4

A

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

24
Q

tx: group 5

A

Treat underlying disorder

25
Q

What is cor pulmonale?

A

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

26
Q

What are initial symptoms of cor pulmonale?

A

Coughing, wheezing, weakness, fatigue, dyspnea

27
Q

What are later symptoms of cor pulmonale?

A

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

28
Q

Cor pulmonale is often associated with

A

COPD

29
Q

What’s gold standard in Dx of cor pulmonale?

A

right heart cath

30
Q

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

A

cor pulmonale

31
Q

increased PAP w/ signs of RHF

A

acute cor pulmonale

32
Q

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

A

chronic cor pulmonale

33
Q

How do you treat cor pulmonale?

A

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)