Pulmonary HTN Flashcards

1
Q

Pulmonary Hypertension is defined by what?

A

mPAP > 20 mmHg

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2
Q

Pre-capillary PH is defined as?

A

mPAP > 20 mmHG
PAWP </= 15 mmHg
PVR > 2WU

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3
Q

Isolated Post-capillary PH is defined as?

A

mPAP > 20 mmHg
PAWP > 15 mmHg
PVR </= 2 WU

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4
Q

Combined pre and post capillary PH is defined as?

A

mPAP > 20 mmHg
PAWP > 15 mmHg
PVR > 2 WU

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5
Q

Exercise PH is defined as?

A

mPAP/CO slope between rest and exercise > 3 mmHg/L/min

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6
Q

Risk Factors for PH

A

age > 65 years old
Left Heart Disease
COPD
CHD
Some infectious diseases (schistosomiasis, HIV)
high altitude

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7
Q

Clinical Presentation of PH
Early

A

Dyspnea on Exertion
Fatigue and rapid exhaustion
Dyspnea when bending forward
Palpitations
Hemoptysis
Exercise-induced abdominal distension and nausea
Weight gain d/t fluid retention
Syncope (during or shortly after physical exertion

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8
Q

Clinical Presentation of PH
Late

A

Exertional chest pain: dynamic compression of the left main coronary artery
Hoarseness (dysphonia): compression o fthe left laryngeal recurrent nerve)
SOB, wheezing, cough, lower respiratory tract infection, atelectasis: compression of the bronchi

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9
Q

The cardinal symptom of PH is

A

dyspnea on progressively minor exertion

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10
Q

Signs of PH

A

Central, peripheral or mixed cyanosis
accentuated pulmonary component of the second heart sound
RV third heart sound
Systolic murmur of the tricuspid regurgitation
Diastolic murmur of pulmonary regurgitation

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11
Q

Signs of RV backward failure

A

Distended and pulsating jugular veins
Abdominal distention
Hepatomegaly
Ascites
Peripheral edema

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12
Q

Signs pointing towards underlying cause of PH

A

Digital Clubbing
Sclerodactyly
Raynaud’s Phenomenon
digital ulceration
GERD

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13
Q

Signs pointing towards underlying cause of PH
Digital Clubbing

A

Cyanotic CHD, fibrotic lung disease, bronchiectasis, PVOD, or liver disease

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14
Q

Signs pointing towards underlying cause of PH
Differential clubbing/cyanosis

A

PDA/Eisenmenger’s syndrome

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15
Q

Signs pointing towards underlying cause of PH
Auscultory findings

A

crackles or wheezing
murmurs
Lung or heart disease related

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16
Q

Signs pointing towards underlying cause of PH
Sequelae of DVT, venous insufficiency

A

CTEPH

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17
Q

Signs pointing towards underlying cause of PH
Telangiectasia

A

Hereditary Hemorrhagic Telangiectasia or systemic sclerosis

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18
Q

Signs pointing towards underlying cause of PH
Sclerodactyly, Raynaud’s Phenomenon, digital ulceration, GORD

A

systemic sclerosis

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19
Q

Signs of RV forward failure

A

Peripheral cyanosis (blue lips and tips)
Dizziness
Pallor
Cool Extremities
Prolonged Cap refill

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20
Q

Typical ECG abnormalities in PH

A

P pulmonale (P > 0.25 mV in lead II)
R or sagittal axis deviation
RV hypertrophy (R/S > 1, w/ R > 0.5 mV in V1; R in V1 + S in lead V5 > 1mV)
RBBB
RV strain pattern (ST depression/ T-wave inversion in the right pre-cordial V1-4 and inferior II, III, aVF)
Prolonged QTc

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21
Q

Radiographic Signs of PH and concomitant abnormalities

A

R heart enlargement
PA enlargement
Pruning of the peripheral vessels
‘Water-bottle’ shape of cardiac silhouette

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22
Q

Radiographic Signs of LHD/Pulmonary Congestion

A

Central air space opacification
Interlobular septal thickening ‘Kerley B’ lines
Pleural effusions
LA enlargement
LV dilation

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23
Q

Radiographic signs of lung disease

A

Flattening of diaphragm (COPD/Emphysema)
Hyperlucency (COPD/Emphysema)
Lung volume loss (fibrotic lung disease)
Reticular opacification (fibrotic lung disease)

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24
Q

PFT findings in patients w/ PAH

A

usually normal or may show mild restrictive, obstructive or combined abnormalities.

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25
Q

More severe PFT abnormalities are occasionally found in patients w/ ?

A

PAH associated w/ CHD

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26
Q

A severely reduced Diffusing Capacity for Carbon Monoxide (<45% of predicted value) in the presence of otherwise normal PFTs can ve found in what patients?

A

PAH w/ Systemic Sclerosis

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27
Q

Patients w/ PAH have what PaO2 levels?

A

slightly reduced or normal

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28
Q

Severe reduction of PaO2 in PAH might raise suspicion of what?

A

patent foramen ovale
hepatic disease
other abnormalities w/ R-to-L shunt (e.g. septal defect)
low-DLCO- associated conditions

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29
Q

Patients w/ PAH have what PaCO2 levels?

A

typically lower than normal d/t hyperventilation

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30
Q

Elevated PaCO2 is very unusual in PAH and reflects what?
What is recommended in this case?

A

alveolar hypoventilation, which may be a cause of PAH in itself
Overnight oximetry or polysomnography for suspicion of sleep d/o breathing or hypoventilation

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31
Q

Echo Signs of RV dysfunction on four chamber view

A

Dilated RV
Enlarged RA area (> 18 cubic cm)
Presence of pericardial effusion

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32
Q

Echo Signs of RV dysfunction on parasternal long axis view

A

Enlarged RV

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33
Q

Echo Signs of RV dysfunction on parasternal short axis view

A

Flattened interventricular septum leading to “D-shaped” LV
Presence of pericardial effusion

34
Q

Echo Signs of RV dysfunction on subcostal view

A

Distended inferior vena cava w/ diminished inspiratory collapsibility
Presence of pericardial effusion

35
Q

Other Imaging that can help diagnose PH

A

Non-con CT
CTPA
Dual Energy CT
Digital Subtraction Angiography
Cardiac MRI

36
Q

What is the gold standard for diagnosing and classifying PH

A

R heart cath

37
Q

Normal Hemodynamic Measures obtained during R heart cath
R atrial pressure?
systolic PAP?
diastolic PAP?
mean PAP?
PAWP?
CO
SvO2
SaO2
Systemic BP

A

2-6 mmHg
15-30 mmHg
4-12 mmHg
8-20 mmHg
</= 15 mmHg
4-8 L/min
65-80%
95-100%
120/80

38
Q

Calculated Parameters obtained during R heart cath
PVR
PVRI
TPR
CI
SV
SVI
PAC

A

0.3 - 2.0 WU
3-3.5 WUm2
< 3 WU
2.5-4 L/min m2
60-100 mL
33-47mL/m2
<2.3 mL/mmHg

39
Q

Non-pharmacologic recommendations for PAH

A

Physical activity and supervised rehab w/n symptom limits

40
Q

Vaccines for PAH

A

Covid-19
influenza
Streptococcus Pneumoniae

41
Q

When are diuretics recommended in patients w/ PAH?

A

With signs of RV failure and fluid retention

42
Q

When is long term O2 therapy recommended in patients w/ PAH?

A

PaO2 < 60 mmHg

43
Q

What is suggested in patients w/ iron-deficiency anemia?

A

correction of iron status

44
Q

What medications are not recommended in patients with PAH unless required by comorbidities such as HTN, CAD, L Heart Failure, or arrhythmias?

A

ACEis
ARBs
ARNIs
SGLT-2is
beta blockers
ivabradine

45
Q

If a patient w/ PH undergoes R heart cath w/ pulmonary vasoreactivity testing and has >/= 10mmHg mPAP drop from baseline to </= 40 mmHg w/ increased or unchanged CO, what is the suggested therapy?

When to reasses?

A

CCB and titrate to optimized individual dose

3-6 months

46
Q

How to reassess response to CCB for PAH?

If reassessment shows good response, when to reassess again?

A

WHO-FC I/II
BNP < 50 ng/L or NT-proBNP < 300 ng/L
normal or near normal resting hemodynamics

6-12 months

47
Q

WHO Classification
Patients with PH but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnoea or fatigue, chest pain, or near syncope

A

WHO-FC I

48
Q

WHO Classification
Patients with PH resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnoea or fatigue, chest pain, or near syncope

A

WHO-FC II

49
Q

WHO Classification
Patients with PH resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes undue dyspnoea or fatigue, chest pain, or near syncope

A

WHO-FC III

50
Q

WHO Classification
Patients with PH with an inability to carry out any physical activity without symptoms. These patients manifest signs of right HF. Dyspnoea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity

A

WHO-FC IV

51
Q

CCB’s used to treat PAH
include starting dose and target dose

A

Amlodipine start
Diltiazem start
Felodipine start
Nifedipine start

52
Q

Amlodipine Starting dose for PAH?
Target dose for PAH?

A

5 mg once daily
15-30 mg once daily

53
Q

Diltiazem Starting dose for PAH?
Target dose for PAH?

A

60 mg BID
120-360 mg BID

54
Q

Felodipine Starting dose for PAH?
Target dose for PAH?

A

5 mg once daily
15-30mg once daily

55
Q

Nifedipine Starting dose for PAH?
Target dose for PAH?

A

10 mg TID
20-60 mg BID or TID

56
Q

Endothelin receptor antagonists

A

Ambrisentan
Bosentan
Macitentan

57
Q

Ambrisentan Starting dose for PAH?
Target dose for PAH?

A

5 mg once daily
10 mg once daily

58
Q

Bosentan Starting dose for PAH?
Target dose for PAH?

A

62.5 mg BID
125 mg BID

59
Q

Macitentan Starting dose for PAH?
Target dose for PAH?

A

10 mg once daily
10 mg once daily

60
Q

Phosphodiesterase 5 inhibitors

A

Sildenafil
Tadalafil

61
Q

Sildenafil Starting dose for PAH?
Target dose for PAH?

A

20 mg TID
20 mg TID

62
Q

Tadalafil Starting dose for PAH?
Target dose for PAH?

A

20 or 40 mg once daily
40 mg once daily

63
Q

Prostacyclin analogues (oral admin)

A

Beraprost sodium
Beraprost extended release
Treprostinil

64
Q

Prostacyclin receptor agonists

A

selexipag

65
Q

Soluble guanylate cyclase stimulators

A

Riociguat

66
Q

Proscacyclin analogues (inhaled admin)

A

Iloprost
Treprostinil

67
Q

Prostacyclin analogues (iv or sc admin)

A

epoprostenol iv
Treprostinil sc

68
Q

What is the recommended treatment for in patients w/ IPAH, HPAH, or DPAH who are responders to acute vasoreactivity testing?

A

CCBs

69
Q

What is the recommended treatment for patients w/ IPAH, HPAH, or DPAH in WHO-FC I or II w/ marked hemodynamic improvement

A

Continue high doses of CCBs

70
Q

Initiating PAH therapy is recommended in patients who?

A

remain in WHO-FC III or IV or those w/o marked hemodynamic improvement after high doses of CCBs

71
Q

What is recommended initial treatment for patients w/ IPAH/HPAH/DPAH and are non-vasoreactive, who present at low or intermediate risk of death?

A

Combination therapy w/ a PDE5i and an ERA is recommended

72
Q

What is the recommended initial oral drug combo for patients w/ IPAH/HPAH/DPAH w/o cardiopulmonary comorbidities?

A

Combination of ambrisentan and tadalafil
Combination of macitentan and tadalafil

73
Q

What is recommended to base treatment escalations on?

A

risk assessment and general treatment strategies

74
Q

The addition of what to PDE5is or oral/inhaled PCA is recommended to reduce the risk of morbidity/mortality events?

A

macitentan

75
Q

The addition of what to EERAs and/or PDE5is is recommended to reduce the risk of morbidity/mortality events?

A

selexipag

76
Q

The addition of what to ERA or PDE5is/riociguat monotherapy is recommended to reduce the risk of morbidity/mortality?

A

treprostinil

77
Q

The addition of what to epoprostenol is recommended to improve exercise capacity

A

sildenafil

78
Q

When managing patients with right HF in the ICU, it is recommended to?

A

Involve physicians w/ expertise, treat causative factors and use supportive measures, including inotropes and vasopressors, fluid management and PAH drugs as appropriate

79
Q

A combination fo what is recommended to diagnose PAH w/ signs of venous and/or capillary involvement (PVOD/PCH)a

A

clinical and radiological findings, ABG, PFTs and genetic testing

80
Q
A