Pulmonary HTN Flashcards

(80 cards)

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
More severe PFT abnormalities are occasionally found in patients w/ ?
PAH associated w/ CHD
26
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?
PAH w/ Systemic Sclerosis
27
Patients w/ PAH have what PaO2 levels?
slightly reduced or normal
28
Severe reduction of PaO2 in PAH might raise suspicion of what?
patent foramen ovale hepatic disease other abnormalities w/ R-to-L shunt (e.g. septal defect) low-DLCO- associated conditions
29
Patients w/ PAH have what PaCO2 levels?
typically lower than normal d/t hyperventilation
30
Elevated PaCO2 is very unusual in PAH and reflects what? What is recommended in this case?
alveolar hypoventilation, which may be a cause of PAH in itself Overnight oximetry or polysomnography for suspicion of sleep d/o breathing or hypoventilation
31
Echo Signs of RV dysfunction on four chamber view
Dilated RV Enlarged RA area (> 18 cubic cm) Presence of pericardial effusion
32
Echo Signs of RV dysfunction on parasternal long axis view
Enlarged RV
33
Echo Signs of RV dysfunction on parasternal short axis view
Flattened interventricular septum leading to "D-shaped" LV Presence of pericardial effusion
34
Echo Signs of RV dysfunction on subcostal view
Distended inferior vena cava w/ diminished inspiratory collapsibility Presence of pericardial effusion
35
Other Imaging that can help diagnose PH
Non-con CT CTPA Dual Energy CT Digital Subtraction Angiography Cardiac MRI
36
What is the gold standard for diagnosing and classifying PH
R heart cath
37
Normal Hemodynamic Measures obtained during R heart cath R atrial pressure? systolic PAP? diastolic PAP? mean PAP? PAWP? CO SvO2 SaO2 Systemic BP
2-6 mmHg 15-30 mmHg 4-12 mmHg 8-20 mmHg
38
Calculated Parameters obtained during R heart cath PVR PVRI TPR CI SV SVI PAC
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
Non-pharmacologic recommendations for PAH
Physical activity and supervised rehab w/n symptom limits
40
Vaccines for PAH
Covid-19 influenza Streptococcus Pneumoniae
41
When are diuretics recommended in patients w/ PAH?
With signs of RV failure and fluid retention
42
When is long term O2 therapy recommended in patients w/ PAH?
PaO2 < 60 mmHg
43
What is suggested in patients w/ iron-deficiency anemia?
correction of iron status
44
What medications are not recommended in patients with PAH unless required by comorbidities such as HTN, CAD, L Heart Failure, or arrhythmias?
ACEis ARBs ARNIs SGLT-2is beta blockers ivabradine
45
If a patient w/ PH undergoes R heart cath w/ pulmonary vasoreactivity testing and has >/= 10mmHg mPAP drop from baseline to
CCB and titrate to optimized individual dose 3-6 months
46
How to reassess response to CCB for PAH? If reassessment shows good response, when to reassess again?
WHO-FC I/II BNP < 50 ng/L or NT-proBNP < 300 ng/L normal or near normal resting hemodynamics 6-12 months
47
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
WHO-FC I
48
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
WHO-FC II
49
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
WHO-FC III
50
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
WHO-FC IV
51
CCB's used to treat PAH include starting dose and target dose
Amlodipine start Diltiazem start Felodipine start Nifedipine start
52
Amlodipine Starting dose for PAH? Target dose for PAH?
5 mg once daily 15-30 mg once daily
53
Diltiazem Starting dose for PAH? Target dose for PAH?
60 mg BID 120-360 mg BID
54
Felodipine Starting dose for PAH? Target dose for PAH?
5 mg once daily 15-30mg once daily
55
Nifedipine Starting dose for PAH? Target dose for PAH?
10 mg TID 20-60 mg BID or TID
56
Endothelin receptor antagonists
Ambrisentan Bosentan Macitentan
57
Ambrisentan Starting dose for PAH? Target dose for PAH?
5 mg once daily 10 mg once daily
58
Bosentan Starting dose for PAH? Target dose for PAH?
62.5 mg BID 125 mg BID
59
Macitentan Starting dose for PAH? Target dose for PAH?
10 mg once daily 10 mg once daily
60
Phosphodiesterase 5 inhibitors
Sildenafil Tadalafil
61
Sildenafil Starting dose for PAH? Target dose for PAH?
20 mg TID 20 mg TID
62
Tadalafil Starting dose for PAH? Target dose for PAH?
20 or 40 mg once daily 40 mg once daily
63
Prostacyclin analogues (oral admin)
Beraprost sodium Beraprost extended release Treprostinil
64
Prostacyclin receptor agonists
selexipag
65
Soluble guanylate cyclase stimulators
Riociguat
66
Proscacyclin analogues (inhaled admin)
Iloprost Treprostinil
67
Prostacyclin analogues (iv or sc admin)
epoprostenol iv Treprostinil sc
68
What is the recommended treatment for in patients w/ IPAH, HPAH, or DPAH who are responders to acute vasoreactivity testing?
CCBs
69
What is the recommended treatment for patients w/ IPAH, HPAH, or DPAH in WHO-FC I or II w/ marked hemodynamic improvement
Continue high doses of CCBs
70
Initiating PAH therapy is recommended in patients who?
remain in WHO-FC III or IV or those w/o marked hemodynamic improvement after high doses of CCBs
71
What is recommended initial treatment for patients w/ IPAH/HPAH/DPAH and are non-vasoreactive, who present at low or intermediate risk of death?
Combination therapy w/ a PDE5i and an ERA is recommended
72
What is the recommended initial oral drug combo for patients w/ IPAH/HPAH/DPAH w/o cardiopulmonary comorbidities?
Combination of ambrisentan and tadalafil Combination of macitentan and tadalafil
73
What is recommended to base treatment escalations on?
risk assessment and general treatment strategies
74
The addition of what to PDE5is or oral/inhaled PCA is recommended to reduce the risk of morbidity/mortality events?
macitentan
75
The addition of what to EERAs and/or PDE5is is recommended to reduce the risk of morbidity/mortality events?
selexipag
76
The addition of what to ERA or PDE5is/riociguat monotherapy is recommended to reduce the risk of morbidity/mortality?
treprostinil
77
The addition of what to epoprostenol is recommended to improve exercise capacity
sildenafil
78
When managing patients with right HF in the ICU, it is recommended to?
Involve physicians w/ expertise, treat causative factors and use supportive measures, including inotropes and vasopressors, fluid management and PAH drugs as appropriate
79
A combination fo what is recommended to diagnose PAH w/ signs of venous and/or capillary involvement (PVOD/PCH)a
clinical and radiological findings, ABG, PFTs and genetic testing
80