Pulmonary HTN Flashcards

1
Q

what is pulmonary artery hypertension?

A

relatively rare form of pulmonary HTN characterized by:

  • dyspnea
  • chest pain
  • syncope
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2
Q

what is the primary treatment for Group 3 PH?

A

treat underlying cause of hypoxemia and correction of hypoxemia with supplemental oxygen

<b>(oxygen is the only modality with proven mortality benefit)</b>

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3
Q

what are the signs/sx’s of pulmonary HTN?

A
  • initially asymptomatic
  • DOE (often mistaken for reconditioning or related to other condition like COPD)
  • dyspnea at rest (advanced disease)
  • fatigue
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4
Q

txts for pulmonary artery HTN?

A
  • Diuretics
  • Anticoagulation
  • Calcium Channel Blockers
  • Endothelin Receptor Blockers
  • Phosphodiesterase-5 Inhibitors
  • Prostacyclin
  • Lung Transplant
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5
Q

what are the PE findings of pulmonary HTN?

A
  • Initially increased intensity of S2 in pulmonic area, may be narrowly split
  • R ventricle failure widens the splitting of S2
  • R ventricular S3
  • High pitched systolic murmur of tricuspid regurgitation
  • Elevated JVP
  • Hepatomegaly
  • Peripheral edema
  • Ascites
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6
Q

what is the most common cause of pulmonary hypertension in adults?

A

lung disease

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7
Q

advanced therapy in group 5 PH?

A

favorable response in sarcoidosis

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8
Q

what is group 1 for WHO functional classification for pulmonary HTN

A

<b>No limitations of physical activity</b>. No fatigue or dyspnea, CP, or heart syncope

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9
Q

what is the pathophysiology of pulmonary HTN?

A

Decreased compliance of pulmonary vasculature
-Progressive increase in the right ventricular afterload results in right ventricular hypertrophy

-Eventually right ventricle dilates resulting in decreased contractility -> cardiac output will fall

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10
Q

what is group 2 for WHO functional classification for pulmonary HTN

A

<b>Slight limitation of physical activity, comfortable at rest.</b> Ordinary physical activity results in undue fatigue or dyspnea, chest pain, or heart syncope

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11
Q

is r heart disease secondary to l heart disease cor pulmonale?

A

NO!!!

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12
Q

what is the normal physiology of pulmonary circulation?

A

Pulmonary circulation accommodates varying amounts of blood delivered from the R heart & facilitates gas exchange

-Thin walled compliant vessels

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13
Q

what is vasoreactivity test? what agents are used?

A

Administration of short-acting vasodilator, measure hemodynamic response using right heart catheter.

Agents commonly used: epoprostenol, adenosine and inhaled nitric oxide

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14
Q

what are the advanced treatments?

A

Prostanoid formulations, endothelia receptor antagonists, PDE-5 inhibitors

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15
Q

what is group 5 classified as for pulmonary HTN?

A

Group 5 – unclear multifactorial mechanisms

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16
Q

who gets advanced therapy for PH?

A

Persistent PH & WHO functional class II, III, or IV despite adequate primary therapy

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17
Q

what is the primary treatment for Group 2 PH?

A

heart failure txt

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18
Q

what is the main cause of death in patients with pulmonary arterial hypertension?

A

circulatory collapse (existence in group 1 PAH is bad prognosis)

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19
Q

what are the prostanoid formulations?

A

Epoprostenol (prostacyclin)

  • Improves hemodynamics, functional capacity, and survival in IPAH
  • Continuous infusion with permanent implanted central venous catheter with portable infusion pump
  • Only therapy that has been shown to prolong survival
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20
Q

what is group 4 classified as for pulmonary HTN?

A

Group 4 – secondary to thromboembolic disease

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21
Q

what is the pathophysiology of cor pulmonale?

A
  • R heart failure resulting from long standing pulmonary hypertension
  • R ventricular hypertrophy develops due to the high pulmonary pressure that it pumps against
  • Eventually R ventricle loses it’s contractility
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22
Q

what are the causes of pulmonary HTN group 5?

A

Pulmonary hypertension with unclear multifactorial mechanisms.

  • Hematologic disorders (myeloproliferative disorders)
  • Systemic disorders (Sarcoidosis)
  • Metabolic disorders (Glycogen storage disease)
  • Miscellaneous (Sickle cell disease)
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23
Q

what can echocardiogram do for pulmonary HTN?

A

can’t diagnose PH, but can estimate pulmonary artery systolic pressure & therefore determine if PH is likely

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24
Q

what are the endothelin receptor antagonists?

A

Endothelin is a potent vasoconstrictor found in high concentrations in lungs of patients with IPAH and other group 1 PAH.

  • Bosentan, macitentan
  • Ambrisentan and sitaxsentan

*improve exercise capacity, dyspnea and hemodynamic measures

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25
what is group 3 classified as for pulmonary HTN?
Group 3 –secondary to lung disease or hypoxemia
26
what is pulmonary HTN?
abnormal elevation in pulmonary artery pressure (PAP) - mean pulmonary artery pressure ≥25mmHg - (21-24mmHg borderline)
27
what are causes of pulmonary HTN group 4?
Chronic thromboembolic disease: - Multiple small pulmonary emboli developing over time - A decrease in the number (volume) of functional blood vessels -> fewer vessels now need to accommodate more blood
28
what is the primary treatment for Group 5 PH?
Target specific underlying cause
29
what does the prostanoid formulation improve?
prostanoid formulation = epoprostenol (prostacyclin) -Improves hemodynamics, functional capacity, and survival in IPAH
30
what symptom of PH is associated with advanced disease?
dyspnea at rest
31
what are things to consider when treating all groups of PH?
- Diuretics treat fluid retention - Oxygen may benefit all groups - Anticoagulation for IPAH, hereditary PAH, drug-induced PAH or group 4 PH * - Digoxin improves RV EF of patients with group 3 PH & helps control HR in patients who have SVTs associated with RV dysfunction - Exercise
32
digoxin improves what for which groups with PH?
Digoxin improves RV EF of patients with group 3 PH & helps control HR in patients who have SVTs associated with RV dysfunction
33
what is pulmonary HTN almost always caused by?
Increased pulmonary vascular resistance (pulmonary arteries)
34
what is group 1 classified as for pulmonary HTN?
Group 1 – secondary to diseases that localize to small pulmonary arteries/arterioles (PAH)
35
how long until >20% of pts have symptoms of PH before PH is recognized?
>2 years
36
what criteria must be met to diagnose Group 1 PH?
- Mean pulmonary artery pressure >25 mmHg at rest - Mean pulmonary capillary wedge pressure <15 mm Hg (exclude PH owing to L heart disease) - Chronic lung disease is mild to absent (exclude PH owing to lung disease/hypoxia) - Venous thromboembolic disease is absent (exclude PH owing to multiple small PEs) - Other disorders are absent (rheum, heme, metabolic)
37
what are signs & symptoms of right heart failure?
- angina (r heart requires more oxygen) - exertional syncope (inability to increase CO) - peripheral edema (R heart failure) - abd discomfort due to hepatic congestion
38
what is the pathophysiology/cause of pulmonary HTN group 2?
Left heart disease -L atrial HTN requires an increased pulmonary artery systolic pressure to maintain an adequate driving force across the pulmonary vasculature
39
what do the endothelia receptor antagonists improve?
exercise capacity, dyspnea and hemodynamic measures
40
what is primary therapy for pulmonary HTN txt directed at?
directed at the underlying cause
41
at what pulmonary artery pressure is PH diagnosed as well as the PASP?
- PH is diagnosed when mean PAP =≥25 mmHg at rest | - PASP between 21mmHg – 24mmHg are of uncertain clinical significance
42
what are the PDE-5 inhibitors?
- Sildenafil - Tadalafil - Vardenafil - *Prolong vasodilatory effect of nitric oxide. - *Also used to treat erectile dysfunction - *Improves pulmonary hemodynamics and exercise capacity.
43
what is pulmonary HTN usually caused by?
long standing pulmonary disease/hypoxia
44
what is group 2 classified as for pulmonary HTN?
Group 2 –secondary to L heart disease
45
what is advanced therapy for pulmonary HTN txt directed at?
the pulmonary HTN itself
46
what does pulmonary HTN result in?
right sided heart failure/Cor Pulmonale
47
what is the pathophysiology of pulmonary arterial hypertension/Group 1 PH?
Intrinsic to arteries themselves; abnormalities in pulmonary vascular endothelial and smooth muscle cells (affects all three layers of the vascular wall) - Vasoconstriction - Vascular proliferation - Thrombosis - Inflammation
48
what is group 3 for WHO functional classification for pulmonary HTN
Marked limitation of physical activity. They are comfortable at rest. Less than ordinary physical activity causes undue fatigue or dyspnea, chest pain, or heart syncope.
49
what are causes of Group 1 pulmonary arterial HTN?
- Idiopathic - Hereditary - Drugs or toxins (Appetite suppressants, cocaine, amphetamines, medications) - Connective tissue diseases (systemic sclerosis)->Narrowing arteries/arterioles, interstitial fibrosis - HIV - Portal hypertension (liver disease) - Congenital heart disease (VSD, ASD)
50
how do CCBs help PH pts?
- Prolonged survival - Sustained functional improvement - Hemodynamic improvement
51
when is PH unlikely according to pulmonary artery systolic pressure?
<36mmHg & TRV <=2.8 m/sec
52
anticoagulation treatment is good for which groups with PH?
Anticoagulation for IPAH, hereditary PAH, drug-induced PAH or group 4 PH
53
EKG in pt with pulmonary artery HTN & RVH
right axis deviation & poor R wave progression
54
where should advanced therapy for PH be used?
should be administered at specialized centers where clinicians are experienced
55
what does the Echo for pulmonary HTN assess?
assesses PASP, R heart size and L to R shunts (PFO) | -diagnose left ventricular dysfunction
56
advanced therapy in group 3 PH?
not FDA approved, not recommended, occasionally considered
57
advanced therapy in group 4 PH?
can be considered
58
what are the causes of pulmonary HTN?
1. Increased pulmonary vascular resistance (pulmonary arteries) (** almost always caused by this) 2. Elevated L atrial pressure (L heart disease; valve disease, systolic dysfunction etc.) 3. Increased pulmonary blood flow (volume) – (not a significant cause– pulm vascular bed vasodilates & recruits vessels in response to increased flow)
59
dx tests for pulmonary HTN?
- Chest radiograph (CXR) - Electrocardiography (EKG) - Echocardiography (Echo)-Assess PASP, R heart size and assess for L to R shunts (PFO). (Diagnose left ventricular dysfunction) - Pulmonary Function Tests (PFTs) - Overnight Oximetry - Polysomnography (Dx OSA) - V/Q scan (more sensitive than CTA in detecting chronic thromboembolic pulmonary disease)
60
how many classifications of pulmonary HTN are there?
5 groups
61
who is vasoreactivity testing for?
for pts who are selected for advanced therapy to determine if they will benefit from CCB's (less expensive, fever side effects)
62
what is the first test to order for pulmonary HTN and that is also useful for screening?
TTE -useful for screening
63
when is PH likely according to pulmonary artery systolic pressure?
>50mmHg & Tricuspid regurgitation velocity (TRV) is >3.4 m/sec
64
what is group 4 for WHO functional classification for pulmonary HTN
Inability to carry on any physical activity without symptoms, signs of right heart failure. Dyspnea and/or fatigue may be present even at rest.
65
why is early identification and treatment of pulmonary HTN ideal?
Early identification and treatment is ideal because advanced disease may be less responsive to therapy -also need to assess disease severity
66
advanced therapy in group 1 PAH?
first line
67
what are causes of pulmonary HTN group 3?
- Chronic obstructive pulmonary disease (COPD) - Interstitial lung disease (ILD) - Obstructive sleep apnea (OSA) - Other causes of hypoxemia **Generally only caused by severe lung disease
68
what is normal pulmonary artery pressure (PAP) and measured by what?
8-20mmHg measured by R heart catheterization
69
what is the definition of cor pulmonale? causes?
Altered structure and/or impaired function of the right ventricle that results from pulmonary hypertension. - Pulmonary disease (COPD) - Vasculature (PAH) - Upper airway (OSA) - Chest wall (kyphoscoliosis) (Note: R heart dz secondary to L heart dz is NOT Cor Pulmonale)
70
advanced therapy in group 2 PH?
``` possibly harmful (epoprostenol) -L heart unable to handle increased flow ```
71
what is the gold standard for diagnosing pulmonary hypertension and is also necessary to confirm diagnosis?
R heart catheterization – gold standard & necessary to confirm diagnosis
72
what is the primary treatment for Group 4 PH?
Anticoagulation, surgical thromboendarterectomy for selected patients