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Clinical Medicine II > Pulmonary HTN > Flashcards

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
Q

what is group 3 classified as for pulmonary HTN?

A

Group 3 –secondary to lung disease or hypoxemia

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26
Q

what is pulmonary HTN?

A

abnormal elevation in pulmonary artery pressure (PAP)

  • mean pulmonary artery pressure ≥25mmHg
  • (21-24mmHg borderline)
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27
Q

what are causes of pulmonary HTN group 4?

A

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
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28
Q

what is the primary treatment for Group 5 PH?

A

Target specific underlying cause

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29
Q

what does the prostanoid formulation improve?

A

prostanoid formulation = epoprostenol (prostacyclin)

-Improves hemodynamics, functional capacity, and survival in IPAH

30
Q

what symptom of PH is associated with advanced disease?

A

dyspnea at rest

31
Q

what are things to consider when treating all groups of PH?

A
  • 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
Q

digoxin improves what for which groups with PH?

A

Digoxin improves RV EF of patients with group 3 PH & helps control HR in patients who have SVTs associated with RV dysfunction

33
Q

what is pulmonary HTN almost always caused by?

A

Increased pulmonary vascular resistance (pulmonary arteries)

34
Q

what is group 1 classified as for pulmonary HTN?

A

Group 1 – secondary to diseases that localize to small pulmonary arteries/arterioles (PAH)

35
Q

how long until >20% of pts have symptoms of PH before PH is recognized?

A

> 2 years

36
Q

what criteria must be met to diagnose Group 1 PH?

A
  • 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
Q

what are signs & symptoms of right heart failure?

A
  • angina (r heart requires more oxygen)
  • exertional syncope (inability to increase CO)
  • peripheral edema (R heart failure)
  • abd discomfort due to hepatic congestion
38
Q

what is the pathophysiology/cause of pulmonary HTN group 2?

A

Left heart disease
-L atrial HTN requires an increased pulmonary artery systolic pressure to maintain an adequate driving force across the pulmonary vasculature

39
Q

what do the endothelia receptor antagonists improve?

A

exercise capacity, dyspnea and hemodynamic measures

40
Q

what is primary therapy for pulmonary HTN txt directed at?

A

directed at the underlying cause

41
Q

at what pulmonary artery pressure is PH diagnosed as well as the PASP?

A
  • PH is diagnosed when mean PAP =≥25 mmHg at rest

- PASP between 21mmHg – 24mmHg are of uncertain clinical significance

42
Q

what are the PDE-5 inhibitors?

A
  • Sildenafil
  • Tadalafil
  • Vardenafil
  • *Prolong vasodilatory effect of nitric oxide.
  • *Also used to treat erectile dysfunction
  • *Improves pulmonary hemodynamics and exercise capacity.
43
Q

what is pulmonary HTN usually caused by?

A

long standing pulmonary disease/hypoxia

44
Q

what is group 2 classified as for pulmonary HTN?

A

Group 2 –secondary to L heart disease

45
Q

what is advanced therapy for pulmonary HTN txt directed at?

A

the pulmonary HTN itself

46
Q

what does pulmonary HTN result in?

A

right sided heart failure/Cor Pulmonale

47
Q

what is the pathophysiology of pulmonary arterial hypertension/Group 1 PH?

A

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
Q

what is group 3 for WHO functional classification for pulmonary HTN

A

<b>Marked limitation of physical activity.</b> They are comfortable at rest. Less than ordinary physical activity causes undue fatigue or dyspnea, chest pain, or heart syncope.

49
Q

what are causes of Group 1 pulmonary arterial HTN?

A
  • 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
Q

how do CCBs help PH pts?

A
  • Prolonged survival
  • Sustained functional improvement
  • Hemodynamic improvement
51
Q

when is PH unlikely according to pulmonary artery systolic pressure?

A

<36mmHg & TRV <=2.8 m/sec

52
Q

anticoagulation treatment is good for which groups with PH?

A

Anticoagulation for IPAH, hereditary PAH, drug-induced PAH or group 4 PH

53
Q

EKG in pt with pulmonary artery HTN & RVH

A

right axis deviation & poor R wave progression

54
Q

where should advanced therapy for PH be used?

A

should be administered at specialized centers where clinicians are experienced

55
Q

what does the Echo for pulmonary HTN assess?

A

assesses PASP, R heart size and L to R shunts (PFO)

-diagnose left ventricular dysfunction

56
Q

advanced therapy in group 3 PH?

A

not FDA approved, not recommended, occasionally considered

57
Q

advanced therapy in group 4 PH?

A

can be considered

58
Q

what are the causes of pulmonary HTN?

A
  1. Increased pulmonary vascular resistance (pulmonary arteries) <b>(** almost always caused by this)</b>
  2. Elevated L atrial pressure (L heart disease; valve disease, systolic dysfunction etc.)
  3. Increased pulmonary blood flow (volume) – <b>(not a significant cause– pulm vascular bed vasodilates & recruits vessels in response to increased flow)</b>
59
Q

dx tests for pulmonary HTN?

A
  • 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
Q

how many classifications of pulmonary HTN are there?

A

5 groups

61
Q

who is vasoreactivity testing for?

A

for pts who are selected for advanced therapy to determine if they will benefit from CCB’s (less expensive, fever side effects)

62
Q

what is the first test to order for pulmonary HTN and that is also useful for screening?

A

TTE

-useful for screening

63
Q

when is PH likely according to pulmonary artery systolic pressure?

A

> 50mmHg & Tricuspid regurgitation velocity (TRV) is >3.4 m/sec

64
Q

what is group 4 for WHO functional classification for pulmonary HTN

A

<b>Inability to carry on any physical activity without symptoms, signs of right heart failure.</b> Dyspnea and/or fatigue may be present even at rest.

65
Q

why is early identification and treatment of pulmonary HTN ideal?

A

Early identification and treatment is ideal because advanced disease may be less responsive to therapy

-also need to assess disease severity

66
Q

advanced therapy in group 1 PAH?

A

first line

67
Q

what are causes of pulmonary HTN group 3?

A
  • Chronic obstructive pulmonary disease (COPD)
  • Interstitial lung disease (ILD)
  • Obstructive sleep apnea (OSA)
  • Other causes of hypoxemia

<b>**Generally only caused by severe lung disease</b>

68
Q

what is normal pulmonary artery pressure (PAP) and measured by what?

A

8-20mmHg measured by R heart catheterization

69
Q

what is the definition of cor pulmonale? causes?

A

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)

<b>(Note: R heart dz secondary to L heart dz is NOT Cor Pulmonale)</b>

70
Q

advanced therapy in group 2 PH?

A 
possibly harmful (epoprostenol)
-L heart unable to handle increased flow
71
Q

what is the gold standard for diagnosing pulmonary hypertension and is also necessary to confirm diagnosis?

A

R heart catheterization – gold standard & necessary to confirm diagnosis

72
Q

what is the primary treatment for Group 4 PH?

A

Anticoagulation, surgical thromboendarterectomy for selected patients

Clinical Medicine II (10 decks)

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