SM 165 Pulmonary Hypertension Flashcards

1
Q

What is Type I Pulmonary Hypertension?

A

Pulmonary Arterial Hypertension

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2
Q

Which form of Pulmonary Hypertension is rare?

A

Type I

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3
Q

What can cause Type I Pulmonary Hypertension?

A

Heredity, drugs, infections, idiopathic

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4
Q

What is Type II Pulmonary Hypertension?

A

Pulmonary Hypertension due to Left Heart Disease

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5
Q

What can cause Type II Pulmonary Hypertension?

A

Heart Failure: systolic or diastolic dysfunction, as well as valvular disease

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6
Q

Which is more common, Type I or Type II Pulmonary Hypertension?

A

Type II

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7
Q

What is Type III Pulmonary Hypertension?

A

Pulmonary Hypertension due to intrinsic lung disease

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8
Q

What can cause Type III Pulmonary Hypertension?

A

COPD, Interstitial Lung Disease, Sleep Disorders

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9
Q

What is Type IV Pulmonary Hypertension?

A

Chronic Thromboembolic Pulmonary Hypertension

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10
Q

What can cause Type IV Pulmonary Hypertension?

A

A persistent pulmonary embolism that raises pressure in the pulmonary circulation

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11
Q

What is Type V Pulmonary Hypertension?

A

Multifactorial Pulmonary Hypertension

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12
Q

What can cause Type V Pulmonary Hypertension?

A

A variety of factors, such as systemic and metabolic disorders

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13
Q

What does PCWP approximate and why is it relevant for Pulmonary Hypertension?

A

PCWP approximates LA Pressure and is used to rule out Left Heart failure

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14
Q

What is used to diagnose Pulmonary Hypertension?

A

A PA catheter measurement of Pulmonary Artery Pressure

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15
Q

What are the diagnostic criteria for Type I Pulmonary Hypertension?

A

PAP >= 25mmHg + PCWP <= 15mmHg

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16
Q

What are the diagnostic criteria for Type II Pulmonary Hypertension?

A

PAP >= 25mmHg + PCWP >= 15mmHg

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17
Q

What differentiates the diagnostic criteria for Type I and Type II Pulmonary Hypertension, and why?

A

Both require elevated PAP >= 25mmHg but Type II involves left heart failure so PCWP >= 15mmHg while Type I does not so PCWP <= 15mmHg

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18
Q

What are the diagnostic criteria for Type III Pulmonary Hypertension?

A

PAP >= 25mmHg + underlying chronic lung disease

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19
Q

What are the diagnostic criteria for Type IV Pulmonary Hypertension?

A

PAP >= 25mmHg + PCWP <= 15mmHg + Pulmonary Embolism

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20
Q

Which type of Pulmonary Hypertension can be cured?

A

Type IV can be cured because the clot can be removed

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21
Q

How is Pulmonary Vascular Resistance calculated?

A

(R. Heart Pressure - L. Heart Pressure)/CO

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22
Q

What sorts of changes accompany Type I Pulmonary Hypertension?

A

Smooth muscle hypertrophy, neointimal thickening, and endothelial proliferation of the pulmonary vasculature (pulmonary artery)

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23
Q

Which portion of the pulmonary circulation is affected by Type I Pulmonary Hypertension?

A

Precapillary arterioles

24
Q

Which portion of the pulmonary circulation is affected by Type II Pulmonary Hypertension?

A

Post-capillary muscles and the left heart

25
Q

What portion of the pulmonary circulation is affected by Type III Pulmonary Hypertension?

A

The parenchemal tissue that forms the pulmonary capillary bed

26
Q

Which portion of the pulmonary circulation is affected by Type IV Pulmonary Hypertension?

A

Precapillary arterioles occluded by an embolism

27
Q

What is the major screening test for Pulmonary Hypertension?

A

Transthoracic Echo

28
Q

What sorts of EKG changes would be expected with Pulmonary Hypertension?

A

RV Hypertrophy and Right Axis Deviation

29
Q

What Echocardiogram findings would correlate with Type I Pulmonary Hypertension?

A

Normal Left Heart with Dilated Right Ventricle

30
Q

What Echocardiogram findings would correlate with Type II Pulmonary Hypertension?

A

Dilated Left Heart with Normal Right Ventricle

31
Q

Should pressure estimates from an Echocardiogram be trusted in Pulmonary Hypertension?

A

No; they are prone to error, and the focus during Echo should be on the structure and function of the heart

32
Q

What test is used to diagnose Type V Pulmonary Hypertension?

A

V/Q Scan, which identifies thromboembolic pulmonary malperfusion

33
Q

What ad-hoc tests should be used in the work up of Pulmonary Hypertension?

A

After diagnosis of Pulmonary Hypertension, use serology tests and sleep tests to identify underlying infectious or sleep-dependent causes

34
Q

What is the gold standard for diagnosing Pulmonary Hypertension?

A

Right Heart Catheterization, which directly measures the PAP, PCWP, and CO as well as indirectly calculating PVR

35
Q

Why is characterizing the type of PH important?

A

Treatments for Type I can hurt Type II and III, and the treatment is expensive

36
Q

Is Pulmonary Hypertension common?

A

Yes, but Type I Pulmonary Hypertension is not

37
Q

What are the symptoms of Pulmonary Hypertension?

A

Non-specific; Progressive dyspnea on exertion, fatigue, chest pain

38
Q

Which group is mostly affected by Pulmonary Hypertension?

A

Females

39
Q

What is the treatment for Type I Pulmonary Hypertension?

A

Type I is driven by abnormal vessel smooth muscle proliferation, so treat it with a Pulmonary Vasodilator to decrease resistance

40
Q

Are Pulmonary Vasodilators curative?

A

No, because they do not address the underlying cause of smooth muscle proliferation; they just dilate to decrease resistance

41
Q

What are the 3 classes of Pulmonary Vasodilators?

A

Endothelin blockers, PDE Inhibitors, and Prostacyclin agonists

42
Q

How do Endothelin blockers work?

A

Prevent the vasoconstrictive effects of Endothelin to cause pulmonary vasodilation

43
Q

How do PDE inhibitors work?

A

Prevent PDE from degrading Nitric Oxide to cause pulmonary vasodilation

44
Q

How do Prostacyclin agonists work?

A

Increase Prostacyclin to cause vasodilation, and administered via IV 24/7, to cause pulmonary vasodilation

45
Q

How is Type II Pulmonary Hypertension treated?

A

Optimize heart failure medications and diurese to lower pulmonary pressure

46
Q

Are pulmonary vasodilators indicated for Type II Pulmonary Hypertension, and why/why not?

A

Pulmonary vasodilators are not indicated for Type II Pulmonary Hypertension, because decreasing the arterial resistance will cause greater blood flow to a failing left heart, which results in more blood backing up into the pulmonary circulation and causing pulmonary edema

47
Q

What is the risk of pulmonary vasodilators in Type II Pulmonary Hypertension?

A

Pulmonary Edema

48
Q

What is the treatment for Type III Pulmonary Hypertension and why?

A

Optimize lung disease medications and reverse hypoxia, to treat the underlying cause of capillary damage and prevent hypoxia induced vasoconstriction

49
Q

Are pulmonary vasodilators indicated for Type III Pulmonary Hypertension, and why/why not?

A

Pulmonary vasodilators are not indicated for Type III Pulmonary Hypertension, because they can increase V/Q mismatch; hypoxic regions of the lungs naturally vasoconstrict to avoid wasting blood perfusion, so dilating the pulmonary arterioles and overcoming this resistance leads to perfusion of blood in areas with low ventilation, worsening V/Q mismatch

50
Q

What is the treatment for Type IV Pulmonary Hypertension?

A

Pulmonary Vasodilators, anti-coagulants, and thrombo-endarterectomy

51
Q

Are pulmonary vasodilators indicated for Type IV Pulmonary Hypertension, and why/why not?

A

Pulmonary vasodilators are indicated for Type IV Pulmonary Hypertension, because the clot typically blocks the arterioles and the vasodilators will relax the arterioles, allowing blood to flow around the clot and lower the pulmonary pressure

52
Q

What is Pulmonary Thromboendartectomy?

A

A surgical procedure where the clot is directly removed from the pulmonary circulation, curing Type IV Pulmonary Hypertension

53
Q

What does passive congestion refer to in Pulmonary Hypertension and how does it guide treatment?

A

Passive congestion refers to Pulmonary Hypertension primarily due to Left Heart failure alone, which is best treated with a diuretic to lower pressure; some Pulmonary Hypertension involves vascular remodeling, which extends beyond the HF and may require additional treatment

54
Q

What happens if Pulmonary Hypertension is “out of proportion”?

A

The elevated Pulmonary pressure is beyond what should be expected from Heart Failure alone, suggesting vascular proliferation or dysfunction

55
Q

What distinguishes Lung Disease with Severe Pulmonary Hypertension?

A

Strongly elevated PAP >= 35mmHg indicates an underlying vascular cause to the Pulmonary Hypertension, beyond the Lung Disease itself

56
Q

How is Lung Disease with Pulmonary Hypertension effected by severity of Pulmonary Hypertension?

A

In less severe patients (PAP < 35mmHg), the symptoms are due primarily to the ventilatory impairment of the lung disease itself; in the rare severe patient, symptoms are driven by circulatory impairment from the elevated PAP

57
Q

Which two types of Pulmonary Hypertension have a spectrum of treatment options?

A

Type 2 and Type 3, because they can vary in severity of elevated PAP and by extension the underlying cause