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SM_165a: Pulmonary Hypertension Flashcards

1
Q

Group 1 pulmonary hypertension is _______

A

Group 1 pulmonary hypertension is pulmonary arterial hypertension

(associated with connective tissue disease)

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2
Q

Group 2 pulmonary hypertension is _______

A

Group 2 pulmonary hypertension is pulmonary hypertension owing to left heart disease

(left sided pressures high so right sided pressures high)

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3
Q

Group 3 pulmonary hypertension is _______

A

Group 3 pulmonary hypertension is pulmonary hypertension owing to lung disease or hypoxia

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4
Q

Group 4 pulmonary hypertension is _______

A

Group 4 pulmonary hypertension is chronic thromboembolic pulmonary hypertension

(abnormal V/Q scan)

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5
Q

Group 5 pulmonary hypertension is _______

A

Group 5 pulmonary hypertension is pulmonary hypertension with unclear multifactorial mechanisms

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6
Q

Normal CVP is _____ mmHg

A

Normal CVP is <8 mmHg

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7
Q

Normal RAP is _____ mmHg

A

Normal RAP is 2-8 mmHg

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8
Q

Normal RV pressure is _____ mmHg

A

Normal RV pressure is 20-30 mmHg systolic and less than RAP diastolic

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9
Q

Normal PA pressure is ______

A

Normal PA pressure is 20-25 / 5-15 mmHg

(mean = 14 mmHg)

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10
Q

Normal PCWP is _____ mmHg

A

Normal PCWP is 6-12 mmHg

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11
Q

Normal LV pressure is ____ mmHg

A

Normal LV pressure is 120 / 6-12 mmHg

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12
Q

Normal LAP is ____ mmHg

A

Normal LAP is 6-10 mmHg

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13
Q

Class 1: PAH

  • mPAP at rest:
  • PCWP:
  • PVR:
A

Class 1: PAH

  • mPAP at rest: ≥ 25 mmHg
  • PCWP: ≤ 15 mmHg
  • PVR: > 3 Wood
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14
Q

Class 2: PVH

  • mPAP at rest:
  • PCWP:
A

Class 2: PVH

  • mPAP at rest: ≥ 25 mmHg
  • PCWP: ≥ 15 mmHg
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15
Q

Class 3: PH associated with lung disease

  • mPAP at rest:
A

Class 3: PH associated with lung disease

  • mPAP at rest: > 25 mmHg
  • Underlying chronic lung disease
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16
Q

Class 4: PH CTEPH

  • mPAP at rest:
  • PCWP:
A

Class 4: PH CTEPH

  • mPAP at rest: > 25 mmHg
  • PCWP: ≤ 15 mmHg
  • Evidence of chronic perfusion defects
17
Q

Class 1: PAH

Pathology

A

Class 1: PAH

Pathology

  • Smooth muscle hypertrophy
  • Neointima formation (neo-vascularization)
  • Endothelial cell proliferation (monoclonality)
    *
18
Q

Class 2: PVH

Pathology

  • *
A

Class 2: PVH

Pathology

  • Medial thickening
  • Occlusive venopathy
19
Q

Class 3: PH associated with lung disease

Pathology

  • *
A

Class 3: PH associated with lung disease

Pathology

  • Factor VIII staining
  • Smooth muscle hypertrophy
20
Q

Class 4: PH CTEPH

Ventilation ___ perfusion

A

Class 4: PH CTEPH

Ventilation > perfusion

21
Q

Where is the lesion in each type of PH?

A
  • Group 1: pulmonary arteries (smaller)
  • Group 2: pulmonary veins
  • Group 3: capillaries (parenchymal lung disease)
  • Group 4: arteries in lungs (larger)
22
Q

Echocardiographic changes in Group 1 pulmonary hypertension are _____ sided

A

Echocardiographic changes in Group 1 pulmonary hypertension are right sided

(enlargement of RA and RV, poorly functioning RV)

Group 1 is PAH

23
Q

Echocardiographic changes in Group 2 pulmonary hypertension are _____ sided

A

Echocardiographic changes in Group 2 pulmonary hypertension are left sided

(reduced LV EF and dilated LA)

Group 2 is PVH

24
Q

Compare and contrast features of PAH and PVH

25
Q

Normal V/Q scan makes CTEPH ______

A

Normal V/Q scan makes CTEPH unlikely

(CT scan may underestimate the degree of obstruction in CTEPH)

26
Q

The gold standard in the diagnostic work up of pulmonary hypertension is _____

A

The gold standard in the diagnostic work up of pulmonary hypertension is right heart catheterization

  • direct measurement of RAP, PAP, PCWP, CO
  • calculation of PVR and CI
  • detetermination of intracardiac shunt
  • determination of acute vasodilator activity
27
Q

Why is characterizing the type of pulmonary hypertension important?

A
  • Therapies differ drastically between groups
  • Incorrect treatment can worsen a patient’s clinical status
  • PAH treatments are costly
28
Q

PVH is _____ common, while PAH is _____ common

A

PVH is very common, while PAH is not common

29
Q

Presenting symptoms of PAH are often _____

A

Presenting symptoms of PAH are often non-specific

30
Q

Group 1 pulmonary hypertension (PAH) is treated with _______

A

Group 1 pulmonary hypertension (PAH_ is treated with pulmonary vasodilator therapy

(dilates pre-capillary bed, meaning the small pulmonary arteries)

31
Q

Treatment of Group 1 pulmonary hypertension (PAH) centers on three pathways: ______, ______, and ______

A

Treatment of Group 1 pulmonary hypertension (PAH) centers on three pathways: endothelin pathway, nitric oxide pathway, prostacyclin

  • Endothelin promotes vasoconstriction - downregulated in treatment
  • Nitric oxide promotes vasodilation - upregulated in treatment
  • Prostacyclin promotes vasodilation - upregulated in treatment
32
Q

Group 2 pulmonary hypertension (PVH) is treated by ______ and with _______

A

Group 2 pulmonary hypertension (PVH) is treated by optimizing heart failure medications and with diuresis

(PDE-5 may be useful)

33
Q

Pulmonary vasodilators are not used in treatment of Group 2 pulmonary hypertension (PVH) because of ______

A

Pulmonary vasodilators are not used in treatment of Group 2 pulmonary hypertension (PVH) because of theoretical risk of pulmonary edema

34
Q

Group 3 pulmonary hypertension (PH lung disease) is treated by ______ and _______

A

Group 3 pulmonary hypertension (PH lung disease) is treated by optimizing lung disease medications and reversing hypoxia

(PDE-5 may be useful)

35
Q

Pulmonary vasodilators are not used in treatment of Group 3 pulmonary hypertension (PH lung disease) because of ______

A

Pulmonary vasodilators are not used in treatment of Group 3 pulmonary hypertension (PH lung disease) because of theoretical risk of increasing V/Q mismatching and worsening hypoxemia

(no clear benefit from PAH specific therapy and trend toward increased oxygen requirement for Group 3 patients)

36
Q

Group 4 hypertension (CTEPH) is treated with ______, ______, and ______

A

Group 4 hypertension (CTEPH) is treated with anticoagulation, pulmonary vasodilators in non-operative candidates, and thromboendarterectomy

37
Q

Group 2 pulmonary hypertension (PVH) and Group 3 pulmonary hypertension (PH lung disease) would be sometimes referred to as _______

A

Group 2 pulmonary hypertension (PVH) and Group 3 pulmonary hypertension (PH lung disease) would be sometimes referred to as “out of proportion”

38
Q

What is lung disease with severe PH?

A
  • Minority of chronic lung disease patients suspected to have severe vascular abnormalities
  • Circulatory impairment is primary driver of reduced exercise capacity rather than ventilatory impairment related to lung disease

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