Class 1: PAH mPAP at rest: PCWP: PVR:

Class 1: PAH mPAP at rest: ≥ 25 mmHg PCWP: ≤ 15 mmHg PVR: \> 3 Wood

Class 2: PVH mPAP at rest: PCWP:

Class 2: PVH mPAP at rest: ≥ 25 mmHg PCWP: ≥ 15 mmHg

Class 3: PH associated with lung disease mPAP at rest:

Class 3: PH associated with lung disease mPAP at rest: \> 25 mmHg Underlying chronic lung disease

Class 4: PH CTEPH mPAP at rest: PCWP:

Class 4: PH CTEPH mPAP at rest: \> 25 mmHg PCWP: ≤ 15 mmHg Evidence of chronic perfusion defects

SM_165a: Pulmonary Hypertension Flashcards by Nathan Shlobin

Group 1 pulmonary hypertension is _______

Group 1 pulmonary hypertension is pulmonary arterial hypertension

(associated with connective tissue disease)

How well did you know this?

Not at all

Perfectly

Group 2 pulmonary hypertension is _______

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

(left sided pressures high so right sided pressures high)

How well did you know this?

Not at all

Perfectly

Group 3 pulmonary hypertension is _______

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

How well did you know this?

Not at all

Perfectly

Group 4 pulmonary hypertension is _______

Group 4 pulmonary hypertension is chronic thromboembolic pulmonary hypertension

(abnormal V/Q scan)

How well did you know this?

Not at all

Perfectly

Group 5 pulmonary hypertension is _______

Group 5 pulmonary hypertension is pulmonary hypertension with unclear multifactorial mechanisms

How well did you know this?

Not at all

Perfectly

Normal CVP is _____ mmHg

Normal CVP is <8 mmHg

How well did you know this?

Not at all

Perfectly

Normal RAP is _____ mmHg

Normal RAP is 2-8 mmHg

How well did you know this?

Not at all

Perfectly

Normal RV pressure is _____ mmHg

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

How well did you know this?

Not at all

Perfectly

Normal PA pressure is ______

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

(mean = 14 mmHg)

How well did you know this?

Not at all

Perfectly

Normal PCWP is _____ mmHg

Normal PCWP is 6-12 mmHg

How well did you know this?

Not at all

Perfectly

Normal LV pressure is ____ mmHg

Normal LV pressure is 120 / 6-12 mmHg

How well did you know this?

Not at all

Perfectly

Normal LAP is ____ mmHg

Normal LAP is 6-10 mmHg

How well did you know this?

Not at all

Perfectly

Class 1: PAH

mPAP at rest:
PCWP:
PVR:

Class 1: PAH

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

How well did you know this?

Not at all

Perfectly

Class 2: PVH

mPAP at rest:
PCWP:

Class 2: PVH

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

How well did you know this?

Not at all

Perfectly

Class 3: PH associated with lung disease

mPAP at rest:

Class 3: PH associated with lung disease

mPAP at rest: > 25 mmHg
Underlying chronic lung disease

How well did you know this?

Not at all

Perfectly

Class 4: PH CTEPH

mPAP at rest:
PCWP:

Class 4: PH CTEPH

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

Class 1: PAH

Pathology

Class 1: PAH

Pathology

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

Class 2: PVH

Pathology

Class 2: PVH

Pathology

Medial thickening
Occlusive venopathy

Class 3: PH associated with lung disease

Pathology

Class 3: PH associated with lung disease

Pathology

Factor VIII staining
Smooth muscle hypertrophy

Class 4: PH CTEPH

Ventilation ___ perfusion

Class 4: PH CTEPH

Ventilation > perfusion

Where is the lesion in each type of PH?

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

Echocardiographic changes in Group 1 pulmonary hypertension are _____ sided

Echocardiographic changes in Group 1 pulmonary hypertension are right sided

(enlargement of RA and RV, poorly functioning RV)

Group 1 is PAH

Echocardiographic changes in Group 2 pulmonary hypertension are _____ sided

Echocardiographic changes in Group 2 pulmonary hypertension are left sided

(reduced LV EF and dilated LA)

Group 2 is PVH

Compare and contrast features of PAH and PVH

Normal V/Q scan makes CTEPH \_\_\_\_\_\_

Normal V/Q scan makes CTEPH unlikely (CT scan may underestimate the degree of obstruction in CTEPH)

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

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

Why is characterizing the type of pulmonary hypertension important?

* Therapies differ drastically between groups * Incorrect treatment can worsen a patient's clinical status * PAH treatments are costly

PVH is _____ common, while PAH is _____ common

PVH is very common, while PAH is not common

Presenting symptoms of PAH are often \_\_\_\_\_

Presenting symptoms of PAH are often non-specific

Group 1 pulmonary hypertension (PAH) is treated with \_\_\_\_\_\_\_

Group 1 pulmonary hypertension (PAH\_ is treated with pulmonary vasodilator therapy (dilates pre-capillary bed, meaning the small pulmonary arteries)

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

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

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

Group 2 pulmonary hypertension (PVH) is treated by optimizing heart failure medications and with diuresis (PDE-5 may be useful)

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

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

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

Group 3 pulmonary hypertension (PH lung disease) is treated by optimizing lung disease medications and reversing hypoxia (PDE-5 may be useful)

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

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)

Group 4 hypertension (CTEPH) is treated with \_\_\_\_\_\_, \_\_\_\_\_\_, and \_\_\_\_\_\_

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

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

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

What is lung disease with severe PH?

* 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