Pulmonary Hypertension Flashcards

1
Q

7 subtypes of group 1 PHTN

A
  1. Idiopathic
  2. Heritable
  3. Drug and Toxin induced
  4. PAH associated with
    1. Connective tissue disease
    2. HIV
    3. Portal HTN
    4. Congenital heart disease
    5. Schistosomiasis
  5. PAH long-term responders to CCBs
  6. PAH with overt features of venous/capillaries involvement (PVOD/PCH)
  7. Persistent PHTN of the newborn syndrome
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2
Q

4 subtypes of group 2 PHTN

A
  1. HFpEF
  2. HFrEF
  3. Valvular heart disease
  4. Congenital/Acquired cardiovascular conditions leading to post-capillary PHTN
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3
Q

5 subtypes of group 3 PHTN

A
  1. Obstructive lung disease
  2. Restrictive lung disease
  3. Mixed disease
  4. Hypoxia without lung disease
  5. Developmental lung disorders
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4
Q

2 subtypes of group 4 PHTN

A
  1. CTEPH
  2. Other pulmonary artery obstructions
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5
Q

4 subtypes of group 5 PHTN

A
  1. Hematologic disorders
  2. Systemic and metabolic disorders
  3. Others
  4. Complex congenital heart disease
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6
Q

Hemodynamics of pre and post capillary PHTN

A
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7
Q

Calculation for Transpulmonary Gradient (TPG)

A

TPG = MPAP - PCWP

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8
Q

PVR calculation formula

A

PVR = TPG/CO

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9
Q

Gene mutation responsible for 75% of heritable PAH

A

BMPR2

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10
Q

Genes associated with hereditary hemorrhagic telangiectasia and PAH

A

Alk-1

SMAD9

Endoglin

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11
Q

Gene mutation found in PAH from PVOD or PCH

A

EIF2AK4

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12
Q

Most common connective tissue disease in PAH

A

Scleroderma

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13
Q

DETECT algorithm for performing RHC in scleroderma

A

Get if any of the following:

  1. TTE changes
  2. FVC/DLCO ratio > 1.6
  3. > 2 fold upper limit of normal NT-Pro-BNP
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14
Q

PAH is an independent risk factor for death in what disease?

A

HIV

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15
Q

Echo criteria for RHC in portopulmonary HTN

A
  1. Tricuspid jet > 3.4
  2. RA enlargement
  3. RV enlargement or dysfunction
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16
Q

2 key findings associated with PVOD

A

Low DLCO and out of proportion hypoxemia

17
Q

Patients who develop pulmonary edema with PAH therapy may have what condition?

A

PVOD

18
Q

Treatment for PVOD

A

Transplant

19
Q

Occupational exposure linked to PVOD

A

Trichloroethylene (organic solvent)

20
Q

Most frequent PAH presenting symptom

A

DOE

21
Q

Only 3 types of PAH you do vasoreactivity for

A

IPAH, HPAP, DT PAH

22
Q

Low, Medium, and High risk mortality 6MWT for PHTN

A

> 440

165-440

< 165 m