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ABIM Pulmonary > Pulmonary Hypertension > Flashcards

Pulmonary Hypertension Flashcards

1
Q

Equation for pulmonary vascular resistance?

A

PVR = (mPAP - PCWP)/CO.

-Gives result in Wood’s Units. Multiply by 80 for dynes.

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2
Q

Equation for trans-pulmonary gradient?

A

mPAP - PCWP

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3
Q

How to change from Wood’s Units to dynes?

A

multiply by 80.

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4
Q

Genetic mutation associated with heritable pulmonary hypertension?

A

BMPR2 is most common.

-Also, ALK-1, KCNK3, Endoglin, SMAD9, Caveolin-1

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5
Q

Common drugs for drug-induced pulmonary hypertension (group 1.3)

A
  • Definite; Aminorex, Fenfluramine, Dexfenfluramine, methamphetamines, benfluorex, Dasatinib, Toxic Rapeseed Oil.
  • Possible: Cocaine, amphetamines, phenylpropanolamine, L-tryptophan, St. John’s Wort, IFN, alkylating agents, Bosutinib, Hep C antivirals, Leflonamide, Indirubin (Chinese Qing-Dai herb)
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6
Q

When is RHC indicated in connective tissue disease (according to the DETECT algorithm)?

A

If TTE is abnormal, or FVC/DLCO ratio >1.6.

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7
Q

When to screen HIV patients for pulmonary hypertension?

A

IIf female, cocaine users, or hepatitis C.

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8
Q

When to screen for pulmonary hypertension in cirrhotics?

A

If any portal hypertension present.

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9
Q

Group 1 PAH congenital heart defects?

A
  • Eisenmenger
  • Persistient systemic to pulmonary shunts
  • Small, coincidental defects
  • Defect correction
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10
Q

Treatment for PVOD / PCH (Pulmonary Veno-Occlsuive Disease / Pulmonary Capillary Hemangiomatosis)

A
  • Transplant

- Doesnt’ respond to group 1 PAH therapy, or develops severe pulmonary edema.

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11
Q

TAPSE criteria for pulmonary hypertension?

A

TAPSE<1.6

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12
Q

Genetic mutation for PVOD / PCH?

A

EIF2AK4

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13
Q

Definition of successful vasodilator challenge during RHC for PH?

A

mPAP decrease of at least 10, to rech maximum of 40. Any decreased CO is a failure. Use iNO at 10-20ppm.

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14
Q

5 classes of PAH-specific therapies?

A
  1. Endothelin receptor antagonists
    - Ambrisentan
    - Bosentan
    - Macitentan
  2. PDE5 inhibitors
    - Sildenafil
    - Tadalafil
  3. Soluble Guanylate Cyclase stimulators
    - Riociguat
  4. Prostacyclin Receptor Agonists
    - Selexipag
  5. Prostacyclin Analogs
    - Epoprostanol
    - Iloprost
    - Treprostanil
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15
Q

What are the three Endothelin receptor antagonsits?

A
  1. Endothelin receptor antagonists
    - Ambrisentan
    - Bosentan
    - Macitentan
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16
Q

What is the soluble guanylate cyclase stimulator?

A

Riociguat

17
Q

What is the prostacyclin receptor agonist?

A

Selexipag

18
Q

What are the three prostacyclin analogs?

A

Epoprostanol
Iloprost
Treprostanil

ABIM Pulmonary (37 decks)

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