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Drugs Used in Pulmonary HTN > PAH > Flashcards

PAH Flashcards

1
Q

Class I PAH

A

Pts w/ pulm HTN but w/o resulting limitation of physical activity. Ordinary physical activity does not cause dyspnea or fatigue, CP, or near syncope

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2
Q

Class II PAH

A

Pts w/ pulm HTN resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary activities cause undue dyspnea or fatigue, CP or near syncope

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3
Q

Class III PAH

A

Pts w/ pulm HTN resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes undue dyspnea or fatigue, CP, or near syncope.

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4
Q

Class IV PAH

A

Pts w/ pulm HTN w/ inability to carry out physical activity without sx. These pts manifest signs of RHF. Dyspnea and or fatigue may even be present at rest.

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5
Q

Which pts are recommended to have the vasopressor test?

A

Group I PAH

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6
Q

What types of vasodilators are administered in a vasopressor test?

A

Epoprostenol, adenosine, inhaled nitric oxide

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7
Q

What is a + vasopressor test?

A
  • pulm artery pressure fall greater than or equal to 10 mmHg
  • Mean pulm art pressure is less than or equal to 40 mmHg
  • CO is unchanged or ↑
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8
Q

When to use CCBs?

A

-about 1/2 responders will achieve sustained functional improvement and prolonged survival with calcium channel blockers; CCBs are potentially deleterious in non-responders

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9
Q

PAH pts that have a positive response are those with___?

A

Idiopathic, familial or anorexigenic-induced PAH

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10
Q

PAH pts that are not likely to respond to vasopressor test?

A

Those with PAH caused by a connective tissue dz, HIV, toxins, etc.

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11
Q

What routine treatments have little impact on PAH progression?

A
  • Anticoagulants
  • Diuretics
  • Oxygen therapy (maintain >90%)
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12
Q

How is PAH treated?

A
  • No cure
  • Prognosis influenced by class; starting treatment earlier –> better prognosis w/ delayed progression
  • Thos in Class II can rapidly deteriorate within 6 months to more advanced PAH if untreated
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13
Q

How is PAH diagnosed?

A
  • The Gold Standard is a right heart cath
  • mPAP greater than or equal to 25 mmHg (nml = 14 +/-3)
  • PCWP less than or equal to 15 mmHg
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14
Q

Diagnostic approach & pivotal tests for evaluating Pulm HTN?

A

Initially CXR and EKG –> echo –> final diagnosis made vie R heart cath

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15
Q

Risk factors for pulm art HTN?

A
  1. Family Hx of PAH
  2. Genetic mutation for PAH in pt or 1st-dgree relative: BMPR2, TBXA2, KNCK3, EIF2AK4, Caveolin-1
  3. Limited cutaneous scleroderma or mixed CT dz: FVC/DLCO > 1.6, DLCO < 60%, BNP > 2x nml
  4. HIV infection
  5. Portal HTN
  6. Exposure to drugs or toxins (fen/phen weight loss pills, aminorex, methamphetamine, dastinib)
  7. Congenital heart dz w/ surgically repaired left-to-right shunt within 3-6 months
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16
Q

Early signs and symptoms of PAH?

A

Dyspnea on physical exertion, fatigue, CP, tachycardia, pain on the upper R abd, anorexia

17
Q

Sx when PAH progresses?

A

Syncope/near syncope, swelling in legs and ankles, cyanotic lips and skin

18
Q

Histologic features of PAH?

A
  • Intimal and medial thickening
  • Intimal fibrosis and in situ thrombosis
  • Collateral flux
  • Plexiform lesions (focal proliferation of endothelial and sm m cells; pathognomonic of PAH)
19
Q

Typical script for PAH?

A

Young mother; F>M

20
Q

Trx of pulm HTN bc of L heart dz?

A

Improves with effective trx of L sided HF

21
Q

Trx of pulm HTN bc of lung dz or hypoxia?

A

Treated with oxygen to dilate the pulmonary vasculature

22
Q

Trx of chronic thromboembolic pulm HTN?

A

Anticoag w/ warfarin, surgical thromboendarterectomy in some cases

23
Q

Trx of pulm HTN w/ unclear multifactorial mechanisms?

A

Rare, optimal treatment uncertain

24
Q

What is PAH?

A

Progressive ↑ in pulm vascular resistance (PVR) –> R ventricular overload –> R ventricular failure and premature death; if untrx the median survival is ~2.8 years

  • Progressive ↑ in PVR d/t inter-related changes in the pulm arterioles: vasoconstriction, inflammation, localized thrombosis formation, obstructive remodelling of the pulm vessel wall
25
Q

What is cor pulmonale?

A

Enlargement of the R side of the heart d/t high blood pressure in the pulmonary blood vessels, usually caused by chronic lung dz

26
Q

What to expect of CXR in pt with PAH?

A
  • Peripheral hypovascularity
  • Prominent central pulm art
  • RV enlargement
27
Q

What to expect on EKG in pt w/ PAH?

A

Right atrial enlargement, RVH with strain, and R axis deviation of the QRS complex

Drugs Used in Pulmonary HTN (4 decks)

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