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CVPR Week 6: Pulmonary Hypertension Flashcards

1
Q

Objectives

A
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2
Q

Question 1

A
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3
Q

Why aren’t the incorrect answers correct?

A
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4
Q

Definition of pulmonary hypertension

A

pHTN refers to the presence of abnormally high pulmonary vascular pressure

Normal mPAP: 8 - 20 mmHg at rest

pHTN is defined as mPAP > 20 mmHg at rest

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5
Q

Clinical classifications of pHTN

A
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6
Q

Hemodynamic classification of pHTN

A

Pre-capillary pHTN

or

Post-capillary pHTN

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7
Q

What pressure is defined as PH?

A

MPAP > 20 mmHg

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8
Q

Groups of PH

A
  • Pulmonary arterial hypertension (PAH)
  • PH due to left heart disease
  • PH due to lung disease and/or hypoxia
  • PH due to pulmonary artery obstructions
  • PH with unclear multifactorial mechanisms
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9
Q

PAH AKA

A

Pulmonary Arterial hypertension

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10
Q

Pulmonary Arterial Hypertension cause

A

45% idiopathic (Idiopathic PAH)

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11
Q

PAH is associated with?

5 listed

A
  • connective tissue disease
  • HIV infection
  • Portal hypertension
  • Congenital heart disease
  • Schistomiasis
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12
Q

PPHN AKA

A

Persistent pulmonary hypertension of the newborn

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13
Q

PVOD/PCH

A

PAH with overt features or venous/capillaries involvement

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14
Q

PAH long-term responders to Ca channel blockers

A
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15
Q

PH due to left heart disease

A
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16
Q

PH due to lung disease and/or hypoxia

A
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17
Q

PH due to pulmonary artery obstructions

A
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18
Q

PH with unclear multifactorial mechanisms

A
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19
Q

Hemodynamic classification of PH

A

is it precapillary or postcapillary or is there a little bit of both

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20
Q

Features of Post-capillary PH

4 listed

A

↑ PVP (pulmonary venous pressure)

↑ LAP (Left atrial pressure)

↑ LVEDP (Left ventricular end-diastolic pressure)

However vascular resistance on the arterial side remains normal)

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21
Q

Some common causes of Post-capillary PH

9 listed

A

Myocardial diseases such as

  • Dilated CMP-ischemic/non-ischemic
  • Hypertrophic CMP
  • Restrictive/infiltrative CMP
  • Obesity-related CMP
  • Pericardial disease

Aortic valve disease

Mitral valve disease

Mass can compress pulmonary vein

etc

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22
Q

Pre-capillary PH

A

PCWP <15 mmHg

PVR >= woods units

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23
Q

Question 2

A
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24
Q

WHO Group 1 PH

A

Pulmonary Arterial Hypertension

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25
Q

PAH Definition

A

is a syndrome resulting from restricted blood flow in the pulmonary arterial circulation resulting in increased pulmonary vascular resistance which causes right ventricular strain and ultimately failure

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26
Q

Hemodynamic definition of PAH

A
  • mPAP > 20 mmHg
  • PCWP <= 15 mmHg
  • PVR >= 3 woods units
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27
Q

Prevalence of PAH

A

PAH affects 15 to ~60 people per 1 million inhabitants in countries where studies have been conducted

1000 new cases are diagnosed in the United States every year

PAH incidence is more commonly diagnosed in

  • Women (70% - 80% of cases)
  • Older patients (50-65 years of age)
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28
Q

PAH is most common in

A

Older female patients

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29
Q

PAH Pathogenesis

A

Aberrant pathways

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30
Q

Pathophysiologic changes in PAH

A

Pulmonary vascular remodeling

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31
Q

Prevalence of PAH in connective tissue diseases

A
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32
Q

Drugs and toxins definitely associated with PAH

7 listed

A
  • Aminorex
  • Fenfluramine
  • Dexfenfluramine
  • Toxic rapeseed oil
  • Benfluorex
  • Dasatinib
  • Methamphetamines
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33
Q

Possible drugs and toxins associated with PAH

10 listed

A
  • Cocaine
  • Phenylpropanolamine
  • St. John’s Wort
  • Interferon-alpha and beta
  • Amphetamines
  • Alkylating agents
  • Direct acting antiviral agents against hepititis
  • Bosutinib
  • Leflunomide
34
Q

Question 3

35
Q

Most common presenting symptoms

36
Q

Most examination in PAH

37
Q

The role of ECG in the diagnosis of PAH

38
Q

Pulmonary exam findings in PAH

6 listed

A
  • Accentuated pulmonary component of S2 (audible at apex in over 90%)
  • Early systolic click
  • Midsystolic ejection murmur
  • Left parasternal lift
  • Right ventricular S4
  • Increased jugular a wave
39
Q

PAH Detection and diagnosis

40
Q

PAH Detection and diagnosis

41
Q

WHO Functional classification of PAH

4 listed

42
Q

WHO Class I PAH

A
  • No limitation of usual physical activity
  • Ordinary physical activity does not increase dyspnea, fatigue, chest pain or pre-syncope
43
Q

WHO Class II PAH

A
  • Slight limitation of physical activity
  • No discomfort at rest
  • normal physical activity causes increased dyspnea, fatigue, chest pain or presyncope
44
Q

WHO Class III PAH

A
  • Marked limitation of physical activity
  • No discomfort at rest
  • less than ordinary activity causes increased dyspnea, fatigue, chest pain or pre-syncope
45
Q

WHO Class IV PAH

A
  • Unable to perform any physical activity
  • possible signs of ventricular failure
  • Dyspnea and/or fatigue may be present at rest and symptoms are increased by almost any physical activity
46
Q

Increased risk of PAH

A
  • Systemic Lupus Erythematosis
  • Mixed connective tissue disease
  • Systemic Sclerosis
  • drugs and toxins
47
Q

Diagnostic gold standard of PAH

A
  • Hemodynamics - RAP, mPAP, PCWP, CO/Cl, PVR
  • Saturations to rile out shunts
  • Angiography - vessel properties CTEPH
  • Vasodilator response
48
Q

PAH Hemodynamic diagnostic tests

5 listed

A
  • RAP
  • mPAP
  • PCWP
  • CO/Cl
  • PVR
49
Q

PAH Saturation diagnostic tests

A

Rule out shunts

50
Q

PAH Angiography diagnostic tests

A
  • Vessel properties
  • CTEPH (Chronic Thromboembolic Pulmonary Hypertension)
51
Q

PAH Vasodilator response

52
Q

PAH/CTEPH Fluid/volume control

3 listed

A
  • Diuretics
  • Low sodium diet
  • Daily wgts/I&Os
53
Q

PAH/CTEPH oxygen care

A

hypoxia is a potent pulmonary vasoconstrictor and can elevate PA pressure

54
Q

PAH/CTEPH Anticoagulation treatment

A
  • IPAH/CTEPH patients on anticoagulation
  • consideration in other WHO group 1 patients
55
Q

PAH/CTEPH General care categories and treatment

3 listed

56
Q

Question 4

57
Q

Therapeutic pathways for PAH

3 listed

A
  • Endothelin pathway
  • Nitric oxide pathway
  • Prostacyclin pathway
58
Q

Groups approved for treating PH

A
  • Group 1 (PAH)
  • Group 4 (CTEPH)
  • The other groups require treatment of the underlying condition causing the elevated pressures
59
Q

Therapeutic options for PAH: Drug classes

3 listed

A
  • Prostanoid/Prostanoid analogs
  • ERAs
  • PDE-5 inhibitors
60
Q

Prostanoids description

A

Prostacyclin (PGl2) member of the eicosanoids family, inhibits platelet activation and effective vasodilator

61
Q

Prostacyclin is usually made where?

A

In healthy endothelial cells

62
Q

Level of prostacyclin in PAH patients

A

They have a deficiency of prostacyclins

63
Q

Prostanoids ROA

A

Sever routes of administration

  • IV/Parenteral
  • SQ
  • Inhaled
  • Oral
64
Q

IV Prostanoids

A

Esoprostenol - Flolan and Veletri

Treprostinil - Remodulin

65
Q

Epoprostenol half life

A

Approximately 2-5 minutes

66
Q

Treprostinil half-life

A

several hours

67
Q

Treprostinil and Remodulin administration rate

68
Q

Treprostinil and Remodulin dosing changes

A
  • Dosing never changes even if the weight does
  • The weight at start remains the same throughout the duration of therapy
69
Q

Single lumen Hickman Catheter

A
  • Never stop infusion
  • Never draw labs from line
  • Never flush
70
Q

Oral prostacyclins

A
  • Treprostinil (orinetram)
  • Selexipag (Uptravi)
71
Q

Treprostinil (orinetram)

A

Antiplatelet and vasodilatory actions including pulmonary vasodilation

72
Q

Selexipag (Uptravi)

A
  • Most recently approved in Jan 2016
  • Not a substitute for parenteral therapy
  • Prostacyclin analog
73
Q

Endothelin receptor antagonist for PAH because

A

Endothelin-1 (ET-1) levels are increased in PAH and found in the precapillary pulmonary microvasculature which is the site of the increased vascular resistance in PAH

74
Q

Endothelin receptor antagonist for PAH AGENTS

A
  • Bosentan
  • Ambrisentan
  • Macitentan
75
Q

Phosphodiesterase-5 inhibitors for PAH because

A

PDE5 inhibitor - blocks the degradative action of phosphodiesterase type 5 on cyclic GMP in smooth muscle cells resulting in vasodilation of the vessels

76
Q

Phosphodiesterase-5 inhibitors for PAH Agents

A

Sildenafil

Tadalafil

77
Q

Guanylate Cyclase Stimulator for PAH

A
  • Riociguat has a dual mode of action
  • Synergist with endogenous nitric oxide
  • Directly stimulating guanylate cyclase independent of NO availability
  • FDA approved for both use in PAH as well as inoperable or residual CTEPH
78
Q

Ongoing management for PAH patients

79
Q

PH Discussion

A

PH is a simple hemodynamic definition but is an umbrella term

80
Q

PH is screened with?

81
Q

PH is diagnosed with

A

RHC (right heart catheterization)

CVPR (93 decks)

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