CVPR Week 6: Pulmonary Hypertension Flashcards
1
Q
Objectives
A
2
Q
Question 1
A
3
Q
Why aren’t the incorrect answers correct?
A
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
5
Q
Clinical classifications of pHTN
A
6
Q
Hemodynamic classification of pHTN
A
Pre-capillary pHTN
or
Post-capillary pHTN
7
Q
What pressure is defined as PH?
A
MPAP > 20 mmHg
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
9
Q
PAH AKA
A
Pulmonary Arterial hypertension
10
Q
Pulmonary Arterial Hypertension cause
A
45% idiopathic (Idiopathic PAH)
11
Q
PAH is associated with?
5 listed
A
- connective tissue disease
- HIV infection
- Portal hypertension
- Congenital heart disease
- Schistomiasis
12
Q
PPHN AKA
A
Persistent pulmonary hypertension of the newborn
13
Q
PVOD/PCH
A
PAH with overt features or venous/capillaries involvement
14
Q
PAH long-term responders to Ca channel blockers
A
15
Q
PH due to left heart disease
A
16
Q
PH due to lung disease and/or hypoxia
A
17
Q
PH due to pulmonary artery obstructions
A
18
Q
PH with unclear multifactorial mechanisms
A
19
Q
Hemodynamic classification of PH
A
is it precapillary or postcapillary or is there a little bit of both
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)
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
22
Q
Pre-capillary PH
A
PCWP <15 mmHg
PVR >= woods units
23
Q
Question 2
A
24
Q
WHO Group 1 PH
A
Pulmonary Arterial Hypertension
25
PAH Definition
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
26
Hemodynamic definition of PAH
* mPAP \> 20 mmHg
* PCWP \<= 15 mmHg
* PVR \>= 3 woods units
27
Prevalence of PAH
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)
28
PAH is most common in
Older female patients
29
PAH Pathogenesis
Aberrant pathways
30
Pathophysiologic changes in PAH
Pulmonary vascular remodeling
31
Prevalence of PAH in connective tissue diseases
32
Drugs and toxins definitely associated with PAH
7 listed
* Aminorex
* Fenfluramine
* Dexfenfluramine
* Toxic rapeseed oil
* Benfluorex
* Dasatinib
* Methamphetamines
33
Possible drugs and toxins associated with PAH
10 listed
* Cocaine
* Phenylpropanolamine
* St. John's Wort
* Interferon-alpha and beta
* Amphetamines
* Alkylating agents
* Direct acting antiviral agents against hepititis
* Bosutinib
* Leflunomide
34
Question 3
35
Most common presenting symptoms
36
Most examination in PAH
37
The role of ECG in the diagnosis of PAH
38
Pulmonary exam findings in PAH
6 listed
* 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
PAH Detection and diagnosis
40
PAH Detection and diagnosis
41
WHO Functional classification of PAH
4 listed
42
WHO Class I PAH
* No limitation of usual physical activity
* Ordinary physical activity does not increase dyspnea, fatigue, chest pain or pre-syncope
43
WHO Class II PAH
* Slight limitation of physical activity
* No discomfort at rest
* normal physical activity causes increased dyspnea, fatigue, chest pain or presyncope
44
WHO Class III PAH
* Marked limitation of physical activity
* No discomfort at rest
* less than ordinary activity causes increased dyspnea, fatigue, chest pain or pre-syncope
45
WHO Class IV PAH
* 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
Increased risk of PAH
* Systemic Lupus Erythematosis
* Mixed connective tissue disease
* Systemic Sclerosis
* drugs and toxins
47
Diagnostic gold standard of PAH
* Hemodynamics - RAP, mPAP, PCWP, CO/Cl, PVR
* Saturations to rile out shunts
* Angiography - vessel properties CTEPH
* Vasodilator response
48
PAH Hemodynamic diagnostic tests
5 listed
* RAP
* mPAP
* PCWP
* CO/Cl
* PVR
49
PAH Saturation diagnostic tests
Rule out shunts
50
PAH Angiography diagnostic tests
* Vessel properties
* CTEPH (Chronic Thromboembolic Pulmonary Hypertension)
51
PAH Vasodilator response
52
PAH/CTEPH Fluid/volume control
3 listed
* Diuretics
* Low sodium diet
* Daily wgts/I&Os
53
PAH/CTEPH oxygen care
hypoxia is a potent pulmonary vasoconstrictor and can elevate PA pressure
54
PAH/CTEPH Anticoagulation treatment
* IPAH/CTEPH patients on anticoagulation
* consideration in other WHO group 1 patients
55
PAH/CTEPH General care categories and treatment
3 listed
56
Question 4
57
Therapeutic pathways for PAH
3 listed
* Endothelin pathway
* Nitric oxide pathway
* Prostacyclin pathway
58
Groups approved for treating PH
* Group 1 (PAH)
* Group 4 (CTEPH)
* The other groups require treatment of the underlying condition causing the elevated pressures
59
Therapeutic options for PAH: Drug classes
3 listed
* Prostanoid/Prostanoid analogs
* ERAs
* PDE-5 inhibitors
60
Prostanoids description
Prostacyclin (PGl2) member of the eicosanoids family, inhibits platelet activation and effective vasodilator
61
Prostacyclin is usually made where?
In healthy endothelial cells
62
Level of prostacyclin in PAH patients
They have a deficiency of prostacyclins
63
Prostanoids ROA
Sever routes of administration
* IV/Parenteral
* SQ
* Inhaled
* Oral
64
IV Prostanoids
Esoprostenol - Flolan and Veletri
Treprostinil - Remodulin
65
Epoprostenol half life
Approximately 2-5 minutes
66
Treprostinil half-life
several hours
67
Treprostinil and Remodulin administration rate
ng/kg/min
68
Treprostinil and Remodulin dosing changes
* Dosing never changes even if the weight does
* The weight at start remains the same throughout the duration of therapy
69
Single lumen Hickman Catheter
* Never stop infusion
* Never draw labs from line
* Never flush
70
Oral prostacyclins
* Treprostinil (orinetram)
* Selexipag (Uptravi)
71
Treprostinil (orinetram)
Antiplatelet and vasodilatory actions including pulmonary vasodilation
72
Selexipag (Uptravi)
* Most recently approved in Jan 2016
* Not a substitute for parenteral therapy
* Prostacyclin analog
73
Endothelin receptor antagonist for PAH because
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
Endothelin receptor antagonist for PAH AGENTS
* Bosentan
* Ambrisentan
* Macitentan
75
Phosphodiesterase-5 inhibitors for PAH because
PDE5 inhibitor - blocks the degradative action of phosphodiesterase type 5 on cyclic GMP in smooth muscle cells resulting in vasodilation of the vessels
76
Phosphodiesterase-5 inhibitors for PAH Agents
Sildenafil
Tadalafil
77
Guanylate Cyclase Stimulator for PAH
* 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
Ongoing management for PAH patients
79
PH Discussion
PH is a simple hemodynamic definition but is an umbrella term
80
PH is screened with?
Echo
81
PH is diagnosed with
RHC (right heart catheterization)
