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PAH Flashcards

1
Q

What is the definition of pulmonary hypertension?

A

Pulmonary hypertension (PH) is defined as an increase in the mean pulmonary arterial pressure (mPAP) >20 mmHg as assessed by right heart catheterization (RHC). It is a chronic disorder characterized by progressive elevations in pulmonary artery pressures and can lead to right heart failure.

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2
Q

What is the normal mPAP at rest?

A

The normal mPAP at rest is 14 + 3 mmHg, with an upper limit of normal approximately 20 mmHg.

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3
Q

What is the gold standard for diagnosing pulmonary hypertension?

A

Right Heart Catheterization (RHC) is the gold standard for diagnosing pulmonary hypertension.

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4
Q

Which diagnostic tool is initially used to suspect pulmonary hypertension?

A

2-D echocardiography is initially used to suspect pulmonary hypertension.

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5
Q

What does a Peak Tricuspid Regurgitation Velocity (TRV) of >3.4 m/s indicate?

A

A TRV of >3.4 m/s indicates a high probability of pulmonary hypertension.

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6
Q

What are the four categories of ‘OTHER ECHO SIGNS OF PH’?

A
  • Ventricles
  • Pulmonary Artery
  • Inferior Vena Cava and Right Atrium
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7
Q

What is the probability classification for a TRV of 2.9–3.4 m/s?

A

Intermediate probability of pulmonary hypertension.

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8
Q

What is the significance of the pulmonary artery wedge pressure (PAWP) in pulmonary hypertension diagnosis?

A

PAWP is used to distinguish between different types of pulmonary hypertension, particularly between pre-capillary and post-capillary hypertension.

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9
Q

What are the five clinical classifications of pulmonary hypertension?

A
  • Pulmonary Arterial Hypertension (PAH)
  • Pulmonary hypertension due to left heart disease (PH-LHD)
  • Pulmonary hypertension due to Lung Disease (LD) and/or Hypoxia
  • Chronic thromboembolic pulmonary hypertension and other pulmonary artery obstructions
  • Pulmonary hypertension with unclear and/or multifactorial mechanisms
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10
Q

What is a key factor in the management of pulmonary hypertension?

A

Identifying the clinical classification of pulmonary hypertension is crucial for guiding treatment.

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11
Q

What is the treatment approach for pulmonary arterial hypertension (PAH)?

A

PAH-approved therapies are recommended for patients with Pulmonary Arterial Hypertension (Class 1) and may be used as adjuncts in other conditions presenting with a PAH phenotype.

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12
Q

What does the term ‘vasoreactivity testing’ refer to in pulmonary hypertension?

A

Vasoreactivity testing evaluates the acute vasodilator response, aiming for a reduction of mPAP >10 mmHg with an increased or unchanged cardiac output.

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13
Q

Fill in the blank: The normal mPAP at rest is _______.

A

14 + 3 mmHg

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14
Q

True or False: Right Heart Catheterization (RHC) is recommended for all patients suspected of having pulmonary hypertension.

A

False

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15
Q

What is the aim of the PAPP Pulmonary Hypertension Task Force?

A

To establish a disease registry of pulmonary hypertension and create a clinical pathway for evaluation, diagnosis, management, and follow-up.

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16
Q

What is the purpose of the clinical pathway established by the PAPP Pulmonary Hypertension Task Force?

A

To guide clinicians step by step in the evaluation, diagnosis, management, and follow-up of patients afflicted with pulmonary hypertension.

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17
Q

What is the significance of the 6th World Symposium on Pulmonary Hypertension?

A

It proposed the new definition for pulmonary hypertension and updated guidelines for diagnosis and treatment.

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18
Q

What is the role of echocardiography in the diagnosis of pulmonary hypertension?

A

Echocardiography helps to assess the probability of pulmonary hypertension based on various parameters.

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19
Q

What is the relevance of the clinical pathway completion date?

A

The clinical pathway was completed and presented on November 16, 2022.

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20
Q

What is the primary indication for PAH-approved therapies?

A

Patients with Pulmonary Arterial Hypertension (Class 1)

These therapies may also be used as adjuncts in patients with pulmonary artery obstructions and chronic lung diseases presenting with a PAH phenotype.

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21
Q

What characterizes the ‘PAH phenotype’ in chronic lung diseases?

A

Pulmonary vasoconstriction

This phenotype includes symptoms insufficiently explained by lung mechanical disturbances and severe PH with high PVR and low CO.

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22
Q

What is the first step in the clinical pathway for diagnosing pulmonary hypertension?

A

Screening by 2-D echocardiography

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23
Q

Which echocardiographic parameters are helpful for screening pulmonary hypertension?

A
  • Tricuspid regurgitation gradient (TR gradient)
  • Pulmonic regurgitation gradient (PR gradient)
  • Pulmonary acceleration time (PAT)
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24
Q

What does a mean pulmonary artery pressure (mPAP) greater than 20 mmHg indicate?

A

Pulmonary Hypertension

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25
What is the recommended follow-up for patients with intermediate or high probability of pulmonary hypertension?
Right heart catheterization (RHC)
26
What should be done if Childhood Interstitial Lung Disease is suspected?
Refer to Pediatric Rheumatology for further work-up and management
27
What factors are assessed to classify the severity of pulmonary hypertension?
Mean pulmonary artery pressure (mPAP)
28
What are the classifications of pulmonary hypertension based on etiology?
* Class 1: Pulmonary Arterial Hypertension (PAH) * Class 2: Pulmonary Hypertension due to Left Heart Disease (PH-LHD) * Class 3: Pulmonary Hypertension due to Lung Disease (PH-LD) * Class 4: Chronic Thromboembolic Pulmonary Hypertension (CTEPH) * Class 5: Pulmonary Hypertension with unclear/multifactorial mechanisms
29
What are the general measures recommended for PAH management?
* Physical activity and rehabilitation * Infection prevention (vaccination) * Psychosocial support
30
Fill in the blank: The mean pulmonary artery pressure (mPAP) is computed based on _______.
[formula included in the pathway]
31
What is the definition of severe pulmonary hypertension based on mPAP?
>55 mmHg
32
What treatment is not recommended in the presence of left-heart disease?
PAH-approved therapies
33
What is the initial treatment dose for Nifedipine in PAH?
0.2-0.5 mg/kg/day
34
What should be monitored regularly in patients with pulmonary hypertension?
Functional status and prognosis
35
True or False: Patients with heritable pulmonary arterial hypertension should have their families screened.
True
36
What is the treatment recommendation for pulmonary hypertension due to lung disease with hypoxia?
* Long-term O2 administration * Treatment of underlying lung disease
37
What is the role of Right Heart Catheterization (RHC) in lung disease cases suspected of pulmonary hypertension?
Diagnosis by RHC is not recommended
38
What is the significance of a significant cyanosis in newborns at room air that improves with low oxygen supplementation?
It may indicate persistent pulmonary hypertension of the newborn (PPHN) or related conditions.
39
What is the treatment approach for CTEPH?
* Pulmonary endarterectomy (PEA) * Balloon dilatation of PA (BPA) in non-operable cases * Lifelong anticoagulation
40
What are the common symptoms related to right ventricular (RV) dysfunction?
* Peripheral edema * Anorexia, nausea, abdominal pain * Fatigue, dyspnea
41
What is the classification of pulmonary hypertension severity based on mPAP for mild cases?
21-40 mmHg
42
What is the recommended dosage for Sildenafil in neonates with PPHN?
0.25-0.5 mg/kg/dose 3x a day
43
Fill in the blank: The pulmonary vascular resistance (PVR) must be greater than ________ to classify as PAH.
>3 Wood units
44
What should be done for patients with suspected obstructive or restrictive ventilatory defects?
Pulmonary Function Test (Lung volume and DLCO)
45
What is the treatment recommendation for chronic lung disease presenting as ‘PAH’ phenotype?
Manage as PAH in addition to management of the present lung condition
46
What is the maximum dosage for Epoprostenol in PAH treatment?
20-40 ng/kg/min
47
What is the recommended approach for diagnosing suspected pulmonary hypertension in lung disease?
Diagnosis by RHC is not recommended for suspected PH in lung disease ## Footnote RHC stands for Right Heart Catheterization
48
What should be excluded in the diagnosis of pulmonary hypertension?
Other potential causes should be excluded, including: * LHD * CTEPH ## Footnote LHD stands for Left Heart Disease, and CTEPH stands for Chronic Thromboembolic Pulmonary Hypertension
49
What are the treatment options for CTEPH?
Treatment options include: * Pulmonary Endarterectomy (PEA) * Balloon Dilatation of PA (BPA) in non-operable CTEPH * Cava Filter Placement * Lifelong anticoagulation * Diuretics * Oxygen * Off-label use of PAH-approved therapies in symptomatic, inoperable CTEPH
50
What is the principle behind the phrase 'TREAT THE LUNG NOT THE PRESSURE'?
The principle emphasizes treating the underlying diagnosis rather than just managing the pressure.
51
What is the functional classification of pulmonary hypertension according to the WHO?
The WHO functional classification includes: * Class I: No limitation of physical activity * Class II: Slight limitation of physical activity * Class III: Marked limitation of physical activity * Class IV: Inability to carry out any physical activity without symptoms
52
What are the symptoms of Class I pulmonary hypertension in adults?
Without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain or near syncope.
53
What symptoms characterize Class IV pulmonary hypertension?
Inability to carry out any physical activity without symptoms. Signs of right heart failure may be present.
54
What is the classification for pediatric functional status in children aged 0-0.5 years?
The classification includes: * Class I: Asymptomatic, growing normally * Class II: Slight limitation of physical activity * Class IIIa: Marked limitation of physical activity * Class IIIb: Growth severely compromised * Class IV: Unable to carry out any physical activity without undue symptoms
55
What defines low risk in the prognosis of pulmonary hypertension?
Low risk is defined as: * Estimated 1-yr mortality <5% * Clinical signs of right heart failure absent * No progression of symptoms * No syncope * Normal growth
56
What defines high risk in the prognosis of pulmonary hypertension?
High risk is defined as: * Estimated 1-yr mortality >10% * Clinical signs of right heart failure present * Rapid progression of symptoms * Repeated syncope * Growth failure
57
What are the determinants of prognosis in pulmonary hypertension?
Determinants include: * Clinical signs of right heart failure * Progression of symptoms * Syncope * Growth * WHO/PVRI Functional Class * 6 MWD * Cardiopulmonary exercise testing (CPET) * NT-proBNP plasma levels * Imaging results * Hemodynamics
58
What is the classification of pulmonary hypertension based on etiology?
Classification includes: * Pulmonary Arterial Hypertension (PAH) * Pulmonary hypertension due to left heart disease (PH-LHD) * Pulmonary hypertension due to lung diseases and/or hypoxia * Chronic thromboembolic pulmonary hypertension (CTEPH) * Pulmonary hypertension with unclear and/or multifactorial mechanisms
59
What types of pulmonary arterial hypertension (PAH) are included in the classification?
Types of PAH include: * Idiopathic * Heritable * Drugs and Toxins - Induced * Associated with connective tissue disease, HIV infection, portal hypertension, congenital heart disease, schistosomiasis * Long-term responders to calcium channel blockers * PAH with overt features of venous/capillaries involvement
60
What are the risk factors for developing pulmonary hypertension?
Risk factors include: * Presence of pulmonary arterial hypertension (PAH) * Left to right shunts * Congenital heart defects * Chronic thromboembolic pulmonary hypertension (CTEPH) * Lung diseases and hypoxia
61
What is the mean pulmonary artery pressure threshold for diagnosing pulmonary hypertension?
>20 mmHg
62
What are the clinical signs and symptoms related to right ventricular dysfunction?
* Peripheral edema * Anorexia, nausea, and abdominal pain * Fatigue, dyspnea
63
What is the classification of pulmonary hypertension based on etiology?
5 classifications (6th WSPH Update, Nice, France)
64
What is the significance of TRV in echocardiography for pulmonary hypertension screening?
TRV >2.8 m/s indicates mPAP <27.23 mmHg; TRV 2.9–3.4 m/s indicates intermediate probability
65
What is the role of right heart catheterization (RHC) in pulmonary arterial hypertension?
To confirm PAH in high or intermediate probability cases
66
What are the parameters for confirming pulmonary arterial hypertension (PAH)?
* mPAP >20 mmHg * PAWP <15 mmHg * PVR >3 Wood units
67
What is the function of vasoreactivity testing in PAH diagnosis?
To assess acute vasodilator response in PAH patients
68
What are the recommended treatments for pulmonary arterial hypertension?
* General measures * Supportive therapy * Specific drug therapy
69
What is the recommended initial dose of Nifedipine for treating PAH?
0.2-0.5 mg/kg/day
70
What is the maximum daily dose of Diltiazem for treating PAH?
3.5-6 mg/kg/day
71
What are the characteristics of class I pulmonary hypertension according to WHO functional classification?
Without resulting limitation of physical activity; ordinary physical activity does not cause undue dyspnea
72
Fill in the blank: The probability of pulmonary hypertension is indicated by mean PA Pressure greater than _______.
20 mmHg
73
What is the significance of pulmonary vascular resistance (PVR) in PAH diagnosis?
PVR >3 Wood units indicates PAH
74
True or False: The use of PAH-approved therapies is recommended in PH-LHD.
False
75
What is the treatment approach for chronic lung disease presenting as a 'PAH' phenotype?
* Long-term O2 administration * Treatment of underlying lung disease
76
What is the recommended treatment for chronic thromboembolic pulmonary hypertension (CTEPH)?
* Pulmonary endarterectomy (PEA) * Balloon dilatation of PA * Lifelong anticoagulation
77
What is the role of a ventilation/perfusion scan (V/Q scan) in screening for CTEPH?
To identify mismatched defects indicative of CTEPH
78
What are the symptoms insufficiently explained by lung mechanical disturbances in chronic lung diseases?
Symptoms related to RV dysfunction in the context of chronic lung disease
79
What are non-pharmacological supportive treatments recommended for PAH patients?
* Physical activity and rehabilitation * Infection prevention * Psychosocial support
80
What is the initial treatment recommendation for patients with pulmonary hypertension due to lung disease?
Long-term O2 administration
81
What should be done if a patient presents with cyanosis that improves with low oxygen supplementation?
Consider PPHN (Persistent Pulmonary Hypertension of the Newborn)
82
What is the maximum dose for inhaled Iloprost in PAH treatment?
45 µg/day (inhaled)
83
What is the key principle in treating pulmonary hypertension?
‘TREAT THE LUNG NOT THE PRESSURE' ## Footnote This emphasizes focusing on the underlying lung condition rather than just managing pressure levels.
84
Are there any randomized controlled trials (RCTs) on PAH-approved drugs for treating group 5 disorders?
No RCTs exist ## Footnote This highlights a gap in research for the treatment of specific pulmonary hypertension disorders.
85
What defines WHO Functional Class I of pulmonary hypertension?
Without resulting limitation of physical activity ## Footnote Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain or near syncope.
86
What characterizes WHO Functional Class II of pulmonary hypertension?
Slight limitation of physical activity ## Footnote Comfortable at rest, but ordinary physical activity causes undue dyspnea or fatigue.
87
What are the symptoms of WHO Functional Class III pulmonary hypertension?
Marked limitation of physical activity ## Footnote Comfortable at rest, but less than ordinary activity causes undue dyspnea or fatigue.
88
What defines WHO Functional Class IV of pulmonary hypertension?
Inability to carry out any physical activity without symptoms ## Footnote Patients may show signs of right heart failure and symptoms may be present at rest.
89
What is the pediatric classification for children aged 0-0.5 years in Class I?
Asymptomatic, growing and developing normally ## Footnote No limitation of physical activity and gains head control.
90
What characterizes Class IIIa in pediatric functional classification for children aged 0-0.5 years?
Marked limitation of physical activity ## Footnote Regression of learned physical activities and requires excessive medical attention.
91
In pediatric functional classification for children aged >0.5–1 year, what is Class II?
Slight limitation of physical activity ## Footnote Unduly dyspneic and fatigued when playing, but comfortable at rest.
92
What describes Class IV for children aged >2-5 years?
Unable to carry out any physical activity without undue symptoms ## Footnote Includes severe limitations and inability to attend school.
93
What are the determinants of prognosis in pulmonary hypertension?
Low risk: <5%, Intermediate risk: 5-10%, High risk: >10% ## Footnote These categories are based on clinical signs, symptom progression, and functional class.
94
What is the significance of NT-proBNP plasma levels in prognosis?
BNP <50 ng/l is low risk; BNP >300 ng/l is high risk ## Footnote Levels indicate the severity of pulmonary hypertension.
95
What is included in the classification of pulmonary hypertension based on etiology?
1. Pulmonary Arterial Hypertension (PAH) 2. Pulmonary hypertension due to left heart disease (PH-LHD) 3. Pulmonary hypertension due to lung diseases and/or hypoxia 4. Chronic thromboembolic pulmonary hypertension 5. Pulmonary hypertension with unclear mechanisms ## Footnote Each category has specific subtypes and risk factors.
96
What defines idiopathic pulmonary arterial hypertension?
No known cause for the condition ## Footnote It is classified under Pulmonary Arterial Hypertension.
97
What is the characteristic of pulmonary hypertension due to left heart disease?
Caused by heart failure with preserved or reduced LVEF ## Footnote Includes valvular heart disease and other cardiovascular conditions.
98
What is the definition of chronic thromboembolic pulmonary hypertension (CTEPH)?
Pulmonary hypertension caused by chronic obstruction of pulmonary arteries due to thromboembolic events ## Footnote It affects blood flow and increases pressure in the pulmonary arteries.
99
What are the risk factors for developing pulmonary hypertension?
1. Connective Tissue Disease 2. HIV Infection 3. Portal Hypertension 4. Congenital Heart Disease 5. Schistosomiasis ## Footnote These conditions can lead to pulmonary arterial hypertension.
100
What is the significance of the 6-minute walk distance (6 MWD) in assessing pulmonary hypertension prognosis?
Distance greater than 440 m indicates low risk ## Footnote Distances below certain thresholds correlate with higher mortality risk.
101
What is the outcome of having a peak VO2 <11 ml/min/kg during cardiopulmonary exercise testing?
Indicates high risk for patients ## Footnote This metric is used to assess exercise capacity and prognosis.

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