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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
Q

What is the recommended follow-up for patients with intermediate or high probability of pulmonary hypertension?

A

Right heart catheterization (RHC)

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26
Q

What should be done if Childhood Interstitial Lung Disease is suspected?

A

Refer to Pediatric Rheumatology for further work-up and management

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27
Q

What factors are assessed to classify the severity of pulmonary hypertension?

A

Mean pulmonary artery pressure (mPAP)

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28
Q

What are the classifications of pulmonary hypertension based on etiology?

A
  • 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
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29
Q

What are the general measures recommended for PAH management?

A
  • Physical activity and rehabilitation
  • Infection prevention (vaccination)
  • Psychosocial support
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30
Q

Fill in the blank: The mean pulmonary artery pressure (mPAP) is computed based on _______.

A

[formula included in the pathway]

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31
Q

What is the definition of severe pulmonary hypertension based on mPAP?

A

> 55 mmHg

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32
Q

What treatment is not recommended in the presence of left-heart disease?

A

PAH-approved therapies

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33
Q

What is the initial treatment dose for Nifedipine in PAH?

A

0.2-0.5 mg/kg/day

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34
Q

What should be monitored regularly in patients with pulmonary hypertension?

A

Functional status and prognosis

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35
Q

True or False: Patients with heritable pulmonary arterial hypertension should have their families screened.

A

True

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36
Q

What is the treatment recommendation for pulmonary hypertension due to lung disease with hypoxia?

A
  • Long-term O2 administration
  • Treatment of underlying lung disease
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37
Q

What is the role of Right Heart Catheterization (RHC) in lung disease cases suspected of pulmonary hypertension?

A

Diagnosis by RHC is not recommended

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38
Q

What is the significance of a significant cyanosis in newborns at room air that improves with low oxygen supplementation?

A

It may indicate persistent pulmonary hypertension of the newborn (PPHN) or related conditions.

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39
Q

What is the treatment approach for CTEPH?

A
  • Pulmonary endarterectomy (PEA)
  • Balloon dilatation of PA (BPA) in non-operable cases
  • Lifelong anticoagulation
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40
Q

What are the common symptoms related to right ventricular (RV) dysfunction?

A
  • Peripheral edema
  • Anorexia, nausea, abdominal pain
  • Fatigue, dyspnea
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41
Q

What is the classification of pulmonary hypertension severity based on mPAP for mild cases?

A

21-40 mmHg

42
Q

What is the recommended dosage for Sildenafil in neonates with PPHN?

A

0.25-0.5 mg/kg/dose 3x a day

43
Q

Fill in the blank: The pulmonary vascular resistance (PVR) must be greater than ________ to classify as PAH.

A

> 3 Wood units

44
Q

What should be done for patients with suspected obstructive or restrictive ventilatory defects?

A

Pulmonary Function Test (Lung volume and DLCO)

45
Q

What is the treatment recommendation for chronic lung disease presenting as ‘PAH’ phenotype?

A

Manage as PAH in addition to management of the present lung condition

46
Q

What is the maximum dosage for Epoprostenol in PAH treatment?

A

20-40 ng/kg/min

47
Q

What is the recommended approach for diagnosing suspected pulmonary hypertension in lung disease?

A

Diagnosis by RHC is not recommended for suspected PH in lung disease

RHC stands for Right Heart Catheterization

48
Q

What should be excluded in the diagnosis of pulmonary hypertension?

A

Other potential causes should be excluded, including:
* LHD
* CTEPH

LHD stands for Left Heart Disease, and CTEPH stands for Chronic Thromboembolic Pulmonary Hypertension

49
Q

What are the treatment options for CTEPH?

A

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
Q

What is the principle behind the phrase ‘TREAT THE LUNG NOT THE PRESSURE’?

A

The principle emphasizes treating the underlying diagnosis rather than just managing the pressure.

51
Q

What is the functional classification of pulmonary hypertension according to the WHO?

A

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
Q

What are the symptoms of Class I pulmonary hypertension in adults?

A

Without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain or near syncope.

53
Q

What symptoms characterize Class IV pulmonary hypertension?

A

Inability to carry out any physical activity without symptoms. Signs of right heart failure may be present.

54
Q

What is the classification for pediatric functional status in children aged 0-0.5 years?

A

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
Q

What defines low risk in the prognosis of pulmonary hypertension?

A

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
Q

What defines high risk in the prognosis of pulmonary hypertension?

A

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
Q

What are the determinants of prognosis in pulmonary hypertension?

A

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
Q

What is the classification of pulmonary hypertension based on etiology?

A

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
Q

What types of pulmonary arterial hypertension (PAH) are included in the classification?

A

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
Q

What are the risk factors for developing pulmonary hypertension?

A

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
Q

What is the mean pulmonary artery pressure threshold for diagnosing pulmonary hypertension?

62
Q

What are the clinical signs and symptoms related to right ventricular dysfunction?

A
  • Peripheral edema
  • Anorexia, nausea, and abdominal pain
  • Fatigue, dyspnea
63
Q

What is the classification of pulmonary hypertension based on etiology?

A

5 classifications (6th WSPH Update, Nice, France)

64
Q

What is the significance of TRV in echocardiography for pulmonary hypertension screening?

A

TRV >2.8 m/s indicates mPAP <27.23 mmHg; TRV 2.9–3.4 m/s indicates intermediate probability

65
Q

What is the role of right heart catheterization (RHC) in pulmonary arterial hypertension?

A

To confirm PAH in high or intermediate probability cases

66
Q

What are the parameters for confirming pulmonary arterial hypertension (PAH)?

A
  • mPAP >20 mmHg
  • PAWP <15 mmHg
  • PVR >3 Wood units
67
Q

What is the function of vasoreactivity testing in PAH diagnosis?

A

To assess acute vasodilator response in PAH patients

68
Q

What are the recommended treatments for pulmonary arterial hypertension?

A
  • General measures
  • Supportive therapy
  • Specific drug therapy
69
Q

What is the recommended initial dose of Nifedipine for treating PAH?

A

0.2-0.5 mg/kg/day

70
Q

What is the maximum daily dose of Diltiazem for treating PAH?

A

3.5-6 mg/kg/day

71
Q

What are the characteristics of class I pulmonary hypertension according to WHO functional classification?

A

Without resulting limitation of physical activity; ordinary physical activity does not cause undue dyspnea

72
Q

Fill in the blank: The probability of pulmonary hypertension is indicated by mean PA Pressure greater than _______.

73
Q

What is the significance of pulmonary vascular resistance (PVR) in PAH diagnosis?

A

PVR >3 Wood units indicates PAH

74
Q

True or False: The use of PAH-approved therapies is recommended in PH-LHD.

75
Q

What is the treatment approach for chronic lung disease presenting as a ‘PAH’ phenotype?

A
  • Long-term O2 administration
  • Treatment of underlying lung disease
76
Q

What is the recommended treatment for chronic thromboembolic pulmonary hypertension (CTEPH)?

A
  • Pulmonary endarterectomy (PEA)
  • Balloon dilatation of PA
  • Lifelong anticoagulation
77
Q

What is the role of a ventilation/perfusion scan (V/Q scan) in screening for CTEPH?

A

To identify mismatched defects indicative of CTEPH

78
Q

What are the symptoms insufficiently explained by lung mechanical disturbances in chronic lung diseases?

A

Symptoms related to RV dysfunction in the context of chronic lung disease

79
Q

What are non-pharmacological supportive treatments recommended for PAH patients?

A
  • Physical activity and rehabilitation
  • Infection prevention
  • Psychosocial support
80
Q

What is the initial treatment recommendation for patients with pulmonary hypertension due to lung disease?

A

Long-term O2 administration

81
Q

What should be done if a patient presents with cyanosis that improves with low oxygen supplementation?

A

Consider PPHN (Persistent Pulmonary Hypertension of the Newborn)

82
Q

What is the maximum dose for inhaled Iloprost in PAH treatment?

A

45 µg/day (inhaled)

83
Q

What is the key principle in treating pulmonary hypertension?

A

‘TREAT THE LUNG NOT THE PRESSURE’

This emphasizes focusing on the underlying lung condition rather than just managing pressure levels.

84
Q

Are there any randomized controlled trials (RCTs) on PAH-approved drugs for treating group 5 disorders?

A

No RCTs exist

This highlights a gap in research for the treatment of specific pulmonary hypertension disorders.

85
Q

What defines WHO Functional Class I of pulmonary hypertension?

A

Without resulting limitation of physical activity

Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain or near syncope.

86
Q

What characterizes WHO Functional Class II of pulmonary hypertension?

A

Slight limitation of physical activity

Comfortable at rest, but ordinary physical activity causes undue dyspnea or fatigue.

87
Q

What are the symptoms of WHO Functional Class III pulmonary hypertension?

A

Marked limitation of physical activity

Comfortable at rest, but less than ordinary activity causes undue dyspnea or fatigue.

88
Q

What defines WHO Functional Class IV of pulmonary hypertension?

A

Inability to carry out any physical activity without symptoms

Patients may show signs of right heart failure and symptoms may be present at rest.

89
Q

What is the pediatric classification for children aged 0-0.5 years in Class I?

A

Asymptomatic, growing and developing normally

No limitation of physical activity and gains head control.

90
Q

What characterizes Class IIIa in pediatric functional classification for children aged 0-0.5 years?

A

Marked limitation of physical activity

Regression of learned physical activities and requires excessive medical attention.

91
Q

In pediatric functional classification for children aged >0.5–1 year, what is Class II?

A

Slight limitation of physical activity

Unduly dyspneic and fatigued when playing, but comfortable at rest.

92
Q

What describes Class IV for children aged >2-5 years?

A

Unable to carry out any physical activity without undue symptoms

Includes severe limitations and inability to attend school.

93
Q

What are the determinants of prognosis in pulmonary hypertension?

A

Low risk: <5%, Intermediate risk: 5-10%, High risk: >10%

These categories are based on clinical signs, symptom progression, and functional class.

94
Q

What is the significance of NT-proBNP plasma levels in prognosis?

A

BNP <50 ng/l is low risk; BNP >300 ng/l is high risk

Levels indicate the severity of pulmonary hypertension.

95
Q

What is included in the classification of pulmonary hypertension based on etiology?

A
  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

Each category has specific subtypes and risk factors.

96
Q

What defines idiopathic pulmonary arterial hypertension?

A

No known cause for the condition

It is classified under Pulmonary Arterial Hypertension.

97
Q

What is the characteristic of pulmonary hypertension due to left heart disease?

A

Caused by heart failure with preserved or reduced LVEF

Includes valvular heart disease and other cardiovascular conditions.

98
Q

What is the definition of chronic thromboembolic pulmonary hypertension (CTEPH)?

A

Pulmonary hypertension caused by chronic obstruction of pulmonary arteries due to thromboembolic events

It affects blood flow and increases pressure in the pulmonary arteries.

99
Q

What are the risk factors for developing pulmonary hypertension?

A
  1. Connective Tissue Disease
  2. HIV Infection
  3. Portal Hypertension
  4. Congenital Heart Disease
  5. Schistosomiasis

These conditions can lead to pulmonary arterial hypertension.

100
Q

What is the significance of the 6-minute walk distance (6 MWD) in assessing pulmonary hypertension prognosis?

A

Distance greater than 440 m indicates low risk

Distances below certain thresholds correlate with higher mortality risk.

101
Q

What is the outcome of having a peak VO2 <11 ml/min/kg during cardiopulmonary exercise testing?

A

Indicates high risk for patients

This metric is used to assess exercise capacity and prognosis.

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