Pulmonary Circulatory Disorders & Pleural Disorders
Pulmonary Artery Hypertension (PAH) - ANS-A condition in which pulmonary vessels and often
other lung tissues undergo growth changes that greatly increase pressure in the lung circulatory
system for unknown reasons (also known as idiopathic pulmonary artery hypertension). *Mean
artery pressure ≥ 25 mmHg at rest, considered severe if mPAP ≥ 35 mmHg at rest.* It can be
early missed and can be delayed in diagnosis. Symptoms are more common in other pulmonary
disorders such as asthma or COPD. High mortality rate if left untreated. Most often due to
uncompensated right heart failure. *WHO classification: Group 1 = pulmonary artery
hypertension (PAH) - 2/2 narrowing or the lumen or thickening of the wall of pulmonary arteries.
Group 2 = PH secondary to left heart disease. Group 3 = PH secondary to lung disease and/or
hypoxia. Group 4 = chronic thromboembolic PH. Group 5 = PH secondary to other diseases or
conditions.*
A mean pulmonary artery pressure ≥ 25 mmHg at rest, considered severe if mPAP ≥ 35 mmHg
at rest. - ANS-What is the definition of pulmonary artery hypertension?
• *Group 1* = pulmonary artery hypertension (PAH) - 2/2 narrowing or the lumen or thickening of
the wall of pulmonary arteries.
• *Group 2* = PH secondary to left heart disease.
• *Group 3* = PH secondary to lung disease and/or hypoxia.
• *Group 4* = chronic thromboembolic PH.
• *Group 5* = PH secondary to other diseases or conditions. - ANS-What is the WHO
classification of pulmonary artery hypertension? Groups 1-5?
Group 1 Pulmonary Hypertension - ANS-Pulmonary arterial hypertension due to idiopathic and
heritable causes, HIV, portal hypertension, drug or toxin induced, or congenital heart disease
(ASD, PDA, VSD --> Eisenmenger syndrome). May be due to connective tissue diseases
(systemic sclerosis, Raynaud's disease, SLE, RA). Definite risk if on appetite suppressants
(aminorex, fenfluramine, dexfenfluramine, and diethylpropion) or rapeseed oil. Possible risk if on
cocaine, methamphetamines, amphetamines, leflunomide, phentermine, mazindol, dasatinib, or
interferon therapy for hepatitis.
Group 2 Pulmonary Hypertension - ANS-Pulmonary hypertension secondary to left heart
disease. Associated with increased pressure in the left atrium (> 14 mmHg) - leads to
pulmonary venous hypertension secondary to chronic congestion. Examples: left ventricle
systolic or diastolic dysfunction, mitral and aortic valve disorders, congenital deformities of
inflow/outflow tracts, cardiomyopathies, left atrial myxoma.
Group 3 Pulmonary Hypertension - ANS-Pulmonary hypertension secondary to lung disease.
Due to disorders like COPD, interstitial lung disease (ILD), obstructive sleep apnea (OSA), etc.
Hypoxia leads to vasoconstriction which increases pressure.
, Group 4 Pulmonary Hypertension - ANS-Pulmonary hypertension due to chronic
thromboembolism. May be a connection to underlying hypercoagulable states. Likely to occur if
previous thromboembolic event.
Group 5 Pulmonary Hypertension - ANS-Pulmonary hypertension due to unclear or multifactorial
mechanisms. May be linked to chronic hemolytic anemia (sickle cell disease, beta-thalassemia,
or spherocytosis), myeloproliferative disorders, systemic disorders (sarcoidosis, histiocytosis),
metabolic disorders, chronic kidney disease.
• Difficult to recognize, likely will be some underlying comorbidities that can cloud the diagnostic
picture
• Initial symptoms: exertional dyspnea, lethargy, fatigue, exertional chest pain, exertional
syncope, peripheral edema, anorexia/abdominal pain, rarely cough, hemoptysis, or hoarseness
• PE: increased intensity of second heart sound. Jugular venous distension (A wave in JVP),
may hear holosystolic murmur (tricuspid regurgitation) and in severe PH hear a diastolic murmur
(pulmonary regurgitation).
• CXR: enlargement of pulmonary arteries and R. ventricle
• EKG: RVH, right axis deviation, R wave to S wave ratio greater than 1 on lead V1, increased P
wave amplitude in lead II (P pulmonale).
• Right heart catheterization for definitive dx: mPAP ≥ 25 mmHg at rest (8-20 mmHg is normal). -
ANS-What is the clinical presentation of pulmonary artery hypertension?
• What are some initial symptoms?
• What is seen on PE?
• What is seen on CXR?
• What is seen on EKG?
• What is required for definitive diagnosis?
• Increased intensity of second heart sound (P2)
• Jugular venous distension (A wave in JVP)
• May hear holosystolic murmur (tricuspid regurgitation) and in severe PH hear a diastolic
murmur (pulmonary regurgitation). - ANS-What is observed on physical exam in patient with
pulmonary HTN?
• Right ventricular hypertrophy
• Right axis deviation
• *R wave to S wave ratio greater than 1 on lead V1, increased P wave amplitude in lead II (P
pulmonale).* - ANS-What is observed on EKG in patient with pulmonary HTN?
• *Class I* = Patients without limitation of physical activity. Ordinary physical activity does not
cause undue dyspnea or fatigue, chest pain, or near syncope.
• *Class II* = Patients with slight limitation of physical activity. They are comfortable at rest.
Ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near syncope.
Pulmonary Artery Hypertension (PAH) - ANS-A condition in which pulmonary vessels and often
other lung tissues undergo growth changes that greatly increase pressure in the lung circulatory
system for unknown reasons (also known as idiopathic pulmonary artery hypertension). *Mean
artery pressure ≥ 25 mmHg at rest, considered severe if mPAP ≥ 35 mmHg at rest.* It can be
early missed and can be delayed in diagnosis. Symptoms are more common in other pulmonary
disorders such as asthma or COPD. High mortality rate if left untreated. Most often due to
uncompensated right heart failure. *WHO classification: Group 1 = pulmonary artery
hypertension (PAH) - 2/2 narrowing or the lumen or thickening of the wall of pulmonary arteries.
Group 2 = PH secondary to left heart disease. Group 3 = PH secondary to lung disease and/or
hypoxia. Group 4 = chronic thromboembolic PH. Group 5 = PH secondary to other diseases or
conditions.*
A mean pulmonary artery pressure ≥ 25 mmHg at rest, considered severe if mPAP ≥ 35 mmHg
at rest. - ANS-What is the definition of pulmonary artery hypertension?
• *Group 1* = pulmonary artery hypertension (PAH) - 2/2 narrowing or the lumen or thickening of
the wall of pulmonary arteries.
• *Group 2* = PH secondary to left heart disease.
• *Group 3* = PH secondary to lung disease and/or hypoxia.
• *Group 4* = chronic thromboembolic PH.
• *Group 5* = PH secondary to other diseases or conditions. - ANS-What is the WHO
classification of pulmonary artery hypertension? Groups 1-5?
Group 1 Pulmonary Hypertension - ANS-Pulmonary arterial hypertension due to idiopathic and
heritable causes, HIV, portal hypertension, drug or toxin induced, or congenital heart disease
(ASD, PDA, VSD --> Eisenmenger syndrome). May be due to connective tissue diseases
(systemic sclerosis, Raynaud's disease, SLE, RA). Definite risk if on appetite suppressants
(aminorex, fenfluramine, dexfenfluramine, and diethylpropion) or rapeseed oil. Possible risk if on
cocaine, methamphetamines, amphetamines, leflunomide, phentermine, mazindol, dasatinib, or
interferon therapy for hepatitis.
Group 2 Pulmonary Hypertension - ANS-Pulmonary hypertension secondary to left heart
disease. Associated with increased pressure in the left atrium (> 14 mmHg) - leads to
pulmonary venous hypertension secondary to chronic congestion. Examples: left ventricle
systolic or diastolic dysfunction, mitral and aortic valve disorders, congenital deformities of
inflow/outflow tracts, cardiomyopathies, left atrial myxoma.
Group 3 Pulmonary Hypertension - ANS-Pulmonary hypertension secondary to lung disease.
Due to disorders like COPD, interstitial lung disease (ILD), obstructive sleep apnea (OSA), etc.
Hypoxia leads to vasoconstriction which increases pressure.
, Group 4 Pulmonary Hypertension - ANS-Pulmonary hypertension due to chronic
thromboembolism. May be a connection to underlying hypercoagulable states. Likely to occur if
previous thromboembolic event.
Group 5 Pulmonary Hypertension - ANS-Pulmonary hypertension due to unclear or multifactorial
mechanisms. May be linked to chronic hemolytic anemia (sickle cell disease, beta-thalassemia,
or spherocytosis), myeloproliferative disorders, systemic disorders (sarcoidosis, histiocytosis),
metabolic disorders, chronic kidney disease.
• Difficult to recognize, likely will be some underlying comorbidities that can cloud the diagnostic
picture
• Initial symptoms: exertional dyspnea, lethargy, fatigue, exertional chest pain, exertional
syncope, peripheral edema, anorexia/abdominal pain, rarely cough, hemoptysis, or hoarseness
• PE: increased intensity of second heart sound. Jugular venous distension (A wave in JVP),
may hear holosystolic murmur (tricuspid regurgitation) and in severe PH hear a diastolic murmur
(pulmonary regurgitation).
• CXR: enlargement of pulmonary arteries and R. ventricle
• EKG: RVH, right axis deviation, R wave to S wave ratio greater than 1 on lead V1, increased P
wave amplitude in lead II (P pulmonale).
• Right heart catheterization for definitive dx: mPAP ≥ 25 mmHg at rest (8-20 mmHg is normal). -
ANS-What is the clinical presentation of pulmonary artery hypertension?
• What are some initial symptoms?
• What is seen on PE?
• What is seen on CXR?
• What is seen on EKG?
• What is required for definitive diagnosis?
• Increased intensity of second heart sound (P2)
• Jugular venous distension (A wave in JVP)
• May hear holosystolic murmur (tricuspid regurgitation) and in severe PH hear a diastolic
murmur (pulmonary regurgitation). - ANS-What is observed on physical exam in patient with
pulmonary HTN?
• Right ventricular hypertrophy
• Right axis deviation
• *R wave to S wave ratio greater than 1 on lead V1, increased P wave amplitude in lead II (P
pulmonale).* - ANS-What is observed on EKG in patient with pulmonary HTN?
• *Class I* = Patients without limitation of physical activity. Ordinary physical activity does not
cause undue dyspnea or fatigue, chest pain, or near syncope.
• *Class II* = Patients with slight limitation of physical activity. They are comfortable at rest.
Ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near syncope.
