Cohort Definition Name: “Pulmonary hypertension associated with lung diseases and/or hypoxia (WHO Group 3)”
Contributor name: Joel N. Swerdel
Contributor OrcId: 0000-0001-9491-2737
Logic Description: First occurrence of Pulmonary hypertension associated with lung diseases and/or hypoxia (WHO Group 3).
Recommended study application: outcome, indication
Assertion statement: This cohort definition was executed on at least one real person-level observational health data source and resulted in a cohort with at least 1 person.
Submitted cohort definition:
PH Group 3.txt (112.5 KB)
Target Clinical Description
Group 3 Pulmonary Hypertension refers to a specific classification of pulmonary hypertension caused by lung diseases or chronic hypoxemia (low oxygen levels) that result in increased pressure in the pulmonary arteries. It is associated with conditions such as chronic obstructive pulmonary disease (COPD), interstitial lung disease (ILD), pulmonary fibrosis, sleep apnea, and other respiratory disorders. The underlying lung disease leads to inflammation, scarring, and narrowing of the pulmonary blood vessels, causing increased resistance to blood flow. This, in turn, leads to elevated blood pressure in the pulmonary arteries and potential right heart strain.
Presentation for Group 3 Pulmonary Hypertension:
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Shortness of breath, especially during physical activity
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Fatigue and reduced exercise tolerance
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Chronic cough
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Wheezing
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Chest discomfort or pain
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Cyanosis (bluish discoloration of the lips, skin, or nails) in advanced stages
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Swelling in the ankles and legs (edema)
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Symptoms related to the underlying lung disease (e.g., chronic cough, persistent respiratory infections, progressive breathlessness)
Assessment for Group 3 Pulmonary Hypertension:
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Pulmonary function tests: Assess lung function and identify any obstructive or restrictive lung disease.
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Arterial blood gas analysis: Evaluate oxygen and carbon dioxide levels in the blood.
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Imaging studies: Chest X-ray, computed tomography (CT) scan, or high-resolution CT (HRCT) scan to assess lung structure and identify underlying lung diseases.
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Echocardiogram: Assess the structure and function of the heart, including the measurement of pulmonary artery pressures.
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Sleep study: In cases associated with sleep apnea, a sleep study may be conducted to evaluate the quality of sleep and identify any breathing abnormalities during sleep.
Differential diagnoses for Group 3 Pulmonary Hypertension:
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Group 1 Pulmonary Hypertension (pulmonary arterial hypertension): Pulmonary hypertension caused by abnormalities in the pulmonary arteries themselves, rather than lung disease.
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Group 2 Pulmonary Hypertension: Pulmonary hypertension associated with left heart disease, such as heart failure or valvular heart disease.
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Group 4 Pulmonary Hypertension: Pulmonary hypertension resulting from chronic blood clots in the lungs (chronic thromboembolic pulmonary hypertension).
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Group 5 Pulmonary Hypertension: Pulmonary hypertension associated with various other conditions, such as blood disorders, systemic diseases, or metabolic disorders.
Treatment plan for Group 3 Pulmonary Hypertension:
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Treatment of the underlying lung disease: Managing the underlying lung condition is crucial in managing Group 3 Pulmonary Hypertension. This may involve medications, oxygen therapy, pulmonary rehabilitation, and lifestyle modifications.
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Medications: Depending on the severity of pulmonary hypertension and the response to treatment, specific pulmonary hypertension-targeted therapies may be considered. These may include endothelin receptor antagonists, phosphodiesterase-5 inhibitors, or prostacyclin analogs. However, their use in Group 3 Pulmonary Hypertension is still being studied, and individual assessment is required.
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Oxygen therapy: Supplemental oxygen may be prescribed if arterial blood oxygen levels are low, helping to improve oxygenation and alleviate symptoms.
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Pulmonary rehabilitation: A structured program involving exercise training, breathing techniques, and education to improve lung function, exercise capacity, and overall well-being.
Prognosis for Group 3 Pulmonary Hypertension:
The prognosis for Group 3 Pulmonary Hypertension largely depends on the underlying lung disease and its severity. In some cases, effective management of the underlying lung condition and early intervention can slow the progression of pulmonary hypertension and improve symptoms. However, the prognosis can vary, and the presence of pulmonary hypertension indicates an increased risk of complications and mortality. Regular monitoring, follow-up, and collaboration with a multidisciplinary healthcare team comprising pulmonologists, cardiologists, and other specialists are essential to optimize treatment, manage symptoms, and improve long-term outcomes.