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Phenotype Submission - Pulmonary hypertension associated with left heart disease (WHO Group 2)

Cohort Definition Name: “Pulmonary hypertension associated with left heart disease (WHO Group 2)”
Contributor name: Joel N. Swerdel
Contributor OrcId: 0000-0001-9491-2737
Logic Description: First occurrence of Pulmonary hypertension associated with left heart disease (WHO Group 2).
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 2.txt (145.0 KB)

Target Clinical Description

Group 2 Pulmonary Hypertension refers to a specific classification of pulmonary hypertension characterized by increased blood pressure in the pulmonary arteries due to left heart disease. It is primarily caused by conditions that affect the left side of the heart, such as left ventricular dysfunction, valvular heart disease, or heart failure. The elevated pressure in the pulmonary arteries is a result of increased back pressure from the left side of the heart, leading to the remodeling and narrowing of the pulmonary blood vessels. Group 2 Pulmonary Hypertension can cause symptoms and complications related to both the underlying heart condition and the pulmonary hypertension itself.

Presentation for Group 2 Pulmonary Hypertension:

  • Shortness of breath during physical activity or at rest

  • Fatigue and reduced exercise tolerance

  • Swelling in the ankles, legs, or abdomen

  • Persistent cough

  • Chest pain or pressure

  • Rapid or irregular heartbeat

  • Bluish lips or skin (cyanosis) in advanced stages

  • Signs and symptoms of the underlying left heart disease, such as heart failure symptoms (e.g., fluid retention, difficulty breathing when lying flat)

Assessment for Group 2 Pulmonary Hypertension:

  • Echocardiogram: A non-invasive imaging test to assess the structure and function of the heart, including the measurement of pulmonary artery pressures.

  • Cardiac catheterization: A diagnostic procedure involving the insertion of a catheter into the heart to directly measure pulmonary artery pressures and assess the severity of pulmonary hypertension.

  • Evaluation of underlying heart conditions: Comprehensive assessment of the left side of the heart, including a thorough medical history, physical examination, electrocardiogram (ECG), and other tests (e.g., stress test, cardiac imaging) to identify the underlying cause.

Differential diagnoses for Group 2 Pulmonary Hypertension:

  • Group 1 Pulmonary Hypertension (pulmonary arterial hypertension): Pulmonary hypertension caused by abnormalities in the pulmonary arteries themselves, rather than left heart disease.

  • Group 3 Pulmonary Hypertension: Pulmonary hypertension associated with lung diseases, such as chronic obstructive pulmonary disease (COPD) or interstitial lung disease.

  • Group 4 Pulmonary Hypertension: Pulmonary hypertension resulting from chronic blood clots in the lungs (chronic thromboembolic pulmonary hypertension).

  • Group 5 Pulmonary Hypertension: Pulmonary hypertension associated with various other conditions such as blood disorders, systemic diseases, or metabolic disorders.

Treatment plan for Group 2 Pulmonary Hypertension:

  • Management of the underlying left heart disease: Treating the underlying cause, such as optimizing heart failure management, controlling blood pressure, or repairing/replacing heart valves if necessary.

  • Medications: Depending on the severity and specific characteristics of pulmonary hypertension, medications used in the treatment of pulmonary arterial hypertension (Group 1) may be considered, including calcium channel blockers, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, or prostacyclin analogs. However, caution must be exercised in the use of these medications in Group 2 Pulmonary Hypertension.

  • Lifestyle modifications: Adopting a heart-healthy lifestyle, including regular exercise, maintaining a healthy weight, following a balanced diet, quitting smoking, and managing underlying conditions such as diabetes or high blood pressure.

Prognosis for Group 2 Pulmonary Hypertension:

The prognosis for Group 2 Pulmonary Hypertension depends on various factors, including the severity of the underlying heart disease, the degree of pulmonary hypertension, and the response to treatment. If the underlying left heart disease can be effectively managed, including appropriate medical therapy and lifestyle modifications, it can help alleviate symptoms and improve the overall prognosis. However, the presence of pulmonary hypertension indicates an increased risk for complications and mortality. Regular follow-up with a healthcare team specializing in pulmonary hypertension is essential to monitor the condition, adjust treatment as needed, and optimize long-term outcomes.

imported id 750

Imported to the OHDSI Phenotype Library. It may be expected to be found with id = 771 in the next release. Thank you ‘Joel Swerdel’,‘Eva-maria Didden’;

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