Pulmonary Hypertension (PH)

The high-yield summary of Pulmonary Hypertension (PH) can be divided into five main types, each with unique causes, pathophysiology, and treatment approaches.

Pulmonary Arterial Hypertension (PAH) - Group 1:

Key Points:

• Causes: Idiopathic, heritable, drugs and toxins, associated conditions (e.g., connective tissue diseases, HIV).

• Pathophysiology: Narrowing/stiffening of pulmonary arteries, increased right heart workload.

• Treatment: Endothelin receptor antagonists, phosphodiesterase-5 inhibitors, prostacyclin analogs.

Pulmonary Hypertension due to Left Heart Disease - Group 2:

Key Points:

• Causes: Left ventricular dysfunction, valvular heart diseases.

• Pathophysiology: Increased left atrial pressure leads to increased pulmonary pressure.

• Treatment: Management of underlying heart disease, heart failure medications, and surgical interventions.

Pulmonary Hypertension due to Lung Diseases/Hypoxia - Group 3:

Key Points:

• Causes: COPD, interstitial lung diseases, chronic hypoxia.

• Pathophysiology: Vasoconstriction and remodeling in pulmonary arteries due to chronic lung diseases and hypoxia.

• Treatment: Management of lung conditions, oxygen therapy, pulmonary rehabilitation.

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) - Group 4:

Key Points:

• Causes: Chronic blood clots in the lungs.

• Pathophysiology: Persistent clots increase pressure in pulmonary arteries.

• Treatment: Anticoagulation therapy, pulmonary thromboendarterectomy, PH medications in some cases.

Pulmonary Hypertension due to Unknown Multifactorial Mechanisms - Group 5:

Key Points:

• Causes: Hematologic, systemic, metabolic disorders.

• Pathophysiology: Multifactorial, not well understood.

• Treatment: Manage underlying condition, symptomatic treatment for PH.

Understanding these distinctions is critical for diagnosing and managing PH in clinical practice.