Pulmonary Hypertension (PPT) – Dr. Akif Baig

Pulmonary Hypertension (PH): An Overview

Pulmonary Hypertension (PH) is a condition characterized by elevated blood pressure in the pulmonary arteries, which carry blood from the heart to the lungs. It results in increased resistance to blood flow through the lungs, leading to an overload on the right side of the heart. Over time, this can lead to right heart failure and significant complications if not properly managed.

Types of Pulmonary Hypertension:

Pulmonary Hypertension is classified into five groups based on the underlying cause:

1. Group 1: Pulmonary Arterial Hypertension (PAH)
   • Idiopathic, heritable, or associated with conditions like connective tissue diseases, congenital heart disease, or drug/toxin exposure.
2. Group 2: PH due to Left Heart Disease
   • Commonly caused by left-sided heart conditions such as left ventricular dysfunction, mitral or aortic valve disease.
3. Group 3: PH due to Lung Disease and/or Hypoxia
   • Caused by chronic lung diseases like COPD, interstitial lung disease, or sleep-disordered breathing.
4. Group 4: Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
   • Due to chronic blood clots in the lungs that obstruct blood flow.
5. Group 5: PH with Unclear or Multifactorial Mechanisms
   • Includes conditions like sarcoidosis or metabolic disorders.

Symptoms:

• Dyspnea (shortness of breath), especially during exertion
• Fatigue
• Chest pain
• Palpitations
• Edema (swelling) in the legs and abdomen
• Syncope (fainting) in advanced cases

Diagnosis:

• Echocardiography: Initial screening tool to assess pulmonary artery pressure and heart function.
• Right Heart Catheterization: Gold standard for diagnosing PH by measuring pulmonary artery pressures directly.
• Chest X-ray/CT Scan: To evaluate lung structure and rule out other causes.
• Pulmonary Function Tests: To assess lung disease.
• Ventilation-Perfusion (V/Q) Scan: To detect chronic blood clots in the lungs (CTEPH).

Treatment:

The treatment for pulmonary hypertension depends on its type and underlying cause:

1. Medications for Pulmonary Arterial Hypertension (PAH):
   • Endothelin Receptor Antagonists (ERAs): Bosentan, Ambrisentan
   • Phosphodiesterase-5 Inhibitors (PDE-5i): Sildenafil, Tadalafil
   • Prostacyclin Analogues: Epoprostenol, Treprostinil
   • Soluble Guanylate Cyclase Stimulators: Riociguat
   • Calcium Channel Blockers: For vasoreactive patients
2. Management of Group 2 PH:
   • Focus on treating underlying left heart disease (e.g., diuretics for heart failure).
3. Management of Group 3 PH:
   • Treat underlying lung diseases and provide oxygen therapy if hypoxia is present.
4. Chronic Thromboembolic PH (CTEPH):
   • Anticoagulation therapy is crucial.
   • Surgical treatment such as pulmonary thromboendarterectomy or balloon pulmonary angioplasty may be indicated.
5. General Measures:
   • Oxygen therapy for hypoxic patients.
   • Diuretics to manage fluid overload.
   • Anticoagulation in select patients (e.g., CTEPH).

Prognosis:

Pulmonary Hypertension is a progressive disease, and prognosis varies based on the underlying cause and how early it is detected and treated. With advanced therapies for PAH and better management strategies for other forms, survival rates have improved significantly.

Summary Table: PH Management

Early detection and specialized management are critical to improving outcomes for patients with pulmonary hypertension.