What are the symptoms and treatment options for pulmonary hypertension?

Symptoms of Pulmonary Hypertension

Pulmonary hypertension (PH) presents with non-specific symptoms primarily related to progressive right ventricular dysfunction, with exertional dyspnea being the most common initial symptom. 1

Common Symptoms

• Early/Initial Symptoms (typically exertion-induced):

  • Shortness of breath (dyspnea on exertion)
  • Fatigue
  • Weakness
  • Angina (chest pain)
  • Syncope (fainting)

• Less Common Initial Symptoms:

  • Dry cough
  • Exercise-induced nausea and vomiting

• Advanced Disease Symptoms (occur at rest):

  • Abdominal distension
  • Ankle edema
  • Signs of right ventricular failure

Mechanical Complications and Related Symptoms

Some patients may experience symptoms related to mechanical complications of PH:

• Hemoptysis (coughing up blood) - due to rupture of hypertrophied bronchial arteries
• Hoarseness - from compression of the left recurrent laryngeal nerve by dilated pulmonary arteries
• Wheeze - from large airway compression
• Angina - due to myocardial ischemia from compression of the left main coronary artery
• Signs of cardiac tamponade - if significant pulmonary artery dilation leads to rupture or dissection 1

Physical Examination Findings

Key physical signs that may be present in PH include:

• Left parasternal lift
• Accentuated pulmonary component of the second heart sound (P2)
• Right ventricular third heart sound
• Pansystolic murmur of tricuspid regurgitation
• Diastolic murmur of pulmonary regurgitation

In advanced disease:

• Elevated jugular venous pressure
• Hepatomegaly
• Ascites
• Peripheral edema
• Cool extremities 1

Notably, wheeze and crackles are usually absent in primary pulmonary hypertension, which can help distinguish it from other respiratory conditions 1.

Diagnostic Approach

When PH is suspected based on symptoms:

1. Initial Evaluation: Echocardiography is the first-line non-invasive test 2

2. Further Testing:

   • ECG - may show right ventricular hypertrophy, right axis deviation, and right atrial enlargement
   • Chest radiograph - may reveal central pulmonary arterial dilation with "pruning" of peripheral vessels
   • Pulmonary function tests with DLCO
   • Arterial blood gases

3. Definitive Diagnosis: Right heart catheterization (gold standard) - confirms PH when mean pulmonary artery pressure is ≥25 mmHg at rest 1, 2

Functional Classification

The World Health Organization functional classification helps assess symptom severity:

• Class I: No limitation of physical activity
• Class II: Slight limitation of physical activity; comfortable at rest
• Class III: Marked limitation of physical activity; comfortable at rest
• Class IV: Unable to perform any physical activity without symptoms; signs of right heart failure may be present at rest 1

Important Clinical Considerations

• Symptoms of PH may be modified by underlying or associated diseases
• PH should be considered in any patient with unexplained dyspnea on exertion, syncope, or signs of right ventricular dysfunction 3
• A normal ECG does not exclude the presence of severe PH 1
• Early recognition is crucial as delayed diagnosis can lead to progressive right heart failure and death 4

Remember that symptoms of PH are non-specific and can mimic many other cardiopulmonary conditions, making diagnosis challenging. Any patient with unexplained dyspnea, especially on exertion, should be evaluated for possible PH, particularly if they have risk factors or associated conditions.