Pharmacotherapy Case: Pulmonary Arterial Hypertension (PAH) by Dr. Nehad Ahmed

 

Pharmacotherapy Case: Pulmonary Arterial Hypertension (PAH)

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Patient Information:

• Age: 52
• Gender: Female
• Date of Visit: February 5, 2025

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Chief Complaint:

"I’ve been feeling more short of breath recently, and my legs have been swelling. I’m getting fatigued faster than usual, even doing simple things like walking to the mailbox."

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Subjective Data:

• History of Present Illness: A 52-year-old female presents with progressive dyspnea on exertion and fatigue over the past 4-5 months. She describes shortness of breath with activities that were previously easy, such as walking short distances or climbing one flight of stairs. She also reports bilateral lower extremity edema (ankle swelling) that has worsened over the past month, particularly by the end of the day. The patient denies chest pain, syncope, or dizziness, but states she feels more fatigued and less able to participate in social activities or exercise.

  She denies a history of smoking, alcohol use, or significant lung disease, but she has a history of systemic sclerosis (scleroderma) diagnosed 10 years ago, which has been stable with no significant organ involvement until now. She has also experienced intermittent episodes of Raynaud’s phenomenon. The patient notes no recent weight gain, although her legs have been swollen, especially in the evening.

• Past Medical History:

  • Systemic sclerosis (diagnosed 10 years ago)
  • Hypertension, diagnosed 5 years ago, well-controlled on medication
  • Gastroesophageal reflux disease (GERD), diagnosed 3 years ago

• Medications:

  • Losartan 50 mg daily for hypertension
  • Amlodipine 5 mg daily for hypertension
  • Omeprazole 20 mg daily for GERD
  • Aspirin 81 mg daily for cardiovascular protection

• Allergies:

  • No known drug allergies

• Family History:

  • Mother had hypertension and passed away due to complications of heart failure
  • Father had chronic obstructive pulmonary disease (COPD)

• Social History:

  • Non-smoker
  • Rare alcohol use
  • No recent travel or exposure to infectious diseases
  • Sedentary lifestyle, works as an administrative assistant in an office

• Review of Systems:

  • Respiratory: Shortness of breath with exertion, no history of cough or hemoptysis
  • Cardiovascular: Fatigue, bilateral lower extremity swelling (worse in the evenings), no chest pain or palpitations
  • Gastrointestinal: Occasional heartburn, no nausea or vomiting
  • Neurological: No dizziness or syncope, no recent headaches

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Objective Data:

• Vital Signs:

  • Temperature: 98.4°F (36.9°C)
  • Blood Pressure: 128/76 mmHg (well-controlled hypertension)
  • Heart Rate: 92 bpm (regular rhythm)
  • Respiratory Rate: 18 breaths per minute
  • Oxygen Saturation: 95% on room air

• Physical Examination:

  • General Appearance: The patient appears fatigued but not in acute distress. Mild bilateral lower extremity edema (up to the mid-calf, pitting).
  • HEENT: No signs of jugular venous distention (JVD), no cyanosis
  • Chest and Lungs:
    • Inspection: No visible signs of respiratory distress, no use of accessory muscles
    • Auscultation: Normal breath sounds with no crackles or wheezing, no signs of pleural effusion
  • Cardiovascular:
    • Jugular venous pressure (JVP): Elevated at 6 cm above the sternal angle, suggestive of increased right heart pressure
    • Regular rhythm, no murmurs or gallops
  • Abdomen: Soft, non-tender, no hepatomegaly or splenomegaly
  • Extremities: Mild bilateral pitting edema in lower extremities
  • Neurological: No focal neurological deficits

• Echocardiogram:

  • Right ventricular dilation and right atrial enlargement, suggesting right heart strain
  • Elevated pulmonary artery pressure (PAP) of 45 mmHg, consistent with pulmonary hypertension
  • Normal left ventricular ejection fraction (LVEF)

• Laboratory Results:

  • B-type natriuretic peptide (BNP): 320 pg/mL (elevated, indicative of right heart strain)
  • Complete Blood Count (CBC): Normal
  • Basic Metabolic Panel (BMP): Normal renal function, mild hypoalbuminemia
  • Liver function tests: Normal
  • Anti-centromere antibodies positive (consistent with history of systemic sclerosis)

• Right Heart Catheterization:

  • Mean Pulmonary Artery Pressure (mPAP): 45 mmHg
  • Pulmonary Capillary Wedge Pressure (PCWP): 12 mmHg (normal)
  • Cardiac Output: 4.5 L/min (normal)

• Chest X-ray:

  • Mild enlargement of the right heart border, no significant pulmonary congestion or infiltrates, clear lung fields.

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Assessment:

The patient presents with signs and symptoms consistent with pulmonary arterial hypertension (PAH) likely secondary to systemic sclerosis (a known risk factor for PAH). This diagnosis is supported by:

• Elevated pulmonary artery pressure (PAP) on echocardiogram and right heart catheterization
• Right heart dilation and strain, as evidenced by echocardiogram findings
• Elevated BNP, indicating right heart stress
• The patient’s dyspnea on exertion, fatigue, and lower extremity edema are classic symptoms of PAH.

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Plan:

1. Pharmacotherapy:

   • Start a prostacyclin analog to reduce pulmonary artery pressure:
     • Epoprostenol (Flolan) 2 ng/kg/min via IV infusion, titrated to response. Start low and adjust based on tolerability and clinical response.
   • Endothelin receptor antagonist (ERA) to reduce pulmonary vasoconstriction:
     • Bosentan 62.5 mg orally twice daily (increase to 125 mg twice daily after 4 weeks, based on tolerance).
   • Phosphodiesterase-5 inhibitor to improve exercise tolerance:
     • Sildenafil 20 mg orally three times a day.
   • Diuretics to manage edema:
     • Furosemide 20 mg orally once daily for fluid retention in the lower extremities.
   • Antiplatelet therapy:
     • Continue Aspirin 81 mg daily for cardiovascular protection, as PAH patients have increased risk of thromboembolic events.

2. Oxygen Therapy:

   • Consider home oxygen therapy if oxygen saturation decreases below 90% during exertion or at rest. Reassess oxygen saturation levels during follow-up.

3. Monitoring and Follow-up:

   • Frequent follow-up every 1-2 months to assess treatment response and adjust medications as needed.
   • Monitor for potential side effects of medications (e.g., liver function for bosentan, headache or visual disturbances for sildenafil).

4. Referral:

   • Referral to a pulmonologist and cardiologist specializing in PAH for ongoing management, including further diagnostic workup and advanced therapies if needed (e.g., lung transplantation evaluation).

5. Patient Education:

   • Educate the patient about the chronic nature of PAH and the importance of medication adherence to improve quality of life.
   • Discuss the potential risks and side effects of medications.
   • Encourage lifestyle changes, including salt restriction to help manage fluid retention and regular exercise (as tolerated) to improve cardiovascular health.

6. Vaccinations:

   • Ensure the patient is up-to-date with influenza and pneumococcal vaccinations to reduce the risk of respiratory infections.

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Discharge Instructions:

• Adhere to the prescribed medications and dosages, and monitor for any side effects.
• Keep follow-up appointments with cardiology and pulmonology.
• Report any new symptoms such as increased shortness of breath, chest pain, or dizziness immediately.
• Maintain a low-salt diet to manage fluid retention and swelling.

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Assessment Summary:

• Diagnosis: Pulmonary Arterial Hypertension (PAH), likely secondary to systemic sclerosis.