Pulmonary Arterial Hypertension

Pt with h/o congenital heart disease/failure, OSA, COPD, PE, DVT, systemic sclerosis, HIV, and schistosomiasis presents with dyspnea on exertion and fatigue. Reports recent angina, syncope. SPO2 < 90%, JVD, LE edema on exam.

  • Obtain CBC, BMP, BNP, TSH

  • EKG shows R ventricular enlargement, right bundle branch block, and S1Q3T3 pattern suggestive of PE

  • Echocardiogram indicates pulmonary arterial pressure > 25 mmHg

  • Refer for pulmonary function testing, sleep apnea testing

  • Pulmonary arterial pressure > 35 mmHg: Refer to cardiology for possible R heart catheterization

  • Treatment

    • Vaccination

      • Administer influenza vaccine, PPSV23

      • > 65 y/o: Administer PCV13 followed by PPSV23 in 6 months to 1 year

    • No h/o COPD: Start nifedipine ER 60 mg daily

    • Resting SPO2 < 88% and/or PaO2 < 60 mmHg: Start oxygen therapy

    • Etiology-specific

      • Optimize HFpEF and obstructive lung disease regimens

      • Chronic pulmonary thromboembolic disease: Consider lifelong anticoagulation and/or pulmonary endarterectomy

  • Referral

    • Condition complicated by heart failure: Refer to cardiology

    • Refer to pulmonology based on right heart catheterization results

  • Pt counseled that guidelines advise against pregnancy and recommend long-active reversible contraception

Notes

  • Normal pulmonary artery pressure = 25 mmHg

  • Potential etiologies

    • Group 1

      • Includes congenital conditions, connective tissue disease, iatrogenic

      • Specific risk factors: HIV, systemic sclerosis, congenital heart disease, and schistosomiasis

    • Group 2: Chronic heart failure (left heart disease)

    • Group 3: Obstructive or interstitial lung disease

      • Vasodilators (e.g. nifedipine, sildenafil, bosentan) create ventilation-perfusion mismatch and can worsen symptoms

      • Start supplemental oxygen when PaO2 < 60 mmHg

    • Group 4: Chronic pulmonary thromboemboli (endarterectomy may be indicated)

    • Group 5: Multifactorial, e.g. sickle cell disease

  • Most common cause of death is right heart failure