Clinical Case: Man With COPD and Worsening Shortness of Breath

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Long-term oxygen therapy has been shown to reduce the progression of PH in patients with COPD.
Long-term oxygen therapy has been shown to reduce the progression of PH in patients with COPD.

A 65-year-old man with a 60-pack-year history of cigarette smoking presents with worsening exertional dyspnea. Pulmonary function testing showed significant restrictive lung disease that was thought to be secondary to the patient's chronic obstructive pulmonary disease (COPD). Two-dimensional echocardiography revealed a mean pulmonary artery pressure (PAP) of 53 mm Hg with normal right ventricular size and function. Right heart catheterization at the time of diagnosis confirmed the elevated mean PAP with a capillary wedge pressure of 12 mm Hg and peripheral vascular resistance of 7 wood units. 

The study was positive for vasodilator response with a drop in mean PAP to 40 mm Hg. Cardiac output remained stable. The patient was started on long-acting nifedipine. Ventilation perfusion scan showed no mismatched perfusion defects. Complete metabolic profile and complete blood count were normal, and screening for HIV was negative. High-resolution CT of the lungs showed evidence of pulmonary fibrosis.

The patient was given a diagnosis of concomitant idiopathic pulmonary fibrosis and COPD. Initially, he appeared to do well with the nifedipine with improved symptoms but presented back for evaluation 2 months later with worsening shortness of breath with minimal activity. He feels well at rest but gets markedly short of breath when washing dishes or walking to the bathroom. He is medically optimized for his COPD, with multiple inhaled medications and inhaled corticosteroids.

Physical exam revealed 1+ bilateral lower extremity edema and hepatomegaly. Cardiovascular exam revealed a right ventricular heave, jugular venous distention to his jaw, and lungs that are clear to auscultation. Follow-up labs revealed a N-terminal pro-B-type natriuretic peptide level of 500 pg/mL, and a repeat 2-dimensional echo revealed mild right atrial dilatation, trace pericardial effusion, and a dilated inferior vena cava with partial collapse. Left ventricular function remained reserved

Right ventricular systolic pressure is calculated at 55 mm Hg. The patients is sent for a repeat right heart catheterization, which confirms the elevated mean PAP of 50 mm Hg. His cardiac index is calculated to be 2.0 L/minute/m2. According to the World Health Organization classification for pulmonary hypertension, he is at functional class (WHO-FC) III. Finally, he is referred for cardiopulmonary testing and is found to have a peak VO2 of 14 mL/minute/kg.

What Is the Next Best Step in Management? 

A. Change nifedipine to verapamil

B. Continue nifedipine and start ambrisentan and tadalafil

C. Continue nifedipine and start furosemide, ambrisentan, and tadalafil

D. Stop nifedipine and start furosemide and home oxygen and refer to expert pulmonary hypertension center

E. Stop nifedipine and start ambrisentan and tadalafil

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