Treatment of Underlying Etiology

• Anticoagulation or vena caval interruption are effective for thromboembolic disease.
• CPAP or tracheostomy can be used for obstructive sleep apnea, and correction of cardiac abnormalities is effective if the pulmonary vascular resistance is not substantially elevated.

Afterload Reduction

• Medical therapy is aimed primarily at decreasing right sided afterload. This can be done most effectively with oxygen via reversal of hypoxic pulmonary vasoconstriction. Indeed, 2 large studies of patients with chronic hypoxia (SaO2<90%) and cor pulmonale secondary to COPD demonstrated marked survival benefits with long term oxygen therapy.
• Vasodilating agents used in left heart failure are generally ineffective at decreasing right sided afterload as systemic hypotension is usually encountered before significant decreases in pulmonary vascular resistance are achieved. One clear exception to this is inhaled nitric oxide. The rapid metabolism of this drug prevents systemic vasodilation and hypotension. Furthermore its distribution solely to ventilated regions prevents the worsened hypoxemia due to impaired V/Q mismatching encountered with systemic vasodilators. Investigation in this field is active.

Heart Failure

• Peripheral edema and ascites due to R heart failure can be treated with diuretics. This must be done with care as decreased right ventricular filling can be associated with a catastrophic fall in cardiac output.
• Digoxin is not effective for treating R heart failure.

Lung Transplantation

• Lung transplantation should be considered for patients with end stage COPD, fibrotic lung diseases and primary pulmonary hypertension who have severe symptoms despite maximal medical therapy. Dramatic reversals in pulmonary hypertension and clinical symptoms are seen with this procedure, however, it carries the side effects of long term immunosuppression.