Cor Pulmonale: Drug Therapy

Therapeutic target

• Improvement of prognosis.
• Curative therapy does not exist.

Therapy recommendations

• Treatment of the underlying disease
• Reduction of pressure in the pulmonary circulation:
  • Initial therapy depending on the degree of heart failure/heart failure (NYHA): endothelin receptor antagonists (see below), PDE-5 inhibitors (see below), prostacyclin analogs (see below); note:
    • Therapy should be performed at specialized centers
    • Monotherapy should be started; in the absence of improvement, combination therapy (endothelin receptor antagonist + prostanoid/endothelin receptor antagonist + PDE-5 inhibitor/prostacyclin analog + PDE-5 inhibitor).
    • If responder in vasoreactivity testing, then calcium antagonists high dose in WHO functional class I-III.
    • Follow-up by spiroergometry (measurement of respiratory gases, at rest and under stress, so that statements about cardiac and pulmonary performance are possible), 6-min walking distance.
    • In case of right heart decompensation (cardiac insufficiency, in which there is an insufficient pumping capacity of the right ventricle) if necessary, intensive therapy with inhaled / i.v. prostacyclin analogs.
  • Main therapy: calcium antagonists only in primary pulmonary hypertension/pulmonary hypertension (test out in right heart catheterization (up to 15% responders); pulmonary pressure reduction only with high doses), prostacyclin analogs; endothelin receptor antagonists (pulmonary hypertension NYHA II, III); phosphodiesterase inhibitors (pulmonary hypertension NYHA II, III).
  • Adjunctive (“supportive”) therapy for pulmonary hypertension may include:
    • Cardiac glycosides in concomitant atrial fibrillation (VHF) with tachycardic conduction.
    • Oral anticoagulation (OAC; inhibition of blood clotting).
    • Diuretics (draining drugs) v.a. in right heart failure (right heart weakness).
    • Oxygen at a pO2 < 60 mmHg (target saturation ≥ 92%), oxygen administration (also as long-term therapy) in chronic obstructive pulmonary disease (COPD).
• Lung transplantation (LUTX; last therapeutic option) when conservative treatment options fail.

Initial therapy

NYHA classification – see under heart failure (cardiac insufficiency)/classification.