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New combined treatments avoided transplantation in a child with severe pulmonary hypertension
  1. M Beghetti,
  2. L Nicod,
  3. C Barazzone-Argiroffo,
  4. P C Rimensberger

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Therapeutic options for the management of pulmonary hypertension have been limited for many years. However, recent progresses in the understanding of its pathology have led to the introduction of new treatments that have changed its prognosis, such as the continuous infusion of prostacyclin. Several other treatments (oral, inhaled, or subcutaneous prostacyclin, phosphodiesterase inhibitors, and endothelin receptor blockers) have shown beneficial effects in recent trials. An 8 year old boy with severe primary pulmonary hypertension showed a dramatic improvement following combined treatment with inhaled iloprost (a prostacylin analogue) and oral bosentan (a dual endothelin receptor blocker). In the six minute walk test he increased the distance from 295 m before bosentan to 400 m after three months of combined treatment. Dramatic changes were observed at echocardiography. Echocardiographic short axis views were taken at the same time in the cardiac cycle before combined treatment (panel A) and three months after combined treatment of iloprost and bosentan (panel B). There was a significant reduction in right ventricular (RV) size and changes in interventricular septal curvature (IVS), which went from flat before treatment (panel A) to normal left to right on combined treatment (panel B). Echocardiography showed a decrease in right ventricular surface, from 39 cm2 to 34 cm2. The left ventricle (LV) is squeezed by the dilated right ventricle before (panel A) and is almost normal after three months (panel B). This allowed the lung transplantation to be postponed and improved the patient’s quality of life.

This case shows that combined therapies may offer further improvement in the therapeutic options for the treatment of pulmonary hypertension. Appropriate clinical trials should be performed to confirm this beneficial result.

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