Management of failing Hickman Line for pulmonary hypertension patients on IV Epoprostenol infusion

Objectives

To provide a guideline for the management of a blocked, broken or infected central line in a paediatric patient receiving a continuous infusion of intravenous Epoprostenol for the treatment of pulmonary arterial hypertension.

Scope

This guideline is intended for all healthcare professionals involved in the care of paediatric patients with pulmonary arterial hypertension receiving a continuous infusion of Epoprostenol.

Pulmonary arterial hypertension is a relatively rare condition in the paediatric population and is associated with diverse diseases with onset at any age. It is characterized by pulmonary vascular remodelling leading to elevated pulmonary arterial pressure and, over time, right ventricular dysfunction, under filling/compression of the left ventricle, and terminal heart failure. It is defined as a mean pulmonary artery pressure of > 20 mmHg, measured by cardiac catheterisation.

Treatment for children classified in the high risk groups (WHO functional class III-IV) is a continuous infusion of intravenous Epoprostenol via a central line. This is a life supporting continuous infusion, with a very short half-life of 2-3 minutes. An interruption of Epoprostenol infusion may precipitate a pulmonary hypertensive crisis.

Epoprostenol mechanisms of action:

• Potent vasodilator of pulmonary and systemic vascular arterial beds
• Inhibits platelet aggregation
• Slows the process of scarring and rapid growth of new cells in long vessels

The haemodynamic effects of Epoprostenol include reduced pulmonary vascular resistance, increased cardiac index, and increased oxygen delivery. However, hypotension is also a haemodynamic effect.