Ambulatory norepinephrine treatment of severe autonomic orthostatic hypotension

OBJECTIVES This study was designed to establish a patient-controlled, ambul atory norepinephrine treatment of refractory orthostatic hypotension due to primary autonomic failure. BACKGROUND Autonomic dysfunction leads to disabling postural hypotension. P articularly in primary autonomic dysfunction, repeated syncope and immobili zation can be the result. Medical treatment of orthostatic hypotension ofte n fails in advanced cases. METHODS Ambulatory, patient-controlled norepinephrine therapy was initiated in six patients with orthostatic hypotension due to primary autonomic fail ure that had been refractory to conventional treatment. Before this therapy , three patients were bedridden; one was immobilized in a wheelchair. All h ad recurrent syncope and tolerated upright tilt-table testing for less than 15 min despite extensive medical treatment. For ambulatory treatment, a po rt-a-cath system was implanted and, using a CADD ambulatory infusion pump, norepinephrine was infused in individually adjusted dosages. RESULTS Norepinephrine infusion therapy enabled all patients to sit, stay a nd walk around for more than 45 min. One patient died after a five-year tre atment period, another after nine months because of nonhemorrhagic brain st em infarctions, both in the absence of norepinephrine treatment. The remain ing four patients are still mobile after a period of 19, 10, 9 and 7 months , respectively. None of them has suffered complications due to arterial hyp o- or hypertension, and there has been no infection of the infusion system. CONCLUSIONS In these selected patients with refractory orthostatic hypotens ion due to primary autonomic dysfunction, ambulatory norepinephrine infusio n therapy has proved to be a promising new therapeutic option. Further long -term studies including more patients are necessary to assess additional in dications, reliability and safety of this new method. CT Am Cell Cardiol 20 01;37: 219-23) (C) 2001 by the American College of Cardiology.