Pacemakers in patients with familial dysautonomia
- 57 Downloads
Familial dysautonomia (FD) is a genetic disease associated with a high incidence of sudden death. If fatal bradyarrhythmia is an etiological factor then the incidence of sudden death should decrease after pacemaker placement. Retrospective review of 596 registered FD patients revealed that 22 FD patients (3.7%) had pacemakers placed between December 1984 and June 2003. Clinical and electrocardiographic indications for placement and demographic data were assessed for 20 of the 22 patients (10 males, 10 females, ages 4 to 48 years). Two patients were excluded because of insufficient data. Prior to pacemaker placement, presenting symptoms were syncope and cardiac arrest, 16/20 (80%) and 6/20 (30 %), respectively. Asystole was the most frequent electrocardiographic finding and was documented in 17/20 patients (85 %). Other electrocardiographic abnormalities included bradycardia, AV block, prolonged QTc and prolonged JTc. The average duration of pacemaker utilization was 5.7 years (range 5 months to 14.5 years). Complications included infection (1 patient) and wire migration (2 patients). In the one patient with infection, the pacemaker was permanently removed. This patient then experienced multiple syncopal episodes and death. There were 7 other deaths. Three deaths occurred suddenly without preceding events, and 4 patients had non-cardiac causes of death. None of these 7 deceased patients had recurrence of syncope after pacemaker placement. In the 12 surviving patients, 6 had recurrence of syncope but none had cardiac arrest. Pacemaker placement may protect FD patients from fatal bradyarrhythmia and may decrease the incidence of syncope. However, data are limited and prospective analysis is needed.
Key wordsdysautonomia pacemakers syncope sudden death
Unable to display preview. Download preview PDF.
- 3.Axelrod FB (1996) Autonomic and Sensory Disorders. In: Emory AEH, Rimoin DL (eds) Principles and Practice of Medical Genetics. 3rd ed. Churchill Livingstone, Edinburgh, pp 397–411Google Scholar
- 4.Axelrod FB (2002) Hereditary Sensory and Autonomic Neuropathies: Familial Dysautonomia and other HSANs. Clin Auton Res 12(Supplement 1):2–14Google Scholar
- 8.Axelrod FB, Maayan C,Hazzi C, Bangaru B, Shannon D (1987) Bradycardia associated with hiatal hernia and gastroesophageal reflux relieved by surgery. Am J Gastro 82:159–161Google Scholar
- 19.Grubb BP, Kosinski DJ (2001) Syncope resulting from autonomic insufficiency syndromes associated with orthostatic intolerance. Medical Clinics of NA 85:457–472Google Scholar
- 22.Hilz MJ, Axelrod FB, Sauer P, Hagler A, Russo H, Neundorfer B (1997) Cold face stimulation demonstrates parasympathetic dysfunction in familial dysautonomia. J Auton Nerv Sys 65:111Google Scholar
- 28.Sutton R, Brignole M, Menozzi C, Raviele A, Alboni P, Giani P, Moya A (2000) Dual-chamber pacing in the treatment of neurally mediated tiltpositive cardioinhibitory syncope: pacemaker versus no therapy: a multicenter randomized study. The Vasovagal Syncope International Study (VASIS) Investigators. Circulation 102:294–299PubMedGoogle Scholar