Effect of physical countermaneuvers on orthostatic hypotension in familial dysautonomia
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Familial dysautonomia (FD) patients frequently experience debilitating orthostatic hypotension. Since physical countermaneuvers can increase blood pressure (BP) in other groups of patients with orthostatic hypotension, we evaluated the effectiveness of countermaneuvers in FD patients.
In 17 FD patients (26.4 ± 12.4 years, eight female), we monitored heart rate (HR), blood pressure (BP), cardiac output (CO), total peripheral resistance (TPR) and calf volume while supine, during standing and during application of four countermaneuvers: bending forward, squatting, leg crossing, and abdominal compression using an inflatable belt. Countermaneuvers were initiated after standing up,when systolic BP had fallen by 40mmHg or diastolic BP by 30mmHg or presyncope had occurred.
During active standing, blood pressure and TPR decreased, calf volume increased but CO remained stable.Mean BP increased significantly during bending forward (by 20.0 (17 – 28.5) mmHg; P = 0.005) (median (25th – 75th quartile)), squatting (by 50.8 (33.5 – 56) mmHg; P = 0.002), and abdominal compression (by 5.8 (–1 – 34.7) mmHg; P = 0.04) – but not during leg–crossing. Squatting and abdominal compression also induced a significant increase in CO (by 18.1 (–1.3 – 47.9) % during squatting (P = 0.02) and by 7.6 (0.4 – 19.6) % during abdominal compression (P=0.014)). HR did not change significantly during the countermaneuvers. TPR increased significantly only during squatting (by 37.2 (11.8 – 48.2) %; P = 0.01). However, orthopedic problems or ataxia prevented several patients from performing some of the countermaneuvers. Additionally, many patients required assistance with the maneuvers.
Squatting, bending forward and abdominal compression can improve orthostatic BP in FD patients, which is achieved mainly by an increased cardiac output. Squatting has the greatest effect on orthostatic blood pressure in FD patients. Suitability and effectiveness of a specific countermaneuver depends on the orthopedic or neurological complications of each FD patient and must be individually tested before a therapeutic recommendation can be given.
Key wordsphysical maneuvers familial dysautonomia syncope autonomic failure impedance cardiography
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- 2.Axelrod FB, Hilz MJ (2000) Familial dysautonomia. In: Appenzeller O (ed) Handbook of Clinical Neurology. The Autonomic Nervous System. Part II – Dysfunctions. Elsevier, Amsterdam, pp 143–160 Google Scholar
- 4.Bernstein DP (1986) Continuous noninvasive real–time monitoring of stroke volume and cardiac output by thoracic electrical bioimpedance. Crit Care Medicine 14:898–901Google Scholar
- 11.O'Donnel TV, McIlroy MB (1962) The circulatory effects of squatting. Am Heart J 84:347–358Google Scholar
- 14.Sharpey–Schafer EP (1956) Effects of squatting on the normal and failing circulation. BMJ 1:1072–1074Google Scholar
- 15.Slaugenhaupt SA, Blumenfeld A, Gill SP, Leyne M, Mull J, Cuajungco MP, Liebert CB, Chadwick B, Idelson M, Reznik L, Robbins C, Makalowska I, Brownstein M, Krappmann D, Scheidereit C, Maayan C, Axelrod FB, Gusella JF (2001) Tissue–specific expression of a splicing mutation in the IKBKAP gene causes familial dysautonomia. Am J Hum Genet 68:598–605PubMedCrossRefGoogle Scholar
- 21.The Consensus Committee of the American Autonomic Society and the American Academy of Neurology (1996) Consensus statement on the definition of orthostatic hypotension, pure autonomic failure and multiple system atrophy. Neurology 46:1470Google Scholar