Effect of physical countermaneuvers on orthostatic hypotension in familial dysautonomia

Abstract

■ Abstract 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 40 mmHg or diastolic BP by 30 mmHg 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 (25 th -75 th 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 words physical maneuvers · familial dysautonomia · syncope · autonomic failure · impedance cardiography 1 Published in "Journal of Neurology 253(1): 65-72, 2006" which should be cited to refer to this work.

2017 ACC/AHA/HRS Guideline for the Evaluation and Management of Patients With Syncope: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society

“…The response to beta blockers depends on the genotype, and not all beta blockers are the same. 316,319 Patients with LQTS1 appear to respond better than patients with LQTS2 and LQTS3.…”

“…316,317 Many patients who appear to not respond to beta blockers are poorly compliant or do not tolerate the medication. 317 Therefore, ICD implantation is reasonable in patients with LQTS who continue to have syncope despite beta-blocker therapy and in those who cannot tolerate beta-blocker therapy.…”

Nonpharmacologic Management of Orthostatic Hypotension: A Systematic Review

“…In the long term (time not specified), 3 participants continued, 3 participants performed an abbreviated version, and 2 participants discontinued the program because of the time commitment. 41 Eating smaller, more frequent meals as opposed to less, larger meals does not affect the changes in BP with orthostatic stress but does results in significantly higher supine, sitting, and standing BP and improves orthostatic symptoms Level 1 1 randomized crossover trial 30 PCMs or abdominal compression to 20mmHg improves orthostatic BP Level 1 1 randomized controlled crossover trial 35 Leg crossing with active muscle tensing improves orthostatic BP in individuals with hypoadrenergic OH, whereas leg muscle pumping with tiptoeing does not Level 2 1 prospective controlled trial 38 Sleeping with head up at 12 has a short-term effect but not a long-term effect on improving the orthostatic drop in systolic BP with active standing, but it does improve orthostatic tolerance both in the short term and long term as measured by the Orthostatic Disability Score Level 4 1 pre-post study 39 Drinking 300mL of water 45min prior to HUT does not improves orthostatic BP Level 4 1 pre-post study 40 Rapid drinking of 480mL of tap water 35min prior to standing improves orthostatic drop in BP Level 4 1 pre-post study 39 Ingesting a balanced liquid meal (commercially available Complan with glucose in a milk base) results in lowering of supine and postural BP in individuals with MSA and PAF, but not with DBH deficiency Level 4 1 pre-post study 27 Compression to 40mmHg with a pressure suit improves OH with compression of calves and thighs together, with or without abdominal compression; compression of either calves or thighs alone is not beneficial, whereas abdominal compression on its own is very effective Level 4 1 pre-post study 19 Acute bout of exercise exacerbates OH in the short term Level 4 1 pre-post study 36 Increasing abdominal compression from 20 to 40mmHg or using both abdominal compression and PCMs of the lower extremities concurrently results in improved orthostatic BP response Older adults Level 1 1 randomized controlled crossover trial 25 Active elastic compression bandage of lower extremities (40e60mmHg at ankles, 30e40mmHg at the hip) for 10min followed by additional abdominal compression (20e30mmHg) for 10min improves orthostatic drop in BP and orthostatic symptoms with HUT in community-living older adults Level 1 1 randomized crossover trial 26 Lower-limb compression bandage of 30mmHg pressure applied to both legs (from the ankle to the thigh) when supine prior to standing improves orthostatic symptoms in hospitalized older adults with >36h of bed rest Level 1 1 RCT 37 Sleeping with head up at 5 (6in) has no additional effects on orthostatic BP measured over 2min after standing or symptoms at 6wk when compared with no head elevation Level 4 1 pre-post study 20 8-wk home-based resistance training program does not improve orthostatic drop in BP Level 4 1 pre-post study 24 Graduated elastic compression tights with 20e30mmHg ankle pressure impro...…”

“…Five studies 19,24,[39][40][41] had inclusion criteria of an SBP drop of !20mmHg with varying times at which the drop in SBP was detected after postural challenge. Twelve studies did not have OH as a specific inclusion criterion, but baseline blood pressure postural changes were consistent with an inclusion criterion of SBP drop of !20mmHg [21][22][23]26,[28][29][30][31][32]35 or a fall in SBP to <90mmHg. 33,34 Interventions Eight nonpharmacologic interventions for OH were identified under 2 general categories: physical modalities (exercise, functional electrical stimulation [FES], compression, physical countermaneuvers, compression and physical countermaneuvers, sleeping with head up) (see supplemental table S1) and dietary measures (water intake, meals) (see supplemental table S2).…”

Pathophysiology and Treatment of Orthostatic Hypotension in Parkinsonian Disorders

“…There are several physical counter-maneuvers that patients may use when symptomatic. Leg crossing, squatting, placing one leg on a chair, bending forward with arms crossed over the abdomen, or putting the head between the knees can be tried as a counter-maneuver [40,41]. The hemodynamic purpose of the counter-maneuvers is to increase venous return and cardiac output by increasing intra-abdominal pressure, activating the muscle reflex, increasing vascular tone in the deep vessels, as well as decreasing the height difference between the heart and the brain [41,42].…”