© 2005 The European Society of Cardiology. Published by Elsevier Ltd. All rights reserved.
CASE REPORT
Orthostatic blood pressure control in Marfan's syndrome
aDepartment of Internal Medicine F4-222, Academic Medical Center Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands; bDepartment of Physiology, Academic Medical Center Amsterdam, The Netherlands; cDepartment of Cardiology, Academic Medical Center Amsterdam, The Netherlands
Manuscript submitted 5 February 2004. Accepted after revision 29 May 2004.
*Corresponding author. Tel.: +31 205663161; fax: +31 206919658. E-mail address: w.wieling{at}amc.uva.nl (W. Wieling).
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Abstract |
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A 33-year old male patient, with Marfan's syndrome, reported symptoms of orthostatic intolerance and fatigue as a longstanding problem. Orthostatic cardiovascular examination showed poor orthostatic tolerance, with a rise in heart rate and a fall in arterial blood pressure and cerebral blood flow velocity. Self-discovered physical counterpressure manoeuvers improved symptoms, related to a substantial increase in arterial pressure and cerebral perfusion. When orthostatic complaints are reported by patients with Marfan's syndrome, physical counterpressure manoeuvers should be advised to reduce symptoms of postural hypotension.
Key Words: Marfan's syndrome, orthostatic hypotension, physical counterpressure manoeuvers, cerebral blood flow velocity
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Introduction |
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Height and skeletal muscle tone have since long been associated with poor orthostatic tolerance [1,
2]. Marfan's syndrome is an autosomal dominant condition of abnormal connective tissue. One of the manifestations is a tall asthenic stature and frail musculature. In 58% of the patients height is over 95th percentile for age, mainly related to the length of the legs [3]. The incidence of orthostatic complaints in Marfan patients, therefore, can be expected to be considerably higher compared with controls. However, clinical trials on this subject have not been performed. |
History |
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A 33-year old male patient reported orthostatic intolerance and fatigue as a longstanding problem. Complaints of fatigue, light-headedness and blurred vision directly after standing up and during prolonged standing had been present for 2 years at presentation. He had never experienced syncope. The patient had noted that contraction of the lower limbs or placing a foot on a chair reduced his orthostatic complaints. He was found to have familial Marfan's syndrome 10 years before this presentation, and had a tall asthenic appearance, with a height of 197 cm and a weight of 73 kg (BMI 18.8). His medication consisted of atenolol 50 mg once daily for the last 7 years. The only events in his medical history that could be connected with Marfan's syndrome was a retinal ablation 2 years before the diagnosis: one year before presentation to our unit, a Bentall procedure was performed because of progressive aortic root dilatation. The patient was admitted to the syncope-unit of our centre for evaluation of orthostatic circulatory control.
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Cardiovascular examination |
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Finger arterial blood pressure (BPfinap) was monitored with a Finapres model 5 (Netherlands Organization for Applied Scientific Research, Biomedical Instrumentation (BMI-TNO) Amsterdam, The Netherlands), which adequately reflects changes in arterial pressure [4]
. Heart rate was calculated from the pulse pressure interval. Cerebral blood flow velocity in the right middle cerebral artery (MCAV) was monitored using transcranial Doppler sonography (Multidop X4 DWL, Sipplingen, Germany). Brachial blood pressure (BP) was measured using a Dinamap. In the supine position, brachial blood pressure (BP) was 118/85/68 with a BPfinap of 106/81/68 mmHg. Heart rate was 85 beats per minute (bpm), MCAV 98/59/40 cm s–1 and end-tidal CO2 42 mmHg. Then the patient was instructed to stand up, and stand quietly for several minutes.
The initial orthostatic response in the first 30 s of standing was normal. After 5 min of standing BPfinap was 91/79/72 mmHg. Heart rate had increased to 113 bpm, MCAV reduced to 88/53/36 cm s–1 and end-tidal CO2 to 39 mmHg (panel A). The exaggerated postural rise in heart rate (28 bpm) together with the fall in systolic blood pressure was considered as a sign of poor orthostatic tolerance.
The manoeuvers the patient invented to prevent symptoms were tested. Leg-crossing raised BPfinap to 100/85/75 mmHg, accompanied by a reflex heart rate decrease to 107 bpm, a decrease in the MCAV to 84/49/36 cm s–1 (Fig. 1: panel A) and an end-tidal CO2 of 40 mmHg. Placing his foot on a chair after a period of 8 min of free standing raised BPfinap to 117/88/76 mmHg and MCAV to 96/55/38 cm s–1 (end-tidal CO2 42 mmHg), with a further reduction in HR to values comparable with the supine position (Fig. 1: panel B).
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Discussion |
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Patients as well as their treating physicians state that orthostatic complaints are a major problem in many patients with Marfan's syndrome. In a study of 174 patients with Marfan's syndrome, Peters describes that 70.7% of Marfan patients experience dizziness in daily life [5]
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Height has been described as an independent predictor of orthostatic tolerance [1]. The enhanced gravitational displacement of blood to the lower part of the body may cause the susceptibility to orthostatic symptoms. The smaller skeletal muscle mass, and therefore, less adequate skeletal muscle pump, of patients with Marfan's syndrome could impair venous return. Furthermore diminished baroreflex function due to affected baroreceptors could affect orthostatic tolerance in this group of patients.
Consideration may be made of the use of ß-blockers. Shores et al. reported the beneficial effect of ß-blockers on the rate of aortic dilatation in Marfan patients [6]. A possible side-effect could be the suppression of heart rate increase on change from supine to standing, but the influence of heart rate on orthostatic tolerance is minimal compared with the effects of vascular resistance [7].
Physical counterpressure manoeuvers, like leg-crossing, reduce complaints of orthostatic intolerance. Mechanical compression of the venous vascular beds in the legs, into which blood pools during standing, translocates blood to the chest. This results in an increased cardiac filling pressure, SV and CO and thereby in a rise in systemic arterial pressure and cerebral perfusion [8,9].
The complaints of fatigue, which 88.5% of the patients report [5], could possibly be related to the diminished orthostatic tolerance [10].
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Conclusion |
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This case shows the improvement of BP, heart rate and MCAV during physical manoeuvers which reduce postural hypotension. When considering that a majority of Marfan patients presents with a tall asthenic posture, it is likely that the incidence of orthostatic intolerance is underestimated in the Marfan population. When their history reveals orthostatic complaints, instruction of physical manoeuvers like leg-crossing, squatting or placing a foot on a chair [9]
could benefit their orthostatic tolerance. |
Acknowledgements |
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This study was supported with a grant from the Netherlands Heart Foundation (grant 2003B156).
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References |
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[9] Wieling W, van Lieshout JJ, van Leeuwen AM. Physical manoeuvres that reduce postural hypotension in autonomic failure. Clin Auton Res 1993; 3: 57–65.[CrossRef][Medline]
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