1 Department of Anaesthesiology, University Hospital Eppendorf, Hamburg, Germany. 2 Department of Paediatric Cardiology, University Hospital Eppendorf, Hamburg, Germany
Corresponding author: Department of Anaesthesiology, University Hospital Eppendorf, Martinistrasse 52, D-20246 Hamburg, Germany. E-mail: guschmid@uke.uni-hamburg.de
Accepted for publication: 23 January 2003
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Abstract |
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Br J Anaesth 2003; 90: 8004
Keywords: complications, muscular dystrophy; monitoring, echocardiography; monitoring, electrocardiography
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Introduction |
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Patients with DMD are considered to be at high risk of perioperative complications. These may be related to the administration of succinylcholine or halothane, as the disease is associated with malignant hyperthermia.14 But DMD is also accompanied by heart failure attributable to dystrophic involvement of the myocardium.5 The cardiomyopathy of DMD is characterized by fibrosis of the posterobasal and contiguous lateral wall of the left ventricle, and is associated with arrhythmia, ventricular dilatation and cardiac insufficiency.6 Typical abnormal electrocardiographic patterns are tall R waves in the right chest leads, deep Q waves in the left precordial leads, biventricular hypertrophy and sinus tachycardia. In addition to using trigger-free anaesthesia and testing lung function preoperatively, echocardiography is therefore recommended for patients with DMD.7 We present a case report which demonstrates, however, that normal preoperative echocardiography and electrocardiography cannot exclude the development of heart failure in patients with DMD during anaesthesia for major surgery.
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Case report |
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Discussion |
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Several complications in association with general anaesthesia have been reported in the last two decades in patients with DMD.8 9 Larsen and colleagues2 investigated the frequency and severity of anaesthetic complications in a retrospective study of 84 operations in patients with DMD. No case presenting with acute heart failure was noted. The complications were almost exclusively related to the use of succinylcholine and to malignant hyperthermia. However, in 70.3% of the patients an abnormal electrocardiogram was observed.2 In a recent German study, 444 cases of anaesthesia in 221 patients with muscular dystrophy were investigated. Complications were reported in 15 cases.10 Several complications, including cardiac arrest, occurred in young children with previously undiagnosed muscular dystrophy who received inhalational agents or succinylcholine. Anaesthetic risks and complications in 101 patients with DMD were investigated by Wollinsky and colleagues.11 Several complications could have been prevented by avoiding anaesthetic agents with a high trigger potential for malignant hyperthermia. The risk of cardiac insufficiency or arrhythmia was more pronounced in the older patients and could have been predicted by the findings in the preoperative echocardiograph.11 No patient with a normal preoperative echocardiograph developed heart failure perioperatively.
In contrast, Sethna and colleagues1 pointed out that there is no variable which preoperatively identifies patients who will develop complications during anaesthesia. He reported a 13-yr-old boy scheduled for scoliosis repair. The preoperative echocardiograph showed normal left ventricular size and function. The patient suddenly developed hypotension and bradycardia intraoperatively, which was unresponsive to catecholamines and cardiopulmonary resuscitation. Autopsy showed cardiomyopathy with dilatation, hypertrophy and fibrosis. This case is in concordance with our findings that echocardiography at rest may not be sufficiently sensitive to estimate the intraoperative cardiac risk in patients with DMD. Sethna and colleagues suggested that several complications may be minimized by using narcotics instead of potent volatile anesthetics, and by using non-depolarizing muscle relaxants instead of succinylcholine. However, the present case report demonstrates that, by using propofol and sufentanil for anaesthesia, and rocuronium bromide for muscle paralysis, the perioperative management of patients with DMD can still cause major problems.
Propofol given by infusion may cause hypotension because of its negative inotropic properties.12 In the present case propofol was given in the usual dose range.13 Unfortunately, we do not know the propofol blood concentrations in this patient. It is unclear whether the continued administration of propofol by infusion resulted in overdosage. Routine intraoperative monitoring of brain electrical activity by, for instance, the electroencephalogram may have provided useful information in this respect.
In the last 2 yr there have been several reports about the propofol-infusion syndrome, defined as the constellation of otherwise unexplained myocardial failure, metabolic acidosis and rhabdomyolysis in the setting of prolonged, high-dose propofol infusion (>4.86 mg kg1 h1 for >4872 h).14 15 However, a propofol-infusion syndrome-mediated circulatory insufficiency alone seems unlikely to be the cause of the problem in our patient as the hypotension and tachyarrhythmia occurred only 4 h after starting the propofol administration. But in this case we cannot exclude a contribution from the negative inotropic effects of propofol to the episode of severe heart failure.
Interestingly, female carriers of DMD can develop cardiomyopathy in the absence of skeletal muscle symptoms.16 Larach and colleagues17 pointed out that 48% of paediatric patients with cardiac arrest during anaesthesia had an unrecognized myopathy, and 67% of them were associated with hyperkalaemia. To decrease the incidence of myopathy-associated cardiac arrest during anaesthesia, screening of young male patients for occult myopathy has been recommended.18
As preoperative echocardiography may not reflect the intraoperative ability of the diseased myocardium to respond to stress, a more sensitive method of assessing myocardial dysfunction in patients with DMD is needed. Angermann and colleagues19 proposed that stress echocardiography using angiotensin revealed a significantly reduced fractional shortening in physically disabled patients with DMD. They also demonstrated a correlation between clinical symptoms, abnormal echocardiographic findings and the extent of the skeletal muscle disease. Stress echo cardiography detected latent heart failure in these patients and identified inducible contraction abnormalities in many patients with DMD. It might therefore be beneficial in detecting unrecognized cardiac problems preoperatively.
This case also raises the question of what intraoperative monitoring should be used for detection of cardiac abnormalities and for adjusting therapy. Most importantly, intraoperative transoesophageal echocardiography, pulmonary arterial catheterization and pulse contour analysis (PCCO) may be useful in this respect. Further studies are needed to investigate the importance of these tools, but transoesophageal echocardiography seems to be superior as it gives more detail of the anatomy (e.g. dilatation of the ventricle).
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References |
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