Perioperative hazards in myotonic dystrophy

Mudge, Barbara J.; Taylor, Peter B.; Vanderspek, Abraham F. L.

doi:10.1111/j.1365-2044.1980.tb03827.x

Cited by 45 publications

(17 citation statements)

References 8 publications

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“…Although dilated cardiomyopathy has been reported,5 11 systolic myocardial dysfunction appears to be less prominent than in other forms of muscular dystrophy. However, there are now a number of reports of cardiac decompensation during pregnancy12 13 and, together with the well recognised risks of anaesthesia,14this suggests that more serious covert abnormalities may lie undetected. The highest infant mortality rate was described by Reardon et al ,15 who suggest that there is a 25% chance of death before 18 months of age, with only 50% of patients surviving until their mid-30s.…”

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confidence: 99%

Left ventricular diastolic function in congenital myotonic dystrophy

Bu’Lock

Sood²,

Giovanni³

et al. 1999

Archives of Disease in Childhood

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Objective-Examination of left ventricular function and conduction abnormalities in myotonic dystrophy. Design-Twelve patients (median age, 13.7 years) with myotonic dystrophy had detailed electrocardiography and echocardiography performed. Echocardiographic parameters were compared with body surface area (BSA) matched median normal values. Results-Fractionalshortening was slightly reduced (by 28-29%) in three patients and three patients had mild mitral valve prolapse. Diastolic function was abnormal; isovolumic relaxation time (IVRT) and duration of early filling were prolonged compared with control values (median IVRT, 74 v 61 ms). Peak E velocity was increased (median, 0.82 v 0.78 m/s) but atrial phase filling was normal. Heart rate was reduced (median, 68 v 81 beats/ min). Conduction abnormalities were common but showed no clear relations with diastolic abnormalities. Conclusions-Young patients with myotonic dystrophy have myocardial diastolic dysfunction as well as abnormal electrophysiology. The prognostic implications of such abnormalities require further study. (Arch Dis Child 1999;80:267-270)

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mentioning

confidence: 99%

Left ventricular diastolic function in congenital myotonic dystrophy

Bu’Lock

Sood²,

Giovanni³

et al. 1999

Archives of Disease in Childhood

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“…Although the nondepolarizing muscle relaxants behaved normally [5][6][7][8], exaggerated responses were also reported when dystrophic muscular changes occurred [8,9]. However, to date, there has been no report describing a myotonic patient who was resistant to the nondepolarizing muscle relaxants.…”

Section: Introductionmentioning

confidence: 85%

Unexpected resistance to pancuronium in a patient with myotonic dystrophy (myotonia dystrophica)

Akata

Sasaki

Saito

et al. 2000

Journal of Anesthesia

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baldness, and cataract formation. In addition, he appeared to have cardiac dysrhythmias associated with myotonia dystrophica, and he was diagnosed 7 years previously with Adam-Stokes' syndrome. However, without any cardiac medication, first-degree atrioventricular block (PQ interval, 0.28 s) was the only abnormality found in the resting electrocardiogram, and the echocardiogram was normal. Pulmonary function tests, chest X-rays, and arterial blood gas analysis were all within normal limits (NL). The patient's serum concentrations of electrolytes were all normal. However, the preoperative screening tests on the endocrine system revealed the presence of mild adrenocortical insufficiency due to pituitary dysfunction: urine 17-hydroxycorticosteroids, 0.85 to 2.465 (NL ϭ 3.6-9.0) mg·day Ϫ1 ; 17-ketosteroids, 1.68 to 4.35 (NL ϭ 3.0-13.0) mg·day Ϫ1 ; serum cortisol 6.2 (NL ϭ 5.5-17.0) µg·dl Ϫ1 ; serum ACTH 13 (NL ϭ 30-60) pg·ml Ϫ1 ; ACTH test, within NL. Thyroid function was, however, normal. The preoperative laboratory tests also indicated the presence of hyperlipidemia (triglycerides, 241-384 mg·dl Ϫ1 ; cholesterol, 255-286 mg·dl Ϫ1 ). Other laboratory data, including those on both hepatic and renal function, were normal. The patient had not previously undergone surgery with general anesthesia.The patient was premedicated with intramuscular hydroxyzine hydrochloride (50 mg) and atropine sulfate (0.5 mg) 45 min prior to the induction of anesthesia. Because of the adrenocortical insufficiency, 100 mg of hydrocortisone was intravenously administered immediately before induction of anesthesia. The patient was then mildly sedated with intravenous diazepam (5 mg) and fentanyl (150 µg), and the trachea was successfully intubated without using a neuromuscular blocking agent while the patient was sedated and awake. After endotracheal intubation, the lungs were easily ventilated, and anesthesia was subsequently maintained with 67% N 2 O/1-3% enflurane in oxygen and supplemented by further intravenous administration of fentanyl (total

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“…This may require aggressive therapy with an implantable cardiac defibrillator in appropriate individuals. • It is also of importance to consider cardiac abnormalities in patients with myotonic dystrophy who are to be given a general anesthetic because arrhythmias and conduction abnormalities may be precipitated perioperatively [68].…”

Section: Limb Girdle Muscular Dystrophymentioning

confidence: 99%

Cardiovascular complications of neuromuscular disorders

Sachdev

Elliott

McKenna

2002

Curr Treat Options Cardio Med

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In the past decade, advances in molecular genetics have shown that many familial neuromuscular and cardiovascular diseases share a common pathophysiology. They are caused by inherited mutations in the cellular cytoskeleton of cardiac and skeletal muscle cells. The clinical manifestation of cardiac disease in neuromuscular disorders is common and their management should include both periodic cardiac assessment and appropriate symptomatic and definitive therapy. Dilated cardiomyopathy is a common complication of neuromuscular diseases. Cardiac function may decline progressively as part of the natural history of the disease, but current medical therapy, including angiotensin-converting enzyme inhibitors, beta-blockers, and diuretics, can be used to alleviate symptoms of left ventricular dysfunction. Conduction disturbances may be an important cause of mortality, especially in patients with Emery Dreifuss muscular dystrophy, Kearns-Sayre syndrome, and myotonic dystrophy, and thus pacemaker implantation can be life-saving. Rhythm disturbances, such as atrial fibrillation and ventricular tachyarrhythmias, have been reported in patients with neuromuscular diseases. Treatment is based on preventing sudden death and embolic phenomena and cardioverting or controlling atrial fibrillation. In these patients, problems may arise with anticoagulation and antiarrhythmic therapy due to the inherent locomotor instability associated with the disease, and the presence of concomitant atrioventricular disease. Although uncommon, hypertrophic cardiomyopathy may be a feature of some neuromuscular disorders. Patients should undergo regular risk stratification for sudden cardiac death and symptoms such as heart failure can be treated with medical therapy.

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Perioperative hazards in myotonic dystrophy

Cited by 45 publications

References 8 publications

Left ventricular diastolic function in congenital myotonic dystrophy

Left ventricular diastolic function in congenital myotonic dystrophy

Unexpected resistance to pancuronium in a patient with myotonic dystrophy (myotonia dystrophica)

Cardiovascular complications of neuromuscular disorders

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