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PEDIATRIC ANESTHESIA

Hyperkalemic Cardiac Arrest After Cardiopulmonary Bypass in a Child with Unsuspected Duchenne Muscular Dystrophy

Aruna Nathan, MD^*, Arjunan Ganesh, MBBS^*, Rodolfo I. Godinez, MD, PhD^*, Susan C. Nicolson, MD^*, and William J. Greeley, MD^*

*Department of Anesthesia and Critical Care Medicine, The Children’s Hospital of Philadelphia; and Department of Anesthesia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania

Address correspondence and reprint requests to Arjunan Ganesh, MBBS, Department of Anesthesiology and Critical Care Medicine, The Children’s Hospital of Philadelphia, 34th St. and Civic Center Blvd., Philadelphia, PA 19104-4399. Address e-mail to ganesha{at}email.chop.edu.

	Abstract

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Adverse reactions to volatile anesthetics and depolarizing muscle relaxants can occur in patients with Duchenne muscular dystrophy (DMD) resulting in acute rhabdomyolysis and hyperkalemia. We report a case of hyperkalemic cardiac arrest after cardiac surgery using cardiopulmonary bypass in a child with unsuspected DMD. Early diagnosis and management of hyperkalemia resulted in a successful outcome. Genetic testing confirmed the diagnosis of DMD. We recommend a thorough preoperative investigation, including creatine kinase estimation, in children with a history of unexplained motor delay.

	Introduction

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Of the 150 anesthetic-related cardiac arrests reported in the pediatric perioperative cardiac arrest registry during its first 4 yr, 4 were associated with hyperkalemia at the time of arrest (1). In an earlier study of pediatric cardiac arrests reported to the North American Malignant Hyperthermia Registry, unrecognized myopathy was present in 12 of 25 (48%) patients, 8 of whom had hyperkalemia (2). We report an uncommon cause of hyperkalemic cardiac arrest after cardiac surgery using cardiopulmonary bypass (CPB).

	Case Report

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A 4-yr-old boy, weighing 16.4 kg, Physical Status 1, presented for surgical closure of a large secundum atrial septal defect. There was no family history of congenital heart disease, early deaths, or anesthesia-related problems. The child was premedicated with oral pentobarbital and anesthesia was induced with sevoflurane in oxygen and nitrous oxide. Neuromuscular blockade was achieved with pancuronium before nasotracheal intubation. Anesthesia was maintained with fentanyl (15 µg/kg) and isoflurane given both on and off CPB. Total CPB and cross-clamp times were 29 and 14 min, respectively. The heart was arrested with 25 mL/kg cardioplegia solution containing 15 mEq/L potassium chloride. During and after CPB, the patient was not hyperthermic, acidotic, or hyperkalemic, with serum potassium concentrations ranging from 2.94 to 3.71 mEq/L. The color of urine was also normal during CPB. On arrival to the cardiac intensive care unit, residual neuromuscular blockade was antagonized with atropine and neostigmine, and the trachea extubated. The patient was awake and alert and breathing regularly with an oxygen saturation of 100% at the time of extubation. Twenty minutes later, the electrocardiogram showed broad complex bradycardia that rapidly progressed to ventricular tachycardia and fibrillation, and responded to defibrillation (20 J). The patient was reintubated and serum potassium concentration during resuscitation was >9 mEq/L on the first arterial blood gas with a base deficit of –4.3 mmol/L. The child’s esophageal temperature was 37.7°C. The patient received IV calcium gluconate, sodium bicarbonate, and a glucose-insulin infusion. Serum creatine kinase (CK) within an hour of the arrest was 17,821 U/L, and peaked at 613,120 mEq/L 48 h postoperatively. The urine was noted to be pale pink in color. Administration of dantrolene was considered and the Malignant Hyperthermia (MH) hotline was consulted. Dantrolene was withheld as advised by the MH hotline consultant because there was no evidence of hypermetabolism. The ventilatory requirements were normal and there was no evidence of increased carbon dioxide production. The patient was started on twice maintenance IV fluids with added bicarbonate to help alkalinize the urine. Forced diuresis was achieved with mannitol and furosemide. The patient remained normothermic postoperatively and had a normal acid base status and was tracheally extubated uneventfully the following day. He improved with conservative management and was discharged home on the sixth postoperative day. During further questioning of the family, it was determined that the child did not walk until age 2 yr. Genetic testing of a peripheral blood sample demonstrated a deletion in the dystrophin gene from at least exon 47 to exon 52 (exons 45 and 60 were present) that was consistent with a diagnosis of Duchenne muscular dystrophy (DMD) (3).

	Discussion

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An alarming series of cardiac arrests in children undergoing anesthesia in the 1980s was found to be related to the use of succinylcholine or volatile anesthetics in patients with undiagnosed myopathies (4). More recently, sevoflurane has also been shown to be associated with rhabdomyolysis in patients with DMD (5). Initially, such reactions were thought to be part of MH, and patients with DMD were thought to be MH susceptible. Over the past decade, the consensus has moved toward anesthesia-induced rhabdomyolysis rather than MH being the cause of cardiac arrests in children with heritable myopathies. Acute rhabdomyolysis and hyperkalemia can occur after the use of volatile anesthetics and depolarizing muscle relaxants in DMD patients (6,7), leading some authors to advocate the routine avoidance of these drugs (5). Although it is generally agreed that depolarizing muscle relaxants should not be used in DMD patients, there is no strong evidence to confirm that volatile anesthetics are absolutely contraindicated (8). Interestingly, there are reports of intraoperative cardiac arrests in DMD patients with undiagnosed cardiac disease who have received nontriggering anesthetics with propofol infusions (9,10).

Acute rhabdomyolysis may be caused by succinylcholine, volatile anesthetics, and even CPB (11) and positioning. The trigger for rhabdomyolysis in our patient is not clear, but may have been related to the use of volatile anesthetics or to the institution of CPB, which may have resulted in areas of muscle ischemia resulting in rhabdomyolysis. The patient was on CPB for 29 minutes and was only minimally cooled. Although the period of CPB was short, it is possible that those with DMD may be more prone to develop rhabdomyolysis.

In our patient, only the father was initially available to provide us with preoperative information, which included a history of normal developmental milestones. Routine musculoskeletal examination was essentially normal and the child received the standard of care in our institution for surgical closure of an atrial septal defect, which included an opioid/volatile anesthetic. It is also our institutional policy to avoid volatile anesthetics in patients with known DMD. After the episode, other family members provided a history of delayed walking.

A history of motor delay in children should be thoroughly investigated during the preoperative evaluation, and some have recommended obtaining serum CK levels (2). Breucking et al. (12) obtained information on 444 anesthetic episodes in 200 families with muscular dystrophy, 147 with DMD. Sudden cardiac arrest occurred in six patients, all of whom had undiagnosed disease.

Rhabdomyolysis occurred in 9 patients in this series and in 66 others reported in a literature review (13). The majority of these patients had undiagnosed underlying muscle disease. Our case once again raises the question of screening for DMD and other myopathies, especially in children with unexplained motor delay. Serum CK levels are available within 45 minutes of a blood sample being sent to the clinical laboratory. If CK levels are increased, surgery should be delayed until specific tests are done. Neonatal screening, by adding an inexpensive CK test to the specimen now obtained for inborn metabolic error screening could potentially identify all cases of DMD and other myopathies (2,14).

It has also been suggested that during perianesthetic arrests in children, the pediatric advanced life support protocol should be modified to detect and treat hyperkalemia (2). Usually, rhabdomyolysis and hyperkalemia occur within a short period after exposure to volatile anesthetics. In our patient, the manifestation was delayed by three hours. However, prompt recognition and management of the hyperkalemia resulted in a successful outcome. In conclusion, we describe a case of hyperkalemic cardiac arrest after CPB in a child with unsuspected DMD.

	References

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