Reply from the authors

Abstract

Editor—We thank Dr W. Fish for his interest in our review1Staikou C Chondrogiannis K Mani A Perioperative management of hereditary arrhythmogenic syndromes.Br J Anaesth. 2012; 108: 730-744Abstract Full Text Full Text PDF PubMed Scopus (45) Google Scholar and his constructive comments regarding the role of anaesthesiologists in early diagnosis of hereditary arrhythmogenic syndromes in suspicious cases. We agree that arrhythmogenic syndromes are underdiagnosed, since they may be asymptomatic for a long time. Thus, several anaesthesiologists have probably provided, or will provide at some time, anaesthesia to an undiagnosed patient. Since most of the times the postoperative outcome of these patients is good, an intraoperative arrhythmia, especially if short-lived and self- or pharmacologically resolved, may not be further investigated, either because its clinical significance is underestimated by the physician, because the medical costs will increase, or both. It is true that the anaesthesiologists can be adequately prepared for the perioperative management of patients diagnosed with an arrhythmogenic syndrome, in terms of preoperative clinical optimization of the patient, a multidisciplinary approach, and a case-tailored anaesthetic plan. On the contrary, uncontrollable difficulties may arise during management of undiagnosed patients whose syndrome is unveiled perioperatively. This explains the fact that most reported perioperative deaths have occurred in undiagnosed patients, as shown in the tables of our review.1Staikou C Chondrogiannis K Mani A Perioperative management of hereditary arrhythmogenic syndromes.Br J Anaesth. 2012; 108: 730-744Abstract Full Text Full Text PDF PubMed Scopus (45) Google Scholar Perioperatively, anaesthesiologists should maintain a high level of suspicion for arrhythmogenic syndromes, especially in cases with a personal or family history of arrhythmias, syncope, or sudden cardiac arrest. A detailed history is of paramount importance in revealing suspicious symptoms indicating paroxysmal arrhythmias—such as palpitations, dizzy spells, fatigue, impaired memory, syncopal episodes—especially if they occur under excessive sympathetic or parasympathetic activation, that is, physical exercise, emotional stress, or vagal manoeuvres. If an arrhythmogenic syndrome is suspected before operation, the patient should be further investigated before he/she undergoes a scheduled surgical procedure. This is highlighted by the case reported by Nakamura and colleagues2Nakamura S Nishiyama T Hanaoka K General anesthesia for a patient with asymptomatic sick sinus syndrome.Masui. 2005; 54: 912-913PubMed Google Scholar regarding a patient with asymptomatic bradycardia resistant to atropine manifested just before induction of anaesthesia. The scheduled surgery was postponed and further cardiological investigation revealed sick sinus syndrome. After placement of a temporary cardiac pacemaker, the patient underwent uneventfully a 9 h neurosurgical procedure. Of course, in many cases, neither the medical/anaesthetic history nor the preoperative examinations raise suspicions for an arrhythmogenic syndrome. A characteristic case is the one reported by Hirata and colleagues,3Hirata A Kitagawa H Komoda Y et al.Sick sinus syndrome discovered after induction of general anesthesia in a patient with normal preoperative Holter ECG.Masui. 1994; 43: 1048-1052PubMed Google Scholar regarding a surgical patient with undiagnosed sick sinus syndrome and normal preoperative cardiac examinations, including a Holter electrocardiogram. The syndrome was unveiled after induction of general anaesthesia and was confirmed a few months after operation by a diagnostic new Holter electrocardiogram. In patients with unexplained, suspicious intraoperative arrhythmias, even if they resolved without further complications, postoperative 24 h haemodynamic monitoring and further cardiological investigation, although associated with increased costs, would probably be useful in revealing an arrhythmogenic syndrome. If a sudden perioperative death occurs, postmortem investigation and—if indicated—familial genetic screening should be performed. In these cases, the anaesthesiologists may also play a significant role in announcing the death, explaining, informing, and even guiding the family members towards investigations which may be lifesaving for them, if a hereditary syndrome is diagnosed and thus treated early. None declared.