Anaesthetic management of a patient of Brugada syndrome for an emergency appendicectomy.

Brugada syndrome is a myocardial transmembrane conduction of sodium abnormality and a common cause of sudden cardiac death. It is characterized by a distinctive electrocardiograph pattern with right bundle branch block and ST segment elevation in precordial leads V1-V3. Many factors during general anesthetic management could precipitate malignant dysrhythmia. We report the anesthetic management of a patient with Brugada syndrome for emergency appendectomy uneventfully.

INTRODUCTION

In 1992, Brugada and Brugada[1] described this syndrome. This syndrome is associated with a significant risk of ventricular tachyarrhythmias (Ventricular Fibrillation) and sudden death. Eight patients presented with RBBB and St Segment elevation in leads V1–V3 without any demonstrable structural heart disease were resuscitated from cardiac arrest. It is especially seen in Asian populations.[2] The syndrome is familial with an autosomal dominant mode of transmission and incomplete penetrance. Arrhythmic events are observed at an average age of approximately 40 year, but have been reported over a wide range of ages from 2 to 77 year.[3] Many factors during general anesthesia (medications, bradycardia, and temperature changes) could precipitate malignant dysrhythmia. To date, a few case series and case reports of general anesthesia in Brugada syndrome have been published. We herewith report a patient with Brugada syndrome for emergency appendicectomy under general anesthesia.

CASE REPORT

A 45-year-old male patient Asian origin (—Kerala, India) was admitted with pain in abdomen, nausea, and vomiting with fever since 2 days. Clinical symptoms, signs, and investigations were suggestive of acute appendicitis. Patient was scheduled for an emergency appendicectomy. His weight and height were 70 kg and 173.5 cm, respectively with BMI 23.25. On medical history he had chest pain and for the same was admitted to Hamad general Hospital 1 year back. There was no history of chest pain after that episode, syncope, palpitations in the past. He was investigated with normal blood investigations during that time. But on ECG there was RBBB with ST elevation (coved) in lead V1–V3. Holtermonitorings were done suggestive of atrial premature contractions. On the stress test, as per Bruce protocol, it was negative with the target rate, no ECG abnormality. So cardiologist suggested that baseline ECG suggestive of Brugada syndrome. He was having past history of productive cough, which was diagnosed as asthmatic bronchitis 3 weeks back. He received antibiotics and salbutamol inhaler (as and when required- last used 3 weeks back). There was family history of father's sudden death, but details are not available. No past surgical or allergic history. On general and airway examination was normal except fever. Systemic examination was normal except abdominal tenderness. Chest X-ray was normal. ECG findings same as before RBBB with ST elevation (coved) in lead V1–V3 with QTc 420 msec.

Looking at clinical examination, investigations and as per surgical indication, general anesthesia with avoidance of sodium channel blocker medications was planned. Detail explanation given to patient and consent was taken. Routine monitorings were used. Defibrillator was kept ready. Atropine as per routine was kept ready for abolish any vagal effect. As any vagal stimulation in this type of cases, this leads to dysrrthymias.

General anesthesia was induced with propofol 2.5 mg/ kg and neuromuscular blockade provided through atracurium 0.5 mg/kg. Analgesia was supplemented with fentanyl 2 mcg/kg and patient maintained on sevoflurane with air for controlled ventilation. Paracetamol 1 gm intravenous was given. Spontaneous extubation was carried out with smooth emergence and breathing spontaneously, obeying commands, and satisfactory muscle power. Neostigmine was avoided due to its ability to produce dysrthymia in these cases. Postoperative analgesia provided with morphine in titrated doses. Postoperative care was given in the postanaesthesia care unit. No untoward events (no bradycardia or ventricular fibrillation) occurred during the anesthesia. Total surgery and anaesthesia times were 45 and 65 min, respectively. Invasive monitorings were avoided due to short surgical procedure without much haemodynamic instability. Patient was discharged following an uneventful hospital stay.

DISCUSSION

Brugada syndrome is characterized by abnormal electrocardiogram findings and is also known as sudden unexpected death syndrome or sudden unexpected nocturnal death syndrome.

An estimated 4% of all sudden deaths and at least 20% of sudden deaths in patients with structurally normal hearts are due to the Brugada syndrome.[1-4] Brugada syndrome is genetically linked with mutation of alpha subunits of tetrodotoxin-“insensitive” human cardiac sodium channels (hH1) gene SCN5A.[5] The use of certain antiarrhythmic sodium channel blockers (class IA and IC) accentuate the ECG manifestations.[6]

The following criteria were used to diagnose Brugada syndrome:[7] 1history of cardiac arrest or aborted sudden death or syncope of unknown origin with or without documentation of VF;[2ST segment elevation (coved or saddleback type) in V1–V3 with or without a certain degree of right bundle branch block (RBBB); 3no prolongation of the QT interval (QTc<440 msec); 4no structural and ischemic heart disease demonstrated by echocardiography and cardiac catheterization. In about 20% of the patients, atrial fibrillation is an associated arrhythmia.[8]

According to above favouring criteria, in our patient had ST segment elevation (coved or saddleback type) in V1–V2 with RBBB. There was no prolongation of QTc (420 msec) in ECG [Figure 1]. There was no structural heart disease and stress test negative as per Bruce protocol. He was having history of chest pain 1 year back but no syncope investigated for the same and Halter monitoring was done showed atrial premature contractions. There was family history of father's sudden death but no detail found. As surgery was an emergency, other diagnostic tests were not feasible.

Figure 1

ECG of our patient of Brugada syndrome

Type 1 has a coved-type ST elevation with at least 2-mm J-point elevation, a gradually descending ST segment and a negative T-wave [Figure 2]. The electrocardiogram picture in this study shows the typical Type 1 pattern. Type 2 has a saddle back pattern with a least 2-mm J-point elevation and at least 1-mm ST elevation, with a positive or biphasic T-wave. The type 2 pattern can occasionally be seen in healthy subjects. Type 3 has a saddle-back pattern with <2-mm J-point elevation and <1-mm ST elevation, with a positive T-wave. Type 3 pattern is not uncommon in healthy subjects.[9]

Figure 2

Typical ECG types of Brugada syndrome

Drug challenge with sodium channel blockers such as Pilsicainide and Flacainide is a standard provocative test used to unmask Brugada syndrome. It can induce significant ST segment elevation>0.10 mV. Test has high sensitivity (92%) and specificity (89%) for detection of programmed electrical stimulation (PES) – induced ventricular fibrillation in asymptomatic patients is till controversial.[10]

Fever, vagal stimulation, administration of class Ia, Ic, and III drugs and neostigmine also accentuate the ST segment elevation.[11] So in our patient we have not used neostigmine. Volatile anesthetic has no effect on ECG [Table 1]. Local anesthetics, especially bupivacaine, given by any route (eg. epidural), which cause a sudden rise in the serum concentration can unmask Brugada syndrome.[12] Lignocaine, a class IIb antiarrhythmic drug has no such effect and can be safely used.[13] Acetylcholine, beta antagonists and nicorandil may interfere with the ionic conditions and exacerbate the manifestations. Neostigmine may augment ST-segment elevation in a dose-dependent manner without inducing coronary spasm, while atropine may reduce elevation. Therefore, it may be wise to avoid neostigmine.[14]

Table 1

Acceptable and contraindicated drugs for Brugada syndrome[15, 16]

To date; an implantable cardioverter-defibrillator (ICD) is the sole medical intervention that effectively protects patients with Brugada syndrome from sudden cardiac death.[1516]

CONCLUSION

Vigilance on examination of ECG is required, as RBBB is very common and with normal cardiac function in 10% of patient may nearly miss diagnosis of this syndrome. Such patients are often untreated, but they have the risks of cardiac accidents such as ventricular fibrillation or sudden death. For preoperative evaluation of patients with Brugada syndrome-like ECG, it is important to ask them their experience of syncope and family history. Echocardiography and Holter ECG recording should be done. External defibrillator should be prepared and parasympathetic dominant condition must be avoided during the anesthetic management.

Timely detection of cardiac arrhythmias and immediate treatment is very important. By avoiding agents or conditions that may exacerbate Brugada syndrome during anesthesia, we were able to manage our patient uneventfully.