Case 1
A 31-year-old Filipino labourer had an appendectomy scheduled. It was a minimally invasive procedure called laparoscopy. The patient did not have any significant medical history. Before the surgery, the patient’s vital signs were stable; however, he had a slight fever of 37.9 °C.
The doctors used general anaesthesia prior to the surgery. They used fentanyl, propofol, and rocuronium to induce the anaesthesia. Throughout the surgery, doctors used sevoflurane to maintain the anaesthesia and added additional doses of fentanyl and rocuronium every 30 minutes. The surgery was uneventful, with minimal bleeding, and the patient’s vital signs remained stable. The procedure took two hours.
During the recovery phase, the doctors administered the patient anaesthesia reversal drugs. Soon enough, the patient started breathing on his own. However, suddenly, the electrocardiogram (ECG) monitor showed ventricular tachycardia. The doctors couldn’t detect the patient’s blood pressure or pulse. They immediately started cardiopulmonary resuscitation (CPR) and used a defibrillator to try to restore the heart’s normal rhythm. After administering two doses of epinephrine intravenously and performing cardiac massage and defibrillation, the patient’s ECG returned to a normal sinus rhythm. His vital signs also returned to normal.
The doctors sent the patient’s ABGs and found no signs of severe metabolic acidosis. They moved the patient to the intensive care unit for monitoring. They consulted with a cardiologist. Moreover, doctors performed further tests such as a 12-lead ECG, ck-MB, troponin-T, cardiac echography, and C-reactive protein. None of these tests revealed any significant abnormalities, except for the 12-lead ECG done after the patient recovered, which showed an elevated ST segment in leads V2 and V3, and a saddleback pattern in lead V2 in a later ECG follow-up. This pattern indicated a high suspicion of Brugada syndrome. This is a genetic condition that affects the heart’s electrical activity.
Conclusion
The doctor inquired about the patient’s family history, and they found no cases of sudden death among his first-degree relatives. After four days of observation, the doctors discharged the patient from the hospital and referred him to a medical center for further evaluation. It was later confirmed that he had the genetic trait associated with Brugada syndrome.
Case 2
A 20-year-old from Taiwan presented to the hospital with a right hand injury, by a heavy object at work. The injury compressed his hand. Therefore, he needed surgery to treat it.
The patient and his family informed the doctors that he had a condition called Brugada syndrome. His father had passed away suddenly from a heart attack when he was 36 years old. After this incident, the young patient went to the National Taiwan University Hospital to undergo genetic testing for Brugada syndrome. The results showed that he had a mutation in the SCN5A gene, confirming the presence of the syndrome. However, he had not experienced any symptoms of the condition before.
Before the surgery, doctors conducted a 12-lead ECG test, which showed sinus bradycardia. There were no clear signs of the typical Brugada syndrome ECG pattern. The doctors administered anaesthesia using fentanyl (100 μg), thiamylal sodium (600 mg), and rocuronium (70 mg). They avoided Lidocaine due to its potential to prolong the QT interval. Doctors inserted an arterial line to monitor the patient’s blood pressure and electrolyte levels during the surgery. An external defibrillator was also prepared in case of emergency. The surgery was uneventful, lasting for an hour, with the patient’s vital signs remaining stable. Thereafter, they transferred the patient to the surgical intensive care unit (SICU) for observation and later discharged him.
Discussion: Brugada Syndrome
Brugada syndrome is a genetic condition that affects the electrical activity of the heart. It can differ among different genders, regions, and races. Although the gene related to the syndrome is equally found in males and females, males tend to exhibit more severe symptoms. As a result, the incidence of Brugada syndrome is much higher in males, accounting for about 80% of cases, compared to females. Asians generally have a prevalence that is nine times higher than that of Caucasians and 36 times higher than that of Hispanics. Among the different types of Brugada syndrome, types II and III are more common than type I.
Brugada syndrome can lead to ventricular arrhythmias and sudden cardiac death. Many individuals with Brugada syndrome do not experience any symptoms, but they are at increased risk of ventricular arrhythmias. Fainting episodes can occur due to brief abnormal heart rhythms. If a dangerous heart rhythm persists, it can result in fatal cardiac arrest. The most significant clinical manifestations of Brugada syndrome are ventricular arrhythmias, although patients may also be at risk of atrial arrhythmias, particularly atrial fibrillation. The presence of atrial fibrillation has been associated with increased disease severity and a higher risk of ventricular fibrillation. Fainting episodes can also occur even with a normal heart rhythm, often mistaken for vasovagal syncope, due to a sudden drop in blood pressure.
Brugada syndrome is also known as sudden unexplained nocturnal death syndrome because arrhythmic events are more common at night. Syncope, or sudden death, may be the only symptom during an attack. Brugada syndrome can affect individuals of all ages, but it has the highest incidence between 40 and 45 years old. However, symptoms may first appear in early childhood or old age.
Management of Brugada Syndrome
Brugada syndrome is typically diagnosed through an electrocardiogram (ECG), although the changes may not always be present. They can be manifested by administering sodium channel blockers such as ajmaline or flecainide. The characteristic ECG patterns include saddleback ST-segment elevation and a right bundle branch block pattern in leads V1 to V3. These patterns may be present all the time or appear in specific situations such as fever, certain medical conditions, or the use of certain medications. This can make the diagnosis more challenging.
The latest classification describes three forms of the Brugada ECG pattern. Currently, only the type I ECG pattern with saddle-back ST elevation can be used to definitively diagnose Brugada syndrome. However, if the type I pattern is not present, patients may need to consider their clinical history, and family history, or undergo genetic testing to meet the criteria for diagnosis.
