Over the weekend, I was invited to speak at the Cardio-Connect Asia Pacific Meeting in Hong Kong on why young people, including athletes, die suddenly, without showing any previous symptom related to a heart problem.
There are several possible causes. One of these is what has been labeled as the Brugada Syndrome, a familial or genetic disease first reported by the Spanish family of cardiologists Pedro, Josep and Ramon Brugada in 1992, as cause of sudden unexplained death syndrome (SUDS) in young males. It is characterized by abnormal electrocardiogram (ECG) findings.
Unfortunately, since afflicted individuals are usually young and without any previous symptom, they are not diagnosed early enough before they suffer sudden cardiac arrest.
There’s good reason to believe that many young Filipino males (although females can also be affected) who have died in their sleep were actually victims of Brugada Syndrome.
Local medical literature calls it bangungot syndrome, because the victims moan and groan in their sleep, and are usually found dead in the morning.
Bangungot preceding death was first reported in the Philippines in 1917, with the “sudden night death” attributed to a nightmarish dream, frequently noted after a heavy meal with alcohol. Bangungot is derived from the Tagalog root words bangon (to rise) and ungol (to moan).
Local myths
Although the syndrome has been embellished with local myths, the association with a heavy carbohydrate and fatty meal, as well as an increased intake of alcohol, has scientific basis.
The identification of young males—aged 25 to 44, presumably healthy, without any known cardiac illness—as at-risk individuals is also consistent with international scientific reports on the Brugada Syndrome.
Southeast Asian males seem to have an increased predisposition to it. Similar cases are also seen in Pacific Rim countries and Polynesian populations where Southeast Asians have migrated.
Foreign epidemiologists RG Munger and EA Booton published a report in an international journal in 1998 titled “Bangungut in Manila: sudden and unexplained death in sleep of adult Filipinos,” based on autopsy records from 1948 to 1982.
They noted that the deaths were seasonal, peaking in December-January. The victims were predominantly males, aged 25-44. The deaths generally occurred past midnight, at around 3-4 a.m. The frequency was 26.3 per 100,000 persons per year in the report.
A report later filed by our colleagues, led by Dr. Giselle Gervacio-Domingo, showed a frequency of 43 per 100,000 per year, still predominantly males. This was based on the National Nutrition Health Survey of 2003, which also looked at the prevalence of other medical problems in the Philippines.
In Thailand, they call it lai tai, and their folklore attributes it to the “widow ghost” who forages the night for healthy young men. The prevalence is 26-40 per 100,000 population.
In Japan, it’s called pokkuri, which describes it as peaceful death in one’s sleep. It has a similar prevalence rate.
Based on available local data, we estimate that there are around 180,000 of our young population in the Philippines who have the Brugada Syndrome, and who are at risk of dying suddenly.
Many of these remain undiagnosed because they usually have no symptoms, and because of their young age, they don’t think it’s necessary to consult physicians and rule out this abnormality.
There lies the dilemma. Do we screen our more than 100 million people to find the 180,000 who might have it? Experts are divided as to the cost-effectivity or advisability of such a strategy.
Screening
We agree that it might not be advisable to do an ECG on the entire population. But for those with a history of sudden death in the family, especially deaths occurring during sleep, we recommend that the other family members, especially the males, should be screened.
If one also has a history of fainting (syncope), it’s best to make sure that it’s not related to a heart arrhythmia (irregular heartbeat), which may later lead to sudden cardiac arrest. It’s possible that the life-threatening arrhythmia could only be transient, and terminate spontaneously.
If an individual is diagnosed with Brugada Syndrome, and further tests show increased risk of sudden cardiac arrest, preventive measures may be taken.
Some medicines like good old quinidine have been shown to be effective in preventing life-threatening arrhythmias, but for those who can afford it, an implantable cardiac defibrillator (ICD), similar to an artificial pacemaker battery, is surgically implanted just beneath the skin layer on the chest. It’s hooked to the heart and gives it a mild “shock” (defibrillate) whenever it goes into a life-threatening heartbeat.
One of our young doctors in the hospital has Brugada Syndrome. When he was a surgical resident, he suffered cardiac arrest while doing surgery in the operating room. He was resuscitated and has been on an ICD for more than 10 years now.
He can actually feel the ICD firing from time to time. And every time it does, he pauses and whispers a short prayer, thanking God for saving him from the hundred times he could have died all these years.