DEAR DR. GOTT: My six year-old-grandson was diagnosed with Brugada syndrome after being hospitalized with a kidney infection. It was discovered in an EKG when he was complaining of chest pain. Even his pediatrician has no knowledge of Brugada so we’re trying to get as much information as possible. Could you please help us?
DEAR READER: Brugada syndrome is a fairly newly discovered disorder of the heart’s rhythm that causes the lower chambers (the ventricles) to beat fast, preventing blood from circulating efficiently throughout the body. The first patient diagnosed with the syndrome was a three-year-old child in 1986 following multiple episodes of loss of consciousness.
The heart beat is triggered by an electrical impulse that is generated by cells in the right upper chamber of the heart. Minute pores on each cell direct electrical activity, causing the heart to beat. When a defect in the channels occurs, the heart can beat ineffectively and abnormally. When the remainder of the body fails to receive adequate blood, syncope (fainting) and more critical situations can occur.
The regular cycle of atrial/ventricular contractions effectively pumps blood out of the heart and throughout the body. When the heart beats too fast or too slow, the condition is referred to as an arrhythmia. During an arrhythmia, a patient may be aware of a heart flutter, chest heaviness, or shortness of breath.
Brugada syndrome is often inherited and is more common in men than in women. The condition is rare in young children but has been documented in adolescents and adults. Some people do not exhibit any symptoms at all and may be unaware of the condition. For those who do have symptoms, they may include syncope and noticeable palpitations or an otherwise irregular heart beat.
Causes for Brugada include an imbalance in the chemicals that transmit electrical signals through the body, may be a side effect of certain prescription medications or fever, and can be attributed to the use of cocaine.
Diagnosis can be accomplished through electrocardiogram (EKG), electrophysiology (EP), or through genetic testing. An EP requires that a catheter be threaded through a vein in the groin and up to the heart. This is followed by electrodes passed through the catheter to different areas of the heart. The electrodes don’t shock the heart but can detect electrical signals that run through the heart.
Treatment for high risk patients is commonly performed with the use of an implantable cardioverter/defibrillator (ICD). The ICD performs continuous monitoring of the heart’s rhythm, recognizing ventricular arrhythmias when they occur, and delivers electrical shocks at appropriate times for optimal control. When left untreated, the prognosis is poor.
At one time or another almost everyone might have an irregular heartbeat. Therefore, this information is not in any way designed to have readers draw conclusions regarding a diagnosis. All pediatric cardiologists should be aware of the Brugada Syndrome and should be consulted for further care. There is a self-help group found at www.Brugada.org.
Readers who would like related information can order my Health Report “Coronary Artery Disease” by sending a self-addressed, stamped number 10 envelope and a $2 US check or money order payable to Dr. Peter Gott, PO Box 433, Lakeville, CT 06039-0433. Be sure to mention the title when writing or print out an order form from my website, www.AskDrGottMD.com.
