At 16 years old, she is the picture of good health: a competitive athlete and star of her school’s track team. It seems nothing could stand in her way. But then, fatigue sets in. Not thinking the symptom is anything serious but hoping to find some answers so she can continue to perform her best, the patient makes an appointment with her pediatrician. An electrocardiogram is performed, but an abnormality on the ECG prompts a referral to my office. After examining her ECG, I quickly realize this competitive athlete has a potentially deadly cardiac heart rhythm disorder called long QT syndrome, or LQTS. By current guidelines, this diagnosis means her track and running days are over.
Let me paint the picture for you: This patient had been running for years without incident and now a doctor, whom she’s never met before, is telling her she’s at risk for having a sudden cardiac arrest? It’s as heartbreaking as you can imagine; I see the tears start rolling down her face. Perhaps the most difficult part of my job is sidelining seemingly healthy patients for a disease they cannot see or even feel.
A Closer Look at LQTS
LQTS is caused by ion channel dysfunction. Ion channels are little tiny pores in cell walls that regulate the delicate balance of ions, and without normal ion channel function, life-threatening arrhythmias can occur. The pillars of LQTS therapy include medication (beta-blockers) and avoidance of medications that are known to prolong the corrected QT interval, or QTc. Patients who follow a prescribed treatment strategy tend to be well-protected against arrhythmias and symptoms.
What’s tricky about LQTS is that there are no overt signs of the disease. It’s a silent syndrome that occurs in otherwise healthy individuals, but an ECG can typically diagnose it. Patients with LQTS have predictable arrhythmia triggers, which are frequently caused by physical or emotional stress. Often, patients with this disease will manifest following a syncopal event ( fainting) or an aborted cardiac arrest during physical exertion.
The Changing Nature of the LQTS Sports Guidelines
Because fainting or an aborted cardiac arrest can occur during physical activity, consensus guidelines state that pediatric patients with LQTS are disqualified from most competitive sports. The consensus guidelines were published 10 years ago, and they have somewhat restricted physician and patient autonomy. But fortunately since then, our understanding of LQTS has evolved a great deal. The guidelines have certainly protected many patients, but are they appropriate for everyone? Should a physician, a patient and his or her family members be able to discuss the risk/benefit ratio of sports participation as it pertains to the individual?
In addition, there are so many positive effects of sports participation in young athletes, but excluding young athletes from sports due to LQTS can cause the unintended consequences of obesity, low self-esteem and depression. Are these negative consequences justifiable in every patient?
Recently published data suggests our perception of sports in LQTS may change. More than 100 young patients with LQTS were studied, and we found that none of them had cardiac events during sports participation in a seven-year average follow-up period. Yes, this data has limitations and should not be used to justify the safety of sports participation in LQTS, but it will hopefully serve as a springboard for debate.
Making Decisions for the Future
When it comes to keeping LQTS patients safe, immediate disqualification from sports is always the easier option, since saying “no” requires the least amount of effort. But I didn’t say “no” to this all-star track athlete because I wanted my conversation with her to be different. I wanted her to first focus on calibration and become accustomed to her new lifestyle — a lifestyle that includes taking medication.
Once that transition and understanding occurs, we will then talk about sports, and she and her parents will have an opportunity to express self-determination. This is a decision we will all make together, in the safest and most appropriate fashion for the patient. Perhaps she will decide on her own that sports are not in her best interest, but before jumping the gun, I wanted her to realize she has a major role in that decision.
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Loosening the Reigns on LQTS Sports Guidelines originally appeared on usnews.com
