Arrhythmogenic right ventricular cardiomyopathy

Fast Beats


It is your high school reunion. Despite a packed schedule, you've somehow managed to take the evening off. You have to thank your best friend Andre for this - he just wouldn't take no for an answer. After gulping down some punch, your mood lightens up. You join Andre on the dance floor to recreate all the funky dance moves of your high school days. Enjoying himself enormously, Andre dances around. A short while later though, there is no sign of him on the dance floor. You decide to step out and look for him.


In the corridor outside, you see Andre sitting in a corner, holding his head in his hands. "Are you all right, Andre?" "I feel dizzy and my heart is thumping", he responds. You quickly check his pulse and note that it is around 160 bpm. You also notice that he is tachypneic. Just as you prepare to call 911, Andre opens his eyes and says that he feels better now. The palpitations and dizziness have gone away. You quickly reassess his pulse and realize that it is now only 80 bpm. "Has this happened before, Andre?", you ask. "A couple of times, yeah, but it never lasts long. Forget it - let's go back to the party!" "No way!", you respond. "You're going to the hospital with me!"


Once in the hospital, you obtain a detailed history from Andre. Apparently, the previous attacks were extremely similar to the one he had today, lasting only a few seconds each time. He is otherwise healthy, and extremely fit, being an enthusiastic amateur marathon runner. To the best of his knowledge, there is no history of similar episodes in his family. Aside from his grandfather, who died of a 'heart attack' at age 86, there is no family history of cardiac disease. Nor is there a family history of sudden death at a young age.


You order a 12-lead ECG. The ECG shows T inversions in leads V1-V3, as well as small terminal notches at the end of the QRS complexes.


Two days later, you review the report of the Holter monitor recording. The 24-hour trace shows 520 right ventricular outflow tract (RVOT) ectopics.


You sit with Andre and carefully explain about his diagnosis. He listens to you carefully, his face becoming increasingly grave. At the end, he asks you if he can still continue running marathons.


Andre is devastated by what you have said, and tells you that he wants to be alone for some time. You understand how he feels, and offer to drop him off at his home.


The following day, you receive a call from Andre. He apologizes for his behavior earlier, and thanks you for all the support you have given him. Both of you decide to meet up again at the hospital, to discuss the options for treatment.


You and Andre talk at length about the available management options.


Andre asks if there will be any long-term issues following ICD implantation.


Andre is pleased with your answer and agrees to go ahead with implantation of the ICD. Everything proceeds as planned, and Andre resumes his day to day life.


A few months later.. Your mobile phone rings in the early hours of the morning. Still half asleep, you pick up the phone, to hear an enraged Andre at the end. "You never told me that I would have to replace this damn device! You said it was a once-off thing! I trusted you!"


After hearing this, Andre is initially not so happy about having an ICD implanted. After much deep thought, he reluctantly agrees, saying that it would be preferable to swallowing pills every day of his life. Everything goes according to plan, and Andre makes an uneventful recovery.


A few months later… Andre has opened up a new sports goods shop, and today is the opening day. As you enter the shop, Andre's face lights up. He rushes forward to shake your hand, and introduces you to everyone present as "the friend who saved his life", as you do your best to appear modest. Well Done!


Andre isn't too happy at the prospect of having to take medications for the rest of his life. However, he decides to go ahead with your recommendation.


A few months later... You silently stand by as Andre's coffin is lowered into the grave. Apparently, he suddenly went into cardiac arrest a couple of days ago. You feel empty and unable to experience any emotion. Every now and then, Andre's friends and relatives walk up to you and thank you for helping him, even if you couldn't eventually save him. If only they knew how badly you've messed up .....


Andre agrees with your recommendation. You meet the interventional radiologist to discuss the arrangements for the procedure. She pores over Andre's clinical notes and looks up at you questioningly. "Radiofrequency ablation is not a primary mode of treatment for ARVC. Don't you think that you should consider some other option?" You turn red with embarrassment, and meekly agree. Later on, after double checking the relevant guidelines, you decide to recommend ICD implantation instead.


Right ventricular angiography shows dyskinetic segments in the triangle of dysplasia of the right ventricle. Now, you are absolutely certain that this is indeed arrhythmogenic right ventricular cardiomyopathy.


You are at home lounging with a cup of hot chocolate in hand. Your phone starts ringing. When you pick it up, you are completely bemused to hear nothing but muffled sobs for a few seconds. You suddenly realize that the tearful voice is Andre's wife. The next few minutes of conversation leave you reeling .....