European Heart Journal Advance Access originally published online on October 19, 2007
European Heart Journal 2007 28(22):2820-2821; doi:10.1093/eurheartj/ehm478
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Long-term follow-up of primary prophylactic implantable cardioverter-defibrillator therapy in Brugada syndrome: reply
Heart Rhythm Management Center
Vrije Universiteit Brussel
Brussels
Belgium
Heart Rhythm Management Center
Vrije Universiteit Brussel
Brussels
Belgium
Heart Rhythm Management Center
Vrije Universiteit Brussel
Brussels
Belgium
Heart Rhythm Management Center
Vrije Universiteit Brussel
Brussels
Belgium
Heart Rhythm Management Center
Vrije Universiteit Brussel
Brussels
Belgium
Cardiovascular Research and Teaching Institute
Cardiovascular Center
Moorselbaan 164
9300 Aalst
Belgium
Cardiovascular Research and Teaching Institute
Cardiovascular Center
Moorselbaan 164
9300 Aalst
Belgium
Cardiovascular and Thoracic Surgery
Department Cardiovascular Center
Moorselbaan 164
9300 Aalst
Belgium
Department of Clinical Epidemiology and Biostatistics VU Medical Center
Amsterdam
Holland
Heart Rhythm Management Center
Vrije Universiteit Brussel Brussels
Belgium
E-mail address: andreasarkozy{at}yahoo.ca
We are thankful for the excellent remarks of Dr Bonny regarding the different frequency of spontaneous type I ECG during follow-up among patients with baseline type II, type III, and normal ECGs. First, we would like to answer your question regarding the indication for ICD implantation in the nine patients with normal baseline ECG. Seven of the nine patients had undocumented syncope, which is in itself an indication for an ICD implantation. These seven patients were all adults, two of them also had atrial fibrillation and four of them were inducible to VF during an EP study. The eighth patient was a 6-year-old asymptomatic child identified during family screening of a large family with Brugada syndrome, who developed ventricular fibrillation during the class I AAD test following half dose of the ajmaline and a strongly positive response. This patient was also a carrier of the SCN5A mutation identified in her family. She was the only patient who had an SCN5A mutation identified. The ninth patient was asymptomatic, but had a strongly positive family history of sudden death (three first-degree relatives dying suddenly at a young age) and was inducible to VF during the EPS.
The reason why the importance of the presence of spontaneous type I vs. normal ECG has not been discussed more in detail in our article is we conducted a study focusing only on these issues in this1 and a similar larger ICD patient population. The results of the second study is submitted but not published yet. Shortly, regarding the negative predictive value of the normal ECGs, it seems that even in patients in whom a spontaneous type I ECG is once documented, at least every third ECG is normal. This would suggest that documentation of one or a few normal ECGs does not exclude the presence of spontaneous type I ECG at follow-up. Conversely, in patients in whom baseline only normal ECGs are documented and have no type I ECG during follow-up, every fifth ECG is type II or III. In summary, we completely agree with you that the presence of spontaneous type I ECG is a marker of increased risk as it has also been observed in previous studies.2,3,4 However, we believe that, given the extreme variability of the ECG in this syndrome and the small patient numbers in our study, further studies are necessary to make meaningful conclusion about the predictive value of baseline type II, III, and normal ECGs.
References
- Richter S, Sarkozy A, Chierchia GB, Boussy T, Kourgiannidis G, Geelen P, Wellens F, Brugada P. Variability of the diagnostic coved-type ECG during long-term follow-up of patients with Brugada syndrome and primary prophylactic ICD implantation. Eur Heart J (2006) 27:3666.
- Priori SG, Napolitano C, Gasparini M, Pappone C, Della Bella P, Giordano U, Bloise R, Giustetto C, De Nardis R, Grillo M, Ronchetti E, Faggiano G, Nastoli J. Natural history of Brugada syndrome: insights for risk stratification and management. Circulation (2002) 105:1342–1347.
[Abstract/Free Full Text] - Eckardt L, Probst V, Smits JP, Bahr ES, Wolpert C, Schimpf R, Wichter T, Boisseau P, Heinecke A, Breithardt G, Borggrefe M, LeMarec H, Bocker D, Wilde AA. Long-term prognosis of individuals with right precordial ST-segment-elevation Brugada syndrome. Circulation (2005) 111:257–263.
[Abstract/Free Full Text] - Brugada J, Brugada R, Brugada P. Determinants of sudden cardiac death in individuals with the electrocardiographic pattern of Brugada syndrome and no previous cardiac arrest. Circulation (2003) 108:3092–3096.
[Abstract/Free Full Text]
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