[SCD-FORUM] 59E RE: Brugada patient. Can he work? Dr. Perez Riera
SCD Symposium
info at scd-symposium.org
Thu Oct 19 11:27:18 ART 2006
Dear Marcellus Francis Ramirez from Mannila Philippines: Here Andrés
Ricardo Pérez Riera from Sao Paulo Brazil answer. I think that your
patient has high risk of SCD.
First dear colleague you live in a Country were the disease is
endemic. In Philippines, the entity is known as Bangungut (wailing
followed by SCD during sleep) The death certificate classification
of sudden unexplained nocturnal deaths (SUDS) in Manila has changed
considerably, obscuring an increase in incidence. SUDS appears to be
a regional phenomenon in Southeast Asia and environmental causes are
likely because the deaths are seasonal, increased over the timespan
studied, and are more common among migrants to Manila than among
those born there. A high incidence of SUND has been reported among
young Asian males. These deaths are known as Pokkuri in Japan,
Bangungut in the Philippines and SUND in the USA. SUNDS AND BRUGADA
DISEASE ARE THE SAME DISEASE.
You say that the patient has spontaneous ECG type 1 pattern and
additionally he belongs to male gender. Male gender predicts a more
malignant natural history.
Asymptomatic individuals with a spontaneous abnormal type 1 ECG
developed an arrhythmic event during a mean follow-up period of only
27±29 months. A "spontaneous pattern" is defined as an ECG showing
the patterns established for the first European Consensus about the
syndrome (1): presence of repolarization disorders occurred in the
right precordial leads (V1 and V2) or in the anteroseptal wall (V1 to
V3) with ST-segment elevation coved to the top “coved type” or type
1A of my classification or rectilinear (type 1B of my classification)
equal or mayor than 2mm (0.2mV), and followed by negative T wave
(Brugada phenotype).
See the classification
http://www.scd-symposium.org/files/clasification.pdf
The following are markers of a poor prognosis in BrS patients:
1) Patients with an a spontaneously abnormal ECG type 1 pattern
(Types 1A or 1B);
2) Patients with inducibility of sustained ventricular
arrhythmias at PES: concensus does not exist on the value of PES to
identify the subjects with risk of spontaneous occurrence of VF.
Brugada brothers think that theses patients should receive an ICD.
(2-3) On the other hand, Priori et al from 200 patients using the
life-table method of Kaplan-Meier used to define the cardiac arrest-
free interval in patients undergoing PES failed to demonstrate an
association between PES inducibility and spontaneous occurrence of VF
(4) ; Eckardt et al studied during a mean follow-up of 40 months a
numerous universe of a collaborative large cohort 212 individuals who
presented Brugada type 1 electrocardiographic pattern, from which 125
(59%) was spontaneous, and the rest only after pharmacological test
with a class I drug. The authors verified that 58% were asymptomatic;
31% had suffered >/= 1episodes of syncope with unknown origin and 11%
had been resuscitated from a VF episode (aborted sudden death). A
history of syncope or aborted sudden death was predictor of adverse
outcome. The degree of elevation of the T segment was greater between
symptomatic individuals: 2.3 mm higher than asymptomatic ones (mean
1.9 mm ???: I think that this is a byes of the manuscript because
type 1 is 2mm). In the latter, it was observed that the incidence of
events was very low, and PES had a very low accuracy in predicting
evolution. This paper attempts to clarify the controversial issue,
which still persists, between Priori's group and Brugada's group,
regarding the predictive value of PES, agreeing with the former. The
data regarding the risk of events in patients with BrS are
controversial and depend on the cohort of patients studied. This
collaborative paper describes long-term follow up of a large cohort
of well-identified BrS patients as well as explores predictive value
of PES. In contrary to some previous papers on the topic, in this
study the authors could not demonstrate significant prognostic value
of PES testing. The risk of arrhythmic events in asymptomatic
patients is very low indicating that they could be considered as
patients of much lesser risk than it was previously considered. This
observation might have impact on both diagnostic triage and therapy
approach in BrS patients (pharmacological approach).
3) Male gender predict a more malignant natural history;
4) Symptomatic patients: A history of syncope or aborted sudden
death is predictor of adverse outcome.
5) Spontaneous ST-segment elevation in leads V1 through V3
combined with the history of syncope is a powerful marker to identify
individuals who had cardiac arrest.
6) A spontaneous change in ST segment is associated with the
highest risk for subsequent events in subjects with a Brugada-type 1
ECG. The presence of syncopal episodes, a history of familial sudden
death, and/or LP may increase its value (6).
7) A history of syncope or SCD, the presence of a spontaneous
Type 1 Brugada ECG, and male gender predict a more malignant natural
history. The use of a family history of SCD, the presence of an SCN5A
gene mutation, or EPS to guide the management of patients with a
Brugada ECG is not supported(7);
8) A genetic defect on the SCN5A gene is not associated with a
higher risk of events, suggesting that genetic analysis is a most
useful diagnostic parameter but it is not helpful for risk
stratification(8).
The Brugada phenotype ECG) is much more prevalent than the manifest
BrS. Although invasive electrophysiologic investigations have been
proposed as a risk stratifier, their value is controversial, and
alternative noninvasive techniques may be preferred.
Ikeda et al (6) sought a noninvasive strategy to detect a high-risk
group in a long-term follow-up study of subjects with a Brugada-type
ECG, and no history of cardiac arrest. The study enrolled 124
consecutive subjects with a Brugada-type ECG. Prognostic indices
included: age; sex, a family history of SCD, syncopal episodes, a
spontaneous coved-type ST-segment elevation, maximal magnitude of ST-
segment elevation, a spontaneous change in ST segment, a mean QRSd,
maximal QT interval, QT dispersion, LPs by SA-ECG, and TWAs. Of the
124 subjects, 20 consenting subjects had an ICD before follow-up.
During a 40 +/- 19-month follow-up, 12 subjects (9.7%) reached one of
the endpoints (SCD or VT). Of the 12 risk indices, a family history
of SCD, syncopal episodes, a spontaneous coved-type ST-segment
elevation, a spontaneous change in ST segment, and LP had significant
values. In multivariate analysis, a spontaneous change in ST segment
had the most significance (a relative hazard, 9.2; P = 0.036).
Combined assessment of this index and other significant indices
obtained higher positive predictive values (43-71%). The authors
concluded that a spontaneous change in ST segment is associated with
the highest risk for subsequent events in subjects with a Brugada-
type ECG. The presence of syncopal episodes, a history of familial
sudden death, and/or LP may increase its value.
References
1) Wilde AA, Antzelevitch C, Borggrefe M, Brugada J, Brugada R,
Brugada P, Corrado D, Hauer RNm Kass RS, Nademanee K, Priori SG,
Towbin JA. Proposed diagnostic criteria for the Brugada syndrome Eur
Heart J 2002; 23:1648-1654.
2) Brugada P, Brugada R, Mont L, Rivero M, Geelen P, Brugada J.
Natural history of Brugada syndrome: the prognostic value of
Programmed electrical stimulation of the heart. J Electrophysiol
2003; 14: 455-457.
3) Brugada J, Brugada R, Brugada P. Right bundle-branch block
and ST-segment elevation in leads V1 through V3: a marker for sudden
death in patients without demonstrable structural heart disease.
Circulation. 1998; 97: 457–460.
4) Priori SG, Napolitano C, Gasparini M, et al. Natural history
of Brugada syndrome: insights for risk stratification and management.
Circulation. 2002; 105: 1342-1347.
5) 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: 257-262; 111: 257-263.
6) Ikeda T, Takami M, Sugi K, Noninvasive risk stratification
of subjects with a Brugada-type electrocardiogram and no history of
cardiac arrest. Ann Noninvasive Electrocardiol. 2005; 10:396-403.
7) Gehi AK, Duong TD, Metz LD, et al. Risk stratification of
individuals with the brugada electrocardiogram: a meta-analysis. J
Cardiovasc Electrophysiol. 2006; 17:577-583.
8) Priori SG, Napolitano C, Gasparini M, et al. Natural history
of Brugada syndrome: insights for risk stratification and
management. Circulation. 2002; 105: 1342-1347.
All the best
Andrés Ricardo Pérez Riera.
Chief of Electro-Vectocardiology Sector of the Discipline of
Cardiology, ABC Faculty of Medicine (FMABC), Foundation of ABC
(FUABC) - Santo André - Sao Paulo - Brazil. Rua Sebastiao Afonso
885 - Zip Code: 044417-100- Jardim Miriam S.P Brazil- Phone:
5504-6243 Fax: 5506-0398
El 16/10/2006, a las 15:14, SCD Symposium escribió:
> Forum of the ISHNE Sudden Cardiac Death World-Wide Internet Symposium
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> Congratulations and more power to the organizers of
> this excellent symposium.
> I would like to ask your expert opinion about a recent
> patient I encountered: a male in his mid 30s with type
> 1 brugada ecg pattern, negative EPS for inducible VT
> and no family history of sudden death. The said
> patient is applying as a seaman (works as a ship crew
> member). Can this patient be cleared for employment in
> such occupation without risks?
>
> MARCELLUS FRANCIS RAMIREZ
> Universty of Santo Tomas
> Manila, Philippines
>
> --
> Dr. Sergio Dubner
> President of Scientific Committee
>
> Dr. Edgardo Schapachnik
> President of Steering Committee
>
>
>
>
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--
Dr. Sergio Dubner
President of Scientific Committee
Dr. Edgardo Schapachnik
President of Steering Committee
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