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Idiopathic Brugada-type electrocardiographic pattern in an octogenarian
Journal of Electrocardiology Vol. 37 No. 2 2004
Electrocardiographic Curiosities
Idiopathic Brugada-type Electrocardiographic Pattern in an Octogenarian
Ming-He Huang, MD, PhD, and Frank I. Marcus, MD
Abstract: The prognosis of idiopathic Brugada-type ECG pattern in asymptomatic people is unknown. We report a case of an 85-year-old man who had persistent Brugada-type ECG pattern without associated clinical symptoms. This illustrates that the persistent Brugada-type ECG can be present with normal longevity. Key words: Arrhythmia, Brugada syndrome, sudden death.
Case Report
Brugada syndrome is characterized by ST-segment elevation in leads V1 to V3 associated with arrhythmic death. It may be familial. A genetic defect has been found to be present in SCN5A gene in a minority of patients. Infrequently, the Brugada electrocardiogram (ECG) pattern may be observed as an incidental finding. This poses the problem of determining whether the ECG pattern may be a marker of lethal arrhythmic events in the individual patient. Although the prognosis in patients with idiopathic Brugada-type ECG pattern is still debated, it is instructive to report the case of an 85-year-old asymptomatic man with a Brugadatype ECG pattern.
An 85-year-old white man presented to the emergency department because of mental status change and fever that was found to be due to pneumonia. He was disoriented and agitated. His temperature was 39° C, blood pressure was 130/80 mm Hg, heart rate was 100 beats per minute, and respiratory rate was 18 per minute. His cardiovascular examination was normal. There was no evidence of congestive heart failure. Chest examination revealed rales in the right lower lung field. An ECG showed sinus rhythm with marked ST segment elevation of a coved pattern in V1 through V3 (Fig. 1). A right bundle branch block configuration was observed in V1 and V2. The ECGs obtained 5 and 1 year prior to this admission demonstrated the same pattern. A transthoracic echocardiography showed normal wall motion in both right and left ventricles with a left ventricular ejection fraction of 70%. Mild left ventricular diastolic dysfunction was noted. There was no significant valvular abnormality. Chest X-ray showed right lower lobe infiltration consistent with pneumonia. Troponin I on 2 occasions, electrolytes, and renal function were all
From the Department of Medicine, University of Arizona Medical Center, Sarver heart Center, College of Medicine, Tucson, AZ. Reprint requests: Ming-He Huang, MD, PhD, University of Arizona Medical Center, Sarver Heart Center, 1501 N. Campbell Ave. Tucson, AZ 85724; e-mail: [email protected]. © 2004 Elsevier Inc. All rights reserved. 0022-0736/04/3702-0006$30.00/0 doi:10.1016/j.jelectrocard.2004.01.007
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110 Journal of Electrocardiology Vol. 37 No. 2 April 2004
Fig. 1. An ECG shows sinus rhythm and left axis deviation with marked coved ST-segment elevation in V1 and V2. A right bundle branch block configuration was observed in V1 and V2.
within normal limits. He was treated with antibiotics with resolution of his fever and amelioration of his mental function and was discharged. His past medical history included dementia. He had no history of heart disease, presyncope, or syncope. His only medication was Prozac (Eli Lilly; Florida). There was no family history of sudden death.
Discussion To our knowledge, this is the oldest documented case with a persistent Brugada-type ECG pattern without clinical symptoms. The Brugada syndrome is characterized by 3 types of ECG patterns associated with syncope and/or sudden death (1). This patient’s ECG showed a typical type-I Brugada ECG pattern that is characterized by right bundle branch block morphology and coved ST segment elevation in leads V1 to V3 with negative T waves. In the Brugada syndrome the mean age at which symptoms first occur is in the third or the fourth decade. However, the initial symptom can appear as late as age of 77 (2). It has been postulated that the Brugada-type ECG pattern, per se, is a marker of the risk of arrhythmic death regardless of the presence or absence of clinical symptoms since the initial symptom might occur later in life (2,3). The
incidence of asymptomatic Brugada ECG pattern ranges from 0.02% to 0.7% in large population samples (4). The prognosis in asymptomatic patients with Brugada ECG is controversial. Brugada et al (5). reported 6 of 22 (27%) asymptomatic patients developed sudden death or aborted ventricular fibrillation during 34 ⫾ 32 months of follow-up. Age, sex, family history, and inducibility of arrhythmias were not helpful or stratifying the risk of sudden death. Persistent ST-segment elevation of V1-V3 in healthy individuals was initially reported by Osher and Wolff. (6) They described the ECG pattern in 3 men between the ages of 37 to 40 who remained well during the follow-up periods of up to 3 years. Several recent studies with a relative longterm follow-up (up to 7 years) indicated that the risk for developing malignant arrhythmia, syncope or sudden death in asymptomatic patients was low (4,7–9). None of the patients in these studies had syncope or sudden death.(4,9) The risk of sudden death in the long-term follow-up is not known since symptoms may not appear for many years after the ECG pattern is recognized.(2) Nevertheless, the persistent Brugada-type ECG can be present with normal longevity as reported in this case. The Brugada-type of ST-segment elevation has been suggested to be associated with fever, (10)
Idiopathic Brugada-type ECG •
hyperkalemia, (11) drugs including crack cocaine and overdose of tricyclic antidepressants.(12,13) It may also be related to a variety of right ventricular pathological processes including arrhythmogenic right ventricular dysplasia (14). Mediastinal tumor compressing the right ventricular free wall and hemopericardium of tumor compressing the right ventricle may also induce the ECG changes (15,16). Our patient had none of the above conditions. His previous ECGs as well as the ECG after he became afebrile during the hospitalization displayed the same Brugada-type patterns. The challenge is to be able to properly categorize younger individuals with the Brugada ECG pattern who may have a risk of sudden cardiac death. The highest risks are the individuals with Brugada ECG pattern who have the persistent ECG pattern and have a history of syncope or have been resuscitated from cardiac arrest. Family members with the persistent ECG pattern also are at risk. The asymptomatic individual who does not have a family history of premature sudden death and who has a persistent Brugada ECG pattern is at lower risk. The use of electrophysiological testing is controversial but the data suggests that it is not reliable to indicate patients at high risk (7,8).
5.
6.
7.
8.
9.
10.
11.
12.
References 13. 1. Wilde AAM, Antzelevitch C, Borggrefe M, et al: Proposed diagnostic criteria for the Brugada syndrome (consensus report). Eur Heart J 23:1648, 2002 2. Brugada P, Brugada J: Right bundle branch block, persistent ST segment elevation and sudden cardiac death: A distinct clinical and electrocardiographic syndrome: A multicenter report. Am J Coll Cardiol 20:1391, 1992 3. Brugada P, Brugada J, Brugada R: The Brugada syndrome. ANE 5:88, 2002 4. Littmann L, Monroe MH, Kerns WP, et al: Brugada syndrome and “Brugada sign”: Clinical spectrum
14.
15.
16.
Huang and Marcus 111
with a guide for the clinician. Am Heart J 145:768, 2003 Brugada J, Brugada R, Brugada P: Right bundlebranch block and ST-segment elevation in leads V1 through V3: A marker for sudden death in patients without demonstrable structural heart disease. Circulation 97:457, 1998 Osher HL, Wolff L: Electrocardiographic pattern simulating acute myocardial injury. Am J Med Sci 226: 541, 1953 Morita H, Takenaka-Morita S, Fukushima-Kusano K, et al: Risk stratification for asymptomatic patients with Brugada syndrome. Circ J 67:312, 2003 Priori SG, Napolitano C, Gasparini M, et al: Natural history of Brugada syndrome: Insights for risk stratification and management. Circulation 105:1342, 2002 Takenaka S, Kusano KF, Hisamatsu K, et al: Relative benign clinical course in asymptomatic patients with Brugada-type electrocardiogram without family history of sudden death. J Cardiovasc Electrophysiol 12:2, 2001 Saura D, Garcia-Alberola A, Carrillo P, et al: Brugada-like electrocardiographic pattern induced by fever. PACE 25:856, 2002 Ortega-Carnicer J, Benezet J, Ruiz-Lorenzo F, et al: Transient Brugada-type electrocardiographic abnormalities in renal failure reversed by dialysis. Resuscitation 55:215, 2002 Littmann L, Monroe MH, Svenson RH: Brugada-type electrocardiographic pattern induced by cocaine (review). Mayo Clinic Proc 75:845, 2000 Goldgran-Toledano D, Sideris G, Kevorkian JP: Overdose of cyclic antidepressants and the Brugada syndrome. N Engl J Med 346:1591, 2002 Corrado D, Basso C, Buja G, et al: Right bundle branch block, right precordial st-segment elevation, and sudden death in young people. Circulation 103: 710, 2001 Tarin N, Farre J, Rubio JM, et al: Brugada-like electrocardiographic pattern in a patient with a mediastinal tumor. PACE 22:1264, 1999 Tomcsanyi J, Simor T, Papp L: Images in cardiology. Haemopericardium and Brugada-like ECG pattern in rheumatoid arthritis. Heart 87:234, 2002
Electrocardiographic Curiosities
Idiopathic Brugada-type Electrocardiographic Pattern in an Octogenarian
Ming-He Huang, MD, PhD, and Frank I. Marcus, MD
Abstract: The prognosis of idiopathic Brugada-type ECG pattern in asymptomatic people is unknown. We report a case of an 85-year-old man who had persistent Brugada-type ECG pattern without associated clinical symptoms. This illustrates that the persistent Brugada-type ECG can be present with normal longevity. Key words: Arrhythmia, Brugada syndrome, sudden death.
Case Report
Brugada syndrome is characterized by ST-segment elevation in leads V1 to V3 associated with arrhythmic death. It may be familial. A genetic defect has been found to be present in SCN5A gene in a minority of patients. Infrequently, the Brugada electrocardiogram (ECG) pattern may be observed as an incidental finding. This poses the problem of determining whether the ECG pattern may be a marker of lethal arrhythmic events in the individual patient. Although the prognosis in patients with idiopathic Brugada-type ECG pattern is still debated, it is instructive to report the case of an 85-year-old asymptomatic man with a Brugadatype ECG pattern.
An 85-year-old white man presented to the emergency department because of mental status change and fever that was found to be due to pneumonia. He was disoriented and agitated. His temperature was 39° C, blood pressure was 130/80 mm Hg, heart rate was 100 beats per minute, and respiratory rate was 18 per minute. His cardiovascular examination was normal. There was no evidence of congestive heart failure. Chest examination revealed rales in the right lower lung field. An ECG showed sinus rhythm with marked ST segment elevation of a coved pattern in V1 through V3 (Fig. 1). A right bundle branch block configuration was observed in V1 and V2. The ECGs obtained 5 and 1 year prior to this admission demonstrated the same pattern. A transthoracic echocardiography showed normal wall motion in both right and left ventricles with a left ventricular ejection fraction of 70%. Mild left ventricular diastolic dysfunction was noted. There was no significant valvular abnormality. Chest X-ray showed right lower lobe infiltration consistent with pneumonia. Troponin I on 2 occasions, electrolytes, and renal function were all
From the Department of Medicine, University of Arizona Medical Center, Sarver heart Center, College of Medicine, Tucson, AZ. Reprint requests: Ming-He Huang, MD, PhD, University of Arizona Medical Center, Sarver Heart Center, 1501 N. Campbell Ave. Tucson, AZ 85724; e-mail: [email protected]. © 2004 Elsevier Inc. All rights reserved. 0022-0736/04/3702-0006$30.00/0 doi:10.1016/j.jelectrocard.2004.01.007
109
110 Journal of Electrocardiology Vol. 37 No. 2 April 2004
Fig. 1. An ECG shows sinus rhythm and left axis deviation with marked coved ST-segment elevation in V1 and V2. A right bundle branch block configuration was observed in V1 and V2.
within normal limits. He was treated with antibiotics with resolution of his fever and amelioration of his mental function and was discharged. His past medical history included dementia. He had no history of heart disease, presyncope, or syncope. His only medication was Prozac (Eli Lilly; Florida). There was no family history of sudden death.
Discussion To our knowledge, this is the oldest documented case with a persistent Brugada-type ECG pattern without clinical symptoms. The Brugada syndrome is characterized by 3 types of ECG patterns associated with syncope and/or sudden death (1). This patient’s ECG showed a typical type-I Brugada ECG pattern that is characterized by right bundle branch block morphology and coved ST segment elevation in leads V1 to V3 with negative T waves. In the Brugada syndrome the mean age at which symptoms first occur is in the third or the fourth decade. However, the initial symptom can appear as late as age of 77 (2). It has been postulated that the Brugada-type ECG pattern, per se, is a marker of the risk of arrhythmic death regardless of the presence or absence of clinical symptoms since the initial symptom might occur later in life (2,3). The
incidence of asymptomatic Brugada ECG pattern ranges from 0.02% to 0.7% in large population samples (4). The prognosis in asymptomatic patients with Brugada ECG is controversial. Brugada et al (5). reported 6 of 22 (27%) asymptomatic patients developed sudden death or aborted ventricular fibrillation during 34 ⫾ 32 months of follow-up. Age, sex, family history, and inducibility of arrhythmias were not helpful or stratifying the risk of sudden death. Persistent ST-segment elevation of V1-V3 in healthy individuals was initially reported by Osher and Wolff. (6) They described the ECG pattern in 3 men between the ages of 37 to 40 who remained well during the follow-up periods of up to 3 years. Several recent studies with a relative longterm follow-up (up to 7 years) indicated that the risk for developing malignant arrhythmia, syncope or sudden death in asymptomatic patients was low (4,7–9). None of the patients in these studies had syncope or sudden death.(4,9) The risk of sudden death in the long-term follow-up is not known since symptoms may not appear for many years after the ECG pattern is recognized.(2) Nevertheless, the persistent Brugada-type ECG can be present with normal longevity as reported in this case. The Brugada-type of ST-segment elevation has been suggested to be associated with fever, (10)
Idiopathic Brugada-type ECG •
hyperkalemia, (11) drugs including crack cocaine and overdose of tricyclic antidepressants.(12,13) It may also be related to a variety of right ventricular pathological processes including arrhythmogenic right ventricular dysplasia (14). Mediastinal tumor compressing the right ventricular free wall and hemopericardium of tumor compressing the right ventricle may also induce the ECG changes (15,16). Our patient had none of the above conditions. His previous ECGs as well as the ECG after he became afebrile during the hospitalization displayed the same Brugada-type patterns. The challenge is to be able to properly categorize younger individuals with the Brugada ECG pattern who may have a risk of sudden cardiac death. The highest risks are the individuals with Brugada ECG pattern who have the persistent ECG pattern and have a history of syncope or have been resuscitated from cardiac arrest. Family members with the persistent ECG pattern also are at risk. The asymptomatic individual who does not have a family history of premature sudden death and who has a persistent Brugada ECG pattern is at lower risk. The use of electrophysiological testing is controversial but the data suggests that it is not reliable to indicate patients at high risk (7,8).
5.
6.
7.
8.
9.
10.
11.
12.
References 13. 1. Wilde AAM, Antzelevitch C, Borggrefe M, et al: Proposed diagnostic criteria for the Brugada syndrome (consensus report). Eur Heart J 23:1648, 2002 2. Brugada P, Brugada J: Right bundle branch block, persistent ST segment elevation and sudden cardiac death: A distinct clinical and electrocardiographic syndrome: A multicenter report. Am J Coll Cardiol 20:1391, 1992 3. Brugada P, Brugada J, Brugada R: The Brugada syndrome. ANE 5:88, 2002 4. Littmann L, Monroe MH, Kerns WP, et al: Brugada syndrome and “Brugada sign”: Clinical spectrum
14.
15.
16.
Huang and Marcus 111
with a guide for the clinician. Am Heart J 145:768, 2003 Brugada J, Brugada R, Brugada P: Right bundlebranch block and ST-segment elevation in leads V1 through V3: A marker for sudden death in patients without demonstrable structural heart disease. Circulation 97:457, 1998 Osher HL, Wolff L: Electrocardiographic pattern simulating acute myocardial injury. Am J Med Sci 226: 541, 1953 Morita H, Takenaka-Morita S, Fukushima-Kusano K, et al: Risk stratification for asymptomatic patients with Brugada syndrome. Circ J 67:312, 2003 Priori SG, Napolitano C, Gasparini M, et al: Natural history of Brugada syndrome: Insights for risk stratification and management. Circulation 105:1342, 2002 Takenaka S, Kusano KF, Hisamatsu K, et al: Relative benign clinical course in asymptomatic patients with Brugada-type electrocardiogram without family history of sudden death. J Cardiovasc Electrophysiol 12:2, 2001 Saura D, Garcia-Alberola A, Carrillo P, et al: Brugada-like electrocardiographic pattern induced by fever. PACE 25:856, 2002 Ortega-Carnicer J, Benezet J, Ruiz-Lorenzo F, et al: Transient Brugada-type electrocardiographic abnormalities in renal failure reversed by dialysis. Resuscitation 55:215, 2002 Littmann L, Monroe MH, Svenson RH: Brugada-type electrocardiographic pattern induced by cocaine (review). Mayo Clinic Proc 75:845, 2000 Goldgran-Toledano D, Sideris G, Kevorkian JP: Overdose of cyclic antidepressants and the Brugada syndrome. N Engl J Med 346:1591, 2002 Corrado D, Basso C, Buja G, et al: Right bundle branch block, right precordial st-segment elevation, and sudden death in young people. Circulation 103: 710, 2001 Tarin N, Farre J, Rubio JM, et al: Brugada-like electrocardiographic pattern in a patient with a mediastinal tumor. PACE 22:1264, 1999 Tomcsanyi J, Simor T, Papp L: Images in cardiology. Haemopericardium and Brugada-like ECG pattern in rheumatoid arthritis. Heart 87:234, 2002