Brugada Syndrome: Focus for the General Pediatrician
Brugada Syndrome is an “inherited” channelopathy characterized by a predisposition to syncope and sudden death. It typically presents in young adults but is also known to affect the pediatric population, even if the prevalence is low compared to the adult population. The diagnostic ECG pattern shows...
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| Format: | Article |
| Language: | English |
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MDPI AG
2024-02-01
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| Series: | Children |
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| Online Access: | https://www.mdpi.com/2227-9067/11/3/281 |
| _version_ | 1797241667352264704 |
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| author | Alessia Speranzon Daniela Chicco Paolo Bonazza Raffaele D’Alfonso Marco Bobbo Biancamaria D’Agata Mottolese Egidio Barbi Thomas Caiffa |
| author_facet | Alessia Speranzon Daniela Chicco Paolo Bonazza Raffaele D’Alfonso Marco Bobbo Biancamaria D’Agata Mottolese Egidio Barbi Thomas Caiffa |
| author_sort | Alessia Speranzon |
| collection | DOAJ |
| description | Brugada Syndrome is an “inherited” channelopathy characterized by a predisposition to syncope and sudden death. It typically presents in young adults but is also known to affect the pediatric population, even if the prevalence is low compared to the adult population. The diagnostic ECG pattern shows coved-type ST-segment elevation in the right precordial leads, occurring spontaneously or after provocative drug tests with IV administration of Class I antiarrhythmic drugs. However, the electrocardiographic findings may vary, and transient or concealed forms of the syndrome further complicate diagnosis, necessitating thorough evaluation and close clinical follow-up. The clinical presentation of Brugada Syndrome may range from asymptomatic individuals to patients who have experienced syncope or sudden cardiac arrest. The syndrome remains underdiagnosed due to its elusive symptoms and the absence of abnormal findings between episodes. Additionally, specific triggers such as fever, certain medications and alcohol consumption may unmask the electrocardiographic changes and provoke arrhythmias in susceptible individuals. Given its elusive nature, early diagnosis and risk stratification are crucial in identifying individuals who may benefit from an implantable cardioverter defibrillator, the mainstay of treatment for high-risk patients, or pharmacological interventions. |
| first_indexed | 2024-04-24T18:26:58Z |
| format | Article |
| id | doaj.art-7f93056ba9814dbbbe042db4023969e6 |
| institution | Directory Open Access Journal |
| issn | 2227-9067 |
| language | English |
| last_indexed | 2024-04-24T18:26:58Z |
| publishDate | 2024-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Children |
| spelling | doaj.art-7f93056ba9814dbbbe042db4023969e62024-03-27T13:31:00ZengMDPI AGChildren2227-90672024-02-0111328110.3390/children11030281Brugada Syndrome: Focus for the General PediatricianAlessia Speranzon0Daniela Chicco1Paolo Bonazza2Raffaele D’Alfonso3Marco Bobbo4Biancamaria D’Agata Mottolese5Egidio Barbi6Thomas Caiffa7Department of Medical, Surgical and Health Sciences, University of Trieste, 34127 Trieste, ItalyInstitute for Maternal and Child Health, IRCCS “Burlo Garofolo”, 34127 Trieste, ItalyGeneral Pediatrician, 58100 Grosseto, ItalyGeneral Pediatrician, 58100 Grosseto, ItalyInstitute for Maternal and Child Health, IRCCS “Burlo Garofolo”, 34127 Trieste, ItalyInstitute for Maternal and Child Health, IRCCS “Burlo Garofolo”, 34127 Trieste, ItalyDepartment of Medical, Surgical and Health Sciences, University of Trieste, 34127 Trieste, ItalyInstitute for Maternal and Child Health, IRCCS “Burlo Garofolo”, 34127 Trieste, ItalyBrugada Syndrome is an “inherited” channelopathy characterized by a predisposition to syncope and sudden death. It typically presents in young adults but is also known to affect the pediatric population, even if the prevalence is low compared to the adult population. The diagnostic ECG pattern shows coved-type ST-segment elevation in the right precordial leads, occurring spontaneously or after provocative drug tests with IV administration of Class I antiarrhythmic drugs. However, the electrocardiographic findings may vary, and transient or concealed forms of the syndrome further complicate diagnosis, necessitating thorough evaluation and close clinical follow-up. The clinical presentation of Brugada Syndrome may range from asymptomatic individuals to patients who have experienced syncope or sudden cardiac arrest. The syndrome remains underdiagnosed due to its elusive symptoms and the absence of abnormal findings between episodes. Additionally, specific triggers such as fever, certain medications and alcohol consumption may unmask the electrocardiographic changes and provoke arrhythmias in susceptible individuals. Given its elusive nature, early diagnosis and risk stratification are crucial in identifying individuals who may benefit from an implantable cardioverter defibrillator, the mainstay of treatment for high-risk patients, or pharmacological interventions.https://www.mdpi.com/2227-9067/11/3/281Brugada syndromesudden cardiac deathBrugada patternarrhythmiapediatric population |
| spellingShingle | Alessia Speranzon Daniela Chicco Paolo Bonazza Raffaele D’Alfonso Marco Bobbo Biancamaria D’Agata Mottolese Egidio Barbi Thomas Caiffa Brugada Syndrome: Focus for the General Pediatrician Children Brugada syndrome sudden cardiac death Brugada pattern arrhythmia pediatric population |
| title | Brugada Syndrome: Focus for the General Pediatrician |
| title_full | Brugada Syndrome: Focus for the General Pediatrician |
| title_fullStr | Brugada Syndrome: Focus for the General Pediatrician |
| title_full_unstemmed | Brugada Syndrome: Focus for the General Pediatrician |
| title_short | Brugada Syndrome: Focus for the General Pediatrician |
| title_sort | brugada syndrome focus for the general pediatrician |
| topic | Brugada syndrome sudden cardiac death Brugada pattern arrhythmia pediatric population |
| url | https://www.mdpi.com/2227-9067/11/3/281 |
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