Author + information
- Received October 10, 2017
- Revision received December 17, 2017
- Accepted January 8, 2018
- Published online March 12, 2018.
- Michael Papadakis, MBBS, MDa,b,
- Efstathios Papatheodorou, MDa,b,
- Greg Mellor, MBChB, MDa,b,
- Hariharan Raju, MBChB, PhDa,b,
- Rachel Bastiaenen, PhDa,
- Yanushi Wijeyeratne, BMBSa,
- Sara Wasim, BSca,
- Bode Ensam, MBChBa,b,
- Gherardo Finocchiaro, MDa,b,
- Belinda Gray, BSc(Med), MBBS, PhDa,
- Aneil Malhotra, MBBChir, MA, MSC, PhDa,b,
- Andrew D’Silva, MBBSa,b,
- Nina Edwards, BSca,
- Della Cole, RGN BSca,
- Virginia Attard, MSca,
- Velislav N. Batchvarov, MD, PhDa,
- Maria Tome-Esteban, MD, PhDa,
- Tessa Homfray, MBBSa,
- Mary N. Sheppard, MB, BCh, BAO, MDa,
- Sanjay Sharma, MBChB, MDa,b,∗ ( and )
- Elijah R. Behr, MBBS, MDa
- aCardiology Clinical Academic Group, St. George's, University of London and St. George's University Hospitals NHS Foundation Trust, London, United Kingdom, European Reference Network for rare and low prevalence diseases of the heart, Guard-Heart
- bUniversity Hospital Lewisham, Lewisham, United Kingdom
- ↵∗Address for correspondence:
Dr. Sanjay Sharma, Cardiology Clinical Academic Group, St George’s, University of London, Cranmer Terrace, London SW17 0QT, United Kingdom.
Background Familial evaluation after a sudden death with negative autopsy (sudden arrhythmic death syndrome; SADS) may identify relatives at risk of fatal arrhythmias.
Objectives This study aimed to assess the impact of systematic ajmaline provocation testing using high right precordial leads (RPLs) on the diagnostic yield of Brugada syndrome (BrS) in a large cohort of SADS families.
Methods Three hundred three SADS families (911 relatives) underwent evaluation with resting electrocardiogram using conventional and high RPLs, echocardiography, exercise, and 24-h electrocardiogram monitor. An ajmaline test with conventional and high RPLs was undertaken in 670 (74%) relatives without a familial diagnosis after initial evaluation. Further investigations were guided by clinical suspicion.
Results An inherited cardiac disease was diagnosed in 128 (42%) families and 201 (22%) relatives. BrS was the most prevalent diagnosis (n = 85, 28% of families; n = 140, 15% of relatives). Ajmaline testing was required to unmask the BrS in 97% of diagnosed individuals. The use of high RPLs showed a 16% incremental diagnostic yield of ajmaline testing by diagnosing BrS in an additional 49 families. There were no differences of the characteristics between individuals and families with a diagnostic pattern in the conventional and the high RPLs. On follow-up, a spontaneous type 1 Brugada pattern and/or clinically significant arrhythmic events developed in 17% (n = 25) of the concealed BrS cohort.
Conclusions Systematic use of ajmaline testing with high RPLs increases substantially the yield of BrS in SADS families. Assessment should be performed in expert centers where patients are counseled appropriately for the potential implications of provocation testing.
Dr. Behr has received consulting fees from Medtronic; has received research funding from Biotronik; and has received a research grant from McColl's Ltd. Retail Group and the British Heart Foundation. Drs. Papadakis, Papatheodorou, Mellor, Raju, Ensam, Finocchiaro, Malhotra, and D'Silva were funded by research fellowship grants from a charitable organization called Cardiac Risk in the Young (CRY). Drs. Papadakis, Sheppard, Sharma, and Behr have received research grants from CRY. Dr. Wijeyeratne has received a research fellowship grant from McColl's Ltd. Retail Group. Dr. Raju has received a research fellowship grant from the British Heart Foundation. Drs. Edwards and Batchvarov have received support from the British Heart Foundation. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose. Drs. Papadakis and Papatheodorou contributed equally to this work and are joint first authors. Drs. Sharma and Behr contributed equally to this work and are joint senior authors.
- Received October 10, 2017.
- Revision received December 17, 2017.
- Accepted January 8, 2018.
- 2018 American College of Cardiology Foundation
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