Author + information
- Received August 29, 2018
- Revision received November 27, 2018
- Accepted November 27, 2018
- Published online April 15, 2019.
- Gherardo Finocchiaro, MDa,
- Elijah R. Behr, MA, MBBSa,
- Gaia Tanzarella, MDa,b,
- Michael Papadakis, MBBS, MDa,
- Aneil Malhotra, BSc, MBChB, PhDa,
- Harshil Dhutia, BSc, MBBSa,
- Chris Miles, MBBSa,
- Igor Diemberger, MD, PhDb,
- Sanjay Sharma, BSc, MBChB, MDa and
- Mary N. Sheppard, MBBCh, BAO, BSc, MDc,∗ ()
- aCardiovascular Sciences Research Centre, St. George's, University of London, London, United Kingdom
- bIstituto di Cardiologia, Ospedale Sant’Orsola-Malpighi, Alma Mater Studiorum University of Bologna, Bologna, Italy
- cCardiovascular Pathology Department, St. George's, University of London, London, United Kingdom
- ↵∗Address for correspondence:
Dr. Mary N. Sheppard, Cardiology Clinical and Academic Group, St. George’s University of London, Cranmer Terrace, London SW17 0RE, United Kingdom.
Objectives This study sought to describe the clinical and pathological features of anomalous origin of a coronary artery (AOCA) in sudden cardiac death (SCD) victims.
Background AOCA from the inappropriate sinus of Valsalva or from the pulmonary artery is increasingly diagnosed with current imaging techniques. AOCA is a possible cause of SCD.
Methods We reviewed a database of 5,100 consecutive cases of SCD referred to our specialist cardiac pathology center between January 1994 and March 2017 and identified a subgroup of 30 cases (0.6%) with AOCA. All cases underwent detailed post-mortem evaluation including histological analysis by an expert cardiac pathologist. Clinical information was obtained from referring coroners.
Results The mean age was 28 ± 16 years and 23 individuals were male (77%). In 8 cases (27%), SCD occurred before 18 years of age. Cardiac symptoms were present in 11 individuals (37%), and syncope was the most common (n = 6, 20%). Anomalous left coronary artery arising from the right sinus of Valsalva (ALCA) with interarterial course (n = 11) and anomalous right coronary artery arising from the left sinus of Valsalva (ARCA) with interarterial course (n = 11) were the most common found. ALCA arising from pulmonary artery was present in 7 cases, whereas in 1 case, the left coronary artery arose from the noncoronary cusp. Left ventricular fibrosis was reported in 11 cases (37%) and was mainly subendocardial. There was evidence of acute infarction in 2 cases. Death occurred during exercise or emotional stress in 15 (50%) cases. The AOCA variant where death occurred more frequently during physical activity was ALCA (8 of 11, 73%), followed by ALCA arising from pulmonary artery (4 of 7, 57%) and ARCA (2 of 11, 18%).
Conclusions AOCA is a rare cause of SCD. ALCA and ARCA with interarterial course are the most common anatomical variants recognized at the postmortem of SCD victims. ALCA is more commonly associated with death during exercise. Cardiac arrhythmias causing sudden death seem most likely in the cases without overt myocardial damage.
Dr. Finocchiaro is funded by Cardiac risk in the Young and the Charles Wolfson Charitable Trust. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
All authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC: Clinical Electrophysiology author instructions page.
- Received August 29, 2018.
- Revision received November 27, 2018.
- Accepted November 27, 2018.
- 2019 American College of Cardiology Foundation
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