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High Risk AAOCA Subtypes for SCD
- 1. Sudden Cardiac Death and Aborted SCD in Patients with Anomalous Aortic Origin of a Coronary Artery (AAOCA): A Comprehensive Review of the Literature Dr Hunain Shiwani, BMBS Department of Radiology Leeds Teaching Hospitals NHS Trust 12th March 2018
- 2. Disclosures • No financial relationships or conflicts of interest.
- 3. Background • Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital abnormality (prevalence < 1%) 1. • AAOCA often presents as incidental finding on imaging. • Sudden cardiac death (SCD) may occur in patients with ‘high-risk’ AAOCA. 1 Cheezum MK, et al. JACC. 2017;69(12):1592-608.
- 4. Coronary Anomalies RC A R A Abnormal DestinationAbnormal CourseAbnormal Origin 1. Bridge 2. Subendocardial course 3. Coronary crossing 4. AAOCA subtypes 1. Fistula 2. Anastomotic vessels 3. Inadequate arteriolar / capillary ramifications Abnormal Size or Number of Vessels 1. Absent, hypoplastic or atretic vessel 2. Ectasia or aneurysm 3. Split or duplication vessel 1. Abnormal takeoff 2. Origin outside aorta 3. Absent/multiple ostia 4. Ostia dimple 5. AAOCA from opposite sinus
- 5. Coronary Anomalies Abnormal DestinationAbnormal CourseAbnormal Origin 1. Bridge 2. Subendocardial course 3. Coronary crossing 4. AAOCA subtypes 1. Fistula 2. Anastomotic vessels 3. Inadequate arteriolar / capillary ramifications Abnormal Size or Number of Vessels 1. Absent, hypoplastic or atretic vessel 2. Ectasia or aneurysm 3. Split or duplication vessel 1. Abnormal takeoff 2. Origin outside aorta 3. Absent/multiple ostia 4. Ostia dimple 5. AAOCA from opposite sinus
- 6. Prepulmonic Interarterial Subpulmonic Retroaortic Retrocardiac AAOCA Classification 1 Cheezum MK, et al. JACC. 2017;69(12):1592-608. (Wraparound not shown)
- 7. Not Intramural Intramural Intramural Course 2 Cheezum MK, et al. EHJ CVI. 2017;18:224-35.
- 8. • To date, no summary of evidence in AAOCA-related SCD. • We aimed to provide a comprehensive review of the literature, to describe AAOCA and patient characteristics with potential risk of SCD. Aims
- 9. Methods • Cochrane, MEDLINE, EMBASE, PubMed, Google Scholar • Included: SCD or aborted SCD (cardiac arrest requiring resuscitation) attributed to AAOCA • Excluded: Other cause for SCD/aSCD; Abstract only • All studies independently reviewed by 2 authors
- 10. Results 4676 studies screened 131 studies with individual data (+15 group studies / no individual data) (+35 non-English cases awaiting translation) 4495 excluded
- 11. Age of SCD or Aborted SCD Attributed to AAOCA Age (years) 0 20 40 60 80 #SCDorAbortedSCD 020406080 200 total (73 aborted, 127 SCD*) 71% male Median: 17 years [IQR: 13-24] * 98% with autopsy
- 12. AAOCA Anatomy in SCD/Aborted SCD Patients #SCDorAbortedSCD 0 20 40 60 80 100 Left main (n=116, 57%) RCA (n=78, 39%) LCX (n=5, 2%) LAD (n=3, 1%) * 2 with multiple AAOCA
- 13. Intramural Course Pathology 3CTA 3 Thiene G, Ho SY. Appl. Pathol. 1986.
- 14. Intramural Course in SCD/Aborted SCD Patients #Cases(whendescribed*) 0 20 40 60 80 Intramural (n=75, 82%) Not Intramural (n=16, 18%) * Data not available in 109 cases
- 15. Intramural Course in SCD/Aborted SCD Patients #Cases(whendescribed*) 0 20 40 60 80 Intramural (n=75, 82%) Not Intramural (n=16, 18%) 96% intramural * Data not available in 109 cases
- 16. SCD/Aborted SCD Related to Exercise Exercise (80%) At rest Other 12% 8% • Exercise (n=142, 80%) • At rest (n=21, 12%) • Other (n=14, 8%): - 12 babies (in crib, crying spell) - 2 adults w/ emotional distress * Data not available in 23 cases
- 17. Cardiac Symptoms 66% 43% 0% 20% 40% 60% 80% 100% ^ 8 reported multiple symptoms; * 41 omitted (no data) Symptoms prior to OR on day of event Prior symptoms %WithSymptoms* In patients with symptoms^: - 45% syncope/presyncope - 41% chest pain - 10% dyspnea - 9% palpitations
- 18. Cardiac Testing Echo Before SCD/aSCD After aSCD Total Missed AAOCA Missed AAOCA Found AAOCA 426 21 15 Stress Test Before SCD / aSCD After aSCD Total Normal Abnormal Normal 1310 2 1 64% Negative (n=27/42) 85% Negative (n=11/13)
- 19. Aborted SCD – How was AAOCA Initially Diagnosed*? Angio - 47% CTA - 30% * Unknown in 2% MRI - 3% Echo - 18% • CTA used to improve visualization after 43% of Echo cases • CTA used to improve visualization after 53% of Angio cases
- 20. Aborted SCD – How was AAOCA Initially Diagnosed*? Angio - 47% CTA - 30% * Unknown in 2% MRI - 3% Echo - 18% • CTA used to improve visualization after 43% of Echo cases • CTA used to improve visualization after 53% of Angio cases
- 21. Follow-Up After Aborted SCD (n=73) • Follow-up: 12 [6-24] months* • 1 early death (<30d), 1 late death (4 years), 1 repeat aborted SCD, 1 VT • Initial Management (available in 96%)#: * When duration described Surgical Repair ICD Conservative^ PCI Unknown 82% 8% 5% 5% 4% ^ Medical Rx & Exercise Restrict # 3 PCI + Surgery, 1 ICD + Surgery Unroofing 35% Reimplant 33% CABG 25% 7% Unknown 0% 100%
- 22. Limitations • Publication bias; no control arm • Potential for unrecognized causes of SCD (i.e. channelopathy) • Patient charts and direct pathology not available • Symptom history not standardized / recall bias • Limited follow-up in patients with aborted SCD
- 23. • Young patients (<40 yrs) with interarterial ± intramural ALCA/ARCA have highest reported risk of SCD among AAOCA subtypes. Conclusions
- 24. • Young patients (<40 yrs) with interarterial ± intramural ALCA/ARCA have highest reported risk of SCD among AAOCA subtypes. • Majority of SCD cases related to exercise (80%). Conclusions
- 25. • Young patients (<40 yrs) with interarterial ± intramural ALCA/ARCA have highest reported risk of SCD among AAOCA subtypes. • Majority of SCD cases related to exercise (80%). • Many (66%) had symptoms, including 43% prior to the day of SCD. Conclusions
- 26. • Young patients (<40 yrs) with interarterial ± intramural ALCA/ARCA have highest reported risk of SCD among AAOCA subtypes. • Majority of SCD cases related to exercise (80%). • Many (66%) had symptoms, including 43% prior to the day of SCD. • Long-term studies needed to examine AAOCA prognosis, optimal testing, and risk/benefit of treatment options. Conclusions
- 27. Acknowledgements • Dr Michael Cheezum – Ft. Belvoir Community Hospital, Alexandria VA • Dr Julie Brothers - Children’s Hospital of Philadelphia, Philadelphia PA • Dr Todd Villines - Walter Reed Military Med. Center, Bethesda MD • Dr Richard Liberthson - Massachusetts General Hospital, Boston MA • Dr Ron Blankstein - Brigham and Women’s Hospital, Boston MA • Dr Maria Illina - Royal Hospital for Children, Glasgow • Dr Pierre Aubry - Hôpitaux de Paris, Paris, France
Editor's Notes
- Good morning session chairs and ACC delegates, and thank you for the opportunity to be here. I am Dr Hunain Shiwani from Leeds in the UK and it is my pleasure to present our comprehensive literature review of sudden cardiac death and aborted sudden death in patients with anomalous aortic origin of the coronary artery.
- I have no conflicts of interest.
- Anomalous coronary arteries are rare, with an observed prevalence of less than 1%. While many patients are discovered to have a coronary anomaly as an incidental finding on cardiac imaging, available evidence has demonstrated a potential risk of sudden cardiac death in patients with high-risk subtypes.
- To review, coronary anomalies may be broadly classified as anomalous in their origin, course, destination or the size or the number of vessels.
- In this study, we will focus specifically on anomalous aortic origin of a coronary artery -- or AAOCA -- from the inappropriate sinus of Valsalva.
- AAOCA may arise from the right cusp, left cusp or rarely the noncoronary cusp; and may be further categorized by 1 of 5 course subtypes: prepulmonic, interarterial, subpulmonic, retroaortic and retrocardiac. Additionally, a rare 6th wraparound subtype may arise as a distal collateral from a single coronary artery.
- AAOCA may also have have an early intramural course, as seen here on cardiac CT, where the proximal vessel runs within the aortic wall at its take-off and this results in a slit-like narrowing.
- While reports and case series have examined sudden death in AAOCA patients, to date there has been no summary of sudden death literature in this area. In this study, we aimed to provide a review of the literature and to describe AAOCA and patient characteristics that may confer an increased risk of sudden death.
- For our study we reviewed the major search indeces to identify all published cases of sudden death or aborted sudden death attributed to AAOCA. Two authors independently reviewed studies, excluding cases with another recognized pcause of sudden death.
- Over 4,000 records were screened, identifying 131 full text studies with individual patient data. Another 15 studies had group data only, and we are awaiting translation of 35 foreign papers. In this presentation we will focus on the analysis of the individual patient data
- Among 200 total cases we identified 73 cases of aborted sudden death, and 127 sudden deaths including 98% with autopsy. Patients were predominantly male, with age distribution as demonstrated on the graph and a median age of 17 years. Note that the reported rate of sudden death in patients above the age of 40 is rare, although the range here includes a case in utero, and up to the 6th decade of life.
- In this slide we describe the rate of sudden death or aborted sudden death across course subtypes. The y-axis represents number of patients and note that the vast majority of cases occurred in patients with an interarterial right coronary or a left main, with only rare case reports in the remaining course subtypes.
- The presence of an early intramural course with slit like narrowing has also been described as a potential high risk feature, as demonstrated here on cardiac CT and by direct pathology.
- To summarize available intramural data, this slide demonstrates the reported presence or absence of an intramural course across course subtypes. … [Advance to next slide]
- Notably, in patients with an interarterial course, the vast majority were also found to have an early intramural course.
- Sudden death also appears to have a strong correlation with exercise – as 80% of patients suffered sudden death or aborted sudden death related to physical activity. We categorized adults with emotional distress and babies with sudden death, possibly related to crying spells, as ‘other’, while another 12% of cases occurred at rest.
- As recognition of these patients remains challenging, it is important to note whether symptoms may offer an opportunity to detect AAOCA ante-mortem. Indeed, a majority of patients had symptoms, including 43% with symptoms prior to the day of their event. In these cases, many patients did in fact describe a history of either exertional syncope, presyncope, chest pain, dyspnea or palpitations.
- Multiple non-invasive tests are available to detect and risk stratify AAOCA patients. Unfortunately, echo was unable to recognize 64% of AAOCA vessels, and stress testing was negative in 85% of cases, including 10 normal studies prior to sudden or aborted sudden death. Consequently, a normal stress test cannot be viewed as reassuring in patients with potential high risk anatomic findings.
- In the majority of cases, invasive angiography and CTA were used to detect AAOCA.
- Additionally, coronary CT was used to improve AAOCA visualization in nearly half of cases initially discovered on echo and catheterisation.
- While the amount of available follow-up data was limited, the initial outcomes DO appear favorable with only 2 deaths, 1 aborted SCD and 1 stable VT episode in follow-up after initial management. The majority of cases were managed surgically by a range of approaches including unroofing, reimplantation, and CABG.
- We recognized several limitations to our study. First, the potential for publication bias remains, and our study has no control arm of healthy patients for comparison. Consequently, ability of patient and AAOCA features to predict sudden death remains unknown. Additionally, the true incidence of sudden death and prevalence of AAOCA in the general population is uncertain. While autopsy was available in 98% of sudden death cases, potential remains for unrecognized causes of sudden death such as an ion channelopathy. Patient charts and individual pathology was not available, and symptom histories were limited to details provided in case reports and therefore subject to inherent recall bias. Further, follow-up remains limited in patients after aborted sudden death – limiting our ability to understand the impact of initial management.
- Nevertheless, recognizing these limitations, our study represents the first comprehensive summary of sudden death literature in AAOCA patients, demonstrating that, Number 1, young patients under 40 years of age with an interarterial and intramural anomalous left or anomalous right coronary artery have the highest reported risk of sudden death among AAOCA subtypes.
- Number 2, sudden death appears to be primarily related to exertion in the large majority of patients.
- Number 3, a majority of patients have symptoms, including nearly 40% prior to the day of sudden death – which may aid recognition of these cases ante-mortem.
- Lastly, while our study does recognize patient and AAOCA features that may increase the risk of sudden death, future studies are needed to improve our understanding of AAOCA prognosis and the optimal testing and management of these complex patients.
- I would like to acknowledge my co-authors and contributors to this study, and thank you for the opportunity to present. I am happy to take any questions.