Introduction to Anomalous Aortic Origin of a Coronary Artery (AAOCA) Introduction to Anomalous Aortic Origin of a Coronary Artery (AAOCA)

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1 Introduction to Anomalous Aortic Origin of a Coronary Artery (AAOCA) Julie Brothers, MD Director, Lipid Heart Clinic Children s Hospital of Philadelphia Assistant Professor of Pediatrics Perelman School of Medicine at the University of Pennsylvania Introduction to Anomalous Aortic Origin of a Coronary Artery (AAOCA) Symposium on Coronary Artery Anomalies 2014 December 4, 2014 Insert any picture or image you like here Julie Brothers, M.D. Assistant Professor, Pediatric Cardiology The Children s Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania 1

2 I have no disclosures Disclosures Objectives To recognize the different coronary anomaly courses and identify those believed to confer a higher risk of sudden cardiac death in the young To characterize the potential mechanisms of ischemia To understand the different challenges practitioners encounter with the diagnosis and management of AAOCA 2

3 Normal Coronary Anatomy Right Coronary Artery (RCA) Image courtesy of Robin Smitheus, Tineke Willems, and the Radiology Assistant 3

4 Left Main Coronary Artery (LMCA) Image courtesy of Robin Smitheus, Tineke Willems, and the Radiology Assistant Normal Coronary Arteries Perpendicular Round orifice Image courtesy of Robin Smitheus, Tineke Willems, and the Radiology Assistant 4

5 Virtual Angioscopy Definition of Coronary Anomalies What is normal and what is abnormal or anomalous? According to Angelini, anything that is found in more than 1% of the general popula8on should be considered a normal variant. Angelini P. Circulation 2007;115:

6 Coronary Anomalies Most coronary abnormalities of number, origin, and course are of intellectual interest only Practitioners ideally need to Recognize which variant may result in myocardial ischemia and increased risk of sudden cardiac death (SCD) Diagnose the pa8ent before SCD occurs What is AAOCA? Anomalous Aortic Origin of a Coronary Artery When both coronary arteries arise from the same aortic sinus with a single ostium or two separate os-tia On occasion, the aberrant vessel arises above the inappropriate sinus or the commissure, not truly from the sinus itself 6

7 Course of Coronary and SCD Risk: Pre-pulmonic Anomalous aortic origin of the left main coronary artery (ALCA-R) or right coronary artery (ARCA-L) can course Anterior to right ventricular ouhlow tract (prepulmonic) Likely benign Anterior to Right Ventricular Outflow Tract Likely Benign 7

8 Course of Coronary and SCD Risk: Retroaortic Anomalous aortic origin of the left main coronary artery (ALCA-R) or right coronary artery (ARCA-L) can course Posterior to the aorta (posterior/retroaor8c) Usually benign Rare case reports of ischemia with ALCA- R Kejiriwil NK 2004; Murphy DA 1978 Retroaortic Usually Benign 8

9 Course of Coronary and SCD Risk: Intraseptal Anomalous aortic origin of the left coronary artery (ALCA-R) Course caudad to the pulmonary valve, behind the RVOT, through the conal septum (intraseptal or intraconal or intramyocardial) LMCA or only LAD can course intraseptal May arise as a branch of the RCA ( common trunk ) Os8um is remote and dis8nct from the septal commissure Likely benign Intraseptal Course: ALCA-R RCA LCA RCA RVOT Conal septum PA LAD AO Circ Ao LV Conal septum LCA LAD Circ Courtesy of Matt Harris, MD 9

10 Intraseptal LAD Courtesy of Matt Harris, MD Course of Coronary and SCD Risk: Interarterial Anomalous aortic origin of the left main coronary artery (ALCA-R) or right coronary artery (ARCA-L) can course Interarterial and oren intramural Anomalous coronary os8um located juxtacommissural or just above the commissure Subtype most commonly associated with increased risk of SCD ALCA- R>>ARCA- L Cheitlin MD et al. Circulation. 1974;50:780-7;.Kragel AH et al. Am J Cardiol. 1988;62: Harris MA et al. Circulation 2011; 124:A16138 (oral abstr). 10

11 Interarterial and Intramural ARCA-L ALCA-R Malignant ECHO: Interarterial ARCA-L PA Ao Anomalous RCA Left Main CA 11

12 MRI: Interarterial ARCA-L Cardiac MR: Interarterial ALCA-R 12

13 Myocardial Ischemia and SCD One or several factors leads to: Coronary occlusion or compression Myocardial ischemia Ventricular tachycardia or fibrilla8on Likely cumulative over time Ischemia is intermittent Most common during or just after intense exercise Potential Mechanisms of Ischemia Intramural course of proximal anomalous coronary Stretched out/flaxened as aorta dilates with exercise Hill & Sheppard 2013: 2304 cases of SCD, 18 year period 17 AAOCA (0.7%): 10 ARCA- L and 7 ALCA- R Mean age of SCD: ARCA- L 31yr (5-63); ALCA- R 15yr (11-19) All had interarterial course 8 intramural and 7 with ellip8cal orifice Acute angle take-off Majority of cases of sudden death in a large autopsy series had acute angle take- off but not those with single coronary os8um Taylor et al., J Am Coll Cardiol 1992; Basso et al., J Am Coll Cardiol 2000; Frescura et al., Hum Pathol 1998; Kragel et al., Am J Cardiol 1988;Hill & Sheppard Br J Sports Med 2014 ;48:

14 MRI Virtual Angioscopy or Coronary Fly-Through ARCA-L Slit-like Commissure Round LCA Courtesy of Matt Harris, MD Potential Mechanisms of Ischemia In adults, intravascular ultrasound (IVUS) Hypoplasia and lateral luminal compression of the proximal coronary by the aorta Compression varies with cardiac cycle Worse in systole Greater compression during exercise Flow restriction through pericommissural area that is relatively non-compliant Interarterial AAOCA commonly arises near intercoronary commissure Angelini P J Invasive Cardiol. 2003;15:507-14; Penalvier BMC Cardiovasc Disord. 2012;12:

15 Challenges with AAOCA Diagnosis Physical examination and ECG almost always normal Multiple ways patients present Asymptoma8c: murmur; family history of SCD, AAOCA, or congenital heart disease; abnormal ECG Cardiac- type symptoms: chest pain, palpita8ons, dizziness, syncope at rest or with exercise SCD or SCA Best modality for confirming diagnosis? Best way to screen for AAOCA? Brothers JA et al. Pediatr Cardiol. 2009;30:

16 Risk Stratification Ideally, when a patient is diagnosed, we could risk stratify to high- or low-risk group High- risk group would be referred for surgery or exercise restric8on Low- risk group would be poten8ally cleared for compe88ve athle8cs Risk Stratification: Symptoms Cardiac-type symptoms, such as chest pain, palpitations, dizziness and syncope are frequent in children in general as well as in AAOCA Poynter et al. Syncope with exercise posi)vely associated with interarterial ALCA- R, nega)vely associated with interarterial ARCA- L Associa8on is not causa8on Correla8ons expected Poynter JA et al. World J Pediatr Congenit Heart Surg. 2014;5:

17 Risk Stratification: Anatomy AAOCA course Interarterial highest risk for SCD Features thought to contribute to high-risk anatomy Slit- like orifice, acute angle of take- off, intramural course, os8al ridge, vessel spasm, intussuscep8on, non- compliant pericommissural area Taylor et al looked at 30 hearts with interarterial ALCA- R and ARCA- L No unifying characteris8c with increased risk of SCD Age > 30 years decreased SCD risk Taylor AJ et al., Am Heart J. 1997;133: Risk Stratification: IVUS Angelini et al: IVUS in adults showed certain factors correlated with clinical severity Amount of hypoplasia of intramural por8on Amount of lateral compression of intussuscepted segment Lateral compression during exercise Use of IVUS limited in pediatrics Invasive Catheter size Lack of experience Angelini P. Catheter Cardiovasc Interv 2007;69:

18 Risk Stratification: Exercise Tests Unreliable in assessing for ischemia Posi8ve predic8ve value is low in children More useful if used with an imaging study Basso et al. autopsy series Young athletes, mean age=16 yr 23 ALCA- R, 4 ARCA- L All events exer8onal 6 with EST normal < 6 months before SCD Basso et al. J Am Coll Cardiol. 2000;35: Exercise Test #1 Brothers J, et al. J Thorac Cardiovasc Surg

19 Exercise Test #2 Risk Stratification: Exercise Stress Test Research An off protocol exercise test may be helpful in reproducing symptoms and/or ischemia Longer 8me on treadmill and/or mul8ple sprint efforts Successful at CHOP with elici8ng symptoms Research project being developed to further evaluate Look at maximal VO2 (oxygen consumption) in patients with SCD or evidence of ischemia Does higher VO2 (increased stroke volume) correlate with increased risk of ischemia? 19

20 Risk Stratification: MBF and CFR Quantify myocardial blood flow (MBF) and coronary flow reserve (CFR) using adenosine MRI or PET If there is coronary os8al stenosis, then distal por8on of the anomalous coronary may be maximally dilated at rest Myocardial ischemia may develop with exercise if this vessel cannot dilate further Goal to identify those children with diminished MBF and CFR during adenosine stress testing Management Best treatment option is unknown Notably for the asymptoma8c ARCA- L pa8ent Lack of consistency Significant variability among prac88oners between and within ins8tu8ons Importance of mee8ngs to discuss these differences Use of similar clinical pathways or algorithms Allow for collabora8on of data Brothers JA et al. Pediatr Cardiol. 2009;30:

21 Management of Patients Not Referred for Surgery Figure 4. Management of Patients Awaiting or Not Undergoing Surgery % of Respondents Restrict from exercise Exercise but not competitively Beta-blockers w/o exercise restriction Beta-blockers and cannot exercise competititvely Beta-blockers and restrict all exercise No exercise restrictions and no medical therapy Other Brothers J et al. Pediatr Cardiol : Management: Exercise Restriction Current AHA/ACC guidelines recommend exercise restriction if no surgical repair and prior to surgery Is this always necessary? Is a game of pick up basketball safer than a high school game of basketball Does this encourage a sedentary lifestyle? Does this diminish pa8ent s quality of life (QOL)? Current research study at CHOP to evaluate QOL in those restricted from exercise Graham Jr. TP et al. J Am Coll Cardiol. 2005;45:

22 Management: Unroofing Procedure Most common surgical procedure for interarterial, intramural AAOCA ARCA-L with exertional chest pain, interarterial ALCA-R positively associated with surgery Rare cases of SCD after surgery Presented with collapse/aborted SCD Short- and mid-term complications Mild aor8c insufficiency, aor8c valve replacement, pericardial effusion, ischemic changes on post- opera8ve provoca8ve tes8ng Poynter JA et al. World J Pediatr Congenit Heart Surg. 2014; Nguyen AL, et al, Neth Heart J. Nov 2012; Osaki M et al. Pediatr Cardiol Brothers JA et al. J Am Coll Cardiol. 2007; Romp RL Ann Thorac Surg Wittlieb- Weber C et al. J Thorac Cardiovasc Surg Management: Medical vs Surgical Krasuski et al older adults with interarterial AAOCA Mean age 52.2 years At 10 years of follow- up, no difference in survival between those followed medically vs surgery This is in adults long-term outcome in children and young adults is unknown Krasuski RA et al., Circulation. 2011;123:

23 Management: Surgical Efficacy Risk of post-operative SCD is very low Should be lower than general athle8c popula8on To determine if there is a difference in SCD arer surgery compared to the general athle8c popula8on 5,000 pa8ent years for ALCA- R and 10,000 for ARCA- L All these years need to be doing compe88ve sports To determine if these athletes are safe to play we need to allow them to play Multi-center AAOCA Registry of the Congenital Heart Surgeons Society (CHSS) Summary Most coronary anomalies are benign Do not require surgery or exercise restric8on In the higher risk anomalies, such as interarterial AAOCA, many challenges still remain Diagnosis, risk stra8fica8on, management More research and data are needed Collaboration between centers is essential 23

24 Questions/Comments? 24