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4.4.7 Coronary Anomalies
Coronary abnormalities are among the most common congenital cardiovascular anomalies, surpassing in prevalence nearly all others combined. Coronary anomalies include anomalous aortic origin of a coronary artery (AAOCA), coronary fistula, and myocardial bridge. Many congenital coronary abnormalities have a benign outcome. In contrast, natural history studies of anomalous coronary artery from the PA (particularly anomalous left coronary artery from the PA) suggest poor outcome in untreated patients; similar natural history studies are lacking regarding untreated patients with AAOCA, but other evidence raises concern. See and for a diagnostic and treatment algorithm for AAOCA.
Table 34
Factors That May Relate to the Clinical Importance of AAOCA and Risk of SCD
Age
AAOCA is more commonly invoked as the cause of SCD in patients <35 y of age than in patients >35 y of age, in whom atherosclerotic coronary disease becomes a more prevalent cause. However, death has been attributed to AAOCA in patients of all ages; there does not seem to be an age beyond which the AAOCA may not be relevant, even in the setting of atherosclerotic coronary disease and other concomitant conditions [1], [2].
Anatomy of coronary ostium and proximal coronary course
Slit-like/fish-mouth-shaped orifice, acute angle takeoff, intramural course, interarterial course and hypoplasia of the proximal coronary artery have all been proposed as reasons for symptoms, ischemia and SCD in patients with AAOCA. The slit-like orifice is more commonly seen in anomalous right coronary artery arising from the left sinus. Each of these anatomic findings offers a pathophysiological mechanism for intermittent ischemia, particularly at times of high cardiac output and/or increased aortic wall tension, such as during exercise [1], [2], [3], [4].
Anomalous origin
Left coronary artery arising from the right cusp is less common than the right coronary artery arising from the left cusp but is more often found in autopsy series of SCD [1], [2], [3]. This suggests that anomalous origin of the left coronary artery from the right cusp is more likely to cause SCD than anomalous origin of the right coronary artery from the left cusp. This may be due either to anatomic features that make anomalous aortic origin of the left coronary artery prone to coronary compromise or because a larger proportion of myocardium is supplied by the left coronary artery, or both.
Exercise
Autopsy series suggest a most patients die during, or in close temporal association with, exercise [1], [2], [3].
Ischemia
Autopsy series demonstrate myocardial fibrosis in a significant number of patients whose deaths were attributed to AAOCA, particularly in patients with anomalous left coronary artery arising from the right cusp (S4.4.7-5). Surgical series describe patients with ischemia or MI before surgical repair in the absence of other CAD, suggesting a relation of the coronary anomaly to the ischemia (S4.4.7-16). This suggests that had perfusion imaging been obtained before SCD, ischemia would have been found in such patients [1], [2]. However, other data indicate that a normal stress test does not preclude a SCD event, with the proviso that most of those studies used only stress ECG, rather than the more sensitive and specific modalities of nuclear perfusion imaging or stress echocardiography. In addition, postoperative studies have shown that ischemia may be found after surgical repair in the distribution not supplied by the abnormal coronary artery and may not persist on repeat testing (S4.4.7-19).
Symptoms
In autopsy and surgical series, a significant number of patients reported cardiovascular symptoms, including before SCD events [1], [2], [3], [4], [5]. Symptoms are more commonly reported in patients in whom the left coronary artery arises from the right sinus. Surgical series have described improvement in symptoms after surgical repair [1], [2], [3], [4], [5], [6].
AAOCA indicates anomalous aortic origin of the coronary artery; CAD, coronary artery disease; ECG, electrocardiogram; MI, myocardial infarction; and SCD, sudden cardiac death.
Figure 5
Anomalous Aortic Origin of the Coronary Artery
*Surgical intervention to involve unroofing or coronary revascularization for patients with concomitant fixed obstruction.
Assessment of the risk of SCD in patients with AAOCA and of the role of AAOCA in causing ischemia or symptoms is difficult because available data do not adequately capture the clinical spectrum of these anomalies. Autopsy series are available that help describe the anomalies found in patients who suffered SCD contrasted to other causes of death [1], [2], [3], [4], [5]. There are surgical case series that describe findings before operation, operative anatomy and postoperative course [1], [2], [3], [4], [5]. There are imaging studies describing the anatomy and potential pathophysiological abnormalities associated with AAOCA [1], [2], [3], [4]. There are surgical series describing improvement in symptoms after operation [1], [2], [3]. There are surveys and registries that describe the heterogeneous management strategies applied to AAOCA [1], [2], [3]. What is lacking are data proving that any particular management strategy prevents SCD. As a consequence, decisions regarding whether surgery is necessary or exercise restriction or medical therapy might be beneficial are all based on synthesizing limited data and applying to an individual patient. Clinicians commonly extrapolate to assist in medical decision-making, but the consequences of being “wrong” for a young patient with AAOCA may be perceived to be greater than for many other conditions. Consequently, there is often a clinical urge to seek a reason to do something like surgical repair, because the available data do not identify clinical features that provide reassurance that a patient is at low risk of cardiovascular events. Unfortunately, evidence demonstrating that surgical repair ameliorates SCD risk, derived from large enough cohorts followed over a sufficient period of time, is not available.
4.4.7.1 Anomalous Coronary Artery Evaluation
Recommendations for Anomalous Coronary Artery Evaluation
Coronary angiography, using catheterization, CT, or CMR, is recommended for evaluation of anomalous coronary artery[1], [2], [3].
I
C-LD
2.
Anatomic and physiological evaluation should be performed in patients with anomalous aortic origin of the left coronary from the right sinus and/or right coronary from the left sinus[1], [2], [3], [4], [5], [6].
Recommendation-Specific Supportive Text
1.
CTA, CMR, and catheterization can all delineate the proximal course of the coronary artery and relationship to other structures. CTA is generally preferred because it has superior spatial and temporal resolution, although CMR may also provide adequate delineation of the relationship of the coronary artery to the aorta, PA and other structures, including whether the proximal course appears to be intramural. Coronary angiography by catheterization can be helpful when there is concern about stenosis in the coronary artery or when concomitant hemodynamic evaluation for shunt assessment or intravascular ultrasonography/flow evaluation is needed.
2.
Assessment of AAOCA is enhanced when the precise anatomy and physiological impact of the coronary artery anomaly are understood. As described in , the specific anomalous origin, anatomy of the orifice and proximal vessel and presence of ischemia may all influence the clinical course and thus the management options. Understanding these issues as precisely as possible will better inform clinical decisions.
4.4.7.2 Anomalous Aortic Origin of Coronary Artery
Recommendations for Anomalous Aortic Origin of Coronary Artery
Surgery is recommended for AAOCA from the left sinus or AAOCA from the right sinus for symptoms or diagnostic evidence consistent with coronary ischemia attributable to the anomalous coronary artery[1], [2], [3].
IIa
C-LD
2.
Surgery is reasonable for anomalous aortic origin of the left coronary artery from the right sinus in the absence of symptoms or ischemia[1], [2], [3].
IIa
C-EO
3.
Surgery for AAOCA is reasonable in the setting of ventricular arrhythmias.
IIb
B-NR
4.
Surgery or continued observation may be reasonable for asymptomatic patients with an anomalous left coronary artery arising from the right sinus or right coronary artery arising from the left sinus without ischemia or anatomic or physiological evaluation suggesting potential for compromise of coronary perfusion (e.g., intramural course, fish-mouth-shaped orifice, acute angle)[1], [2], [3].
Recommendation-Specific Supportive Text
1.
In patients with symptoms related to AAOCA, repair of the anomaly should alleviate symptoms. In autopsy and surgical series, cardiac symptoms are more common in patients with a left coronary artery arising from the right coronary cusp. In autopsy studies of patients who died because of an anomalous coronary artery, fibrosis is a common finding, suggesting that ischemia preceded the terminal event. However, there are patients in whom a SCD event occurred despite normal stress ECG, and consequently absence of ischemia is not reassuring. Autopsy series show that many patients whose death is attributed to anomalous coronary arteries are young, thus management of patients should take age into account, with heightened concern about the risk of sudden death in younger patients [1], [2], [3].
2.
Anomalous left coronary from the right sinus is less common than anomalous right coronary from the left sinus (S4.4.7.2-10), but anomalous left coronary artery from the right is more commonly found in autopsy series of athletes and military recruits who had nontraumatic death than right coronary from the left sinus [1], [2], [3], [4]. The overrepresentation of the anomalous left coronary from the right sinus suggests a higher risk of SCD, particular at extremes of exertion and in patients <35 years of age.
There are some anatomic features that are thought to be associated with increased risk of compromise of coronary flow and/or SCD, including a fish-mouth-shaped or slit-like orifice, or intramural course (S4.4.7.2-14), although the slit-like orifice is more commonly encountered in a right coronary arising from the left cusp. It is difficult to quantitate the absolute risk of SCD associated with anomalous aortic origin of the left coronary from the right sinus, and data demonstrating that surgery ameliorates the SCD risk have not been published. Until studies suggest otherwise, limited data and expert consensus suggest that it is reasonable that adults with this malformation should undergo surgical unroofing unless there are extenuating circumstances that would make surgery high risk.
3.
In patients with ventricular arrhythmias presumed related to ischemia caused by anomalous origin of a coronary artery, repair is an option to alleviate the ischemia and presumably mitigate the recurrence of ventricular arrhythmias. However, care should be individualized, as there may be other factors (e.g., CAD, cardiomyopathy, residual ischemia) contributing to ventricular arrhythmias that warrant continued vigilance and additional therapy.
4.
Anomalous aortic origin of the right coronary from the left sinus is more common than anomalous aortic origin of the left coronary from the right sinus. The risk of SCD with the former malformation is difficult to quantitate. There is some physiological rationale to believe that asymptomatic patients without evidence of compromised blood flow would benefit from unroofing, but there are not data to demonstrate that surgical interventions alter the risk of SCD. Thus, watchful waiting may be an appropriate course as well, particularly for a patient with an anomalous right coronary arising from the left sinus.
4.4.7.3 Anomalous Coronary Artery Arising From the PA
Recommendations for Anomalous Coronary Artery Arising From the PA
Surgery is recommended for anomalous left coronary artery from the PA[1], [2], [3], [4], [5], [6], [7].
I
C-EO
2.
In a symptomatic adult with anomalous right coronary artery from the PA with symptoms attributed to the anomalous coronary, surgery is recommended.
IIa
C-EO
3.
Surgery for anomalous right coronary artery from the PA is reasonable in an asymptomatic adult with ventricular dysfunction or with myocardial ischemia attributed to anomalous right coronary artery from the PA.
Recommendation-Specific Supportive Text
1.
Surgery can include reimplantation of the left coronary artery directly into the aorta with or without an interposition graft. Ligation or closure of the left coronary artery at the level of the PA with coronary artery bypass grafting can also be performed, usually using the left internal mammary artery anastomosed to the left anterior descending.
2.
Surgery can include reimplantation of the right coronary artery directly into the aorta with or without an interposition graft. Ligation or closure of the right coronary artery at the level of the PA with coronary artery bypass grafting can also be performed, usually using the right internal mammary artery anastomosed to the right coronary or posterior descending coronary artery.
3.
Surgery to alleviate ischemia or ventricular dysfunction is reasonable if the anomalous coronary artery is thought to be the cause. Surgery can include reimplantation of the right coronary artery directly into the aorta with or without an interposition graft. Ligation or closure of the right coronary artery at the level of the PA with coronary artery bypass grafting can also be performed, usually using the right internal mammary artery anastomosed to the right coronary or posterior descending coronary artery.
Markup:<div data-v-198296ba="" data-v-aafd3410="" class="block swg-result"><div data-v-198296ba=""><section id="hl0006474"><a id="sec7"></a><section id="hl0008811"><a id="sec7.4"></a><section id="hl0009869"><a id="sec7.4.7"></a><a id="sectitle0465"></a><h3 id="hl0009871"><span class="section-label">4.4.7</span> Coronary Anomalies</h3><p id="hl0009872">Coronary abnormalities are among the most common congenital cardiovascular anomalies, surpassing in prevalence nearly all others combined. Coronary anomalies include anomalous aortic origin of a coronary artery (AAOCA), coronary fistula, and myocardial bridge. Many congenital coronary abnormalities have a benign outcome. In contrast, natural history studies of anomalous coronary artery from the PA (particularly anomalous left coronary artery from the PA) suggest poor outcome in untreated patients; similar natural history studies are lacking regarding untreated patients with AAOCA, but other evidence raises concern. See and for a diagnostic and treatment algorithm for AAOCA. </p><div id="tbl34" class="table"><div class="inline-table-label">Table 34</div><div class="inline-table-caption">Factors That May Relate to the Clinical Importance of AAOCA and Risk of SCD</div><div><table id="hl0002855"><tbody><tr><td id="hl0002860" class="table-align-">Age</td><td id="hl0002861" class="table-align-">AAOCA is more commonly invoked as the cause of SCD in patients <35 y of age than in patients >35 y of age, in whom atherosclerotic coronary disease becomes a more prevalent cause. However, death has been attributed to AAOCA in patients of all ages; there does not seem to be an age beyond which the AAOCA may not be relevant, even in the setting of atherosclerotic coronary disease and other concomitant conditions <a class="inline-reference" href="#bib816">[1]</a>, <a class="inline-reference" href="#bib817">[2]</a>. </td></tr><tr><td id="hl0002864" class="table-align-">Anatomy of coronary ostium and proximal coronary course</td><td id="hl0002865" class="table-align-">Slit-like/fish-mouth-shaped orifice, acute angle takeoff, intramural course, interarterial course and hypoplasia of the proximal coronary artery have all been proposed as reasons for symptoms, ischemia and SCD in patients with AAOCA. The slit-like orifice is more commonly seen in anomalous right coronary artery arising from the left sinus. Each of these anatomic findings offers a pathophysiological mechanism for intermittent ischemia, particularly at times of high cardiac output and/or increased aortic wall tension, such as during exercise <a class="inline-reference" href="#bib821">[1]</a>, <a class="inline-reference" href="#bib824">[2]</a>, <a class="inline-reference" href="#bib825">[3]</a>, <a class="inline-reference" href="#bib826">[4]</a>. </td></tr><tr><td id="hl0002868" class="table-align-">Anomalous origin</td><td id="hl0002869" class="table-align-">Left coronary artery arising from the right cusp is less common than the right coronary artery arising from the left cusp but is more often found in autopsy series of SCD <a class="inline-reference" href="#bib816">[1]</a>, <a class="inline-reference" href="#bib818">[2]</a>, <a class="inline-reference" href="#bib830">[3]</a>. This suggests that anomalous origin of the left coronary artery from the right cusp is more likely to cause SCD than anomalous origin of the right coronary artery from the left cusp. This may be due either to anatomic features that make anomalous aortic origin of the left coronary artery prone to coronary compromise or because a larger proportion of myocardium is supplied by the left coronary artery, or both. </td></tr><tr><td id="hl0002872" class="table-align-">Exercise</td><td id="hl0002873" class="table-align-">Autopsy series suggest a most patients die during, or in close temporal association with, exercise <a class="inline-reference" href="#bib818">[1]</a>, <a class="inline-reference" href="#bib819">[2]</a>, <a class="inline-reference" href="#bib820">[3]</a>. </td></tr><tr><td id="hl0002876" class="table-align-">Ischemia</td><td id="hl0002877" class="table-align-">Autopsy series demonstrate myocardial fibrosis in a significant number of patients whose deaths were attributed to AAOCA, particularly in patients with anomalous left coronary artery arising from the right cusp <a class="inline-reference" href="#bib820">(S4.4.7-5)</a>. Surgical series describe patients with ischemia or MI before surgical repair in the absence of other CAD, suggesting a relation of the coronary anomaly to the ischemia <a class="inline-reference" href="#bib831">(S4.4.7-16)</a>. This suggests that had perfusion imaging been obtained before SCD, ischemia would have been found in such patients <a class="inline-reference" href="#bib832">[1]</a>, <a class="inline-reference" href="#bib833">[2]</a>. However, other data indicate that a normal stress test does not preclude a SCD event, with the proviso that most of those studies used only stress ECG, rather than the more sensitive and specific modalities of nuclear perfusion imaging or stress echocardiography. In addition, postoperative studies have shown that ischemia may be found after surgical repair in the distribution not supplied by the abnormal coronary artery and may not persist on repeat testing <a class="inline-reference" href="#bib834">(S4.4.7-19)</a>. </td></tr><tr><td id="hl0002883" class="table-align-">Symptoms</td><td id="hl0002884" class="table-align-">In autopsy and surgical series, a significant number of patients reported cardiovascular symptoms, including before SCD events <a class="inline-reference" href="#bib819">[1]</a>, <a class="inline-reference" href="#bib822">[2]</a>, <a class="inline-reference" href="#bib823">[3]</a>, <a class="inline-reference" href="#bib835">[4]</a>, <a class="inline-reference" href="#bib836">[5]</a>. Symptoms are more commonly reported in patients in whom the left coronary artery arises from the right sinus. Surgical series have described improvement in symptoms after surgical repair <a class="inline-reference" href="#bib818">[1]</a>, <a class="inline-reference" href="#bib819">[2]</a>, <a class="inline-reference" href="#bib820">[3]</a>, <a class="inline-reference" href="#bib821">[4]</a>, <a class="inline-reference" href="#bib822">[5]</a>, <a class="inline-reference" href="#bib823">[6]</a>. </td></tr></tbody></table></div><div class="inline-table-caption">AAOCA indicates anomalous aortic origin of the coronary artery; CAD, coronary artery disease; ECG, electrocardiogram; MI, myocardial infarction; and SCD, sudden cardiac death.</div></div><div class="inline-image figure" id="fig5"><figure><figcaption class="inline-image-label">Figure 5</figcaption><img src="//" alt="" data-eid="1-s2.0-S0735109718368463" data-locator="gr5" data-attachment-filename="07351097/S0735109718X00187/S0735109718368463/gr5-t.gif"><div class="caption-holder"><div class="inline-image-caption">Anomalous Aortic Origin of the Coronary Artery</div><div class="inline-image-caption">*Surgical intervention to involve unroofing or coronary revascularization for patients with concomitant fixed obstruction.</div></div></figure></div><p id="hl0009877">Assessment of the risk of SCD in patients with AAOCA and of the role of AAOCA in causing ischemia or symptoms is difficult because available data do not adequately capture the clinical spectrum of these anomalies. Autopsy series are available that help describe the anomalies found in patients who suffered SCD contrasted to other causes of death <a class="inline-reference" href="#bib816">[1]</a>, <a class="inline-reference" href="#bib817">[2]</a>, <a class="inline-reference" href="#bib818">[3]</a>, <a class="inline-reference" href="#bib819">[4]</a>, <a class="inline-reference" href="#bib820">[5]</a>. There are surgical case series that describe findings before operation, operative anatomy and postoperative course <a class="inline-reference" href="#bib817">[1]</a>, <a class="inline-reference" href="#bib820">[2]</a>, <a class="inline-reference" href="#bib821">[3]</a>, <a class="inline-reference" href="#bib822">[4]</a>, <a class="inline-reference" href="#bib823">[5]</a>. There are imaging studies describing the anatomy and potential pathophysiological abnormalities associated with AAOCA <a class="inline-reference" href="#bib822">[1]</a>, <a class="inline-reference" href="#bib824">[2]</a>, <a class="inline-reference" href="#bib825">[3]</a>, <a class="inline-reference" href="#bib826">[4]</a>. There are surgical series describing improvement in symptoms after operation <a class="inline-reference" href="#bib821">[1]</a>, <a class="inline-reference" href="#bib822">[2]</a>, <a class="inline-reference" href="#bib823">[3]</a>. There are surveys and registries that describe the heterogeneous management strategies applied to AAOCA <a class="inline-reference" href="#bib827">[1]</a>, <a class="inline-reference" href="#bib828">[2]</a>, <a class="inline-reference" href="#bib829">[3]</a>. What is lacking are data proving that any particular management strategy prevents SCD. As a consequence, decisions regarding whether surgery is necessary or exercise restriction or medical therapy might be beneficial are all based on synthesizing limited data and applying to an individual patient. Clinicians commonly extrapolate to assist in medical decision-making, but the consequences of being “wrong” for a young patient with AAOCA may be perceived to be greater than for many other conditions. Consequently, there is often a clinical urge to seek a reason to do something like surgical repair, because the available data do not identify clinical features that provide reassurance that a patient is at low risk of cardiovascular events. Unfortunately, evidence demonstrating that surgical repair ameliorates SCD risk, derived from large enough cohorts followed over a sufficient period of time, is not available. </p><section id="hl0009883"><a id="sec7.4.7.1"></a><a id="sectitle0470"></a><h3 id="hl0009885"><span class="section-label">4.4.7.1</span> Anomalous Coronary Artery Evaluation</h3><div id="undtbl46" class="table"><div class="inline-table-caption">Recommendations for Anomalous Coronary Artery Evaluation</div><div class="inline-table-caption">Referenced studies that support recommendations are summarized in <a id="hl0009892" href="http://jaccjacc.acc.org/Clinical_Document/ACHD_Guideline_ES-FT_Data_Supplements_08-02-18.pdf" target="_blank">Online Data Supplement 51</a>. </div><div><table id="hl0009893"><thead><tr><th id="hl0009899" scope="col" class="table-align-">COR</th><th id="hl0009900" scope="col" class="table-align-">LOE</th><th id="hl0009901" scope="col" class="table-align-">Recommendations</th></tr></thead><tbody><tr><td id="hl0009904" class="table-align-center" colspan="3">Diagnostic</td></tr><tr><td id="hl0009906" class="table-align-"><b>I</b></td><td id="hl0009908" class="table-align-"><b>C-LD</b></td><td id="hl0009910" class="table-align-"><ul id="hl0009911"><li id="hl0009912"><span class="list-item-label">1.</span><p id="hl0009914"><b>Coronary angiography, using catheterization, CT, or CMR, is recommended for evaluation of anomalous coronary artery</b><a class="inline-reference" href="#bib837"><b>[1]</b></a>, <a class="inline-reference" href="#bib838"><b>[2]</b></a>, <a class="inline-reference" href="#bib839"><b>[3]</b></a><b>.</b></p></li></ul></td></tr><tr><td id="hl0009920" class="table-align-"><b>I</b></td><td id="hl0009922" class="table-align-"><b>C-LD</b></td><td id="hl0009924" class="table-align-"><ul id="hl0009925"><li id="hl0009926"><span class="list-item-label">2.</span><p id="hl0009928"><b>Anatomic and physiological evaluation should be performed in patients with anomalous aortic origin of the left coronary from the right sinus and/or right coronary from the left sinus</b><a class="inline-reference" href="#bib840"><b>[1]</b></a>, <a class="inline-reference" href="#bib841"><b>[2]</b></a>, <a class="inline-reference" href="#bib842"><b>[3]</b></a>, <a class="inline-reference" href="#bib843"><b>[4]</b></a>, <a class="inline-reference" href="#bib844"><b>[5]</b></a>, <a class="inline-reference" href="#bib845"><b>[6]</b></a><b>.</b></p></li></ul></td></tr></tbody></table></div></div><p id="hl0009933"><b>Recommendation-Specific Supportive Text</b></p><ul id="hl0009935"><li id="hl0009936"><span class="list-item-label">1.</span><p id="hl0009938">CTA, CMR, and catheterization can all delineate the proximal course of the coronary artery and relationship to other structures. CTA is generally preferred because it has superior spatial and temporal resolution, although CMR may also provide adequate delineation of the relationship of the coronary artery to the aorta, PA and other structures, including whether the proximal course appears to be intramural. Coronary angiography by catheterization can be helpful when there is concern about stenosis in the coronary artery or when concomitant hemodynamic evaluation for shunt assessment or intravascular ultrasonography/flow evaluation is needed.</p></li><li id="hl0009939"><span class="list-item-label">2.</span><p id="hl0009941">Assessment of AAOCA is enhanced when the precise anatomy and physiological impact of the coronary artery anomaly are understood. As described in , the specific anomalous origin, anatomy of the orifice and proximal vessel and presence of ischemia may all influence the clinical course and thus the management options. Understanding these issues as precisely as possible will better inform clinical decisions. </p></li></ul></section><section id="hl0009943"><a id="sec7.4.7.2"></a><a id="sectitle0475"></a><h3 id="hl0009945"><span class="section-label">4.4.7.2</span> Anomalous Aortic Origin of Coronary Artery</h3><div id="undtbl47" class="table"><div class="inline-table-caption">Recommendations for Anomalous Aortic Origin of Coronary Artery</div><div class="inline-table-caption">Referenced studies that support recommendations are summarized in <a id="hl0009952" href="http://jaccjacc.acc.org/Clinical_Document/ACHD_Guideline_ES-FT_Data_Supplements_08-02-18.pdf" target="_blank">Online Data Supplement 51</a>. </div><div><table id="hl0009953"><thead><tr><th id="hl0009959" scope="col" class="table-align-">COR</th><th id="hl0009960" scope="col" class="table-align-">LOE</th><th id="hl0009961" scope="col" class="table-align-">Recommendations</th></tr></thead><tbody><tr><td id="hl0009964" class="table-align-center" colspan="3">Therapeutic</td></tr><tr><td id="hl0009966" class="table-align-"><b>I</b></td><td id="hl0009968" class="table-align-"><b>B-NR</b></td><td id="hl0009970" class="table-align-"><ul id="hl0009971"><li id="hl0009972"><span class="list-item-label">1.</span><p id="hl0009974"><b>Surgery is recommended for AAOCA from the left sinus or AAOCA from the right sinus for symptoms or diagnostic evidence consistent with coronary ischemia attributable to the anomalous coronary artery</b><a class="inline-reference" href="#bib846"><b>[1]</b></a>, <a class="inline-reference" href="#bib847"><b>[2]</b></a>, <a class="inline-reference" href="#bib848"><b>[3]</b></a><b>.</b></p></li></ul></td></tr><tr><td id="hl0009980" class="table-align-"><b>IIa</b></td><td id="hl0009982" class="table-align-"><b>C-LD</b></td><td id="hl0009984" class="table-align-"><ul id="hl0009985"><li id="hl0009986"><span class="list-item-label">2.</span><p id="hl0009988"><b>Surgery is reasonable for anomalous aortic origin of the left coronary artery from the right sinus in the absence of symptoms or ischemia</b><a class="inline-reference" href="#bib849"><b>[1]</b></a>, <a class="inline-reference" href="#bib850"><b>[2]</b></a>, <a class="inline-reference" href="#bib851"><b>[3]</b></a><b>.</b></p></li></ul></td></tr><tr><td id="hl0009994" class="table-align-"><b>IIa</b></td><td id="hl0009996" class="table-align-"><b>C-EO</b></td><td id="hl0009998" class="table-align-"><ul id="hl0009999"><li id="hl0010000"><span class="list-item-label">3.</span><p id="hl0010002"><b>Surgery for AAOCA is reasonable in the setting of ventricular arrhythmias.</b></p></li></ul></td></tr><tr><td id="hl0010005" class="table-align-"><b>IIb</b></td><td id="hl0010007" class="table-align-"><b>B-NR</b></td><td id="hl0010009" class="table-align-"><ul id="hl0010010"><li id="hl0010011"><span class="list-item-label">4.</span><p id="hl0010013"><b>Surgery or continued observation may be reasonable for asymptomatic patients with an anomalous left coronary artery arising from the right sinus or right coronary artery arising from the left sinus without ischemia or anatomic or physiological evaluation suggesting potential for compromise of coronary perfusion (e.g., intramural course, fish-mouth-shaped orifice, acute angle)</b><a class="inline-reference" href="#bib849"><b>[1]</b></a>, <a class="inline-reference" href="#bib850"><b>[2]</b></a>, <a class="inline-reference" href="#bib851"><b>[3]</b></a><b>.</b></p></li></ul></td></tr></tbody></table></div></div><p id="hl0010018"><b>Recommendation-Specific Supportive Text</b></p><ul id="hl0010020"><li id="hl0010021"><span class="list-item-label">1.</span><p id="hl0010023">In patients with symptoms related to AAOCA, repair of the anomaly should alleviate symptoms. In autopsy and surgical series, cardiac symptoms are more common in patients with a left coronary artery arising from the right coronary cusp. In autopsy studies of patients who died because of an anomalous coronary artery, fibrosis is a common finding, suggesting that ischemia preceded the terminal event. However, there are patients in whom a SCD event occurred despite normal stress ECG, and consequently absence of ischemia is not reassuring. Autopsy series show that many patients whose death is attributed to anomalous coronary arteries are young, thus management of patients should take age into account, with heightened concern about the risk of sudden death in younger patients <a class="inline-reference" href="#bib852">[1]</a>, <a class="inline-reference" href="#bib853">[2]</a>, <a class="inline-reference" href="#bib854">[3]</a>. </p></li><li id="hl0010025"><span class="list-item-label">2.</span><p id="hl0010027">Anomalous left coronary from the right sinus is less common than anomalous right coronary from the left sinus <a class="inline-reference" href="#bib855">(S4.4.7.2-10)</a>, but anomalous left coronary artery from the right is more commonly found in autopsy series of athletes and military recruits who had nontraumatic death than right coronary from the left sinus <a class="inline-reference" href="#bib846">[1]</a>, <a class="inline-reference" href="#bib856">[2]</a>, <a class="inline-reference" href="#bib857">[3]</a>, <a class="inline-reference" href="#bib858">[4]</a>. The overrepresentation of the anomalous left coronary from the right sinus suggests a higher risk of SCD, particular at extremes of exertion and in patients <35 years of age. </p><p id="hl0010030">There are some anatomic features that are thought to be associated with increased risk of compromise of coronary flow and/or SCD, including a fish-mouth-shaped or slit-like orifice, or intramural course <a class="inline-reference" href="#bib859">(S4.4.7.2-14)</a>, although the slit-like orifice is more commonly encountered in a right coronary arising from the left cusp. It is difficult to quantitate the absolute risk of SCD associated with anomalous aortic origin of the left coronary from the right sinus, and data demonstrating that surgery ameliorates the SCD risk have not been published. Until studies suggest otherwise, limited data and expert consensus suggest that it is reasonable that adults with this malformation should undergo surgical unroofing unless there are extenuating circumstances that would make surgery high risk. </p></li><li id="hl0010032"><span class="list-item-label">3.</span><p id="hl0010034">In patients with ventricular arrhythmias presumed related to ischemia caused by anomalous origin of a coronary artery, repair is an option to alleviate the ischemia and presumably mitigate the recurrence of ventricular arrhythmias. However, care should be individualized, as there may be other factors (e.g., CAD, cardiomyopathy, residual ischemia) contributing to ventricular arrhythmias that warrant continued vigilance and additional therapy.</p></li><li id="hl0010035"><span class="list-item-label">4.</span><p id="hl0010037">Anomalous aortic origin of the right coronary from the left sinus is more common than anomalous aortic origin of the left coronary from the right sinus. The risk of SCD with the former malformation is difficult to quantitate. There is some physiological rationale to believe that asymptomatic patients without evidence of compromised blood flow would benefit from unroofing, but there are not data to demonstrate that surgical interventions alter the risk of SCD. Thus, watchful waiting may be an appropriate course as well, particularly for a patient with an anomalous right coronary arising from the left sinus.</p></li></ul></section><section id="hl0010038"><a id="sec7.4.7.3"></a><a id="sectitle0480"></a><h3 id="hl0010040"><span class="section-label">4.4.7.3</span> Anomalous Coronary Artery Arising From the PA</h3><div id="undtbl48" class="table"><div class="inline-table-caption">Recommendations for Anomalous Coronary Artery Arising From the PA</div><div class="inline-table-caption">Referenced studies that support recommendations are summarized in <a id="hl0010047" href="http://jaccjacc.acc.org/Clinical_Document/ACHD_Guideline_ES-FT_Data_Supplements_08-02-18.pdf" target="_blank">Online Data Supplement 51</a>. </div><div><table id="hl0010048"><thead><tr><th id="hl0010054" scope="col" class="table-align-">COR</th><th id="hl0010055" scope="col" class="table-align-">LOE</th><th id="hl0010056" scope="col" class="table-align-">Recommendations</th></tr></thead><tbody><tr><td id="hl0010059" class="table-align-center" colspan="3">Therapeutic</td></tr><tr><td id="hl0010061" class="table-align-"><b>I</b></td><td id="hl0010063" class="table-align-"><b>B-NR</b></td><td id="hl0010065" class="table-align-"><ul id="hl0010066"><li id="hl0010067"><span class="list-item-label">1.</span><p id="hl0010069"><b>Surgery is recommended for anomalous left coronary artery from the PA</b><a class="inline-reference" href="#bib860"><b>[1]</b></a>, <a class="inline-reference" href="#bib861"><b>[2]</b></a>, <a class="inline-reference" href="#bib862"><b>[3]</b></a>, <a class="inline-reference" href="#bib863"><b>[4]</b></a>, <a class="inline-reference" href="#bib864"><b>[5]</b></a>, <a class="inline-reference" href="#bib865"><b>[6]</b></a>, <a class="inline-reference" href="#bib866"><b>[7]</b></a><b>.</b></p></li></ul></td></tr><tr><td id="hl0010075" class="table-align-"><b>I</b></td><td id="hl0010077" class="table-align-"><b>C-EO</b></td><td id="hl0010079" class="table-align-"><ul id="hl0010080"><li id="hl0010081"><span class="list-item-label">2.</span><p id="hl0010083"><b>In a symptomatic adult with anomalous right coronary artery from the PA with symptoms attributed to the anomalous coronary, surgery is recommended.</b></p></li></ul></td></tr><tr><td id="hl0010086" class="table-align-"><b>IIa</b></td><td id="hl0010088" class="table-align-"><b>C-EO</b></td><td id="hl0010090" class="table-align-"><ul id="hl0010091"><li id="hl0010092"><span class="list-item-label">3.</span><p id="hl0010094"><b>Surgery for anomalous right coronary artery from the PA is reasonable in an asymptomatic adult with ventricular dysfunction or with myocardial ischemia attributed to anomalous right coronary artery from the PA.</b></p></li></ul></td></tr></tbody></table></div></div><p id="hl0010096"><b>Recommendation-Specific Supportive Text</b></p><ul id="hl0010098"><li id="hl0010099"><span class="list-item-label">1.</span><p id="hl0010101">Surgery can include reimplantation of the left coronary artery directly into the aorta with or without an interposition graft. Ligation or closure of the left coronary artery at the level of the PA with coronary artery bypass grafting can also be performed, usually using the left internal mammary artery anastomosed to the left anterior descending.</p></li><li id="hl0010102"><span class="list-item-label">2.</span><p id="hl0010104">Surgery can include reimplantation of the right coronary artery directly into the aorta with or without an interposition graft. Ligation or closure of the right coronary artery at the level of the PA with coronary artery bypass grafting can also be performed, usually using the right internal mammary artery anastomosed to the right coronary or posterior descending coronary artery.</p></li><li id="hl0010105"><span class="list-item-label">3.</span><p id="hl0010107">Surgery to alleviate ischemia or ventricular dysfunction is reasonable if the anomalous coronary artery is thought to be the cause. Surgery can include reimplantation of the right coronary artery directly into the aorta with or without an interposition graft. Ligation or closure of the right coronary artery at the level of the PA with coronary artery bypass grafting can also be performed, usually using the right internal mammary artery anastomosed to the right coronary or posterior descending coronary artery.</p></li></ul></section></section></section></section></div></div>
