**12.2 Recommendations**

218 Congenital Heart Disease – Selected Aspects

the proximal path of ectopic CX coronary arteries. Angiographic demonstration of a reduction of the lumen is not rare. However, besides atherosclerotic narrowing, a distortion of CX coronary artery may also be suspected in case of enlarged aortic root due to their close relationships. The mechanism of a potentially higher incidence of CAD of ectopic CX

If most of ANOCOR are benign and require no specific investigation or treatment, other abnormalities such ANOCOR classified as high-risk or symptomatic ANOCOR require a meticulous and accurate analysis in order to offer the best management in the present state

First of all, the coronary abnormality should be clearly defined with a correct interpretation of ectopic origin, initial course regarding to aortic wall, and ectopic course regarding to adjacent structures. Uncertain diagnosis or high-risk ANOCOR need always complementary imaging investigation allowing a useful confrontation. All cardiologists and radiologists are not familiar with the large spectrum of congenital coronary abnormalities, and the opinion of a practitioner experienced in the field of ANOCOR should be mandatory before decisionmaking. Recent investigation conducted by the Anomalous Coronary Artery Working Group of the Congenital Heart Surgeon's Society showed a heterogeneous management of young adults with ANOCOR associated with a preaortic course (Brothers et al., 2009). In 36th Bethesda Conference focused on trained athlete with an identified cardiovascular abnormality, detection of coronary anomalies of wrong sinus origin in which a coronary artery passes between the aorta and pulmonary artery should result in exclusion from all participations in competitive sport (Graham et al., 2005). Participation in all sport 3 months after successful operation would be permitted for an athlete without ischemia, ventricular or tachyarrhythmia, or dysfunction during maximal exercise testing was another recommendation of the 36th Bethesda Conference. As previously mentioned, presence of symptoms, high-risk anatomical features and young age are the main criteria requiring a special attention in order to prevent a sudden cardiac death. Even if false-negative cases are frequent, stress exercise tests with nuclear imaging are necessary in this population exposed. More aggressive investigations, such pharmacological tests simulating extreme exercise have been suggested but are not without dangers (Angelini et al., 2003). According to current understanding, only an intramural segment seems to be clearly related with a highrisk of sudden death, and the best means in identifying intramural segment is IVUS. The definition of a cut-off age in deciding a population as high-risk remains difficult in practice. If the literature gives relatively clear information in < 30-year old and > 50-year old patients, the management of patients between 30 and 50 years of age is often problematic. Restriction of activity, particularly competitive sport and intensive exertion, is often recommended if a surgical repair is not indicated. Medical treatment with essentially beta-blockers is sometimes associated. Due to possible but rare late deaths or subclinical myocardial ischemia after surgical repair of ANOCOR, long-term follow-up with regular cardiovascular evaluation is needed (Brothers et al., 2007, Brothers et al., 2009). Presence of significant atherosclerotic coronary disease or valvular disease requiring cardiac surgery permits sometimes a concomitant treatment of a high-risk ANOCOR. Furthermore, identification of

coronary artery in the retroaortic segment remains unclear.

of our knowledge.

**12.1 Management** 

**12. Management, recommendations, and treatments** 

ACC/AHA 2008 guidelines for the management of adults with congenital heart disease (Warnes et al., 2008) give recommendations for congenital coronary abnormalities of ectopic aortic origin and for anomalous left coronary artery from the pulmonary artery (table 10).


Table 10. ACC/AHA 2008 recommendations for anomalous connections with aorta and pulmonary artery.

Surgical repair is indicated for LMCA with preaortic course regardless of symptoms. Surgical repair is also indicated for RCA with preaortic course in association with symptoms and/or inducible ischemia. A conservative approach is recommended in asymptomatic in patients with ectopic RCA in association with preaortic course. Position of PCI with limited experience and without long-term follow-up is not established in the ACC/AHA guidelines. Furthermore, the risk stratification with age is not clearly exposed. Only, a young patient is

Proximal Anomalous Connections of Coronary Arteries in Adults 221

numerous and current therapeutic strategies may vary among clinicians due to marked heterogeneity in physician opinions. Obviously, the left high-risk ANOCOR are easier entrusted to surgeon. Nevertheless, surgical repair of right high-risk ANOCOR has been reported (Garcia-Rinaldi et al., 2004). In contrast to anomalous connections with pulmonary artery, direct aortic reimplantation is rarely possible due to an, almost without exception, intramural segment. Several techniques have been proposed (Said et al. 2010): unroofing of the coronary artery, creation of a neo-ostium, reimplantation of the coronary artery, translocation of the pulmonary artery with patch angioplasty of the coronary artery and coronary bypass grafting. Unroofing consists to a longitudinal excision of the common wall between the aorta and the ectopic coronary artery coursing tangentially with an intramural segment (figure 23). Excision starts at the anomalous ostium and continues into the appropriate sinus. To create a neo-ostium, a probe passed through the intramural segment and the coronary artery is opened at the location at which the probe exits the aortic wall (figure 23). In the absence of intramural segment, reimplantation of the coronary artery is possible. The ectopic ostium is excised and implanted in the appropriate sinus above the sinotubular junction. Another technique may be used in the absence of intramural segment, with a translocation of the pulmonary trunk anteriorly and leftward to avoid a compression of the ectopic artery. This technique may be associated with patch angioplasty of the ectopic coronary artery (Karl et al., 2010). The coronary artery is open well beyond the intramural segment and a patch of autologous pericardium creates a large neo-ostium. Finally, coronary artery bypass grafting with saphenous vein or internal mammary artery is another possibility. Thus, correction of intramural segment is not systematically. The choice of the technique depends of surgeon's preference, anatomical pattern of ANOCOR, and existence of CAD requiring myocardial revascularization. Coronary artery bypass grafting is proposed in older adults with concomitant CAD. The use of venous or arterial conduits in young people exposes to a long-term patency concern, because of competitive flow. For most operators, the creation of a neo-ostium is the more physiological treatment without the risk of take-down of the commissural junction between the right and left coronary sinuses observed sometimes with the unroofing technique. No comparative data exist about these different surgical methods. Surgical practice has evolved in the time from coronary artery bypass grafting to direct surgical repair. To date, unroofing technique seems the more used in young people (Davies et al., 2009, Frommelt et al., 2011, Mainwaring et al. 2011). The rate of perioperative death is near to zero in small series of children and young people (Davies et al., 2009, Erez et al., 2006, Karl et al., 2010). Recently, Krasuski et al. reported the impact of surgery in patients with ANOCOR from the opposite sinus (Krasuski, et al., 2011). A cohort of 301 adults from 210,700 cardiac catherizations performed over a 35-year period was retrospectively analysed. The incidence of anomalous connections with the opposite sinus was of 0.14%, and in 54 of 301 patients (18%) an interarterial course between the aorta and pulmonary artery was identified, thus an incidence of 3/10.000 of high-risk ANOCOR with 18 left ANOCOR (33%) and 36 right ANOCOR (67%). Surgical management was chosen in 28 of 54 patients (52%), and in 8 of 18 left ANOCOR (44%) and 20 of 36 right ANOCOR (56%). Coronary artery bypass grafting was used as treatment in most of cases (71%). At 10 years, no difference in survival was observed between interarterial ANOCOR managed surgically or medically. Some characteristics of the cohort studied may explain the lack of benefit associated with surgical repair. As aforementioned, the study population consisted of relatively old patients with a mean age of 58 years and with a high prevalence of CAD.

defined as a patient with an age less than 50 years. Despite of absence of randomized studies and limited information about long term follow-up in high risk patients, current guidelines are fairly directive in favor of large surgical indications in high–risk ANOCOR with ectopic origin from the aorta. In accordance with its well-described natural history, an anomalous LCA connected with pulmonary artery should be repaired surgically regardless of age. Surprisingly, congenital coronary abnormalities do not appear on the list of specific congenital heart defects in the recent European 2010 guidelines (Baumgartner et al., 2010).

#### **12.3 Treatments of anomalous connections with the pulmonary artery**

Correction of anomalous connections with the pulmonary artery should always be proposed regardless symptoms and age, with as possible establishment of a dual coronary system. The restitution of an antegrade flow in the LCA is associated with an improvement of the left ventricular function and a reduction of the ischemic mitral regurgitation. At mid-term, regression of the intercoronary collateral network and decrease in size of dilated RCA are observed. Numerous surgical methods have been attempted to repair coronary abnormality, including Takeuchi tunnel procedure, venous or arterial bypass grafts, or direct aortic reimplantation. The latter, although technically more challenging, appears today as the method of choice in patients with favorable anatomy (Kottayil et al., 2011). The implantation of the LMCA into the aorta is a more physiological correction and avoids the late risks of venous grafts. Takeuchi procedure is a technique of repair with the creation of a baffle within the pulmonary artery to divert the blood from the origin of the ectopic LMCA to the aorta (Takeuchi et al., 1979). Due to a high rate of reoperations for supravalvular pulmonary stenosis, baffle leak or obstruction of the intrapulmonary channel, the Takeuchi procedure is almost no longer used. Ectopic LCA usually originates from the left posterior pulmonary sinus facing the left side of the aorta. The excised ectopic coronary is directly implanted just above the sinotubular junction. The site of pulmonary excision is repaired with autologous pericardium. Sometimes, strategies to lengthen the anomalous coronary artery are necessary. Mitral repair or replacement is recommended in patients with severe mitral regurgitation. Establishment of a satisfactory left myocardial perfusion may resolve mild and some moderate mitral insufficiencies (Fehrenbacher et al., 2010). Mechanical circulatory support should be considered in patients with intractable left ventricular failure in postoperative setting (Dodge-Khatami et al., 2002). With increased experience with direct aortic implantation technique and better postoperative management, the perioperative mortality rate, initially up to 20%, decreased dramatically of about 10%. Due to the rare frequency of patients with a coronary artery arising from pulmonary artery who survive into childhood, large prospective studies in adults who underwent surgical repair are lacking. Studies, pooling generally children and adult populations, reported a 10-year survival rate between 85 and 95% (Ben Ali et al., 2009, Brown et al., 2008, Fehrenbacher et al., 2010). Close long-term follow-up after surgical repair is recommended to detect residual ischemia.

#### **12.4 Treatments of anomalous connections with the aorta 12.4.1 Surgical treatment**

Surgical treatment is recommended in patients with high-risk anomalous connections with the aorta (Warnes et al, 2008). However, an inhomogeneous management of high-risk ANOCOR is observed in practice (Brothers et al., 2009). Surgical repair techniques are

defined as a patient with an age less than 50 years. Despite of absence of randomized studies and limited information about long term follow-up in high risk patients, current guidelines are fairly directive in favor of large surgical indications in high–risk ANOCOR with ectopic origin from the aorta. In accordance with its well-described natural history, an anomalous LCA connected with pulmonary artery should be repaired surgically regardless of age. Surprisingly, congenital coronary abnormalities do not appear on the list of specific congenital heart defects in the recent European 2010 guidelines (Baumgartner et al., 2010).

Correction of anomalous connections with the pulmonary artery should always be proposed regardless symptoms and age, with as possible establishment of a dual coronary system. The restitution of an antegrade flow in the LCA is associated with an improvement of the left ventricular function and a reduction of the ischemic mitral regurgitation. At mid-term, regression of the intercoronary collateral network and decrease in size of dilated RCA are observed. Numerous surgical methods have been attempted to repair coronary abnormality, including Takeuchi tunnel procedure, venous or arterial bypass grafts, or direct aortic reimplantation. The latter, although technically more challenging, appears today as the method of choice in patients with favorable anatomy (Kottayil et al., 2011). The implantation of the LMCA into the aorta is a more physiological correction and avoids the late risks of venous grafts. Takeuchi procedure is a technique of repair with the creation of a baffle within the pulmonary artery to divert the blood from the origin of the ectopic LMCA to the aorta (Takeuchi et al., 1979). Due to a high rate of reoperations for supravalvular pulmonary stenosis, baffle leak or obstruction of the intrapulmonary channel, the Takeuchi procedure is almost no longer used. Ectopic LCA usually originates from the left posterior pulmonary sinus facing the left side of the aorta. The excised ectopic coronary is directly implanted just above the sinotubular junction. The site of pulmonary excision is repaired with autologous pericardium. Sometimes, strategies to lengthen the anomalous coronary artery are necessary. Mitral repair or replacement is recommended in patients with severe mitral regurgitation. Establishment of a satisfactory left myocardial perfusion may resolve mild and some moderate mitral insufficiencies (Fehrenbacher et al., 2010). Mechanical circulatory support should be considered in patients with intractable left ventricular failure in postoperative setting (Dodge-Khatami et al., 2002). With increased experience with direct aortic implantation technique and better postoperative management, the perioperative mortality rate, initially up to 20%, decreased dramatically of about 10%. Due to the rare frequency of patients with a coronary artery arising from pulmonary artery who survive into childhood, large prospective studies in adults who underwent surgical repair are lacking. Studies, pooling generally children and adult populations, reported a 10-year survival rate between 85 and 95% (Ben Ali et al., 2009, Brown et al., 2008, Fehrenbacher et al., 2010). Close long-term follow-up after surgical repair is

**12.3 Treatments of anomalous connections with the pulmonary artery** 

recommended to detect residual ischemia.

**12.4.1 Surgical treatment** 

**12.4 Treatments of anomalous connections with the aorta** 

Surgical treatment is recommended in patients with high-risk anomalous connections with the aorta (Warnes et al, 2008). However, an inhomogeneous management of high-risk ANOCOR is observed in practice (Brothers et al., 2009). Surgical repair techniques are

numerous and current therapeutic strategies may vary among clinicians due to marked heterogeneity in physician opinions. Obviously, the left high-risk ANOCOR are easier entrusted to surgeon. Nevertheless, surgical repair of right high-risk ANOCOR has been reported (Garcia-Rinaldi et al., 2004). In contrast to anomalous connections with pulmonary artery, direct aortic reimplantation is rarely possible due to an, almost without exception, intramural segment. Several techniques have been proposed (Said et al. 2010): unroofing of the coronary artery, creation of a neo-ostium, reimplantation of the coronary artery, translocation of the pulmonary artery with patch angioplasty of the coronary artery and coronary bypass grafting. Unroofing consists to a longitudinal excision of the common wall between the aorta and the ectopic coronary artery coursing tangentially with an intramural segment (figure 23). Excision starts at the anomalous ostium and continues into the appropriate sinus. To create a neo-ostium, a probe passed through the intramural segment and the coronary artery is opened at the location at which the probe exits the aortic wall (figure 23). In the absence of intramural segment, reimplantation of the coronary artery is possible. The ectopic ostium is excised and implanted in the appropriate sinus above the sinotubular junction. Another technique may be used in the absence of intramural segment, with a translocation of the pulmonary trunk anteriorly and leftward to avoid a compression of the ectopic artery. This technique may be associated with patch angioplasty of the ectopic coronary artery (Karl et al., 2010). The coronary artery is open well beyond the intramural segment and a patch of autologous pericardium creates a large neo-ostium. Finally, coronary artery bypass grafting with saphenous vein or internal mammary artery is another possibility. Thus, correction of intramural segment is not systematically. The choice of the technique depends of surgeon's preference, anatomical pattern of ANOCOR, and existence of CAD requiring myocardial revascularization. Coronary artery bypass grafting is proposed in older adults with concomitant CAD. The use of venous or arterial conduits in young people exposes to a long-term patency concern, because of competitive flow. For most operators, the creation of a neo-ostium is the more physiological treatment without the risk of take-down of the commissural junction between the right and left coronary sinuses observed sometimes with the unroofing technique. No comparative data exist about these different surgical methods. Surgical practice has evolved in the time from coronary artery bypass grafting to direct surgical repair. To date, unroofing technique seems the more used in young people (Davies et al., 2009, Frommelt et al., 2011, Mainwaring et al. 2011). The rate of perioperative death is near to zero in small series of children and young people (Davies et al., 2009, Erez et al., 2006, Karl et al., 2010). Recently, Krasuski et al. reported the impact of surgery in patients with ANOCOR from the opposite sinus (Krasuski, et al., 2011). A cohort of 301 adults from 210,700 cardiac catherizations performed over a 35-year period was retrospectively analysed. The incidence of anomalous connections with the opposite sinus was of 0.14%, and in 54 of 301 patients (18%) an interarterial course between the aorta and pulmonary artery was identified, thus an incidence of 3/10.000 of high-risk ANOCOR with 18 left ANOCOR (33%) and 36 right ANOCOR (67%). Surgical management was chosen in 28 of 54 patients (52%), and in 8 of 18 left ANOCOR (44%) and 20 of 36 right ANOCOR (56%). Coronary artery bypass grafting was used as treatment in most of cases (71%). At 10 years, no difference in survival was observed between interarterial ANOCOR managed surgically or medically. Some characteristics of the cohort studied may explain the lack of benefit associated with surgical repair. As aforementioned, the study population consisted of relatively old patients with a mean age of 58 years and with a high prevalence of CAD.

Proximal Anomalous Connections of Coronary Arteries in Adults 223

Coronary angioplasty with stenting is a more recent therapeutic method in the field of ANOCOR. So far, only cases report or small groups of ANOCOR have been reported. Percutaneous coronary intervention (PCI) in ANOCOR with and without associated CAD must be distinguished. In fact, most of PCI are performed in ANOCOR with significant atherosclerotic lesions within or distal to ectopic course, and sometimes during acute coronary syndrome (Jaffe et al., 2009). PCI may be attractive as an alternative to surgical repair in young patients without CAD. Nevertheless, the capacities of a stent to adapt to anatomical characteristics of an ANOCOR with an intramural segment should be accurately analyzed before its use can be recommended. Hariharan et al. displayed potential concerns with canulation difficult, incomplete coverage of ostium without protruding into the aorta, uncertainly regarding the true diameter, risk of aortic dissection, and unknown restenosis risk (Hariharan et al. 2002). Angelini et al. suggest the use of PCI in patients with symptomatic right ANOCOR associated with ischemia induced by a nuclear stress test (Angelini et al., 2007). During cardiac catheterization, IVUS is crucial to evaluate the minimal lumen area, length of intramural segment, arterial diameters and results after stent deployment. However, evaluation data of interventional treatment of right ANOCOR are currently too poor to envisage on extension of this technique to left ANOCOR. In AHA/ACC 2008 guidelines, PCI

Among the wide spectrum of congenital abnormalities of coronary arteries, proximal anomalous connections of coronary arteries (ANOCOR) represent a significant subgroup, which continues to promote debate. A more simple classification, and recognized by all, would have the advantage to avoid confusions. A non insignificant prevalence of about 1% in the general population and, in particular, the presence of anatomical patterns associated with a risk of sudden death require that the current research supplies data sufficiently robust in order to improve the management of ANOCOR. Young competitive athletes are a well-known population which pays a heavy toll to high-risk ANOCOR. Cooperation between physicians is necessary in order to prevent sport-related fatalities and to determine the modalities of cost-effective screening. From a diagnostic point of view, tomographic techniques, especially CT angiography, represent today an unrivaled tool by their ability to supply multiple volumetric reconstructions. Therefore, accurate diagnosis of the ectopic origin and non-ambiguous interpretation of the ectopic course are possible in most of cases. However, ANOCOR are still often discovered fortuitously in adult populations during selective coronary angiography by angiographers with sometimes a limited experience in the field of ANOCOR. It seems that the knowledge of angiographers should be improved in order to limit the risk of misinterpretation. Systematic usage of tomographic imaging solves this problem, while at the same time educating angiographers. Currently, high-risk ANOCOR are identified well, however the management of them is heterogeneous, specially the timing and mode of treatment. There clearly remains a gap between the practices and the recommendations, sometimes due to misunderstanding, but also relating to physician opinion. The absence of long-term follow-up after surgical repair and the difficulties of undertaking randomized studies, and as the unknown natural history of ANOCOR all may explain the divergence between clinical practice and current guidelines. Surgical treatment demonstrates a preference for the unroofing technique in ANOCOR arising from the aorta

is not recommended in therapeutic management (Warnes et al., 2008).

**12.4.2 Interventional treatment** 

**13. Perspectives** 

The latter was present in 86% of ANOCOR managed surgically and in 50% of ANOCOR managed medically. Abnormal stress tests were more frequent in surgical patients (90%) versus 43% in patients with medical management. Moreover, whether the primary indication for surgery was CAD or coronary abnormality was not clarified. The authors concluded that the results of their study must not be applied to younger patients because the mortality risk is not the same. Mainwaring et al. reported medium-term results after surgical repair of ANOCOR in 50 patients with a mean age of 14 years (Mainwaring, et al., 2011). Congenital cardiac abnormalities were associated in 14 patients. Interarterial and intramural courses were present in 100% and 70% of patients, respectively. An unroofing procedure was performed in 36 of 50 patients (72%). All patients (n=9) with single coronary ostium and without an intramural path underwent pulmonary artery translocation. With a mean follow-up of 5.3 years, satisfactory follow-up was obtained in 47 patients, and all have remained free of cardiac symptoms. Functional results of modern surgical methods are recognized as good in young people with most of patients free of symptoms at a mediumterm follow-up (Erez et al., 2006, Karl, et al. 2010). However, Brothers et al. reported that subclinical ischemia may occur after surgical repair of anomalous aortic origin of a coronary artery (Brothers et al., 2007). In this study, 9 asymptomatic children or adolescents (5 to 18 years) had post operative evaluations (range 2 to 48 months) suggestive of silent ischemia. This data highlight the need of long-term follow-up in young people undergoing surgical repair of ANOCOR.

Fig. 23. Diagram representation of surgical repair of an anomalous left main coronary artery (LMCA) arising from the right sinus and associated with an intramural segment. A and B: unroofing technique. C and D: creation of a neo-ostium. RCA: right coronary artery.

#### **12.4.2 Interventional treatment**

222 Congenital Heart Disease – Selected Aspects

The latter was present in 86% of ANOCOR managed surgically and in 50% of ANOCOR managed medically. Abnormal stress tests were more frequent in surgical patients (90%) versus 43% in patients with medical management. Moreover, whether the primary indication for surgery was CAD or coronary abnormality was not clarified. The authors concluded that the results of their study must not be applied to younger patients because the mortality risk is not the same. Mainwaring et al. reported medium-term results after surgical repair of ANOCOR in 50 patients with a mean age of 14 years (Mainwaring, et al., 2011). Congenital cardiac abnormalities were associated in 14 patients. Interarterial and intramural courses were present in 100% and 70% of patients, respectively. An unroofing procedure was performed in 36 of 50 patients (72%). All patients (n=9) with single coronary ostium and without an intramural path underwent pulmonary artery translocation. With a mean follow-up of 5.3 years, satisfactory follow-up was obtained in 47 patients, and all have remained free of cardiac symptoms. Functional results of modern surgical methods are recognized as good in young people with most of patients free of symptoms at a mediumterm follow-up (Erez et al., 2006, Karl, et al. 2010). However, Brothers et al. reported that subclinical ischemia may occur after surgical repair of anomalous aortic origin of a coronary artery (Brothers et al., 2007). In this study, 9 asymptomatic children or adolescents (5 to 18 years) had post operative evaluations (range 2 to 48 months) suggestive of silent ischemia. This data highlight the need of long-term follow-up in young people undergoing surgical

Fig. 23. Diagram representation of surgical repair of an anomalous left main coronary artery (LMCA) arising from the right sinus and associated with an intramural segment. A and B: unroofing technique. C and D: creation of a neo-ostium. RCA: right coronary artery.

repair of ANOCOR.

Coronary angioplasty with stenting is a more recent therapeutic method in the field of ANOCOR. So far, only cases report or small groups of ANOCOR have been reported. Percutaneous coronary intervention (PCI) in ANOCOR with and without associated CAD must be distinguished. In fact, most of PCI are performed in ANOCOR with significant atherosclerotic lesions within or distal to ectopic course, and sometimes during acute coronary syndrome (Jaffe et al., 2009). PCI may be attractive as an alternative to surgical repair in young patients without CAD. Nevertheless, the capacities of a stent to adapt to anatomical characteristics of an ANOCOR with an intramural segment should be accurately analyzed before its use can be recommended. Hariharan et al. displayed potential concerns with canulation difficult, incomplete coverage of ostium without protruding into the aorta, uncertainly regarding the true diameter, risk of aortic dissection, and unknown restenosis risk (Hariharan et al. 2002). Angelini et al. suggest the use of PCI in patients with symptomatic right ANOCOR associated with ischemia induced by a nuclear stress test (Angelini et al., 2007). During cardiac catheterization, IVUS is crucial to evaluate the minimal lumen area, length of intramural segment, arterial diameters and results after stent deployment. However, evaluation data of interventional treatment of right ANOCOR are currently too poor to envisage on extension of this technique to left ANOCOR. In AHA/ACC 2008 guidelines, PCI is not recommended in therapeutic management (Warnes et al., 2008).

#### **13. Perspectives**

Among the wide spectrum of congenital abnormalities of coronary arteries, proximal anomalous connections of coronary arteries (ANOCOR) represent a significant subgroup, which continues to promote debate. A more simple classification, and recognized by all, would have the advantage to avoid confusions. A non insignificant prevalence of about 1% in the general population and, in particular, the presence of anatomical patterns associated with a risk of sudden death require that the current research supplies data sufficiently robust in order to improve the management of ANOCOR. Young competitive athletes are a well-known population which pays a heavy toll to high-risk ANOCOR. Cooperation between physicians is necessary in order to prevent sport-related fatalities and to determine the modalities of cost-effective screening. From a diagnostic point of view, tomographic techniques, especially CT angiography, represent today an unrivaled tool by their ability to supply multiple volumetric reconstructions. Therefore, accurate diagnosis of the ectopic origin and non-ambiguous interpretation of the ectopic course are possible in most of cases. However, ANOCOR are still often discovered fortuitously in adult populations during selective coronary angiography by angiographers with sometimes a limited experience in the field of ANOCOR. It seems that the knowledge of angiographers should be improved in order to limit the risk of misinterpretation. Systematic usage of tomographic imaging solves this problem, while at the same time educating angiographers. Currently, high-risk ANOCOR are identified well, however the management of them is heterogeneous, specially the timing and mode of treatment. There clearly remains a gap between the practices and the recommendations, sometimes due to misunderstanding, but also relating to physician opinion. The absence of long-term follow-up after surgical repair and the difficulties of undertaking randomized studies, and as the unknown natural history of ANOCOR all may explain the divergence between clinical practice and current guidelines. Surgical treatment demonstrates a preference for the unroofing technique in ANOCOR arising from the aorta

Proximal Anomalous Connections of Coronary Arteries in Adults 225

Angelini, P.; Villason, S.; Chan, C. & Diez, J. (1999). Normal and anomalous coronary

Angelini, P.; Walmsley, R.; Cheong, B, & Ott, A. (2010). Coronary artery originating from the

Angelini, P.; Walmsley, R.; Libreros, A. & Ott, D. (2006). Symptomatic anomalous

Aydinlar, A.; Cicek, D.; Sentruk, T.; Gemici, K.; Serdar, O.; Kazazoglu, A.; Kumbay, E. &

in young competitive athletes. *J Am Coll Cardiol*; Vol. 35, pp. 1493–1501. Baumgartner, H.; Bonhoeffer, P.; De Groot, N.; de Haan, F.; Deanfield, J.; Galie, N.;

Ben Ali, W.; Metton, O.; Roubertie, F.; Pouard, P.; Sidi, D.; Raisky, O. & Vouhé P. (2009).

Bland, E.; White, P. & Garland, J. (1933). Congenital anomalies of the coronary arteries:

Bogers, A.; Gittenberger-de Groot, A.; Poelman, R.; Péault, B. & Huysmans, H. (1989).

Brothers, J.; Carter, C.; McBride, M.; Spry, T. & Paridon, S. (2010). Anomalous left coronary

Brothers, J.; Gaynor, J.; Jacobs, J.; Caldarone, C.; Jegatheeswaran, A. & Jacobs, M. (2010). The

Brothers, J.; Gaynor, J.; Paridon, S.; Lorber, R. & Jacobs, M. (2009). Anomalous aortic origin

strategies in children and young adults. *Pediatr Cardiol*, Vol. 30, pp. 911-921. Brothers, J.; McBride, M.; Seliem, M.; Marino, B.; Tomlinson, R.; Pampaloni, M.; Gaynor,J.;

Surgeons' Society. *Cardio Young*, Vol. 20 (Suppl. 3), pp. 50-58.

pediatric patients. *J Am Coll Cardiol*, Vol 50, pp. 2078-2082.

outgrowth? *Anat Embryol*, Vol. 180, pp. 437-441.

*J Thorac Cardiovasc Surg*, Vol. 140, pp. e27-e29.

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stenosis. *Tex Heart Inst J*, Vol. 37, pp. 221-225.

*Ann Cardiol Angeiol*, Vol. 57, pp. 327-334.

Vol. 31, pp. 2915-2957.

pp. 787-801.

arteries in humans. In: *Coronary artery anomalies: a comprehensive approach*. Angelini,

normal sinus but with acute angulation and intramural course, leading to critical

origination of the left coronary artery from the opposite sinus of Valsalva. Clinical presentations, diagnosis, and surgical repair. *Tex Heart Inst J*, Vol. 33, pp. 171-179. Aubry, P.; Joudinaud, T. & Hyafil, F. (2008). Anomalous origin of coronary arteries in adults.

Cordan, J. Primary congenital anomalies of the coronary arteries. A coronary arteriographic study in western Turkey. (2005). *Int Heart J*, Vol. 46, pp. 97-103. Basso, C; Maron ,B; Corrado, D. & Thiene, G. (2000). Clinical profile of congenital coronary

artery anomalies with origin from the wrong aortic sinus leading to sudden death

Gatzoulis, M.; Gohleke-Barewolf C.; Kaemmerer, H.; Kilner, P.; Meijboom, F.; Mulder, B.; Oechsli, E.; Oliver, J.; Serraf, A.; Szatmari, A.; Thaulow, E.; Vouhe, P. & Walma, E. (2010). ESC Guidelines for the management of grow-up congenital heart disease (new version 2010). The Task Force on the Management of Grow-up Congenital Heart Disease of the European Society of Cardiology (ESC). *Eur Heart J*,

Anomalous origin of the left coronary artery from the pulmonary artery: late results with special attention to the mitral valve. *Eur J Cardiothorac Surg*, Vol. 36, pp. 244-248.

report of an unusual case associated with cardiac hypertrophy. *Am Heart J*, Vol. 8,

Development of the origin of the coronary arteries, a matter of ingrowth or

artery origin from the opposite sinus of Valsalva: evidence of intermittent ischemia.

registry of anomalous aortic origin of the coronary artery of The Congenital Heart

of a coronary artery with an interatrial course: understanging current management

Spray, T. & Paridon, S. (2007). Evaluation of myocardial ischemia following surgical repair of anomalous aortic origin of a coronary artery in a series of

and direct aortic implantation for ANOCOR connected with the pulmonary artery. Perioperative risks are low, except in some anomalous connections with the pulmonary artery, but the long-term evaluation is lacking. To date, the role of PCI remains undetermined. The low incidence of ANOCOR requiring a percutaneous or a surgical repair would justify that these congenital abnormalities being taken care off in a center specialized in the management of ANOCOR. Several attempts have been made to set up observational registries to determine the outcome of different strategies in the field of ANOCOR (Angelini 2007, Aubry et al., 2008, Brothers et al., 2007, Pelliccia, 2001). To date, two registries are ongoing, one in North and South America, and one in France. The registry of anomalous aortic origin of the coronary artery of The Congenital Heart Surgeons' Society (www.chssdc.com) has been set up to determine the outcome of children or young adults (≤ 30-year old) with high-risk ANOCOR. This registry includes those managed conservatively and with surgical intervention. The ANOCOR with interarterial, intramural, and/or intraseptal courses are classified as high-risk. The registry consists of a retrospective cohort of patients diagnosed between 1 January, 1998 and 20 January, 2009 and a prospective cohort of patients newly diagnosed from 21 January, 2009. The registry of proximal anomalous connections of coronary arteries (ANOCOR Registry) of the French Society of Cardiology (www.sfcardio.fr) is a prospective observational study of patients (≥ 15-year old) diagnosed with an ANOCOR. The main objective of this registry is to describe the chosen therapeutic strategies according to the type of ANOCOR. The secondary objectives are to describe the cardiac morbidity and mortality and to estimate the impact of different therapeutic strategies at a 5-year follow-up. The ANOCOR registry started 31 January, 2010 with an inclusion period of 3 years. With such multicenter registries dedicated to ANOCOR, evidence-based guidelines will probably be easier to establish in an attempt to achieve a better understanding of the clinical profile and the impact of interventional correction on the natural history of these congenital coronary abnormalities.

#### **14. References**


and direct aortic implantation for ANOCOR connected with the pulmonary artery. Perioperative risks are low, except in some anomalous connections with the pulmonary artery, but the long-term evaluation is lacking. To date, the role of PCI remains undetermined. The low incidence of ANOCOR requiring a percutaneous or a surgical repair would justify that these congenital abnormalities being taken care off in a center specialized in the management of ANOCOR. Several attempts have been made to set up observational registries to determine the outcome of different strategies in the field of ANOCOR (Angelini 2007, Aubry et al., 2008, Brothers et al., 2007, Pelliccia, 2001). To date, two registries are ongoing, one in North and South America, and one in France. The registry of anomalous aortic origin of the coronary artery of The Congenital Heart Surgeons' Society (www.chssdc.com) has been set up to determine the outcome of children or young adults (≤ 30-year old) with high-risk ANOCOR. This registry includes those managed conservatively and with surgical intervention. The ANOCOR with interarterial, intramural, and/or intraseptal courses are classified as high-risk. The registry consists of a retrospective cohort of patients diagnosed between 1 January, 1998 and 20 January, 2009 and a prospective cohort of patients newly diagnosed from 21 January, 2009. The registry of proximal anomalous connections of coronary arteries (ANOCOR Registry) of the French Society of Cardiology (www.sfcardio.fr) is a prospective observational study of patients (≥ 15-year old) diagnosed with an ANOCOR. The main objective of this registry is to describe the chosen therapeutic strategies according to the type of ANOCOR. The secondary objectives are to describe the cardiac morbidity and mortality and to estimate the impact of different therapeutic strategies at a 5-year follow-up. The ANOCOR registry started 31 January, 2010 with an inclusion period of 3 years. With such multicenter registries dedicated to ANOCOR, evidence-based guidelines will probably be easier to establish in an attempt to achieve a better understanding of the clinical profile and the impact of interventional correction on the

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**Part 4** 

**Management of Congenital Heart Disease** 

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