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Annuloaortic Ectasia in a Patient With Congenital Absence of the Left Pericardium

2013; Elsevier BV; Volume: 96; Issue: 6 Linguagem: Inglês

10.1016/j.athoracsur.2013.04.106

1552-6259

Chigozirim N. Ekeke, Curt J. Daniels, Subha V. Raman, Charles L. Hitchcock, Steven E. Katz, Juan A. Crestanello,

Cardiac Structural Anomalies and Repair

We report a patient with congenital absence of the left pericardium with development of progressive annuloaortic ectasia and aortic insufficiency during a 12-year period. The patient was treated with a Bentall procedure. Pathologic examination of the aorta revealed cystic medial necrosis. The surgical management and a possible association between congenital absence of pericardium and Marfan syndrome are discussed. We report a patient with congenital absence of the left pericardium with development of progressive annuloaortic ectasia and aortic insufficiency during a 12-year period. The patient was treated with a Bentall procedure. Pathologic examination of the aorta revealed cystic medial necrosis. The surgical management and a possible association between congenital absence of pericardium and Marfan syndrome are discussed. Congenital absence of the left pericardium (CAP) usually presents as an incidental finding during evaluation for associated cardiac anomalies [1Nasser W. Feigenbaum H. Helmen C. Congenital absence of the left pericardium.Circulation. 1966; 34: 100-104Crossref PubMed Scopus (5) Google Scholar]. The extreme cardiac displacement and abnormal location of cardiac and mediastinum structures can make cardiac procedures challenging [2Gatzoulis M. Munk M. Merchant N. Van Arsdell G. McCrindle B. Webb G. Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management.Ann Thorac Surg. 2000; 69: 1209-1215Abstract Full Text Full Text PDF PubMed Scopus (130) Google Scholar]. Coronary artery bypass grafting and valve operations have been described in patients with CAP [2Gatzoulis M. Munk M. Merchant N. Van Arsdell G. McCrindle B. Webb G. Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management.Ann Thorac Surg. 2000; 69: 1209-1215Abstract Full Text Full Text PDF PubMed Scopus (130) Google Scholar, 3Muthialu N. Kostolny M. Rees P. Phrenic nerve at greater risk: congenital absence of pericardium exposing the nerve.Ann Thorac Surg. 2012; 94: 27Abstract Full Text Full Text PDF Scopus (1) Google Scholar]. CAP can be an isolated defect or can be associated with intracardiac and extracardiac defects [1Nasser W. Feigenbaum H. Helmen C. Congenital absence of the left pericardium.Circulation. 1966; 34: 100-104Crossref PubMed Scopus (5) Google Scholar, 2Gatzoulis M. Munk M. Merchant N. Van Arsdell G. McCrindle B. Webb G. Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management.Ann Thorac Surg. 2000; 69: 1209-1215Abstract Full Text Full Text PDF PubMed Scopus (130) Google Scholar]. The association of CAP with Marfan syndrome or with other connective disorders that predispose patients to aortic aneurysms is not well established.We present a case of CAP in a patient with marfanoid features. Progressive annuloaortic ectasia and congestive heart failure, secondary to aortic insufficiency, developed over a 12-year period.A 48-year-old man diagnosed with congenital absence of the pericardium (CAP) 12 years earlier [4Raman S. Daniels C. Katz S. Ryan J. King M. Congenital absence of the pericardium.Circulation. 2001; 104: 1447-1448Crossref PubMed Scopus (14) Google Scholar] presented with dyspnea upon exertion and increased neck pulsations. A type A aortic dissection had occurred in the patient's brother when he was in his early 40s.The physical examination revealed an increased pulse pressure and a diastolic murmur. Echocardiography demonstrated severe aortic insufficiency and annuloaortic ectasia. The sonographic windows were poor. Cardiac magnetic resonance imaging confirmed severe aortic insufficiency, with a regurgitant fraction of 50%. The aortic valve was trileaflet. The left ventricular ejection fraction was 0.45, and the left ventricle dimensions were 76 mm in end-diastole and 47 mm in end-systole. As expected, there was displacement of the heart into the left chest, with the apex pointing posteriorly.Computed tomography angiography showed that the aortic annulus, the sinuses of Valsalva, and the sinotubular junction were aneurysmal (Fig 1). The arch and descending aorta were normal in diameter.We recommended surgical intervention based on the presence of severe symptomatic aortic regurgitation with decreased left ventricular ejection fraction and dilated left ventricle and the presence of annuloaortic ectasia. Upon sternotomy, the heart was covered by the right lung. No adhesions were present. The left phrenic nerve was located anteriorly to the right atrioventricular groove. The aorta, pulmonary artery, and superior and inferior vena cava were in normal position. The location of the aortic sinuses and coronary ostium was normal. The ventricular cavities were displaced toward the left and posteriorly. There was a free pericardial edge next to the right atrium covering the right phrenic nerve.Cardiopulmonary bypass was instituted by cannulating the distal ascending aorta and the right atrium. The ascending aorta, sinuses of Valsalva, and aortic leaflets were excised. Buttons containing the coronary arteries were fashioned. A Bentall procedure was performed with a 32-mm valved conduit. Histologic examination of the ascending aortic wall demonstrated medial necrosis (Fig 1). The patient recovered well and is doing well 1 year after the procedure.CommentCAP is uncommon. It is usually diagnosed as an incidental finding in patients being evaluated for chest pain or other cardiac symptoms [1Nasser W. Feigenbaum H. Helmen C. Congenital absence of the left pericardium.Circulation. 1966; 34: 100-104Crossref PubMed Scopus (5) Google Scholar]. Coronary artery bypass grafting and ascending aorta replacement for type A dissection have been reported in these patients [2Gatzoulis M. Munk M. Merchant N. Van Arsdell G. McCrindle B. Webb G. Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management.Ann Thorac Surg. 2000; 69: 1209-1215Abstract Full Text Full Text PDF PubMed Scopus (130) Google Scholar, 3Muthialu N. Kostolny M. Rees P. Phrenic nerve at greater risk: congenital absence of pericardium exposing the nerve.Ann Thorac Surg. 2012; 94: 27Abstract Full Text Full Text PDF Scopus (1) Google Scholar, 5Amiri A. Weber C. Schlosser V. Meinertz T.H. Coronary artery disease in a patient with a congenital pericardial defect.Thorac Cardiovasc Surg. 1989; 37: 379-381Crossref PubMed Scopus (7) Google Scholar, 6Galicia-Tornell M.M. Marín-Solís B. Fuentes-Orozco C. Martínez-Martínez M. Villalpando-Mendoza E. Ramírez-Orozco F. Bentall procedure in ascending aortic aneurysm: hospital mortality.Cir Cir J. 2010; 78: 43-49Google Scholar]. Cardiac operations in these patients can be challenging because of the abnormal location of the cardiac structures. Particular attention should be paid to the location of the left phrenic nerve, which is usually located anterior to the heart and the great vessels.During coronary artery bypass grafting, measures should be taken to prevent kinking of the grafts, particularly the left internal mammary artery. The venous grafts to the lateral wall coronary vessels need to be longer because the heart is displaced posteriorly and laterally.Aortic root replacement and the anatomy of the aortic root in the setting of CAP with extreme left and posterior rotation of the heart have not been well described. The aortic root in this patient had the usual anatomic configuration, and the locations of the coronary and noncoronary sinuses, coronary ostia, and coronary artery origins were normal. Arterial and venous cannulation, retrograde cardioplegia cannula, left ventricular vent, and root vent placement were conducted routinely. The left phrenic nerve needed to be mobilized laterally because it was running in front of the aorta, right atrial appendage, and right ventricle.CAP occurs as an isolated defect in most patients. However, it can be associated with cardiac or extracardiac defects in 30% of patients [1Nasser W. Feigenbaum H. Helmen C. Congenital absence of the left pericardium.Circulation. 1966; 34: 100-104Crossref PubMed Scopus (5) Google Scholar]. A link between CAP and aortic connective tissue disorders is not established, but two previous studies have reported that association [7Abbas A. Appleton C. Liu P. Sweeney J. Congenital absence of the pericardium: case presentation and review of literature.Int J Cardiol. 2005; 98: 21-25Abstract Full Text Full Text PDF PubMed Scopus (81) Google Scholar, 8Corti P. Pagnoni N. Mangano G. Bordiga G. Lorini G. Congenital absence of pericardium in a case of a variant of Marfan syndrome.Recenti Prog Med. 1989; 80: 63-66PubMed Google Scholar]. In addition, several cases of aortic dissections have been described in these patients. The current patient had a family history of aortic dissection at a young age, and progressive dilatation of the aortic root had occurred during a 12-year period. Histologic findings were similar to those in patients with connective tissue disorder.Medial necrosis is the histologic hallmark of patients with Marfan syndrome. It is also commonly seen in other groups who present with aortic dissections or aneurysms [6Galicia-Tornell M.M. Marín-Solís B. Fuentes-Orozco C. Martínez-Martínez M. Villalpando-Mendoza E. Ramírez-Orozco F. Bentall procedure in ascending aortic aneurysm: hospital mortality.Cir Cir J. 2010; 78: 43-49Google Scholar]. Non-Marfan patients with medial necrosis are at high risk of late adverse aortic events and should be aggressively monitored and treated to avoid acute aortic syndromes in other territories [8Corti P. Pagnoni N. Mangano G. Bordiga G. Lorini G. Congenital absence of pericardium in a case of a variant of Marfan syndrome.Recenti Prog Med. 1989; 80: 63-66PubMed Google Scholar]. This patient did not meet the Gent criteria for the diagnosis of Marfan syndrome [9Loeys B. Dietz H. Braverman A. et al.The revised Ghent nosology for the Marfan syndrome.J Med Genet. 2010; 47: 476-485Crossref PubMed Scopus (1289) Google Scholar], but his family history, presentation, and histologic assessment were highly suggestive of Marfan syndrome or another aortic connective tissue disorder. Lifetime surveillance is recommended for aortic dilatation.In conclusion, the aortic root anatomy in patients with CAP is preserved. Therefore, aortic root replacement can be safely performed without the need for technical modifications. Surgeons should be aware of the abnormal anterior location of the left phrenic nerve. CAP may be associated with abnormalities of the aortic wall that predispose patients to aortic aneurysm or dissection. Patients with CAP should be screened for root and ascending aorta aneurysms. Additional studies are necessary to further define this association. Congenital absence of the left pericardium (CAP) usually presents as an incidental finding during evaluation for associated cardiac anomalies [1Nasser W. Feigenbaum H. Helmen C. Congenital absence of the left pericardium.Circulation. 1966; 34: 100-104Crossref PubMed Scopus (5) Google Scholar]. The extreme cardiac displacement and abnormal location of cardiac and mediastinum structures can make cardiac procedures challenging [2Gatzoulis M. Munk M. Merchant N. Van Arsdell G. McCrindle B. Webb G. Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management.Ann Thorac Surg. 2000; 69: 1209-1215Abstract Full Text Full Text PDF PubMed Scopus (130) Google Scholar]. Coronary artery bypass grafting and valve operations have been described in patients with CAP [2Gatzoulis M. Munk M. Merchant N. Van Arsdell G. McCrindle B. Webb G. Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management.Ann Thorac Surg. 2000; 69: 1209-1215Abstract Full Text Full Text PDF PubMed Scopus (130) Google Scholar, 3Muthialu N. Kostolny M. Rees P. Phrenic nerve at greater risk: congenital absence of pericardium exposing the nerve.Ann Thorac Surg. 2012; 94: 27Abstract Full Text Full Text PDF Scopus (1) Google Scholar]. CAP can be an isolated defect or can be associated with intracardiac and extracardiac defects [1Nasser W. Feigenbaum H. Helmen C. Congenital absence of the left pericardium.Circulation. 1966; 34: 100-104Crossref PubMed Scopus (5) Google Scholar, 2Gatzoulis M. Munk M. Merchant N. Van Arsdell G. McCrindle B. Webb G. Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management.Ann Thorac Surg. 2000; 69: 1209-1215Abstract Full Text Full Text PDF PubMed Scopus (130) Google Scholar]. The association of CAP with Marfan syndrome or with other connective disorders that predispose patients to aortic aneurysms is not well established. We present a case of CAP in a patient with marfanoid features. Progressive annuloaortic ectasia and congestive heart failure, secondary to aortic insufficiency, developed over a 12-year period. A 48-year-old man diagnosed with congenital absence of the pericardium (CAP) 12 years earlier [4Raman S. Daniels C. Katz S. Ryan J. King M. Congenital absence of the pericardium.Circulation. 2001; 104: 1447-1448Crossref PubMed Scopus (14) Google Scholar] presented with dyspnea upon exertion and increased neck pulsations. A type A aortic dissection had occurred in the patient's brother when he was in his early 40s. The physical examination revealed an increased pulse pressure and a diastolic murmur. Echocardiography demonstrated severe aortic insufficiency and annuloaortic ectasia. The sonographic windows were poor. Cardiac magnetic resonance imaging confirmed severe aortic insufficiency, with a regurgitant fraction of 50%. The aortic valve was trileaflet. The left ventricular ejection fraction was 0.45, and the left ventricle dimensions were 76 mm in end-diastole and 47 mm in end-systole. As expected, there was displacement of the heart into the left chest, with the apex pointing posteriorly. Computed tomography angiography showed that the aortic annulus, the sinuses of Valsalva, and the sinotubular junction were aneurysmal (Fig 1). The arch and descending aorta were normal in diameter. We recommended surgical intervention based on the presence of severe symptomatic aortic regurgitation with decreased left ventricular ejection fraction and dilated left ventricle and the presence of annuloaortic ectasia. Upon sternotomy, the heart was covered by the right lung. No adhesions were present. The left phrenic nerve was located anteriorly to the right atrioventricular groove. The aorta, pulmonary artery, and superior and inferior vena cava were in normal position. The location of the aortic sinuses and coronary ostium was normal. The ventricular cavities were displaced toward the left and posteriorly. There was a free pericardial edge next to the right atrium covering the right phrenic nerve. Cardiopulmonary bypass was instituted by cannulating the distal ascending aorta and the right atrium. The ascending aorta, sinuses of Valsalva, and aortic leaflets were excised. Buttons containing the coronary arteries were fashioned. A Bentall procedure was performed with a 32-mm valved conduit. Histologic examination of the ascending aortic wall demonstrated medial necrosis (Fig 1). The patient recovered well and is doing well 1 year after the procedure. CommentCAP is uncommon. It is usually diagnosed as an incidental finding in patients being evaluated for chest pain or other cardiac symptoms [1Nasser W. Feigenbaum H. Helmen C. Congenital absence of the left pericardium.Circulation. 1966; 34: 100-104Crossref PubMed Scopus (5) Google Scholar]. Coronary artery bypass grafting and ascending aorta replacement for type A dissection have been reported in these patients [2Gatzoulis M. Munk M. Merchant N. Van Arsdell G. McCrindle B. Webb G. Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management.Ann Thorac Surg. 2000; 69: 1209-1215Abstract Full Text Full Text PDF PubMed Scopus (130) Google Scholar, 3Muthialu N. Kostolny M. Rees P. Phrenic nerve at greater risk: congenital absence of pericardium exposing the nerve.Ann Thorac Surg. 2012; 94: 27Abstract Full Text Full Text PDF Scopus (1) Google Scholar, 5Amiri A. Weber C. Schlosser V. Meinertz T.H. Coronary artery disease in a patient with a congenital pericardial defect.Thorac Cardiovasc Surg. 1989; 37: 379-381Crossref PubMed Scopus (7) Google Scholar, 6Galicia-Tornell M.M. Marín-Solís B. Fuentes-Orozco C. Martínez-Martínez M. Villalpando-Mendoza E. Ramírez-Orozco F. Bentall procedure in ascending aortic aneurysm: hospital mortality.Cir Cir J. 2010; 78: 43-49Google Scholar]. Cardiac operations in these patients can be challenging because of the abnormal location of the cardiac structures. Particular attention should be paid to the location of the left phrenic nerve, which is usually located anterior to the heart and the great vessels.During coronary artery bypass grafting, measures should be taken to prevent kinking of the grafts, particularly the left internal mammary artery. The venous grafts to the lateral wall coronary vessels need to be longer because the heart is displaced posteriorly and laterally.Aortic root replacement and the anatomy of the aortic root in the setting of CAP with extreme left and posterior rotation of the heart have not been well described. The aortic root in this patient had the usual anatomic configuration, and the locations of the coronary and noncoronary sinuses, coronary ostia, and coronary artery origins were normal. Arterial and venous cannulation, retrograde cardioplegia cannula, left ventricular vent, and root vent placement were conducted routinely. The left phrenic nerve needed to be mobilized laterally because it was running in front of the aorta, right atrial appendage, and right ventricle.CAP occurs as an isolated defect in most patients. However, it can be associated with cardiac or extracardiac defects in 30% of patients [1Nasser W. Feigenbaum H. Helmen C. Congenital absence of the left pericardium.Circulation. 1966; 34: 100-104Crossref PubMed Scopus (5) Google Scholar]. A link between CAP and aortic connective tissue disorders is not established, but two previous studies have reported that association [7Abbas A. Appleton C. Liu P. Sweeney J. Congenital absence of the pericardium: case presentation and review of literature.Int J Cardiol. 2005; 98: 21-25Abstract Full Text Full Text PDF PubMed Scopus (81) Google Scholar, 8Corti P. Pagnoni N. Mangano G. Bordiga G. Lorini G. Congenital absence of pericardium in a case of a variant of Marfan syndrome.Recenti Prog Med. 1989; 80: 63-66PubMed Google Scholar]. In addition, several cases of aortic dissections have been described in these patients. The current patient had a family history of aortic dissection at a young age, and progressive dilatation of the aortic root had occurred during a 12-year period. Histologic findings were similar to those in patients with connective tissue disorder.Medial necrosis is the histologic hallmark of patients with Marfan syndrome. It is also commonly seen in other groups who present with aortic dissections or aneurysms [6Galicia-Tornell M.M. Marín-Solís B. Fuentes-Orozco C. Martínez-Martínez M. Villalpando-Mendoza E. Ramírez-Orozco F. Bentall procedure in ascending aortic aneurysm: hospital mortality.Cir Cir J. 2010; 78: 43-49Google Scholar]. Non-Marfan patients with medial necrosis are at high risk of late adverse aortic events and should be aggressively monitored and treated to avoid acute aortic syndromes in other territories [8Corti P. Pagnoni N. Mangano G. Bordiga G. Lorini G. Congenital absence of pericardium in a case of a variant of Marfan syndrome.Recenti Prog Med. 1989; 80: 63-66PubMed Google Scholar]. This patient did not meet the Gent criteria for the diagnosis of Marfan syndrome [9Loeys B. Dietz H. Braverman A. et al.The revised Ghent nosology for the Marfan syndrome.J Med Genet. 2010; 47: 476-485Crossref PubMed Scopus (1289) Google Scholar], but his family history, presentation, and histologic assessment were highly suggestive of Marfan syndrome or another aortic connective tissue disorder. Lifetime surveillance is recommended for aortic dilatation.In conclusion, the aortic root anatomy in patients with CAP is preserved. Therefore, aortic root replacement can be safely performed without the need for technical modifications. Surgeons should be aware of the abnormal anterior location of the left phrenic nerve. CAP may be associated with abnormalities of the aortic wall that predispose patients to aortic aneurysm or dissection. Patients with CAP should be screened for root and ascending aorta aneurysms. Additional studies are necessary to further define this association. CAP is uncommon. It is usually diagnosed as an incidental finding in patients being evaluated for chest pain or other cardiac symptoms [1Nasser W. Feigenbaum H. Helmen C. Congenital absence of the left pericardium.Circulation. 1966; 34: 100-104Crossref PubMed Scopus (5) Google Scholar]. Coronary artery bypass grafting and ascending aorta replacement for type A dissection have been reported in these patients [2Gatzoulis M. Munk M. Merchant N. Van Arsdell G. McCrindle B. Webb G. Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management.Ann Thorac Surg. 2000; 69: 1209-1215Abstract Full Text Full Text PDF PubMed Scopus (130) Google Scholar, 3Muthialu N. Kostolny M. Rees P. Phrenic nerve at greater risk: congenital absence of pericardium exposing the nerve.Ann Thorac Surg. 2012; 94: 27Abstract Full Text Full Text PDF Scopus (1) Google Scholar, 5Amiri A. Weber C. Schlosser V. Meinertz T.H. Coronary artery disease in a patient with a congenital pericardial defect.Thorac Cardiovasc Surg. 1989; 37: 379-381Crossref PubMed Scopus (7) Google Scholar, 6Galicia-Tornell M.M. Marín-Solís B. Fuentes-Orozco C. Martínez-Martínez M. Villalpando-Mendoza E. Ramírez-Orozco F. Bentall procedure in ascending aortic aneurysm: hospital mortality.Cir Cir J. 2010; 78: 43-49Google Scholar]. Cardiac operations in these patients can be challenging because of the abnormal location of the cardiac structures. Particular attention should be paid to the location of the left phrenic nerve, which is usually located anterior to the heart and the great vessels. During coronary artery bypass grafting, measures should be taken to prevent kinking of the grafts, particularly the left internal mammary artery. The venous grafts to the lateral wall coronary vessels need to be longer because the heart is displaced posteriorly and laterally. Aortic root replacement and the anatomy of the aortic root in the setting of CAP with extreme left and posterior rotation of the heart have not been well described. The aortic root in this patient had the usual anatomic configuration, and the locations of the coronary and noncoronary sinuses, coronary ostia, and coronary artery origins were normal. Arterial and venous cannulation, retrograde cardioplegia cannula, left ventricular vent, and root vent placement were conducted routinely. The left phrenic nerve needed to be mobilized laterally because it was running in front of the aorta, right atrial appendage, and right ventricle. CAP occurs as an isolated defect in most patients. However, it can be associated with cardiac or extracardiac defects in 30% of patients [1Nasser W. Feigenbaum H. Helmen C. Congenital absence of the left pericardium.Circulation. 1966; 34: 100-104Crossref PubMed Scopus (5) Google Scholar]. A link between CAP and aortic connective tissue disorders is not established, but two previous studies have reported that association [7Abbas A. Appleton C. Liu P. Sweeney J. Congenital absence of the pericardium: case presentation and review of literature.Int J Cardiol. 2005; 98: 21-25Abstract Full Text Full Text PDF PubMed Scopus (81) Google Scholar, 8Corti P. Pagnoni N. Mangano G. Bordiga G. Lorini G. Congenital absence of pericardium in a case of a variant of Marfan syndrome.Recenti Prog Med. 1989; 80: 63-66PubMed Google Scholar]. In addition, several cases of aortic dissections have been described in these patients. The current patient had a family history of aortic dissection at a young age, and progressive dilatation of the aortic root had occurred during a 12-year period. Histologic findings were similar to those in patients with connective tissue disorder. Medial necrosis is the histologic hallmark of patients with Marfan syndrome. It is also commonly seen in other groups who present with aortic dissections or aneurysms [6Galicia-Tornell M.M. Marín-Solís B. Fuentes-Orozco C. Martínez-Martínez M. Villalpando-Mendoza E. Ramírez-Orozco F. Bentall procedure in ascending aortic aneurysm: hospital mortality.Cir Cir J. 2010; 78: 43-49Google Scholar]. Non-Marfan patients with medial necrosis are at high risk of late adverse aortic events and should be aggressively monitored and treated to avoid acute aortic syndromes in other territories [8Corti P. Pagnoni N. Mangano G. Bordiga G. Lorini G. Congenital absence of pericardium in a case of a variant of Marfan syndrome.Recenti Prog Med. 1989; 80: 63-66PubMed Google Scholar]. This patient did not meet the Gent criteria for the diagnosis of Marfan syndrome [9Loeys B. Dietz H. Braverman A. et al.The revised Ghent nosology for the Marfan syndrome.J Med Genet. 2010; 47: 476-485Crossref PubMed Scopus (1289) Google Scholar], but his family history, presentation, and histologic assessment were highly suggestive of Marfan syndrome or another aortic connective tissue disorder. Lifetime surveillance is recommended for aortic dilatation. In conclusion, the aortic root anatomy in patients with CAP is preserved. Therefore, aortic root replacement can be safely performed without the need for technical modifications. Surgeons should be aware of the abnormal anterior location of the left phrenic nerve. CAP may be associated with abnormalities of the aortic wall that predispose patients to aortic aneurysm or dissection. Patients with CAP should be screened for root and ascending aorta aneurysms. Additional studies are necessary to further define this association.