Portal de Periódicos da CAPES

Anomalous Left Coronary Artery, Ventricular Septal Defect, and Double Aortic Arch

2005; Elsevier BV; Volume: 80; Issue: 1 Linguagem: Inglês

10.1016/j.athoracsur.2003.12.050

1552-6259

Ganesh Shanmugam, Alistair J. McLennan, James Pollock, Kenneth MacArthur,

Vascular anomalies and interventions

Anomalous origin of the left coronary artery from the pulmonary artery generally occurs in isolation. We report an infant diagnosed with double aortic arch and ventricular septal defect who was found to have an anomalous origin of the left coronary artery from the pulmonary artery at surgery. Successful ventricular septal defect repair, division of the arch, and aortic reimplantation of the left coronary artery was performed. This article describes a combination of lesions previously not described, highlights the altered presentation when multiple lesions coexist, discusses the propensity to miss the anomalous coronary, and reviews the literature that demonstrates the consequences of overlooking this defect. Anomalous origin of the left coronary artery from the pulmonary artery generally occurs in isolation. We report an infant diagnosed with double aortic arch and ventricular septal defect who was found to have an anomalous origin of the left coronary artery from the pulmonary artery at surgery. Successful ventricular septal defect repair, division of the arch, and aortic reimplantation of the left coronary artery was performed. This article describes a combination of lesions previously not described, highlights the altered presentation when multiple lesions coexist, discusses the propensity to miss the anomalous coronary, and reviews the literature that demonstrates the consequences of overlooking this defect. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) or Garland-Bland-White syndrome is a congenital coronary malformation generally occurring as an isolated lesion, although there are a few reports of its association with other intracardiac defects. The presence of ALCAPA in complex congenital heart disease significantly increases the morbidity of the associated malformation. Conversely, correction of the associated defect may decrease the pulmonary artery (PA) pressure resulting in reduced left coronary artery flow and myocardial ischemia.A 4-month-old infant was admitted with stridor and obstructive apnea. Bronchoscopy revealed marked tracheal compression on the right lateral aspect. Magnetic resonance imaging revealed a double aortic arch with tracheal narrowing (Fig 1). Electrocardiogram showed biventricular hypertrophy. Transthoracic echocardiogram revealed a large ventricular septal defect with a large volume left to right shunt. The patient had a volume-loaded left ventricle, heart failure with pulmonary edema, and required ventilation for 5 days before surgery.A median sternotomy was performed and the double aortic arch was identified. The right arch was divided between vascular clamps and the ends were over sewn. Cardiopulmonary bypass was established through aorto-bicaval cannulation with caval snares. Cardioplegic arrest was induced by the antegrade administration of cold cardioplegia into the aortic root.A large 9-mm ventricular septal defect (VSD) was closed using a Dacron patch (Band, AZ) through a right atriotomy. Initial attempts to wean the patient off bypass failed and were associated with high left atrial and pulmonary arterial pressures and poor ventricular contraction. Intraoperative epicardial echocardiography revealed severe left ventricular dysfunction and an anomalous left coronary artery arising from the posterior sinus of the PA (Fig 2).Fig 2Intraoperative echocardiogram reveals the left coronary arising from the pulmonary artery. (AO = aorta; LCA = left coronary artery; PA = pulmonary artery; RCA = right coronary artery.)View Large Image Figure ViewerDownload (PPT)Cardiopulmonary bypass was reinstituted, the right and left pulmonary arteries were taped, and a pericardial patch was harvested. Cardioplegia was infused through the aortic root with branch pulmonary artery occlusion. The main PA was transected to expose the anomalous coronary, which was explanted along with a cuff of pulmonary artery and translocated to the aorta where a trap-door anastomosis was performed using a pericardial patch to complete the anastomosis. The sinus defect in the main PA was reconstructed with an autologous pericardial patch.The patient was weaned off bypass using dopamine and milrinone. Postoperatively her ventricular function gradually improved and the ionotropes were weaned over the next 10 days. Serial echocardiograms showed improvement in ventricular function and antegrade flow in the implanted coronary. She was extubated 2 days later. Thereafter she made an uneventful recovery and was discharged home 3 weeks postoperatively. On postoperative follow-up she remains well with substantial recovery of ventricular function. Symptoms due to the arch were completely relieved. Left ventricular end-diastolic dimension reduced postoperatively and ejection fraction improved. Fractional shortening improved from 34% preoperatively to 39% postoperatively at 1 year.CommentAnomalous origin of the left coronary artery from the pulmonary artery ALCAPA is a rare and usually isolated congenital anomaly [1Backer C.L. Stout M.J. Zales V.R. et al.Anomalous origin of the left coronary artery. A twenty-year review of surgical management.J Thorac Cardiovasc Surg. 1992; 103: 1049-1058PubMed Google Scholar]. It may result from abnormal septation of the conotruncus, from persistence of the pulmonary buds combined with involution of the aortic buds that form the coronary arteries [2Heifetz S.A. Robinowitz M. Mueller K.H. Virmani R. Total anomalous origin of the coronary arteries from the pulmonary artery.Paediatr Cardiol. 1986; 7: 11-18Crossref PubMed Scopus (52) Google Scholar], or from abnormal growth of the peritruncal coronary vasculature ring [3Bogers A.J.J.C. Gittebberger-de Groot A.C. Poelmann R.E. Huysmans H.A. Development of the origin of the coronary arteries, a matter of ingrowth or outgrowth?.Anat Embryol. 1989; 180: 437-441Crossref PubMed Scopus (219) Google Scholar]. Immediate surgical correction with the aim of restoring a two-coronary system is the current standard in patients with ALCAPA. Several techniques have been described to address the anomalous left coronary.Hemodynamic alterations resulting from associated intracardiac lesions may obscure the features of an ALCAPA. An ALCAPA with VSD differs in clinical presentation from an isolated ALCAPA [4Pinsky W.W. Gillette P.C. Duff D.F. et al.Anomalous origin of left coronary artery from the pulmonary artery with ventricular septal defect.Circulation. 1978; 57: 1026-1030Crossref PubMed Scopus (16) Google Scholar]. Patients with both these anomalies present in infancy with manifestations of a large left-right shunt and pulmonary hypertension. The volume overload due to the VSD increases the stress on an ischemic ventricle with poor collaterals.A VSD with pulmonary hypertension and an ALCAPA can mask the ALCAPA. The presence of oxygenated blood under high pressure in the PA, which perfuses the ALCAPA, can initially be beneficial. However when the VSD closes spontaneously or is surgically closed, the PA pressures and oxygen saturation drop, causing coronary hypoperfusion and ischemic ventricular dysfunction and even infarction. This was the probable mechanism for intraoperative ventricular dysfunction after VSD closure in our patient. However, intraoperative diagnosis led to reimplantation of the anomalous artery and eventually a successful surgical outcome.Ideally the ALCAPA should have been picked up preoperatively, thereby preventing the occurrence of ischemic ventricular dysfunction consequent to VSD closure. Left ventricular dysfunction in such patients cannot be explained, by the VSD or the double arch alone, and should raise the possibility of an associated ALCAPA. The preoperative detection of an ALCAPA iscrucial in such patients, and further angiography or directed echocardiography should be performed as necessary. A complete preoperative diagnosis would have resulted in an uncomplicated operative procedure that would have consisted of division of the arch, aortic implantation of the ALCAPA, and closure of the VSD in the first instance.In these patients there is also a prolonged phase of antegrade coronary flow. Consequently adequate collateralization may not be established. Inadequate collateralization would imply that simple ligation of the left coronary artery in these patients, even in an emergency, could be catastrophic, which is a point of surgical significance.Pinsky and colleagues [4Pinsky W.W. Gillette P.C. Duff D.F. et al.Anomalous origin of left coronary artery from the pulmonary artery with ventricular septal defect.Circulation. 1978; 57: 1026-1030Crossref PubMed Scopus (16) Google Scholar] reported 2 patients with VSD and ALCAPA who died after surgical repair despite adequate left coronary perfusion postoperatively. In these cases, an operation was attempted only after extensive but subtle myocardial damage had already occurred. If the anomalous coronary had been picked up earlier, surgical risk would have been substantially reduced.Cottrill and colleagues [5Cottrill C.M. Davis D. McMillen M. O'Connor W.N. Noonan J.A. Todd E.P. Anomalous left coronary artery from the pulmonary artery significance of associated intracardiac defects.J Am Coll Cardiol. 1985; 6: 237-242Abstract Full Text PDF PubMed Scopus (17) Google Scholar] reported 2 patients with anomalous origins of the left coronary artery from the pulmonary artery and an associated defect that masked the anomalous origins. Two other reports in the literature document the combination of an ALCAPA in association with coarctation [6Levin S.E. Dansky R. Kinsley R.H. Origin of left coronary artery from right pulmonary artery co-existing with coarctation of the aorta.Int J Cardiol. 1990; 27: 31-36Abstract Full Text PDF PubMed Scopus (17) Google Scholar] and interrupted aortic arch [7Ma J.S. Choe G. Hwang T.J. Oh B.S. Nam J.H. Anomalous origin of the left anterior descending coronary artery from the pulmonary trunk associated with type B interrupted aortic arch.Pediatr Cardiol. 1994; 15: 143-145PubMed Google Scholar], wherein the ALCAPA was diagnosed after surgery or at autopsy.Our patient had a combination of lesions, previously not reported. The presence of preoperative cardiac dysfunction should alert one to the possibility of associated lesions like ALCAPA.In conclusion, patients with VSD and ALCAPA may have few findings, if any, in the history or examination to suggest a coronary anomaly. It is crucial in these patients to preempt the occurrence of extensive myocardial damage to improve their chances of survival. Diagnostic technology does exist. What is required is a high index of suspicion especially when multiple lesions are involved. Although this represents an unusual constellation of findings and presents a challenging surgical repair, the infant had an excellent outcome. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) or Garland-Bland-White syndrome is a congenital coronary malformation generally occurring as an isolated lesion, although there are a few reports of its association with other intracardiac defects. The presence of ALCAPA in complex congenital heart disease significantly increases the morbidity of the associated malformation. Conversely, correction of the associated defect may decrease the pulmonary artery (PA) pressure resulting in reduced left coronary artery flow and myocardial ischemia. A 4-month-old infant was admitted with stridor and obstructive apnea. Bronchoscopy revealed marked tracheal compression on the right lateral aspect. Magnetic resonance imaging revealed a double aortic arch with tracheal narrowing (Fig 1). Electrocardiogram showed biventricular hypertrophy. Transthoracic echocardiogram revealed a large ventricular septal defect with a large volume left to right shunt. The patient had a volume-loaded left ventricle, heart failure with pulmonary edema, and required ventilation for 5 days before surgery. A median sternotomy was performed and the double aortic arch was identified. The right arch was divided between vascular clamps and the ends were over sewn. Cardiopulmonary bypass was established through aorto-bicaval cannulation with caval snares. Cardioplegic arrest was induced by the antegrade administration of cold cardioplegia into the aortic root. A large 9-mm ventricular septal defect (VSD) was closed using a Dacron patch (Band, AZ) through a right atriotomy. Initial attempts to wean the patient off bypass failed and were associated with high left atrial and pulmonary arterial pressures and poor ventricular contraction. Intraoperative epicardial echocardiography revealed severe left ventricular dysfunction and an anomalous left coronary artery arising from the posterior sinus of the PA (Fig 2). Cardiopulmonary bypass was reinstituted, the right and left pulmonary arteries were taped, and a pericardial patch was harvested. Cardioplegia was infused through the aortic root with branch pulmonary artery occlusion. The main PA was transected to expose the anomalous coronary, which was explanted along with a cuff of pulmonary artery and translocated to the aorta where a trap-door anastomosis was performed using a pericardial patch to complete the anastomosis. The sinus defect in the main PA was reconstructed with an autologous pericardial patch. The patient was weaned off bypass using dopamine and milrinone. Postoperatively her ventricular function gradually improved and the ionotropes were weaned over the next 10 days. Serial echocardiograms showed improvement in ventricular function and antegrade flow in the implanted coronary. She was extubated 2 days later. Thereafter she made an uneventful recovery and was discharged home 3 weeks postoperatively. On postoperative follow-up she remains well with substantial recovery of ventricular function. Symptoms due to the arch were completely relieved. Left ventricular end-diastolic dimension reduced postoperatively and ejection fraction improved. Fractional shortening improved from 34% preoperatively to 39% postoperatively at 1 year. CommentAnomalous origin of the left coronary artery from the pulmonary artery ALCAPA is a rare and usually isolated congenital anomaly [1Backer C.L. Stout M.J. Zales V.R. et al.Anomalous origin of the left coronary artery. A twenty-year review of surgical management.J Thorac Cardiovasc Surg. 1992; 103: 1049-1058PubMed Google Scholar]. It may result from abnormal septation of the conotruncus, from persistence of the pulmonary buds combined with involution of the aortic buds that form the coronary arteries [2Heifetz S.A. Robinowitz M. Mueller K.H. Virmani R. Total anomalous origin of the coronary arteries from the pulmonary artery.Paediatr Cardiol. 1986; 7: 11-18Crossref PubMed Scopus (52) Google Scholar], or from abnormal growth of the peritruncal coronary vasculature ring [3Bogers A.J.J.C. Gittebberger-de Groot A.C. Poelmann R.E. Huysmans H.A. Development of the origin of the coronary arteries, a matter of ingrowth or outgrowth?.Anat Embryol. 1989; 180: 437-441Crossref PubMed Scopus (219) Google Scholar]. Immediate surgical correction with the aim of restoring a two-coronary system is the current standard in patients with ALCAPA. Several techniques have been described to address the anomalous left coronary.Hemodynamic alterations resulting from associated intracardiac lesions may obscure the features of an ALCAPA. An ALCAPA with VSD differs in clinical presentation from an isolated ALCAPA [4Pinsky W.W. Gillette P.C. Duff D.F. et al.Anomalous origin of left coronary artery from the pulmonary artery with ventricular septal defect.Circulation. 1978; 57: 1026-1030Crossref PubMed Scopus (16) Google Scholar]. Patients with both these anomalies present in infancy with manifestations of a large left-right shunt and pulmonary hypertension. The volume overload due to the VSD increases the stress on an ischemic ventricle with poor collaterals.A VSD with pulmonary hypertension and an ALCAPA can mask the ALCAPA. The presence of oxygenated blood under high pressure in the PA, which perfuses the ALCAPA, can initially be beneficial. However when the VSD closes spontaneously or is surgically closed, the PA pressures and oxygen saturation drop, causing coronary hypoperfusion and ischemic ventricular dysfunction and even infarction. This was the probable mechanism for intraoperative ventricular dysfunction after VSD closure in our patient. However, intraoperative diagnosis led to reimplantation of the anomalous artery and eventually a successful surgical outcome.Ideally the ALCAPA should have been picked up preoperatively, thereby preventing the occurrence of ischemic ventricular dysfunction consequent to VSD closure. Left ventricular dysfunction in such patients cannot be explained, by the VSD or the double arch alone, and should raise the possibility of an associated ALCAPA. The preoperative detection of an ALCAPA iscrucial in such patients, and further angiography or directed echocardiography should be performed as necessary. A complete preoperative diagnosis would have resulted in an uncomplicated operative procedure that would have consisted of division of the arch, aortic implantation of the ALCAPA, and closure of the VSD in the first instance.In these patients there is also a prolonged phase of antegrade coronary flow. Consequently adequate collateralization may not be established. Inadequate collateralization would imply that simple ligation of the left coronary artery in these patients, even in an emergency, could be catastrophic, which is a point of surgical significance.Pinsky and colleagues [4Pinsky W.W. Gillette P.C. Duff D.F. et al.Anomalous origin of left coronary artery from the pulmonary artery with ventricular septal defect.Circulation. 1978; 57: 1026-1030Crossref PubMed Scopus (16) Google Scholar] reported 2 patients with VSD and ALCAPA who died after surgical repair despite adequate left coronary perfusion postoperatively. In these cases, an operation was attempted only after extensive but subtle myocardial damage had already occurred. If the anomalous coronary had been picked up earlier, surgical risk would have been substantially reduced.Cottrill and colleagues [5Cottrill C.M. Davis D. McMillen M. O'Connor W.N. Noonan J.A. Todd E.P. Anomalous left coronary artery from the pulmonary artery significance of associated intracardiac defects.J Am Coll Cardiol. 1985; 6: 237-242Abstract Full Text PDF PubMed Scopus (17) Google Scholar] reported 2 patients with anomalous origins of the left coronary artery from the pulmonary artery and an associated defect that masked the anomalous origins. Two other reports in the literature document the combination of an ALCAPA in association with coarctation [6Levin S.E. Dansky R. Kinsley R.H. Origin of left coronary artery from right pulmonary artery co-existing with coarctation of the aorta.Int J Cardiol. 1990; 27: 31-36Abstract Full Text PDF PubMed Scopus (17) Google Scholar] and interrupted aortic arch [7Ma J.S. Choe G. Hwang T.J. Oh B.S. Nam J.H. Anomalous origin of the left anterior descending coronary artery from the pulmonary trunk associated with type B interrupted aortic arch.Pediatr Cardiol. 1994; 15: 143-145PubMed Google Scholar], wherein the ALCAPA was diagnosed after surgery or at autopsy.Our patient had a combination of lesions, previously not reported. The presence of preoperative cardiac dysfunction should alert one to the possibility of associated lesions like ALCAPA.In conclusion, patients with VSD and ALCAPA may have few findings, if any, in the history or examination to suggest a coronary anomaly. It is crucial in these patients to preempt the occurrence of extensive myocardial damage to improve their chances of survival. Diagnostic technology does exist. What is required is a high index of suspicion especially when multiple lesions are involved. Although this represents an unusual constellation of findings and presents a challenging surgical repair, the infant had an excellent outcome. Anomalous origin of the left coronary artery from the pulmonary artery ALCAPA is a rare and usually isolated congenital anomaly [1Backer C.L. Stout M.J. Zales V.R. et al.Anomalous origin of the left coronary artery. A twenty-year review of surgical management.J Thorac Cardiovasc Surg. 1992; 103: 1049-1058PubMed Google Scholar]. It may result from abnormal septation of the conotruncus, from persistence of the pulmonary buds combined with involution of the aortic buds that form the coronary arteries [2Heifetz S.A. Robinowitz M. Mueller K.H. Virmani R. Total anomalous origin of the coronary arteries from the pulmonary artery.Paediatr Cardiol. 1986; 7: 11-18Crossref PubMed Scopus (52) Google Scholar], or from abnormal growth of the peritruncal coronary vasculature ring [3Bogers A.J.J.C. Gittebberger-de Groot A.C. Poelmann R.E. Huysmans H.A. Development of the origin of the coronary arteries, a matter of ingrowth or outgrowth?.Anat Embryol. 1989; 180: 437-441Crossref PubMed Scopus (219) Google Scholar]. Immediate surgical correction with the aim of restoring a two-coronary system is the current standard in patients with ALCAPA. Several techniques have been described to address the anomalous left coronary. Hemodynamic alterations resulting from associated intracardiac lesions may obscure the features of an ALCAPA. An ALCAPA with VSD differs in clinical presentation from an isolated ALCAPA [4Pinsky W.W. Gillette P.C. Duff D.F. et al.Anomalous origin of left coronary artery from the pulmonary artery with ventricular septal defect.Circulation. 1978; 57: 1026-1030Crossref PubMed Scopus (16) Google Scholar]. Patients with both these anomalies present in infancy with manifestations of a large left-right shunt and pulmonary hypertension. The volume overload due to the VSD increases the stress on an ischemic ventricle with poor collaterals. A VSD with pulmonary hypertension and an ALCAPA can mask the ALCAPA. The presence of oxygenated blood under high pressure in the PA, which perfuses the ALCAPA, can initially be beneficial. However when the VSD closes spontaneously or is surgically closed, the PA pressures and oxygen saturation drop, causing coronary hypoperfusion and ischemic ventricular dysfunction and even infarction. This was the probable mechanism for intraoperative ventricular dysfunction after VSD closure in our patient. However, intraoperative diagnosis led to reimplantation of the anomalous artery and eventually a successful surgical outcome. Ideally the ALCAPA should have been picked up preoperatively, thereby preventing the occurrence of ischemic ventricular dysfunction consequent to VSD closure. Left ventricular dysfunction in such patients cannot be explained, by the VSD or the double arch alone, and should raise the possibility of an associated ALCAPA. The preoperative detection of an ALCAPA iscrucial in such patients, and further angiography or directed echocardiography should be performed as necessary. A complete preoperative diagnosis would have resulted in an uncomplicated operative procedure that would have consisted of division of the arch, aortic implantation of the ALCAPA, and closure of the VSD in the first instance. In these patients there is also a prolonged phase of antegrade coronary flow. Consequently adequate collateralization may not be established. Inadequate collateralization would imply that simple ligation of the left coronary artery in these patients, even in an emergency, could be catastrophic, which is a point of surgical significance. Pinsky and colleagues [4Pinsky W.W. Gillette P.C. Duff D.F. et al.Anomalous origin of left coronary artery from the pulmonary artery with ventricular septal defect.Circulation. 1978; 57: 1026-1030Crossref PubMed Scopus (16) Google Scholar] reported 2 patients with VSD and ALCAPA who died after surgical repair despite adequate left coronary perfusion postoperatively. In these cases, an operation was attempted only after extensive but subtle myocardial damage had already occurred. If the anomalous coronary had been picked up earlier, surgical risk would have been substantially reduced. Cottrill and colleagues [5Cottrill C.M. Davis D. McMillen M. O'Connor W.N. Noonan J.A. Todd E.P. Anomalous left coronary artery from the pulmonary artery significance of associated intracardiac defects.J Am Coll Cardiol. 1985; 6: 237-242Abstract Full Text PDF PubMed Scopus (17) Google Scholar] reported 2 patients with anomalous origins of the left coronary artery from the pulmonary artery and an associated defect that masked the anomalous origins. Two other reports in the literature document the combination of an ALCAPA in association with coarctation [6Levin S.E. Dansky R. Kinsley R.H. Origin of left coronary artery from right pulmonary artery co-existing with coarctation of the aorta.Int J Cardiol. 1990; 27: 31-36Abstract Full Text PDF PubMed Scopus (17) Google Scholar] and interrupted aortic arch [7Ma J.S. Choe G. Hwang T.J. Oh B.S. Nam J.H. Anomalous origin of the left anterior descending coronary artery from the pulmonary trunk associated with type B interrupted aortic arch.Pediatr Cardiol. 1994; 15: 143-145PubMed Google Scholar], wherein the ALCAPA was diagnosed after surgery or at autopsy. Our patient had a combination of lesions, previously not reported. The presence of preoperative cardiac dysfunction should alert one to the possibility of associated lesions like ALCAPA. In conclusion, patients with VSD and ALCAPA may have few findings, if any, in the history or examination to suggest a coronary anomaly. It is crucial in these patients to preempt the occurrence of extensive myocardial damage to improve their chances of survival. Diagnostic technology does exist. What is required is a high index of suspicion especially when multiple lesions are involved. Although this represents an unusual constellation of findings and presents a challenging surgical repair, the infant had an excellent outcome.