Ann Thorac Surg 2014;97:707–10
CASE REPORT ILIC ET AL ANOMALOUS ORIGIN OF LEFT CORONARY ARTERY
707
described for patients at high risk of rupture [3]. Other reported methods to facilitate safe sternal reentry include adjunctive carotid cannulation, endocardiopulmonary bypass, thoracoscopy for improved visualization, and endovascular techniques [1, 4–6]. The present technique allows for safe entry of the mediastinum while avoiding the risks associated with carotid cannulation and prolonged periods of cardiopulmonary bypass and systemic hypothermia. The size of the ascending aortic aneurysm (>55 mm) has been shown to be a predictor of catastrophic hemorrhage during sternotomy [7]. Our 2 patients had massive 9-cm and 13-cm pseudoaneurysms, respectively, and would be considered at high risk for hemorrhage during reentry. We have found that the described technique is safe, efficient, reliable, and reproducible, and uses surgical equipment that is readily available at most centers. It is now our preferred technique for all of our redo operations.
References 1. Mohammadi S, Bonnet N, Leprince P, et al. Reoperation for false aneurysm of the ascending aorta after its prosthetic replacement: surgical strategy. Ann Thorac Surg 2005;79: 147–52. 2. Eddy AC, Miller D, Johnson D, et al. Anterior sternal retraction for reoperative median sternotomy. Am J Surg 1991;5: 556–9. 3. Atik FA, Navia JL, Svensson LG, et al. Surgical treatment of pseudoaneurysm of the thoracic aorta. J Thorac Cardiovasc Surg 2006;132:379–85. Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc
4. Bachet J, Pirotte M, Laborde F, Guilmet D. Reoperation for giant false aneurysm of the thoracic aorta: how to reenter the chest? Ann Thorac Surg 2007;83:1610–4. 5. Gazzaniga AB, Palafox BA. Substernal thoracoscopic guidance during sternal reentry. Ann Thorac Surg 2001;72:289–90. 6. Reyes KG, Pettersson GB, Mihaljevic T, Roselli EE. A strategy for safe sternal reentry in patients with pseudoaneurysms of the ascending aorta using the PORT-ACCESS EndoCPB system. Interact Cardiovasc Thorac Surg 2009;9:893–5. 7. Villavicencio MA, Orszulak TA, Sundt TM, et al. Thoracic aorta false aneurysm: what surgical strategy should be recommended? Ann Thorac Surg 2006;82:81–9.
Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery, Scimitar Syndrome, and Aortic Coarctation Slobodan Ilic, MD, PhD, Djordje Hercog, MD, Milan Vucicevic, MD, Irena Vulicevic, MD, Branko Mimic, MD, Milan Djukic, MD, PhD, Ida Jovanovic, MD, PhD, Vojislav Parezanovic, MD, PhD, and Tamara Ilisic, MD School of Medicine, University of Belgrade, and University Children’s Hospital, Belgrade, Serbia
Accepted for publication May 15, 2013. Address correspondence to Dr Ilic, Milutina Milankovica 62/35, 11070 Belgrade, Serbia; e-mail: [email protected].
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.05.109
FEATURE ARTICLES
Fig 4. Key aspects of the of the dissection technique. (A) Use of the Rultract and Weitlaner retractors to expose the subxyphoid region. (B) The approach allows for a standard sternotomy. (C) The actual dissection technique with electrocautery and inferior retraction with Yankauer suction to free tissue from the posterior sternal table (see text for details).
708
CASE REPORT ILIC ET AL ANOMALOUS ORIGIN OF LEFT CORONARY ARTERY
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) represents one of the most common causes of myocardial ischemia in infants and if left untreated results in a high mortality rate. When ALCAPA coexists with other congenital malformations, particularly those associated with pulmonary hypertension, the initial presentation can be quite confusing and is often misinterpreted. We report an infant with ALCAPA associated with scimitar syndrome and aortic coarctation whose clinical course illustrates the complexities and difficulties of management with a successful outcome. (Ann Thorac Surg 2014;97:707–10) Ó 2014 by The Society of Thoracic Surgeons
A
FEATURE ARTICLES
nomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is usually seen as an isolated lesion, but there are some reports of its association with tetralogy of Fallot, complete atrioventricular septal defect, aortic coarctation, ventricular septal defect, aortopulmonary window, and transposition of the great arteries [1–3]. Associated malformations can affect the hemodynamics and natural history of the ALCAPA [1]. Surgical correction of these associated anomalies can unmask the coronary anomaly by unexpected cardiac decompensation. Failure to anticipate coronary anomaly can result in cardiogenic shock and death [4]. A full-term infant was referred to our institution in the infant’s eighth day of life (body weight 3.1 kg) with tachypnea, dyspnea, and peripheral cyanosis. Radiography of the chest showed a normal heart position with cardiomegaly. An echocardiogram demonstrated mildly hypoplastic left ventricle with normal fractional shortening, pulmonary hypertension, small atrial septal defect, and patent ductus with possible aortic coarctation. Moderate anticongestive therapy commenced, but owing to echocardiographic progression of the aortic coarctation, the baby was given prostaglandin E1 and a surgical procedure was indicated. Through a left thoracotomy, the coarctation was resected and an extended end-to-end anastomosis was performed. The aberrant right subclavian artery was divided. The postoperative course was complicated by severe respiratory distress and minor pulmonary hypertensive crisis. The baby was treated with hyperventilation, high fraction of inspired oxygen, nitric oxide, and sildenafil, but without considerable improvement. Radiographic images of the chest and ultrasound images were highly suggestive of eventration of the right side of the diaphragm, and a thoracotomy was performed to plicate the diaphragm. However, the diaphragm was normal in structure and insertion, but an abnormal arterial vessel for the right lower lobe and an anomalous pulmonary vein were identified along with abnormal appearance of the basal lung segments, suggestive of scimitar syndrome with pulmonary sequestration. Otherwise, the right lung was well developed. An abnormal arterial supply coursing
Ann Thorac Surg 2014;97:707–10
through the inferior pulmonary ligament was divided just above the diaphragm. It was decided not to proceed with a complex operation without further diagnostic evaluation. The cardiac catheterization and computed tomography confirmed scimitar syndrome and pulmonary sequestration but also an ALCAPA. Subsequent echocardiographic studies showed severe left ventricle dysfunction (fractional shortening 0.16). Intracardiac repair was done with the patient supported by extracorporeal circulation with use of bicaval cannulation and deep hypothermia without circulatory arrest. Myocardial protection included cold crystalloid cardioplegia, and the ischemic time was 114 minutes. Intraoperative findings generally correlated with the diagnosis except the high origin of ALCAPA arising from the proximal left pulmonary artery, immediately adjacent to the main pulmonary artery bifurcation (Fig 1). Because of the high origin and rather long left main coronary, there was no need to extend the ALCAPA, and direct reimplantation into the posterolateral aortic wall was performed. The scimitar syndrome was repaired by enlarging the atrial septal defect and baffling the anomalous vein to the left atrium, with use of autologous pericardium along
Fig 1. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) button excised high and to the left of the main pulmonary artery (MPA) bifurcation. (Ao ¼ aorta.)
Ann Thorac Surg 2014;97:707–10
CASE REPORT ILIC ET AL ANOMALOUS ORIGIN OF LEFT CORONARY ARTERY
709
with augmentation of both the right atrial wall and the inferior vena cava by a bovine pericardial patch. The operation was uneventful, and the child was successfully weaned from mechanical ventilation on the 15th postoperative day and discharged 1 month after the corrective operation, 4 months after referral. One year after the operation, heart catheterization confirmed a patent left coronary artery (Fig 2A). Five years after repair, the child was doing well without medication, with normal fractional shortening and mitral valve function. Echocardiography 5 years after repair confirmed a patent left coronary artery (Fig 2B), without signs of baffle obstruction (Fig 3A). Magnetic resonance imaging also showed a patent right pulmonary vein (Fig 3B).
Comment Symptoms in children with ALCAPA develop days to weeks after birth when the ductus arteriosus closes and pulmonary vascular resistance falls. This results in the left ventricular myocardium being perfused by desaturated blood under low pressure, leading to myocardial ischemia. Further increase in myocardial oxygen consumption and inadequate collateral flow results in left ventricular infarction and causes ischemic damage to the papillary muscles and mitral regurgitation. Surgically establishment of a two-coronary system by transferring the anomalous coronary artery into the aorta is the gold standard in therapy for ALCAPA.
Scimitar syndrome is a rare congenital anomaly most commonly consisting of partial anomalous pulmonary venous connection of the right lung to the inferior vena cava, right lung hypoplasia, dextroposition of the heart, anomalous arterial supply from the aorta to the right lung (with or without sequestration), and anomalies of the diaphragm (hernia, eventration, duplication). There is variable presentation of this syndrome, ranging from severe congestive heart failure in infancy to mild symptoms in childhood. Multiple factors are responsible for the severe symptoms and pulmonary hypertension in infants with scimitar syndrome. These include left-to-right shunt from the anomalous pulmonary venous connection, pulmonary hypoplasia, stenosis of the anomalous pulmonary vein, systemic arterial blood supply to the right lower lung, and associated cardiovascular defects [5]. Simple ligation or coil embolization of the abnormal arterial vessels has been advocated as the best and simplest type of treatment, especially in symptomatic infants. For patients with congestive heart failure, repeated pneumonia, and pulmonary hypertension, it is important to reroute the anomalous right pulmonary veins and repair the associated cardiac defects. Surgical repair includes intraatrial baffle repair or reimplantation of the scimitar vein into the left atrium. According to a multicentric study by the European Congenital Heart Surgeons Association [6], surgical treatment of this rare syndrome is associated with a relatively high incidence of residual scimitar drainage stenosis. Despite aggressive management, the association of scimitar syndrome and
Fig 3. (A) Echocardiography 5 years after repair showing a wide and patent baffle of the anomalous scimitar vein. (B) Magnetic resonance image 5 years after correction showing a patent right pulmonary vein (RPV). (LA ¼ left atrium; LPV ¼ left pulmonary vein; RA ¼ right atrium.)
FEATURE ARTICLES
Fig 2. (A) Aortography 1 year after repair, showing a patent left coronary artery (LCA) reimplanted above the sinus of Valsalva. (B) Echocardiography 5 years after repair, showing a patent LCA.
710
CASE REPORT NAKAMURA ET AL VALSALVA SINUS ANEURYSM WITH TRUNCUS
FEATURE ARTICLES
complex cardiovascular anomalies still carries a poor prognosis for survival [5, 6]. There are only two reports describing ALCAPA associated with scimitar syndrome [1, 5]. To our knowledge, the world literature has never yet described the combination of ALCALPA with both scimitar syndrome and coarctation of the aorta. Gao and colleagues [5] reported 7 of 13 infants with scimitar syndrome and left-sided lesions, including hypoplastic left ventricle, arch obstruction, subaortic stenosis, pulmonary vein stenosis, and anomalous left coronary artery, all of which were associated with poor outcome. An infant in their series with scimitar syndrome and ALCAPA died during ligation of both the patent ductus arteriosus and ALCAPA [5]. ALCAPA was associated with fatal outcome in 1 patient reported previously [1]. In our patient, long-term mechanical ventilation and subsequent chronic lung disease and the scimitar-related pulmonary hypertension most likely increased pulmonary vascular resistance during the first weeks of life. This probably explains why the child had no mitral insufficiency or any evidence of myocardial ischemia preoperatively. After coarctation repair, particularly after the second operation in which the abnormal arterial supply to the affected right lung was divided, subsequent amelioration of pulmonary hypertension revealed the coronary anomaly. In unusual circumstances, the anomalous coronary may arise from a branch pulmonary artery [7], making echocardiographic diagnosis difficult, even with Doppler, as shown in our case. We describe our diagnostic pitfalls of this rare combination. Although this represents an unusual constellation of findings and presented a technically challenging surgical repair, the infant had an excellent outcome.
References 1. Boning U, Sauer U, Mocellin H, Schumacher G, Buhlmeyer K. Anomalous coronary drainage from the pulmonary artery with associated heart and vascular abnormalities: report on 3 patients and review of the literature. Herz 1983;8:93–104. 2. McMahon CJ, DiBardino DJ, Undar A, Fraser CD. Anomalous origin of left coronary artery from the right pulmonary artery in association with type III aortopulmonary window and interrupted aortic arch. Ann Thorac Surg 2002;74: 919–21. 3. Murala JS, Cooper S, Duffy B, Matbah N, Argent E, Nunn G. Anomalous left coronary artery arising from the left pulmonary artery, aortic coarctation, and a large ventricular septal defect. J Thorac Cardiovasc Surg 2006;131: 911–2. 4. Sreeram N, Hunter S, Wren C. Acute myocardial infarction in infancy: unmasking of anomalous origin of the left coronary artery by ligation of an arterial duct. Br Heart J 1989;61:307–8. 5. Gao YA, Burrows PE, Benson LN, Rabinovitch M, Freedom RM. Scimitar syndrome in infancy. J Am Coll Cardiol 1993;22:873–82. 6. Vida VL, Padalino MA, Boccuzzo G, et al. Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study. Circulation 2010;122:1159–66. Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc
Ann Thorac Surg 2014;97:710–2
7. Radha AS, Dharan BS, Kumar RK, Rao SG. Anomalous origin of left coronary artery from right pulmonary artery in an infant with coarctation of the aorta. Ann Thorac Surg 2004;78:324–6.
Case of Congenital Aneurysm of Sinus of Valsalva With Common Arterial Trunk Yuki Nakamura, MD, Mitsuru Aoki, MD, Ikuo Hagino, MD, Hiroshi Koshiyama, MD, Tadashi Fujiwara, MD, and Hiromichi Nakajima, MD Departments of Cardiovascular Surgery, and Pediatric Cardiology, Chiba Children’s Hospital, Chiba, Japan
We describe an unusual case of a patient with common arterial trunk and interrupted aortic arch, complicated by congenital aneurysm of the sinus of Valsalva, who underwent successful repair. To our knowledge, no such case has been reported in the English literature. The combination of common arterial trunk and congenital aneurysm of the sinus of Valsalva may yield insights into the morphogenesis of congenital aneurysm of the sinus of Valsalva. (Ann Thorac Surg 2014;97:710–2) Ó 2014 by The Society of Thoracic Surgeons
A
n aneurysm of the sinus of Valsalva (ASV) is a thinwalled enlargement of the aortic wall that arises from the base of a single aortic sinus. It is rare in infancy and childhood, and there are only a few reports in the English literature which describe neonatal patients with ASV. A 1-month-old male infant diagnosed with common arterial trunk (CAT) and interrupted aortic arch (IAA) was referred to us for surgical indication. He was born at 38 weeks of gestation with a birth weight of 2,210 g. On admission, he presented signs of congestive cardiac failure. Further examination revealed low-set ears, micrognathia, simian crease, and overlapping fingers, with a deletion within chromosome 5q23 to q31. Transthoracic echocardiography revealed a diagnosis of CAT, IAA type A, a patent arterial duct, and a juxtaarterial ventricular septal defect (VSD). The truncal valve was quadricuspid with little regurgitation. An image captured from a parasternal window demonstrated an irregular hypoechoic lesion 5 mm in diameter that was adjacent to a truncal sinus of Valsalva, suggesting an aneurysmal cavity in the right ventricle (RV) outflow tract (Fig 1). However, the exact spatial relationship between the lesion and the truncal root could not be delineated because of poor echocardiographic window. Color flow Doppler
Accepted for publication June 3, 2013. Address correspondence to Dr Nakamura, Department of Cardiovascular Surgery, Chiba Children’s Hospital, 579-1, Heta, Midori, Chiba 266-0007, Japan; e-mail: [email protected].
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.06.107
CASE REPORT ILIC ET AL ANOMALOUS ORIGIN OF LEFT CORONARY ARTERY
707
described for patients at high risk of rupture [3]. Other reported methods to facilitate safe sternal reentry include adjunctive carotid cannulation, endocardiopulmonary bypass, thoracoscopy for improved visualization, and endovascular techniques [1, 4–6]. The present technique allows for safe entry of the mediastinum while avoiding the risks associated with carotid cannulation and prolonged periods of cardiopulmonary bypass and systemic hypothermia. The size of the ascending aortic aneurysm (>55 mm) has been shown to be a predictor of catastrophic hemorrhage during sternotomy [7]. Our 2 patients had massive 9-cm and 13-cm pseudoaneurysms, respectively, and would be considered at high risk for hemorrhage during reentry. We have found that the described technique is safe, efficient, reliable, and reproducible, and uses surgical equipment that is readily available at most centers. It is now our preferred technique for all of our redo operations.
References 1. Mohammadi S, Bonnet N, Leprince P, et al. Reoperation for false aneurysm of the ascending aorta after its prosthetic replacement: surgical strategy. Ann Thorac Surg 2005;79: 147–52. 2. Eddy AC, Miller D, Johnson D, et al. Anterior sternal retraction for reoperative median sternotomy. Am J Surg 1991;5: 556–9. 3. Atik FA, Navia JL, Svensson LG, et al. Surgical treatment of pseudoaneurysm of the thoracic aorta. J Thorac Cardiovasc Surg 2006;132:379–85. Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc
4. Bachet J, Pirotte M, Laborde F, Guilmet D. Reoperation for giant false aneurysm of the thoracic aorta: how to reenter the chest? Ann Thorac Surg 2007;83:1610–4. 5. Gazzaniga AB, Palafox BA. Substernal thoracoscopic guidance during sternal reentry. Ann Thorac Surg 2001;72:289–90. 6. Reyes KG, Pettersson GB, Mihaljevic T, Roselli EE. A strategy for safe sternal reentry in patients with pseudoaneurysms of the ascending aorta using the PORT-ACCESS EndoCPB system. Interact Cardiovasc Thorac Surg 2009;9:893–5. 7. Villavicencio MA, Orszulak TA, Sundt TM, et al. Thoracic aorta false aneurysm: what surgical strategy should be recommended? Ann Thorac Surg 2006;82:81–9.
Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery, Scimitar Syndrome, and Aortic Coarctation Slobodan Ilic, MD, PhD, Djordje Hercog, MD, Milan Vucicevic, MD, Irena Vulicevic, MD, Branko Mimic, MD, Milan Djukic, MD, PhD, Ida Jovanovic, MD, PhD, Vojislav Parezanovic, MD, PhD, and Tamara Ilisic, MD School of Medicine, University of Belgrade, and University Children’s Hospital, Belgrade, Serbia
Accepted for publication May 15, 2013. Address correspondence to Dr Ilic, Milutina Milankovica 62/35, 11070 Belgrade, Serbia; e-mail: [email protected].
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.05.109
FEATURE ARTICLES
Fig 4. Key aspects of the of the dissection technique. (A) Use of the Rultract and Weitlaner retractors to expose the subxyphoid region. (B) The approach allows for a standard sternotomy. (C) The actual dissection technique with electrocautery and inferior retraction with Yankauer suction to free tissue from the posterior sternal table (see text for details).
708
CASE REPORT ILIC ET AL ANOMALOUS ORIGIN OF LEFT CORONARY ARTERY
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) represents one of the most common causes of myocardial ischemia in infants and if left untreated results in a high mortality rate. When ALCAPA coexists with other congenital malformations, particularly those associated with pulmonary hypertension, the initial presentation can be quite confusing and is often misinterpreted. We report an infant with ALCAPA associated with scimitar syndrome and aortic coarctation whose clinical course illustrates the complexities and difficulties of management with a successful outcome. (Ann Thorac Surg 2014;97:707–10) Ó 2014 by The Society of Thoracic Surgeons
A
FEATURE ARTICLES
nomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is usually seen as an isolated lesion, but there are some reports of its association with tetralogy of Fallot, complete atrioventricular septal defect, aortic coarctation, ventricular septal defect, aortopulmonary window, and transposition of the great arteries [1–3]. Associated malformations can affect the hemodynamics and natural history of the ALCAPA [1]. Surgical correction of these associated anomalies can unmask the coronary anomaly by unexpected cardiac decompensation. Failure to anticipate coronary anomaly can result in cardiogenic shock and death [4]. A full-term infant was referred to our institution in the infant’s eighth day of life (body weight 3.1 kg) with tachypnea, dyspnea, and peripheral cyanosis. Radiography of the chest showed a normal heart position with cardiomegaly. An echocardiogram demonstrated mildly hypoplastic left ventricle with normal fractional shortening, pulmonary hypertension, small atrial septal defect, and patent ductus with possible aortic coarctation. Moderate anticongestive therapy commenced, but owing to echocardiographic progression of the aortic coarctation, the baby was given prostaglandin E1 and a surgical procedure was indicated. Through a left thoracotomy, the coarctation was resected and an extended end-to-end anastomosis was performed. The aberrant right subclavian artery was divided. The postoperative course was complicated by severe respiratory distress and minor pulmonary hypertensive crisis. The baby was treated with hyperventilation, high fraction of inspired oxygen, nitric oxide, and sildenafil, but without considerable improvement. Radiographic images of the chest and ultrasound images were highly suggestive of eventration of the right side of the diaphragm, and a thoracotomy was performed to plicate the diaphragm. However, the diaphragm was normal in structure and insertion, but an abnormal arterial vessel for the right lower lobe and an anomalous pulmonary vein were identified along with abnormal appearance of the basal lung segments, suggestive of scimitar syndrome with pulmonary sequestration. Otherwise, the right lung was well developed. An abnormal arterial supply coursing
Ann Thorac Surg 2014;97:707–10
through the inferior pulmonary ligament was divided just above the diaphragm. It was decided not to proceed with a complex operation without further diagnostic evaluation. The cardiac catheterization and computed tomography confirmed scimitar syndrome and pulmonary sequestration but also an ALCAPA. Subsequent echocardiographic studies showed severe left ventricle dysfunction (fractional shortening 0.16). Intracardiac repair was done with the patient supported by extracorporeal circulation with use of bicaval cannulation and deep hypothermia without circulatory arrest. Myocardial protection included cold crystalloid cardioplegia, and the ischemic time was 114 minutes. Intraoperative findings generally correlated with the diagnosis except the high origin of ALCAPA arising from the proximal left pulmonary artery, immediately adjacent to the main pulmonary artery bifurcation (Fig 1). Because of the high origin and rather long left main coronary, there was no need to extend the ALCAPA, and direct reimplantation into the posterolateral aortic wall was performed. The scimitar syndrome was repaired by enlarging the atrial septal defect and baffling the anomalous vein to the left atrium, with use of autologous pericardium along
Fig 1. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) button excised high and to the left of the main pulmonary artery (MPA) bifurcation. (Ao ¼ aorta.)
Ann Thorac Surg 2014;97:707–10
CASE REPORT ILIC ET AL ANOMALOUS ORIGIN OF LEFT CORONARY ARTERY
709
with augmentation of both the right atrial wall and the inferior vena cava by a bovine pericardial patch. The operation was uneventful, and the child was successfully weaned from mechanical ventilation on the 15th postoperative day and discharged 1 month after the corrective operation, 4 months after referral. One year after the operation, heart catheterization confirmed a patent left coronary artery (Fig 2A). Five years after repair, the child was doing well without medication, with normal fractional shortening and mitral valve function. Echocardiography 5 years after repair confirmed a patent left coronary artery (Fig 2B), without signs of baffle obstruction (Fig 3A). Magnetic resonance imaging also showed a patent right pulmonary vein (Fig 3B).
Comment Symptoms in children with ALCAPA develop days to weeks after birth when the ductus arteriosus closes and pulmonary vascular resistance falls. This results in the left ventricular myocardium being perfused by desaturated blood under low pressure, leading to myocardial ischemia. Further increase in myocardial oxygen consumption and inadequate collateral flow results in left ventricular infarction and causes ischemic damage to the papillary muscles and mitral regurgitation. Surgically establishment of a two-coronary system by transferring the anomalous coronary artery into the aorta is the gold standard in therapy for ALCAPA.
Scimitar syndrome is a rare congenital anomaly most commonly consisting of partial anomalous pulmonary venous connection of the right lung to the inferior vena cava, right lung hypoplasia, dextroposition of the heart, anomalous arterial supply from the aorta to the right lung (with or without sequestration), and anomalies of the diaphragm (hernia, eventration, duplication). There is variable presentation of this syndrome, ranging from severe congestive heart failure in infancy to mild symptoms in childhood. Multiple factors are responsible for the severe symptoms and pulmonary hypertension in infants with scimitar syndrome. These include left-to-right shunt from the anomalous pulmonary venous connection, pulmonary hypoplasia, stenosis of the anomalous pulmonary vein, systemic arterial blood supply to the right lower lung, and associated cardiovascular defects [5]. Simple ligation or coil embolization of the abnormal arterial vessels has been advocated as the best and simplest type of treatment, especially in symptomatic infants. For patients with congestive heart failure, repeated pneumonia, and pulmonary hypertension, it is important to reroute the anomalous right pulmonary veins and repair the associated cardiac defects. Surgical repair includes intraatrial baffle repair or reimplantation of the scimitar vein into the left atrium. According to a multicentric study by the European Congenital Heart Surgeons Association [6], surgical treatment of this rare syndrome is associated with a relatively high incidence of residual scimitar drainage stenosis. Despite aggressive management, the association of scimitar syndrome and
Fig 3. (A) Echocardiography 5 years after repair showing a wide and patent baffle of the anomalous scimitar vein. (B) Magnetic resonance image 5 years after correction showing a patent right pulmonary vein (RPV). (LA ¼ left atrium; LPV ¼ left pulmonary vein; RA ¼ right atrium.)
FEATURE ARTICLES
Fig 2. (A) Aortography 1 year after repair, showing a patent left coronary artery (LCA) reimplanted above the sinus of Valsalva. (B) Echocardiography 5 years after repair, showing a patent LCA.
710
CASE REPORT NAKAMURA ET AL VALSALVA SINUS ANEURYSM WITH TRUNCUS
FEATURE ARTICLES
complex cardiovascular anomalies still carries a poor prognosis for survival [5, 6]. There are only two reports describing ALCAPA associated with scimitar syndrome [1, 5]. To our knowledge, the world literature has never yet described the combination of ALCALPA with both scimitar syndrome and coarctation of the aorta. Gao and colleagues [5] reported 7 of 13 infants with scimitar syndrome and left-sided lesions, including hypoplastic left ventricle, arch obstruction, subaortic stenosis, pulmonary vein stenosis, and anomalous left coronary artery, all of which were associated with poor outcome. An infant in their series with scimitar syndrome and ALCAPA died during ligation of both the patent ductus arteriosus and ALCAPA [5]. ALCAPA was associated with fatal outcome in 1 patient reported previously [1]. In our patient, long-term mechanical ventilation and subsequent chronic lung disease and the scimitar-related pulmonary hypertension most likely increased pulmonary vascular resistance during the first weeks of life. This probably explains why the child had no mitral insufficiency or any evidence of myocardial ischemia preoperatively. After coarctation repair, particularly after the second operation in which the abnormal arterial supply to the affected right lung was divided, subsequent amelioration of pulmonary hypertension revealed the coronary anomaly. In unusual circumstances, the anomalous coronary may arise from a branch pulmonary artery [7], making echocardiographic diagnosis difficult, even with Doppler, as shown in our case. We describe our diagnostic pitfalls of this rare combination. Although this represents an unusual constellation of findings and presented a technically challenging surgical repair, the infant had an excellent outcome.
References 1. Boning U, Sauer U, Mocellin H, Schumacher G, Buhlmeyer K. Anomalous coronary drainage from the pulmonary artery with associated heart and vascular abnormalities: report on 3 patients and review of the literature. Herz 1983;8:93–104. 2. McMahon CJ, DiBardino DJ, Undar A, Fraser CD. Anomalous origin of left coronary artery from the right pulmonary artery in association with type III aortopulmonary window and interrupted aortic arch. Ann Thorac Surg 2002;74: 919–21. 3. Murala JS, Cooper S, Duffy B, Matbah N, Argent E, Nunn G. Anomalous left coronary artery arising from the left pulmonary artery, aortic coarctation, and a large ventricular septal defect. J Thorac Cardiovasc Surg 2006;131: 911–2. 4. Sreeram N, Hunter S, Wren C. Acute myocardial infarction in infancy: unmasking of anomalous origin of the left coronary artery by ligation of an arterial duct. Br Heart J 1989;61:307–8. 5. Gao YA, Burrows PE, Benson LN, Rabinovitch M, Freedom RM. Scimitar syndrome in infancy. J Am Coll Cardiol 1993;22:873–82. 6. Vida VL, Padalino MA, Boccuzzo G, et al. Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study. Circulation 2010;122:1159–66. Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc
Ann Thorac Surg 2014;97:710–2
7. Radha AS, Dharan BS, Kumar RK, Rao SG. Anomalous origin of left coronary artery from right pulmonary artery in an infant with coarctation of the aorta. Ann Thorac Surg 2004;78:324–6.
Case of Congenital Aneurysm of Sinus of Valsalva With Common Arterial Trunk Yuki Nakamura, MD, Mitsuru Aoki, MD, Ikuo Hagino, MD, Hiroshi Koshiyama, MD, Tadashi Fujiwara, MD, and Hiromichi Nakajima, MD Departments of Cardiovascular Surgery, and Pediatric Cardiology, Chiba Children’s Hospital, Chiba, Japan
We describe an unusual case of a patient with common arterial trunk and interrupted aortic arch, complicated by congenital aneurysm of the sinus of Valsalva, who underwent successful repair. To our knowledge, no such case has been reported in the English literature. The combination of common arterial trunk and congenital aneurysm of the sinus of Valsalva may yield insights into the morphogenesis of congenital aneurysm of the sinus of Valsalva. (Ann Thorac Surg 2014;97:710–2) Ó 2014 by The Society of Thoracic Surgeons
A
n aneurysm of the sinus of Valsalva (ASV) is a thinwalled enlargement of the aortic wall that arises from the base of a single aortic sinus. It is rare in infancy and childhood, and there are only a few reports in the English literature which describe neonatal patients with ASV. A 1-month-old male infant diagnosed with common arterial trunk (CAT) and interrupted aortic arch (IAA) was referred to us for surgical indication. He was born at 38 weeks of gestation with a birth weight of 2,210 g. On admission, he presented signs of congestive cardiac failure. Further examination revealed low-set ears, micrognathia, simian crease, and overlapping fingers, with a deletion within chromosome 5q23 to q31. Transthoracic echocardiography revealed a diagnosis of CAT, IAA type A, a patent arterial duct, and a juxtaarterial ventricular septal defect (VSD). The truncal valve was quadricuspid with little regurgitation. An image captured from a parasternal window demonstrated an irregular hypoechoic lesion 5 mm in diameter that was adjacent to a truncal sinus of Valsalva, suggesting an aneurysmal cavity in the right ventricle (RV) outflow tract (Fig 1). However, the exact spatial relationship between the lesion and the truncal root could not be delineated because of poor echocardiographic window. Color flow Doppler
Accepted for publication June 3, 2013. Address correspondence to Dr Nakamura, Department of Cardiovascular Surgery, Chiba Children’s Hospital, 579-1, Heta, Midori, Chiba 266-0007, Japan; e-mail: [email protected].
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.06.107
