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The Large Window Ductus: A Surgical Trap
1790
¨ NENFELDER ET AL CASE REPORT GRU LARGE WINDOW DUCTUS
References 1. Citak M, Rees A, Mavroudis C. Surgical management of infective endocarditis in children. Ann Thorac Surg 1992;54: 755– 60. 2. Tolan RW, Kleiman MB, Frank M, King H, Brown JW. Operative intervention in active endocarditis in children: report of a series of cases and review. Clin Inf Dis 1992;14: 852– 62. 3. Mavroudis C, Wampler J, Hodsden JE, Rees AH, Solinger RE, Elbl F. Modified aortoseptoplasty for annular abscess and erosion of the membranous septum. Chest 1984;85:442– 4. 4. David TE, Komeda M, Brofman PR. Surgical treatment of aortic root abscess. Circulation 1989;80(Suppl 1):269–74. 5. Chauvaud S, Jebara V, Chachques JC, et al. Valve extension with glutaraldehyde-preserved autologous pericardium. Results in mitral valve repair. J Thorac Cardiovasc Surg 1991; 102:171– 8. 6. Dearani JA, Orszulak TA, Schaff HV, Daly RC, Anderson BJ, Danielson GK. Results of allograft aortic valve replacement for complex endocarditis. J Thorac Cardiovasc Surg 1997;113: 285–91. 7. Kadoba K, Jonas RA, Mayer JE, Castan˜eda AR. Mitral valve replacement in the first year of life. J Thorac Cardiovasc Surg 1990;100:762– 8.
The Large Window Ductus: A Surgical Trap Ju¨rg Gru¨nenfelder, MD, Ulrike Bartram, MD, Richard Van Praagh, MD, Kevin E. Bove, MD, Warren W. Bailey, MD, Richard A. Meyer, MD, Peter R. Koenig, MD, Angel R. Perez, MD, and David C. Schwartz, MD Departments of Pathology and Cardiology, Children’s Hospital, and Departments of Pathology and Pediatrics, Harvard Medical School, Boston, Massachusetts; and Department of Pathology and Divisions of Cardiology and Cardiothoracic Surgery, Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio
A rare window type of patent ductus arteriosus is reported that was large (15 mm in maximal transverse dimension) but had virtually no length and hence was externally invisible. The smaller aortic isthmus (4 mm in diameter), which was intrapericardial, was mistaken for the ductus and was inadvertently clip-occluded, leading to death. After a specific diagnosis is made, the large window ductus should be patched on cardiopulmonary bypass with a transpulmonary approach. (Ann Thorac Surg 1998;65:1790 –1) © 1998 by The Society of Thoracic Surgeons
W
hen a large patent ductus arteriosus (PDA) is wide in diameter and very short in length, upon external inspection it may not be visible. This is a serious surgical problem, as the following case illustrates.
Accepted for publication Jan 13, 1998. Address reprint requests to Dr Richard Van Praagh, Cardiac Registry, Children’s Hospital, 300 Longwood Ave, Boston, MA 02115.
© 1998 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
Ann Thorac Surg 1998;65:1790 –1
An 18-month-old boy with multiple congenital anomalies underwent echocardiography and cardiac catheterization that were interpreted as showing cor triatriatum, persistent left superior vena cava, unroofed coronary sinus, secundum atrial septal defect, a hemodynamically large but structurally ill-defined PDA, and pulmonary hypertension. Cardiac surgical repair at 19 months of age consisted of excision of an obstructive diaphragm within the left atrium, rerouting of the persistent left superior vena cava to the right atrium via a Gore-Tex (W. L. Gore & Assoc, Flagstaff, AZ) tunnel, patch closure of the secundum atrial septal defect and of the enlarged coronary sinus ostium using bovine pericardium, and occlusion by two clips of the intrapericardial structure interpreted to be the PDA. The postoperative course was characterized by hypoxemia, acute renal failure, hepatic and intestinal ischemia, poor peripheral perfusion, and absent pulses in the left leg, leading to death 1 day postoperatively. Autopsy revealed right atrial hypertrophy (2 to 3 mm in thickness/1 mm in normal controls), marked right ventricular hypertrophy and enlargement (6 to 10 mm/2 to 3 mm), and moderate dilatation of the main pulmonary artery and branches (Fig 1A). A large window-type PDA lay between the main pulmonary artery and the descending thoracic aorta, above the ostium of the left pulmonary artery (see Fig 1A). The maximal transverse dimension of this oval window ductus was 15 mm, the perimeter being 30 mm. Because this large window ductus had virtually no length (Fig 1B), it could not be seen on external inspection. The main pulmonary artery and the descending thoracic aorta appeared to be one continuous artery, with no intervening PDA. Internally, no thickened wrinkled ductal intimal pads were seen grossly (see Fig 1B), unlike typical PDA in which the ductal intimal pads are readily apparent on gross inspection. Histologically, however, ductal intimal pads and ductal medial musculature were well seen, confirming that this large aortopulmonary communication was indeed a very large PDA. Although the aortic isthmus normally is extrapericardial, in this patient the isthmus lay within the pericardial sac, was 4 mm in internal diameter, and was occluded by two clips (see Fig 1B). The left atrium was mildly hypertrophied (1 to 2 mm/1 mm) and the left ventricle was moderately hypertrophied (12 mm/6 to 8 mm). Autopsy also revealed a persistent left superior vena cava connecting with the coronary sinus and draining into the right atrium, a small left innominate vein, partial unroofing of the coronary sinus consisting of a 10-mmlong coronary sinus septal defect adjacent to the left atrial appendage, totally anomalous pulmonary venous connection to the coronary sinus, no evidence of cor triatriatum, bilateral severe cerebral dysplasia with asymmetric megalencephaly and polygyria (greater of the left cerebrum than of the right), almost total absence of the left cerebellar hemisphere, and histologic evidence of widespread ischemic changes below the diaphragm (involv0003-4975/98/$19.00 PII S0003-4975(98)00280-X
Ann Thorac Surg 1998;65:1790 –1
Fig 1. (A) External frontal view of the specimen of heart and lungs. Note the two surgical clips (Clips) occluding the aortic arch just proximal to the large patent ductus arteriosus (PDA). (B) Frontal view of opened right ventricle (RV), pulmonary valve (PV), main pulmonary artery (MPA), PDA, and descending thoracic aorta (Desc Ao) to show very large and extremely short window ductus. Note the absence of wrinkled ductal intimal pads. The MPA is confluent via the PDA with the Desc Ao. (Asc Ao 5 ascending aorta; Innom 5 innominate artery; LA 5 left atrium; LCC 5 left common carotid artery; LS 5 left subclavian artery; LV 5 left ventricle; RA 5 right atrium.)
ing the liver, gastrointestinal tract, pancreas, kidneys, lumbar spinal cord, and skeletal muscles), but none above the diaphragm.
Comment The large window ductus is a surgical trap because the presence of a huge, very short PDA is not apparent on external inspection. Instead, the main pulmonary artery appears to be directly continuous with the descending thoracic aorta, resembling one continuous artery with no visible interposed ductus. If the relatively small aortic isthmus is also intrapericardial, as in the present rare case, then the aortic isthmus strongly resembles a PDA.
CASE REPORT
¨ NENFELDER ET AL GRU LARGE WINDOW DUCTUS
1791
This combination of (1) a large but externally invisible window ductus and (2) a relatively small and intrapericardial aortic isthmus together constitutes a potentially lethal surgical trap. Mistaken for the PDA, the aortic isthmus can be erroneously occluded, as in the present case. The surgeon needs to know that it is anatomically and developmentally impossible for the main pulmonary artery to be directly continuous with the descending thoracic aorta, despite external appearances. The main pulmonary artery always communicates with the descending thoracic aorta via the PDA, not directly. When the PDA is very large and unconstricted, as in the normal embryo and fetus, then the PDA is as wide as the main pulmonary artery and the descending thoracic aorta. Hence, upon external inspection, the PDA “disappears,” particularly if it is extremely short, as in the present case. The surgeon should also understand that the PDA is located directly above the ostium of the left pulmonary artery (see Fig 1B). Another potential source of confusion is that when viewed from the front as via a median sternotomy, a very large PDA obscures the origin of the left pulmonary artery (see Fig 1A). It is also helpful to understand that the left recurrent laryngeal branch of the left vagus nerve curls under the aortic arch from left to right, distal to the PDA—not proximal to the PDA and hence not beneath the preductal portion of the aortic arch. Consequently, the recurrent laryngeal nerve may be regarded as a surgical guide to the PDA: no recurrent laryngeal nerve, no ductus arteriosus. Postoperatively, the presence of aortic isthmic interruption can be masked by right-to-left blood flow through the large unoccluded window ductus into the descending thoracic aorta. Consequently, two-dimensional echocardiography with color-flow Doppler may not detect total iatrogenic interruption of the aortic isthmus. However, the lower body is perfused postoperatively by unsaturated venous blood, as was confirmed histologically in the present case. Simulating a very distal aortopulmonary window, the large window ductus appears to be exceedingly rare. Among the 3,400 postmortem cases of congenital heart disease currently retained in the Cardiac Registry of the Children’s Hospital, Boston, there is not a single case of large window ductus. In the literature, we have been able to find only 1 similar case that was reported in 1953 by Lev (his Figure 98b, page 130) [1]. Because a large externally invisible window ductus cannot be ligated and divided, or clip-occluded in the usual way, we propose that appropriate surgical management should consist of patch occlusion on cardiopulmonary bypass with a transpulmonary approach.
Reference 1. Lev M. Autopsy diagnosis of congenitally malformed hearts. Springfield: Charles C Thomas, 1953:130.
¨ NENFELDER ET AL CASE REPORT GRU LARGE WINDOW DUCTUS
References 1. Citak M, Rees A, Mavroudis C. Surgical management of infective endocarditis in children. Ann Thorac Surg 1992;54: 755– 60. 2. Tolan RW, Kleiman MB, Frank M, King H, Brown JW. Operative intervention in active endocarditis in children: report of a series of cases and review. Clin Inf Dis 1992;14: 852– 62. 3. Mavroudis C, Wampler J, Hodsden JE, Rees AH, Solinger RE, Elbl F. Modified aortoseptoplasty for annular abscess and erosion of the membranous septum. Chest 1984;85:442– 4. 4. David TE, Komeda M, Brofman PR. Surgical treatment of aortic root abscess. Circulation 1989;80(Suppl 1):269–74. 5. Chauvaud S, Jebara V, Chachques JC, et al. Valve extension with glutaraldehyde-preserved autologous pericardium. Results in mitral valve repair. J Thorac Cardiovasc Surg 1991; 102:171– 8. 6. Dearani JA, Orszulak TA, Schaff HV, Daly RC, Anderson BJ, Danielson GK. Results of allograft aortic valve replacement for complex endocarditis. J Thorac Cardiovasc Surg 1997;113: 285–91. 7. Kadoba K, Jonas RA, Mayer JE, Castan˜eda AR. Mitral valve replacement in the first year of life. J Thorac Cardiovasc Surg 1990;100:762– 8.
The Large Window Ductus: A Surgical Trap Ju¨rg Gru¨nenfelder, MD, Ulrike Bartram, MD, Richard Van Praagh, MD, Kevin E. Bove, MD, Warren W. Bailey, MD, Richard A. Meyer, MD, Peter R. Koenig, MD, Angel R. Perez, MD, and David C. Schwartz, MD Departments of Pathology and Cardiology, Children’s Hospital, and Departments of Pathology and Pediatrics, Harvard Medical School, Boston, Massachusetts; and Department of Pathology and Divisions of Cardiology and Cardiothoracic Surgery, Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio
A rare window type of patent ductus arteriosus is reported that was large (15 mm in maximal transverse dimension) but had virtually no length and hence was externally invisible. The smaller aortic isthmus (4 mm in diameter), which was intrapericardial, was mistaken for the ductus and was inadvertently clip-occluded, leading to death. After a specific diagnosis is made, the large window ductus should be patched on cardiopulmonary bypass with a transpulmonary approach. (Ann Thorac Surg 1998;65:1790 –1) © 1998 by The Society of Thoracic Surgeons
W
hen a large patent ductus arteriosus (PDA) is wide in diameter and very short in length, upon external inspection it may not be visible. This is a serious surgical problem, as the following case illustrates.
Accepted for publication Jan 13, 1998. Address reprint requests to Dr Richard Van Praagh, Cardiac Registry, Children’s Hospital, 300 Longwood Ave, Boston, MA 02115.
© 1998 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
Ann Thorac Surg 1998;65:1790 –1
An 18-month-old boy with multiple congenital anomalies underwent echocardiography and cardiac catheterization that were interpreted as showing cor triatriatum, persistent left superior vena cava, unroofed coronary sinus, secundum atrial septal defect, a hemodynamically large but structurally ill-defined PDA, and pulmonary hypertension. Cardiac surgical repair at 19 months of age consisted of excision of an obstructive diaphragm within the left atrium, rerouting of the persistent left superior vena cava to the right atrium via a Gore-Tex (W. L. Gore & Assoc, Flagstaff, AZ) tunnel, patch closure of the secundum atrial septal defect and of the enlarged coronary sinus ostium using bovine pericardium, and occlusion by two clips of the intrapericardial structure interpreted to be the PDA. The postoperative course was characterized by hypoxemia, acute renal failure, hepatic and intestinal ischemia, poor peripheral perfusion, and absent pulses in the left leg, leading to death 1 day postoperatively. Autopsy revealed right atrial hypertrophy (2 to 3 mm in thickness/1 mm in normal controls), marked right ventricular hypertrophy and enlargement (6 to 10 mm/2 to 3 mm), and moderate dilatation of the main pulmonary artery and branches (Fig 1A). A large window-type PDA lay between the main pulmonary artery and the descending thoracic aorta, above the ostium of the left pulmonary artery (see Fig 1A). The maximal transverse dimension of this oval window ductus was 15 mm, the perimeter being 30 mm. Because this large window ductus had virtually no length (Fig 1B), it could not be seen on external inspection. The main pulmonary artery and the descending thoracic aorta appeared to be one continuous artery, with no intervening PDA. Internally, no thickened wrinkled ductal intimal pads were seen grossly (see Fig 1B), unlike typical PDA in which the ductal intimal pads are readily apparent on gross inspection. Histologically, however, ductal intimal pads and ductal medial musculature were well seen, confirming that this large aortopulmonary communication was indeed a very large PDA. Although the aortic isthmus normally is extrapericardial, in this patient the isthmus lay within the pericardial sac, was 4 mm in internal diameter, and was occluded by two clips (see Fig 1B). The left atrium was mildly hypertrophied (1 to 2 mm/1 mm) and the left ventricle was moderately hypertrophied (12 mm/6 to 8 mm). Autopsy also revealed a persistent left superior vena cava connecting with the coronary sinus and draining into the right atrium, a small left innominate vein, partial unroofing of the coronary sinus consisting of a 10-mmlong coronary sinus septal defect adjacent to the left atrial appendage, totally anomalous pulmonary venous connection to the coronary sinus, no evidence of cor triatriatum, bilateral severe cerebral dysplasia with asymmetric megalencephaly and polygyria (greater of the left cerebrum than of the right), almost total absence of the left cerebellar hemisphere, and histologic evidence of widespread ischemic changes below the diaphragm (involv0003-4975/98/$19.00 PII S0003-4975(98)00280-X
Ann Thorac Surg 1998;65:1790 –1
Fig 1. (A) External frontal view of the specimen of heart and lungs. Note the two surgical clips (Clips) occluding the aortic arch just proximal to the large patent ductus arteriosus (PDA). (B) Frontal view of opened right ventricle (RV), pulmonary valve (PV), main pulmonary artery (MPA), PDA, and descending thoracic aorta (Desc Ao) to show very large and extremely short window ductus. Note the absence of wrinkled ductal intimal pads. The MPA is confluent via the PDA with the Desc Ao. (Asc Ao 5 ascending aorta; Innom 5 innominate artery; LA 5 left atrium; LCC 5 left common carotid artery; LS 5 left subclavian artery; LV 5 left ventricle; RA 5 right atrium.)
ing the liver, gastrointestinal tract, pancreas, kidneys, lumbar spinal cord, and skeletal muscles), but none above the diaphragm.
Comment The large window ductus is a surgical trap because the presence of a huge, very short PDA is not apparent on external inspection. Instead, the main pulmonary artery appears to be directly continuous with the descending thoracic aorta, resembling one continuous artery with no visible interposed ductus. If the relatively small aortic isthmus is also intrapericardial, as in the present rare case, then the aortic isthmus strongly resembles a PDA.
CASE REPORT
¨ NENFELDER ET AL GRU LARGE WINDOW DUCTUS
1791
This combination of (1) a large but externally invisible window ductus and (2) a relatively small and intrapericardial aortic isthmus together constitutes a potentially lethal surgical trap. Mistaken for the PDA, the aortic isthmus can be erroneously occluded, as in the present case. The surgeon needs to know that it is anatomically and developmentally impossible for the main pulmonary artery to be directly continuous with the descending thoracic aorta, despite external appearances. The main pulmonary artery always communicates with the descending thoracic aorta via the PDA, not directly. When the PDA is very large and unconstricted, as in the normal embryo and fetus, then the PDA is as wide as the main pulmonary artery and the descending thoracic aorta. Hence, upon external inspection, the PDA “disappears,” particularly if it is extremely short, as in the present case. The surgeon should also understand that the PDA is located directly above the ostium of the left pulmonary artery (see Fig 1B). Another potential source of confusion is that when viewed from the front as via a median sternotomy, a very large PDA obscures the origin of the left pulmonary artery (see Fig 1A). It is also helpful to understand that the left recurrent laryngeal branch of the left vagus nerve curls under the aortic arch from left to right, distal to the PDA—not proximal to the PDA and hence not beneath the preductal portion of the aortic arch. Consequently, the recurrent laryngeal nerve may be regarded as a surgical guide to the PDA: no recurrent laryngeal nerve, no ductus arteriosus. Postoperatively, the presence of aortic isthmic interruption can be masked by right-to-left blood flow through the large unoccluded window ductus into the descending thoracic aorta. Consequently, two-dimensional echocardiography with color-flow Doppler may not detect total iatrogenic interruption of the aortic isthmus. However, the lower body is perfused postoperatively by unsaturated venous blood, as was confirmed histologically in the present case. Simulating a very distal aortopulmonary window, the large window ductus appears to be exceedingly rare. Among the 3,400 postmortem cases of congenital heart disease currently retained in the Cardiac Registry of the Children’s Hospital, Boston, there is not a single case of large window ductus. In the literature, we have been able to find only 1 similar case that was reported in 1953 by Lev (his Figure 98b, page 130) [1]. Because a large externally invisible window ductus cannot be ligated and divided, or clip-occluded in the usual way, we propose that appropriate surgical management should consist of patch occlusion on cardiopulmonary bypass with a transpulmonary approach.
Reference 1. Lev M. Autopsy diagnosis of congenitally malformed hearts. Springfield: Charles C Thomas, 1953:130.