- Email: [email protected]
Ebstein’s Anomaly of the Tricuspid Valve in Combination of Tetralogy of Fallot: Total Correction in Infancy
304
CASE REPORT LAI ET AL EBSTEIN’S ANOMALY COEXISTS WITH TOF
FEATURE ARTICLES
octreotide and technetium (99mTc). Additional computed tomography or magnetic resonance imaging assists in showing precise anatomic location and relationships. Typical features include a soft fleshy texture, high vascularity, adhesion to surrounding structures, and local invasion making resection challenging [3]. Cardiac pheochromocytomas lack clear planes of dissection and therefore excision of surrounding myocardium and vasculature with reconstruction through pericardial patches and grafts may be required for complete resection. Completeness of resection relates to increased survival at follow-up [4]. These tumors are highly vascular and prone to fatal hemorrhagic complications [3]. Preoperative embolization has a proven role in minimizing bleeding and facilitating dissection in vascularized tumors. Though well described in neck and abdominal pheochromocytomas, there are no previous reports of this procedure used for intrapericardial pheochromocytomas. The only two previously reported cases of preoperative embolization were nonsecretory mediastinal paragangliomas [5, 6]. In both cases embolization was only carried out after unexpected severe hemorrhage forcing the operation to be abandoned at first attempt. For pheochromocytomas, surgery continues to be first line treatment [7] and is based on surgical principles of adequate exposure, meticulous hemostasis, minimal tumor manipulation, and complete resection with intact capsule. The surgical approach to the tumor and use of cardiopulmonary bypass varies, depending on anatomical location, surgeon preference, and experience. The most popular approach is the median sternotomy with cardiopulmonary bypass, which allows adequate vascular control and manipulation [2, 4 –7]. Thoracotomy with or without cardiopulmonary bypass has also been used. Preoperative embolization has a valuable role in the management of cardiac pheochromocytoma. Our experience shows that it reduces hemorrhage, results in greater intraoperative hemodynamic stability, and therefore facilitates handling of the tumor and resection off the surrounding structures. We believe that this multimodality approach helps create a safer operation with reduced risk of complications for both the surgeon and the anesthesiologist.
References 1. Lenders JWM, Eisenhofer G, Mannelli M, Pacak K. Phaeochromocytoma. Lancet 2005;366:665–75. 2. Besterman E, Bromley LL, Peart WS. An intrapericardial pheochromocytoma. Br Heart J 1974;36:318 –20. 3. Orringer MB, Sisson JC, Glazer G, et al. Surgical treatment of cardiac pheochromocytomas. J Thorac Cardiovasc Surg 1985;89:753–7. 4. Lamy AL, Fradet GJ, Luoma A, Nelems B. Anterior and middle mediastinum paraganglioma: complete resection is the treatment of choice. Ann Thorac Surg 1994;57:249 –52. 5. Drucker EA, McLoud TC, Dedrick CG, Hilgenberg AD, Geller SC, Shepard JA. Mediastinal paraganglioma: radio© 2007 by The Society of Thoracic Surgeons Published by Elsevier Inc
Ann Thorac Surg 2007;83:304 – 6
logic evaluation of an unusual vascular tumor. AJR 1987;148:521–2. 6. Rakovich G, Ferraro P, Therasse E, Duranceau A. Preoperative embolization in the management of a mediastinal paraganglioma. Ann Thorac Surg 2001;72:601–3. 7. Gopalakrishnan R, Ticzon AR, Cruz PA, Kennedy FB, Duffy FC, Barmada B, Giacobine JW. Cardiac paraganglioma (chemodectoma): a case report and review of the literature. J Thorac Cardiovasc Surg 1978;76:183–9.
Ebstein’s Anomaly of the Tricuspid Valve in Combination of Tetralogy of Fallot: Total Correction in Infancy Chao-Han Lai, MD, Jing-Ming Wu, MD, and Yu-Jen Yang, MD, PhD Division of Cardiovascular Surgery, Department of Surgery, Department of Pediatrics, National Cheng Kung University Hospital, and Cardiovascular Research Center, Medical College, National Chen Kung University, Tainan, Taiwan
Ebstein’s anomaly of the tricuspid valve in combination with Tetralogy of Fallot is extremely rare. We report a patient with these combined entities in a baby presented with cyanosis and right heart failure soon after birth. Total correction toward biventricular repair was successfully conducted when he was 7 months old. For management of a patient with such rare complex anomalies, we recommend Carpentier’s technique for Ebstein’s anomaly to restore the morphology of the right ventricle, and “one and half ventricle repair” can be a salvage procedure if the right ventricle is not functionally tolerant. (Ann Thorac Surg 2007;83:304 – 6) © 2007 by The Society of Thoracic Surgeons
E
bstein’s anomaly of the tricuspid valve, featured as downward displacement of the septal and posterior leaflets, resulting in an atrialized portion of the right ventricle, is an uncommon congenital malformation [1, 2]. The combination of tetralogy of Fallot (TOF) and Ebstein’s anomaly is extremely rare [3]. In this report, an infant with Ebstein’s anomaly and TOF, manifesting as cyanosis and right heart failure soon after birth, received successful correction of TOF and valvuloplasty of the tricuspid valve at the age of 7 months. A male newborn was referred to our hospital because of cyanosis, heart murmur, and dyspnea noted soon after birth. On physical examination, he presented tachypnea with subcostal retraction, marked hepatomegaly, and general cyanosis. A grade III/VI systolic ejection murmur was audible over the left upper sternal border. Chest roentgenogram showed decreased bilateral lung marking and marked cardiomegaly with 95% cardiothoracic ratio, Accepted for publication April 24, 2006. Address correspondence to Dr Yang, Department of Surgery, National Cheng Kung University Hospital, National Cheng Kung University, 138, Sheng-Li Rd, Tainan 704, Taiwan; e-mail: [email protected].
0003-4975/07/$32.00 doi:10.1016/j.athoracsur.2006.04.078
Fig 1. Preoperative echocardiography showed features of tetralogy of Fallot, including (A) overriding aorta and ventricular septal defect and (B) severe pulmonary stenosis with pressure gradient of 70 mm Hg (arrow).
especially with the right atrial enlargement. Electrocardiogram showed right atrial enlargement, right ventricular hypertrophy, and nonsustained atrial flutter. Echocardiographic studies revealed the typical features of TOF (Fig 1), Ebstein’s anomaly, and an interatrial shunt. The posterior and septal leaflets were displaced 0.7 cm downward from the tricuspid annulus, resulting in severe tricuspid regurgitation (TR) (Fig 2). Although the true right ventricle was mildly underdeveloped, the contractility was not compromised. Medication was prescribed for right heart failure, yet the symptoms of respiratory distress and heart failure became more severe during the follow-up period. Therefore surgical intervention was advised at the age of 7 months, while body weight was 6 kg. The cardiopulmonary bypass was set up with single aortic and bi-caval cannulation. After the aorta was cross clamped and the
CASE REPORT LAI ET AL EBSTEIN’S ANOMALY COEXISTS WITH TOF
305
blood cardioplegic solution was infused, the right atrium was opened parallel to the atrioventricular groove. The anterior leaflet was large and freely movable; however the posterior and septal leaflets were displaced downward about 1 cm from the annulus level. The septal leaflet was hypoplastic and adhered to the septum. Pathology of the atrialized portion of the right ventricle and tricuspid valve leaflets was comparable with that of type B according to Carpentier’s classification [1]. The ventricular septal defect came into sight when the anterior and posterior leaflets of the tricuspid valve were detached from the annulus. However, the outlet extension of the perimembranous ventricular septal defect made its upper margin difficult to repair by this approach. Thus we made the longitudinal incision on the right ventricle outflow tract and performed infundibulectomy. The ventricular septal defect was patch repaired with multiple interrupted stitches. The pulmonary valve was bicuspid with commissural fusion, and the pulmonary annulus was hypoplastic. Therefore, after pulmonary valvotomy, the right ventricle outflow tract was enlarged with a transannular patch. The tricuspid valve was reconstructed using Carpentier’s technique: the atrialized ventricle was plicated vertically with four mattress stitches reinforced with pledgets. The detached leaflets were reattached clockwise to the new annulus to cover the tricuspid orifice beyond the plication site and ending below the coronary sinus, but no prosthetic ring was used [1, 2]. After rewarming, the patient was smoothly weaned from cardiopulmonary bypass with a small amount of inotropic agents. Postoperative echocardiography demonstrated only mild pulmonary stenosis (PS), and mild to moderate TR (Fig 3). The cardiothoracic ratio on chest roentgenogram was reduced to 78%. The postoperative course was uneventful, and he was discharged on postoperative day 10. One month after surgery, mild pulmonary stenosis with a 22 mm Hg pressure gradient between the right ventricle and main pulmonary artery was shown in follow-up echocardiography. Also a mild to moderate degree of TR
Fig 2. Preoperative echocardiography also showed the concomitant Ebstein’s anomaly featured as a downward displacement of the tricuspid valve (arrow) and severe tricuspid regurgitation with a pressure gradient of 70 mm Hg.
FEATURE ARTICLES
Ann Thorac Surg 2007;83:304 – 6
306
CASE REPORT GEORGE ET AL BRIDGE TO TRANSPLANTATION WITH LVAD AFTER MUSTARD PROCEDURE
Fig 3. Postoperative echocardiography showed a mild to moderate tricuspid regurgitation with a pressure gradient of 27 mm Hg.
FEATURE ARTICLES
(the regurgitation jet extending over one third but less than one half of the atrium) with a pressure gradient of 28 mm Hg between the right ventricle and right atrium was shown. The patient was asymptomatic with a moderate amount of diuretics.
Comment Ebstein’s anomaly comprises only about 1% of all congenital heart lesions [1]. It is frequently associated with various cardiac anomalies; however, coexistence with TOF is extremely rare. There has been very scarce surgical experience for patients with such a combination. Chan and colleagues [3] reported the surgical correction in a 7-month-old girl with TOF in the presence of Ebstein’s anomaly, but the tricuspid valve was not touched because the TR was only mild. In our patient, right heart failure due to severe TR necessitated total correction for both lesions in infancy. Successful surgical management for both lesions has not been previously described in the English literature. Clinical presentation of Ebstein’s anomaly varies; it depends mainly on the displacement of the tricuspid valve. The most severe form usually has symptoms presenting in the neonatal stage or early infancy, whereas patients with mild displacement may remain asymptomatic until late adult life. The symptoms of our patient should be moderately severe because the pathologic features were compatible with that of type B according to Carpentier’s classification. However, systemic pressure of the right ventricle resulting from coexistence of TOF aggravated the severity of TR and heart failure. Thus, intractable right heart failure ensued even after maximal medication and made early surgical intervention necessary. How to manage the patient surgically merits some discussion. The right complex heart brought about both heart failure and cyanosis. A palliative aortopulmonary shunt was not advisable because it would not prevent heart failure. Starnes procedure has been reserved only © 2007 by The Society of Thoracic Surgeons Published by Elsevier Inc
Ann Thorac Surg 2007;83:306 – 8
for those with the most severe form, and inevitably the patients had to accept the single ventricle physiology [4]. Even though the right ventricle was slightly hypoplastic in the preoperative evaluation, we preferred the biventricular repair because the right ventricular function seemed adequate. We adopted Carpentier’s technique because the vertical plication may restore the morphology and function of right ventricle better, yet the prosthetic ring was not used due to the infant’s growth potential [2]. Fortunately, the patient could be weaned from the pump smoothly after total correction of both lesions. The hemodynamic data were satisfactory and transesophageal echocardiography showed only mild TR. Therefore the salvage “one and one half ventricle repair” procedure [5] that is designed to unload the hypoplastic and dysplastic right ventricle by means of a bi-directional cavopulmonary shunt was not used. Albeit, long term follow-up is indeed necessary because the postoperative echocardiography revealed mild pulmonary stenosis and mild to moderate TR.
References 1. Carpentier A, Chauvaud S, Mace L, et al. A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1988;96:92–101. 2. Quaegebeur JM, Sreeram N, Fraser AG, et al. Surgery for Ebstein’s anomaly: the clinical and echocardiographic evaluation of a new technique. J Am Coll Cardiol 1991;17:722– 8. 3. Chan KC, Walker DR, Gibbs JL. Surgical correction of tetralogy of Fallot in the presence of Ebstein’s anomaly of the tricuspid valve. Int J Cardiol 1989;25:242–3. 4. Starnes VA, Pitlick PT, Bernstein D, Griffin ML, Choy M, Shumway N. Ebstein’s anomaly appearing in the neonate: a new surgical approach. J Thorac Cardiovasc Surg 1991; 101:1082–7. 5. Stellin G, Vida VL, Milanesi O, et al. Surgical treatment of complex cardiac anomalies: the “one and one half ventricle repair.” Eur J Cardiothorac Surg 2002;22:1043–9.
Bridge to Transplantation With a Left Ventricular Assist Device for Systemic Ventricular Failure After Mustard Procedure Robert S. George, BS, MRCS, Emma J. Birks, MRCP, PhD, Rosemary C. Radley-Smith, FRCP, Asghar Khaghani, FRCS, and Magdi Yacoub, FRS Departments of Transplantation and Mechanical Circulatory Support, and Pediatric Cardiology, Royal Brompton and Harefield NHS Trust, and National Heart and Lung Institute, Harefield, Middlesex, United Kingdom
After Mustard or Senning procedures, transplantation remains the only option for some patients who present at Accepted for publication March 14, 2006. Address correspondence to Dr Birks, Royal Brompton and Harefield NHS Trust, Hill End Rd, Harefield, UB9 6JH Middlesex, UK; e-mail: e.birks@ rbhnt.nhs.uk.
0003-4975/07/$32.00 doi:10.1016/j.athoracsur.2006.03.119
CASE REPORT LAI ET AL EBSTEIN’S ANOMALY COEXISTS WITH TOF
FEATURE ARTICLES
octreotide and technetium (99mTc). Additional computed tomography or magnetic resonance imaging assists in showing precise anatomic location and relationships. Typical features include a soft fleshy texture, high vascularity, adhesion to surrounding structures, and local invasion making resection challenging [3]. Cardiac pheochromocytomas lack clear planes of dissection and therefore excision of surrounding myocardium and vasculature with reconstruction through pericardial patches and grafts may be required for complete resection. Completeness of resection relates to increased survival at follow-up [4]. These tumors are highly vascular and prone to fatal hemorrhagic complications [3]. Preoperative embolization has a proven role in minimizing bleeding and facilitating dissection in vascularized tumors. Though well described in neck and abdominal pheochromocytomas, there are no previous reports of this procedure used for intrapericardial pheochromocytomas. The only two previously reported cases of preoperative embolization were nonsecretory mediastinal paragangliomas [5, 6]. In both cases embolization was only carried out after unexpected severe hemorrhage forcing the operation to be abandoned at first attempt. For pheochromocytomas, surgery continues to be first line treatment [7] and is based on surgical principles of adequate exposure, meticulous hemostasis, minimal tumor manipulation, and complete resection with intact capsule. The surgical approach to the tumor and use of cardiopulmonary bypass varies, depending on anatomical location, surgeon preference, and experience. The most popular approach is the median sternotomy with cardiopulmonary bypass, which allows adequate vascular control and manipulation [2, 4 –7]. Thoracotomy with or without cardiopulmonary bypass has also been used. Preoperative embolization has a valuable role in the management of cardiac pheochromocytoma. Our experience shows that it reduces hemorrhage, results in greater intraoperative hemodynamic stability, and therefore facilitates handling of the tumor and resection off the surrounding structures. We believe that this multimodality approach helps create a safer operation with reduced risk of complications for both the surgeon and the anesthesiologist.
References 1. Lenders JWM, Eisenhofer G, Mannelli M, Pacak K. Phaeochromocytoma. Lancet 2005;366:665–75. 2. Besterman E, Bromley LL, Peart WS. An intrapericardial pheochromocytoma. Br Heart J 1974;36:318 –20. 3. Orringer MB, Sisson JC, Glazer G, et al. Surgical treatment of cardiac pheochromocytomas. J Thorac Cardiovasc Surg 1985;89:753–7. 4. Lamy AL, Fradet GJ, Luoma A, Nelems B. Anterior and middle mediastinum paraganglioma: complete resection is the treatment of choice. Ann Thorac Surg 1994;57:249 –52. 5. Drucker EA, McLoud TC, Dedrick CG, Hilgenberg AD, Geller SC, Shepard JA. Mediastinal paraganglioma: radio© 2007 by The Society of Thoracic Surgeons Published by Elsevier Inc
Ann Thorac Surg 2007;83:304 – 6
logic evaluation of an unusual vascular tumor. AJR 1987;148:521–2. 6. Rakovich G, Ferraro P, Therasse E, Duranceau A. Preoperative embolization in the management of a mediastinal paraganglioma. Ann Thorac Surg 2001;72:601–3. 7. Gopalakrishnan R, Ticzon AR, Cruz PA, Kennedy FB, Duffy FC, Barmada B, Giacobine JW. Cardiac paraganglioma (chemodectoma): a case report and review of the literature. J Thorac Cardiovasc Surg 1978;76:183–9.
Ebstein’s Anomaly of the Tricuspid Valve in Combination of Tetralogy of Fallot: Total Correction in Infancy Chao-Han Lai, MD, Jing-Ming Wu, MD, and Yu-Jen Yang, MD, PhD Division of Cardiovascular Surgery, Department of Surgery, Department of Pediatrics, National Cheng Kung University Hospital, and Cardiovascular Research Center, Medical College, National Chen Kung University, Tainan, Taiwan
Ebstein’s anomaly of the tricuspid valve in combination with Tetralogy of Fallot is extremely rare. We report a patient with these combined entities in a baby presented with cyanosis and right heart failure soon after birth. Total correction toward biventricular repair was successfully conducted when he was 7 months old. For management of a patient with such rare complex anomalies, we recommend Carpentier’s technique for Ebstein’s anomaly to restore the morphology of the right ventricle, and “one and half ventricle repair” can be a salvage procedure if the right ventricle is not functionally tolerant. (Ann Thorac Surg 2007;83:304 – 6) © 2007 by The Society of Thoracic Surgeons
E
bstein’s anomaly of the tricuspid valve, featured as downward displacement of the septal and posterior leaflets, resulting in an atrialized portion of the right ventricle, is an uncommon congenital malformation [1, 2]. The combination of tetralogy of Fallot (TOF) and Ebstein’s anomaly is extremely rare [3]. In this report, an infant with Ebstein’s anomaly and TOF, manifesting as cyanosis and right heart failure soon after birth, received successful correction of TOF and valvuloplasty of the tricuspid valve at the age of 7 months. A male newborn was referred to our hospital because of cyanosis, heart murmur, and dyspnea noted soon after birth. On physical examination, he presented tachypnea with subcostal retraction, marked hepatomegaly, and general cyanosis. A grade III/VI systolic ejection murmur was audible over the left upper sternal border. Chest roentgenogram showed decreased bilateral lung marking and marked cardiomegaly with 95% cardiothoracic ratio, Accepted for publication April 24, 2006. Address correspondence to Dr Yang, Department of Surgery, National Cheng Kung University Hospital, National Cheng Kung University, 138, Sheng-Li Rd, Tainan 704, Taiwan; e-mail: [email protected].
0003-4975/07/$32.00 doi:10.1016/j.athoracsur.2006.04.078
Fig 1. Preoperative echocardiography showed features of tetralogy of Fallot, including (A) overriding aorta and ventricular septal defect and (B) severe pulmonary stenosis with pressure gradient of 70 mm Hg (arrow).
especially with the right atrial enlargement. Electrocardiogram showed right atrial enlargement, right ventricular hypertrophy, and nonsustained atrial flutter. Echocardiographic studies revealed the typical features of TOF (Fig 1), Ebstein’s anomaly, and an interatrial shunt. The posterior and septal leaflets were displaced 0.7 cm downward from the tricuspid annulus, resulting in severe tricuspid regurgitation (TR) (Fig 2). Although the true right ventricle was mildly underdeveloped, the contractility was not compromised. Medication was prescribed for right heart failure, yet the symptoms of respiratory distress and heart failure became more severe during the follow-up period. Therefore surgical intervention was advised at the age of 7 months, while body weight was 6 kg. The cardiopulmonary bypass was set up with single aortic and bi-caval cannulation. After the aorta was cross clamped and the
CASE REPORT LAI ET AL EBSTEIN’S ANOMALY COEXISTS WITH TOF
305
blood cardioplegic solution was infused, the right atrium was opened parallel to the atrioventricular groove. The anterior leaflet was large and freely movable; however the posterior and septal leaflets were displaced downward about 1 cm from the annulus level. The septal leaflet was hypoplastic and adhered to the septum. Pathology of the atrialized portion of the right ventricle and tricuspid valve leaflets was comparable with that of type B according to Carpentier’s classification [1]. The ventricular septal defect came into sight when the anterior and posterior leaflets of the tricuspid valve were detached from the annulus. However, the outlet extension of the perimembranous ventricular septal defect made its upper margin difficult to repair by this approach. Thus we made the longitudinal incision on the right ventricle outflow tract and performed infundibulectomy. The ventricular septal defect was patch repaired with multiple interrupted stitches. The pulmonary valve was bicuspid with commissural fusion, and the pulmonary annulus was hypoplastic. Therefore, after pulmonary valvotomy, the right ventricle outflow tract was enlarged with a transannular patch. The tricuspid valve was reconstructed using Carpentier’s technique: the atrialized ventricle was plicated vertically with four mattress stitches reinforced with pledgets. The detached leaflets were reattached clockwise to the new annulus to cover the tricuspid orifice beyond the plication site and ending below the coronary sinus, but no prosthetic ring was used [1, 2]. After rewarming, the patient was smoothly weaned from cardiopulmonary bypass with a small amount of inotropic agents. Postoperative echocardiography demonstrated only mild pulmonary stenosis (PS), and mild to moderate TR (Fig 3). The cardiothoracic ratio on chest roentgenogram was reduced to 78%. The postoperative course was uneventful, and he was discharged on postoperative day 10. One month after surgery, mild pulmonary stenosis with a 22 mm Hg pressure gradient between the right ventricle and main pulmonary artery was shown in follow-up echocardiography. Also a mild to moderate degree of TR
Fig 2. Preoperative echocardiography also showed the concomitant Ebstein’s anomaly featured as a downward displacement of the tricuspid valve (arrow) and severe tricuspid regurgitation with a pressure gradient of 70 mm Hg.
FEATURE ARTICLES
Ann Thorac Surg 2007;83:304 – 6
306
CASE REPORT GEORGE ET AL BRIDGE TO TRANSPLANTATION WITH LVAD AFTER MUSTARD PROCEDURE
Fig 3. Postoperative echocardiography showed a mild to moderate tricuspid regurgitation with a pressure gradient of 27 mm Hg.
FEATURE ARTICLES
(the regurgitation jet extending over one third but less than one half of the atrium) with a pressure gradient of 28 mm Hg between the right ventricle and right atrium was shown. The patient was asymptomatic with a moderate amount of diuretics.
Comment Ebstein’s anomaly comprises only about 1% of all congenital heart lesions [1]. It is frequently associated with various cardiac anomalies; however, coexistence with TOF is extremely rare. There has been very scarce surgical experience for patients with such a combination. Chan and colleagues [3] reported the surgical correction in a 7-month-old girl with TOF in the presence of Ebstein’s anomaly, but the tricuspid valve was not touched because the TR was only mild. In our patient, right heart failure due to severe TR necessitated total correction for both lesions in infancy. Successful surgical management for both lesions has not been previously described in the English literature. Clinical presentation of Ebstein’s anomaly varies; it depends mainly on the displacement of the tricuspid valve. The most severe form usually has symptoms presenting in the neonatal stage or early infancy, whereas patients with mild displacement may remain asymptomatic until late adult life. The symptoms of our patient should be moderately severe because the pathologic features were compatible with that of type B according to Carpentier’s classification. However, systemic pressure of the right ventricle resulting from coexistence of TOF aggravated the severity of TR and heart failure. Thus, intractable right heart failure ensued even after maximal medication and made early surgical intervention necessary. How to manage the patient surgically merits some discussion. The right complex heart brought about both heart failure and cyanosis. A palliative aortopulmonary shunt was not advisable because it would not prevent heart failure. Starnes procedure has been reserved only © 2007 by The Society of Thoracic Surgeons Published by Elsevier Inc
Ann Thorac Surg 2007;83:306 – 8
for those with the most severe form, and inevitably the patients had to accept the single ventricle physiology [4]. Even though the right ventricle was slightly hypoplastic in the preoperative evaluation, we preferred the biventricular repair because the right ventricular function seemed adequate. We adopted Carpentier’s technique because the vertical plication may restore the morphology and function of right ventricle better, yet the prosthetic ring was not used due to the infant’s growth potential [2]. Fortunately, the patient could be weaned from the pump smoothly after total correction of both lesions. The hemodynamic data were satisfactory and transesophageal echocardiography showed only mild TR. Therefore the salvage “one and one half ventricle repair” procedure [5] that is designed to unload the hypoplastic and dysplastic right ventricle by means of a bi-directional cavopulmonary shunt was not used. Albeit, long term follow-up is indeed necessary because the postoperative echocardiography revealed mild pulmonary stenosis and mild to moderate TR.
References 1. Carpentier A, Chauvaud S, Mace L, et al. A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1988;96:92–101. 2. Quaegebeur JM, Sreeram N, Fraser AG, et al. Surgery for Ebstein’s anomaly: the clinical and echocardiographic evaluation of a new technique. J Am Coll Cardiol 1991;17:722– 8. 3. Chan KC, Walker DR, Gibbs JL. Surgical correction of tetralogy of Fallot in the presence of Ebstein’s anomaly of the tricuspid valve. Int J Cardiol 1989;25:242–3. 4. Starnes VA, Pitlick PT, Bernstein D, Griffin ML, Choy M, Shumway N. Ebstein’s anomaly appearing in the neonate: a new surgical approach. J Thorac Cardiovasc Surg 1991; 101:1082–7. 5. Stellin G, Vida VL, Milanesi O, et al. Surgical treatment of complex cardiac anomalies: the “one and one half ventricle repair.” Eur J Cardiothorac Surg 2002;22:1043–9.
Bridge to Transplantation With a Left Ventricular Assist Device for Systemic Ventricular Failure After Mustard Procedure Robert S. George, BS, MRCS, Emma J. Birks, MRCP, PhD, Rosemary C. Radley-Smith, FRCP, Asghar Khaghani, FRCS, and Magdi Yacoub, FRS Departments of Transplantation and Mechanical Circulatory Support, and Pediatric Cardiology, Royal Brompton and Harefield NHS Trust, and National Heart and Lung Institute, Harefield, Middlesex, United Kingdom
After Mustard or Senning procedures, transplantation remains the only option for some patients who present at Accepted for publication March 14, 2006. Address correspondence to Dr Birks, Royal Brompton and Harefield NHS Trust, Hill End Rd, Harefield, UB9 6JH Middlesex, UK; e-mail: e.birks@ rbhnt.nhs.uk.
0003-4975/07/$32.00 doi:10.1016/j.athoracsur.2006.03.119