Ann Thorac Surg 2008;86:1378-1380. doi:10.1016/j.athoracsur.2008.03.010
© 2008 The Society of Thoracic Surgeons
Case Reports
Norwood Procedure Combined With the Starnes Procedure
Tomohiro Nakata, MD,
Yoshifumi Fujimoto, MD,
Keiichi Hirose, MD, PhD,
Yuko Tosaka, MD,
Yujiro Ide, MD,
Kisaburo Sakamoto, MD*
Department of Cardiovascular Surgery, Shizuoka Children's Hospital, Shizuoka, Japan
Accepted for publication March 5, 2008.
* Address correspondence to Dr Sakamoto, Department of Cardiovascular Surgery, Shizuoka Children's Hospital, 860 Urushiyama Aoi-ku, Shizuoka, 420-8660, Japan (Email: sakamoto{at}jun.ncvc.go.jp).
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Abstract
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We describe the case of 6-day-old baby girl with congenitally corrected transposition of the great arteries, Ebstein anomaly with severe tricuspid valve regurgitation, aortic atresia, hypoplastic aortic arch, and patent ductus arteriosus. She underwent Starnes procedure combined with the Norwood procedure. Postoperative echocardiography demonstrated good left ventricular function, reduced size of the right ventricle, and an unobstructed aortic arch. She was discharged and is currently awaiting a bidirectional Glenn operation.
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Introduction
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Congenitally corrected transposition of the great arteries (cTGA) is characterized by discordant atrioventricular and ventriculo-arterial connections, and is commonly associated with Ebstein anomaly of the tricuspid valve. Because the right ventricle supports the aorta in cTGA, Ebstein anomaly with severe tricuspid valve regurgitation (TR) is often associated with aortic arch obstruction [1–3].
The combination of cTGA with Ebstein anomaly and aortic atresia is extremely rare, and may be considered to be inoperable. We believe that no successful surgical repair has been previously reported. We present the first successful case of Starnes procedure combined with the Norwood procedure.
A 24-year-old gravida 1, para 1 woman was referred to our institution for fetal cardiac evaluation in gestational week 36. Fetal echocardiography revealed normal visceral situs, atrioventricular discordance, ventriculo-arterial discordance, Ebstein anomaly with severe TR, aortic atresia, and hypoplastic aortic arch.
A baby girl (weight, 2,770 g) was delivered by Cesarean section in gestational week 37. She was immediately intubated due to respiratory distress, and prostaglandin E1 infusion was initiated. Postnatal echocardiography confirmed the prenatal diagnosis (Fig 1). The small ascending aorta with retrograde flow was located anterior to the left of the main pulmonary artery (PA). The hypoplastic left-sided aortic arch with normal branching pattern and a small muscular ventricular septal defect were identified. Chest roentgenogram showed severe cardiomegaly (Fig 2).
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Fig 1. Preoperative echocardiography (A, B) demonstrated the small ascending aorta, Ebstein anomaly of the tricuspid valve, and severely dilated left atrium. Postoperative echocardiography (C) demonstrated reduced size of the right ventricle. (AAo = ascending aorta; LA = left atrium; LAA = left atrial appendage; LCCA = left common carotid artery; LV = left ventricle; PA = pulmonary artery; PDA = patent ductus arteriosus; RA = right atrium; RV = right ventricle.)
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Fig 2. Chest roentgenogram (A) before and (B) after the Norwood procedure combined with the Starnes procedure.
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On day 6 after birth, she underwent cardiac surgery. An expanded polytetrafluoroethylene (E-PTFE) graft (3.0-mm in diameter) was sewn to the innominate artery. Cardiopulmonary bypass was commenced by introducing the E-PTFE graft as an arterial line and with bi-caval venous cannulation. After division of the ductus arteriosus, the main PA was opened. We found that the ductal tissue migrated into and surrounded the ostium of the left PA. Therefore, prior to cardioplegic arrest, we performed PA angioplasty with total resection of the ductal tissue and direct end-to-end anastomosis, using the PA wall of the infant.
Then we performed the Starnes procedure as follows: an atrial septostomy was conducted and the tricuspid valve was inspected through the atrial septal opening. The large anterior leaflet was tethered to the right ventricular free wall and this impaired valve competence. The septal and posterior leaflets were displaced from the anatomical atrioventricular groove and formed an atrialized portion of the right ventricle. An E-PTFE sheet of 0.4-mm thickness with a 2.0-mm fenestration was sewn to the tricuspid valve annulus. The severely dilated giant left atrium was resected and plicated.
Then we performed the Norwood procedure as follows: low-flow regional cerebral perfusion (79 min) was maintained during arch reconstruction through the E-PTFE graft. After complete removal of the ductal tissue, the ascending aorta (2-mm diameter) was transected and an aortotomy was extended to the level of the sinotubular junction. The proximal stump of the main PA was sutured to the proximal ascending aorta in a side-to-side fashion, and the posterior wall of the descending aorta was sutured to the aortic arch (3-mm diameter). A Xenomedica patch (Baxter Healthcare Corp, Horw, Switzerland) was used for the minor curvature of aortic augmentation, and the anterior wall of the main PA was sutured to the aortic arch. An arterial cannula was inserted into the proximal stump of the main PA, and the E-PTFE graft was sewn to the right PA. The duration of cardiopulmonary bypass and aortic cross clamping was 351 and 119 min, respectively. The intraoperative arterial oxygen saturation was in the low 80s. The sternum was left open during the immediate postoperative period, and she was hemodynamically stabilized using inotropic agents and sodium nitroprusside. Delayed sternal closure was performed on postoperative day 6. The patient stayed in the intensive care unit for 14 days, mainly due to prolonged pleural drainage, and she was successfully extubated on postoperative day 39.
A postoperative chest roentgenogram showed a decreased cardiothoracic ratio (Fig 2), and follow-up echocardiography demonstrated good left ventricular function, reduced right ventricle dimension, and an unobstructed aortic arch (Fig 1).
She was discharged 2 months after the surgery. Her systemic oxygen saturation was in the low 80s with oxygen at 1 L/min. She is currently awaiting a bidirectional Glenn operation.
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Comment
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The combination of cTGA and Ebstein anomaly with severe TR may compromise forward systemic flow and contribute to hypoplasia of the systemic outflow tract [1–3]. This situation is similar to the association of Ebstein anomaly with severe TR and underdevelopment of the pulmonary outflow tract in the heart with normal segmental connections. The combination of cTGA, Ebstein anomaly with severe TR, and aortic atresia is extremely rare and associated with poor prognosis [1–3]. We believe that no successful surgical repair has been previously reported.
Our goals of first-stage palliation were to provide reliable tracts for both systemic and pulmonary blood flow, to correct severe TR with left atrial dilation, and to prevent left ventricle compression due to a dilated right ventricle. Although Knott-Craig and colleagues [4] proposed aggressive tricuspid valve plasty for Ebstein anomaly in symptomatic neonates, we did not believe that her right ventricle could supply the appropriate forward systemic blood flow due to aortic atresia with only small muscular ventricular septal defect. Therefore, we decided not to use her right ventricle as a systemic ventricle, and selected the Starnes procedure with a fenestration of the tricuspid valve patch to prevent the possible deleterious effects of right ventricular volume overload and assure sufficient left ventricular function. Postoperative echocardiography demonstrated good results.
One may argue that the Starnes procedure with bilateral PA banding might be less invasive than the Starnes procedure combined with the Norwood procedure. Although Michel-Behnke and colleagues [5] reported successful stage I bilateral PA banding with patent ductus arteriosus stent for multiple left heart obstructive lesions, acute ischemia that did not respond to medical and mechanical resuscitation was also reported in patients with an extremely diminutive ascending aorta. The ascending aorta in our patient was so small that we were concerned about potential coronary blood flow insufficiency with bilateral PA banding. Therefore, we chose the Starnes procedure concomitant with the Norwood procedure to secure her coronary blood flow rather than the Starnes procedure with bilateral PA banding. Fortunately, no ischemic event occurred during the surgery and post-surgical periods. In conclusion, this new surgical strategy of the Starnes procedure concomitant with the Norwood procedure for a neonate with cTGA, Ebstein anomaly with severe TR, and aortic atresia can be a reasonable and useful surgical option.
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References
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- Celermajer DS, Cullen S, Deanfield JE, Sullivan ID. Congenitally corrected transposition and Ebstein's anomaly of the systemic atrioventricular valve: association with aortic arch obstruction J Am Coll Cardiol 1991;18:1056-1058.[Abstract]
- Bader R, Perrin D, Yoo SJ. Congenitally corrected transposition of the great arteries with Ebstein malformation and hypoplasia of the aortic arch in a fetus Fetal Pediatr Pathol 2004;23:257-263.[Medline]
- Craig BG, Smallhorn JF, Rowe RD, Williams WG, Trusler GA, Freedom RM. Severe obstruction to systemic blood flow in congenitally corrected transposition (discordant atrioventricular and ventriculo-arterial connexions): an analysis of 14 patients Int J Cardiol 1986;11:209-217.[Medline]
- Knott-Craig CJ, Overholt ED, Ward KE, Ringewald JM, Baker SS, Razook JD. Repair of Ebstein's anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up Ann Thorac Surg 2002;73:1786-1793.[Abstract/Free Full Text]
- Michel-Behnke I, Akintuerk H, Marquardt I, et al. Stenting of the ductus arteriosus and banding of the pulmonary arteries: basis for various surgical strategies in newborns with multiple left heart obstructive lesions Heart 2003;89:645-650.[Abstract/Free Full Text]
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Abstract
Introduction
