Eur J Cardiothorac Surg 2007;31:744-746. doi:10.1016/j.ejcts.2007.01.022
Copyright © 2007, European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved
Emergent David-V operation for a ruptured aortic root aneurysm in a 9-year-old child
Yasuyuki Kato*,
Hirokazu Ohashi,
Yasushi Tsutsumi,
Takahiro Kawai
Department of Cardiovascular Surgery, Fukui Cardiovascular Center, Shinbo 2-228 Fukui, Japan
Received 7 December 2006;
received in revised form 5 January 2007;
accepted 15 January 2007.
* Corresponding author. Tel.: +80 776 54 5660; fax: +80 776 53 2131. (Email: ayak0511{at}yahoo.co.jp).
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Abstract
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Ruptured aortic root aneurysm is very rare in children less than 10 years of age. Isolated dilatation of the ascending aorta and/or aortic root in a child is mostly associated with Marfan's syndrome, and the standard surgical treatment is aortic root replacement with a composite valve graft or homograft. We report here a successful emergent T. David-V operation using two grafts of different sizes for a ruptured aortic root aneurysm in a 9-year-old child with Marfan's syndrome.
Key Words: Aneurysm • Aortic • Aortic root • Rupture • Valve disease
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1. Introduction
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Rupture of an aortic root aneurysm is extremely rare in children less than 10 years of age [1]. The standard surgical treatment for a dilated aortic root is aortic root replacement with a composite valve graft or homograft [2–5]. However, children using composite grafts are exposed to a long-term risk of thromboembolism and oral anticoagulation, while graft failure may occur in those using homografts. We report here a case of a successful emergent valve-sparing T. David-V operation using two grafts of different sizes for a ruptured aortic root aneurysm in a 9-year-old child with Marfan's syndrome.
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2. Case
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A 9-year-old boy was admitted to hospital with chest pain. He had been diagnosed as Marfan's syndrome based on the presence of scoliosis, clubfoot, and dilatation of the aortic root. On admission, he was 111 cm tall and weighted 16 kg. His blood pressure was 110/58 mmHg and pulse 126 beats/min. A holodiastolic murmur of grade 3/6 was heard in the aortic area. An echocardiogram revealed dilatation of the aortic root, severe aortic regurgitation, and pericardial effusion. The maximum diameter of the aortic root was 65 mm and of the aortic annulus 24 mm. A CT scan demonstrated dilatation of the aortic root, pericardial effusion, and deformity of the thorax (Fig. 1a).
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Fig. 1. (a) Chest computed tomography demonstrates marked dilatation of the aortic root, pericardial effusion, and deformity of thorax. (b) Intraoperative view of the large aortic root aneurysm. (c) The tear was located at the posterior wall of the ascending aorta just distal to the sinotubular junction (arrows). (d) The aortic valve reimplanted inside the graft.
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An emergent operation was performed. When the pericardium was opened, a large aortic root aneurysm appeared (Fig. 1b). After establishing cardiopulmonary bypass, cardioplegia was delivered via retrograde fashion. The aortic wall was opened, and a tear was noticed in the posterior wall of the ascending aorta just distal to the sinotubular junction (Fig. 1c). Each sinus of Valsalva was excised, and the coronary buttons were dissected out. The aortic root was mobilized and three horizontal mattress sutures were placed on each commissure. The aortic valve was inspected, including assessment of leaflet thickness and coaptation, and it was decided that it should be preserved. A 34 mm Hemashield graft was selected as the valve leaflets were relatively large for the patient's body size, and it would allow for future patient growth. The proximal side of the graft was necked down to 23 mm with twelve interrupted mattress sutures. Fifteen subannular stitches were placed in a horizontal mattress fashion immediately below the lowest level of the valve leaflets in the left ventricular outflow tract, using 3-0 polyester sutures with pledgets, and then passed through the necked down side of the graft. The valve was reimplanted inside the graft and the subannular stitches were tied (Fig. 1d). The resuspension points of the three commissures were adjusted to obtain good coaptation of the leaflets. The valve was well coapted as tested by saline infusion into the aortic root. The sinus remnant was fixed within the graft, and the coronary buttons were anastomosed in a standard fashion. The graft was amputated just distal to the commissures. The diameter of the distal ascending aorta was 18 mm, and a 20 mm Hemashield graft was anastomosed to it. Finally, the pseudosinus was created by sewing the distal graft to proximal aortic root graft at the neosinotubular junction. An immediate postoperative transesophageal echocardiogram showed trivial aortic regurgitation. At follow-up 6 months later the boy was in good condition, and a routine transthoracic echocardiogram showed trivial aortic regurgitation and bulging pseudosinuses (Fig. 2
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Fig. 2. Postoperative transthoracic echocardiography showing trivial aortic regurgitation and bulging pseudosinuses (arrows).
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3. Comment
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Dilatation of the aortic root and ascending aorta leading to aortic regurgitation and/or dissection are the main causes of premature death in Marfan's syndrome. Except for severely affected infants with mitral valve disease, dilatation of the aortic root remains the most common indication for cardiac operations in children with Marfan's syndrome [1–5]. Operation is not usually required in children less than 10 years of age with an aortic root aneurysm, as rupture or dissection of the aortic root is uncommon in this age group. In a meta-analysis of patients aged less than 20 years with Marfan's syndrome, Kniesh reported aortic dissection in five patients (1.7%) and rupture in three (1.0%), all of whom were 14 years or older [1]. Therefore, our patient is an extremely rare case and a good example of the potential risk for early rupture of a dilated aortic root in Marfan's syndrome.
The standard surgical treatment for children with a dilated aortic root is aortic root replacement with a composite valve graft or homograft, and excellent results have been reported [2–5]. However, children with composite graft replacement require long-term anticoagulation and face the risk of complications inherent to prosthetic valves, while graft failure may occur in patients with homografts, which are difficult to obtain in Japan.
Valve-sparing aortic root replacement has become an attractive alternative option to eliminate long-term anticoagulation and complications accompanying prostheses, especially in younger patients. Few reports, however, are available about valve-sparing surgery for children with a dilated ascending aorta and/or aortic root [3,5,6], and we have found no cases in the literature of emergent valve-sparing operations for a ruptured aortic root aneurysm in children aged 10 years or less.
In this case, we performed a valve-sparing T. David-V procedure using two grafts of different sizes to create pseudosinuses as described by Demers and Miller [7]. This reimplantation technique provides better annular stabilization, facilitates sewing the valve inside the graft, and makes the distal aortic anastomosis a better size match. This technique also enables the surgeon to easily adjust the size of the aortic annulus, the length of the graft pseudosinus, and the diameter of the sinotubular junction. We did not use a Valsalva prosthesis as it was difficult to adjust the length and diameter of the graft sinus to the height of the commissures and the size of leaflets.
We replaced the ascending aorta using a 20 mm graft, which put the patient at risk of future left ventricular hypertrophy due to functional aortic stenosis. Careful long-term follow-up is necessary to assess for the development of left ventricular hypertrophy, and the condition of the aortic valve. The creation of pseudosinuses can be expected to facilitate valve opening and closure, and minimize leaflet stress and strain, which may enhance long-term valve durability [8].
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References
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- Knirsch W, Hillebrand D, Horke A, Lewin MA, Rein J, Uhlemann F. Aortic aneurysm rupture in infantile Marfan's syndrome. Pediatr Cardiol 2001;22:156-159.[CrossRef][Medline]
- Gillinov AM, Zehr KJ, Redmond JM, Gott VL, Deitz HC, Reits BA, Laschinger JC, Cameron DE. Cardiac operations in children with Marfan's syndrome: Indications and results. Ann Thorac Surg 1997;64:1140-1145.[Abstract/Free Full Text]
- Carrel T, Berdat P, Pavlovic M, Sukhanov S, Engliberger L, Pfammatter JP. Surgery of the dilated aortic root and ascending aorta in pediatric patients: techniques and results. Eur J Cardiothorac Surg 2003;24:249-254.[Abstract/Free Full Text]
- Anttila V, Piaszczynski M, Mora B, Hagino I, Lacro RV, Zurakowski D, Jonas RA. Improved outcome with composite graft versus homograft root replacement for children with aortic root aneurysms. Eur J Cardiothorac Surg 2005;27:420-424.[Abstract/Free Full Text]
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Abstract
1. Introduction
