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Interact CardioVasc Thorac Surg 2008;7:1121-1123. doi:10.1510/icvts.2008.183418 © 2008 European Association of Cardio-Thoracic Surgery
Consequences of incomplete repair of acute type A aortic dissectionDepartment of Cardiac, Thoracic and Vascular Surgery, National University Hospital, National University of Singapore, 5 Lower Kent Ridge Road, Level 2, 119074 Singapore Received 21 May 2008; received in revised form 29 July 2008; accepted 30 July 2008
Corresponding author. Tel.: +65 6772 2065/5214; fax: +65 6776 6475.
During emergency repair of acute Stanford type A aortic dissections, surgical compromises in the form of incomplete arch replacement are made due to the unstable condition of the patient and safety issues of the performing team. We report a case of delayed reoperation after previous incomplete surgery for acute type A aortic dissection in a young patient with Marfan's syndrome. He presented again with repetitive chest pain five years after initial surgical treatment. Extensive aneurysmal dilatation of the aorta and remaining dissection led to the decision to replace the ascending aorta and the aortic arch. After a good progress during the first days after surgery, the patient died due to a ruptured thoraco-abdominal aneurysm on the fifth postoperative day. Extensive surgical reconstruction including aortic arch replacement should be considered in patients with Marfan's syndrome who present with aortic dissections type A to avoid unnecessary reoperations and their complications.
Key Words: Aortic dissection; Surgical treatment; Aortic arch replacement; Marfan's syndrome
Aortic dissection is a common complication in Marfan's syndrome patients. Surgery in this cohort of patients often requires extensive resection of the dissected aorta and reconstruction of the aortic root. Compromise in surgical strategy endangers the patient's life and may result in unnecessary repetition of surgical intervention. We report a case of delayed reoperation after previous incomplete surgery for acute type A aortic dissection with fatal out-come.
A 24-year-old male patient with known history of Marfan's syndrome was admitted to our hospital in 2001 with an acute type A aortic dissection extending to the infrarenal abdominal aorta without formation of an aneurysm (diameter of the ascending aorta was 3.1 cm and the descending aorta was 2.9 cm). The patient underwent an emergency repair of his ascending aorta on the same day of admission, with replacement of aortic root and proximal ascending aorta with a 23-mm sized valve conduit. During surgery the aortic arch was cannulated and aortic cross clamp was used without hypothermic circulatory arrest (closed distal anastomosis technique). The entry site was found in the ascending aorta and the tear resected. The patient recovered well and was discharged 12 days after the procedure. A control CT-scan three months after surgery showed persistence of a dissection in the aortic arch and descending thoracic aorta with a large false lumen compressing the true lumen. Additionally, there was evidence of false aneurysm formation at the level of the previous distal anastomosis between the graft and the ascending aorta (Fig. 1). The option to repeat surgery and replace the ascending aorta and aortic arch was offered to him but he denied any surgical intervention.
In 2006 the patient was readmitted to the hospital with repetitive chest pain and progressive dilatation of the ascending aorta, the aortic arch and the thoraco-abdominal aorta was visible on the CT thorax scan (Fig. 2). He consented and underwent the second operation almost six years after the first procedure. Replacement of the ascending aorta and the aortic arch was planned as a first stage procedure. A median sternotomy was performed and the femoral vessels cannulated. The residual distal ascending aorta and the total arch were replaced under circulatory arrest with antegrade cerebral perfusion technique for brain protection. We proceeded with the next planned stage of surgery on the thoraco-abdominal aorta using the elephant trunk technique and a 24-mm Dacron graft. The arch island was anastomosed to the proximal part of the graft after completion of the elephant trunk procedure.
The patient recovered well, but on the fifth day after surgery he collapsed, did not respond to resuscitation, and passed away. Post-mortem evaluation revealed thoraco-abdominal aneurysm rupture.
Surgical treatment for Stanford type A dissections can prevent fatal complications such as cardiac tamponade, myocardial infarction, heart failure, aortic rupture and significantly decreases early mortality [1–3]. However, late results have been disappointing even after an initial successful operation [2, 4]. The remaining aortic dissection that was not primarily replaced is responsible for the considerable number of postoperative complications and deaths [1, 2]. Aggressive surgical approach involving extensive resection of dissected aorta for patients with aortic dissection became more popular over the recent years [3]. According to the authors, extensive replacement of dissected aorta can improve long-term results and decrease requirement for a second intervention. In the presented case, the first operation was limited to the aortic root and proximal part of ascending aorta only and was not optimal for the patient with Marfan's syndrome. Additionally, there is an increasing trend to aortic-valve-sparing surgeries, such as the recent David procedure [5]. Open distal anastomosis has been introduced to increase safety and adequacy of arch surgery [2, 3]. Despite controversy, this technique has the advantage of the aortic lumen revision and leads to a more radical resection of dissected aortic wall. In our case the aortic arch was not revised and the dissected aortic wall was not adequately resected. Furthermore, the first surgery with incomplete resection of ascending aorta made total replacement of the dissected aorta via left thoracotomy impossible. Surgical treatment is advocated for patients with persistent false lumen and development of aneurysm formation after the first operation [1, 2, 6]. Acute rupture of an existing aneurysm has a rate of 3–5% per five years and it is especially frequent with large and persistently growing aneurysms [1, 4]. In our case, anastomotic aneurysm formation occurred in the first three months after first operation and was not repaired for five years due to defaulted follow-up. Kouchoukos et al. have shown in non-Marfan patients with extensive aortic disease and with a variety of entities, that an extensive first surgical approach is similar to the first surgery of a two-stage procedure considering complications and follow-up. Aggressive surgical approach can be an option since in Marfan's patients the risk of rupture complications is even higher. However, as stated, aneurysmal enlargement of the aorta distal to the anastomosis and the extent of bilateral anterior thoracotomy can be deterring factors or limiting factors that have to be assessed individually [7]. The rapid endovascular approach and stent grafting of the aneurysmal enlarged aorta has proven to be safe and useful in non-Marfan patients, but for patients with Marfan's syndrome bigger studies and especially long-term follow-up data are not available yet and a general recommendation cannot be given [8]. This case demonstrates the importance of complete surgical resection of ascending aorta. Extensive surgical reconstruction including aortic arch replacement should be considered in patients with Marfan's syndrome who present with aortic dissections type A to avoid unnecessary reoperations and their complications [9]. Furthermore, consequent regular follow-up with CT-scan after surgery must be mandatory in these patients.
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