Background : The surgical treatment of the mega-aortic syndrome consists in two consecutive interventions. During the first step, the ascending aorta (sometimes including the aortic valve) and aortic arch are replaced using the elephant trunk (ET) in the descending aorta, thus facilitating the second step. The results of this intervention are analysed retrospectively.
Methods : From September 1984 through September 2000, 81 patients with a mean age of 66.4 years (range 21 to 79 years) underwent surgery. Indication for surgery was a chronic aneurysm in 79 patients (elective), and acute type A dissection in 2 patients (emergency). The reconstruction consisted in a Bentall + total ascending aorta + ET in 29 (36%) patients, a supracoronary ascending aorta + total arch + ET in 51 (63%) patients, and a total arch + ET in 1 (1%) patient. Associated procedures included CABG in 14 patients, aortic valve plasty in 3, mitral valve repair or replacement in 2, and a bypass to the supra-aortic vessels in 6. The brain was protected using deep hypothermic circulatory arrest in 7 (9%) patients, antegrade selective cerebral perfusion in 57 (70%) patients, and a combination of the previous techniques in 17 (21%) patients.
Results : There were no intraoperative deaths. Hospital mortality was 7.4% (6 patients). The causes of death were rupture of the distant aneurysm in 2 patients, multi-organ failure in 2, cardiac failure in 1, and tamponade in 1. Postoperative central neurologic damage occurred in 6 patients: it was permanent in 4 (5%) patients, and temporary in 2 (2.5%). Other morbidity included temporary hemodialysis in 3 (3%) patients, myocardial infarction in 1 (1%), tracheotomy in 7 (8%), rethoracotomy for bleeding in 24 (30%) and left recurrent nerve palsy in 16 (22%) patients. To date, thirty six-patients underwent a second stage repair of their thoracic aortic aneurysm (4 patients), or thoracoabdominal aneurysm (2 patients), with a mean interval of 4 months. In five patients (excluding the hospital deaths), the distant aneurysm ruptured before the second step could be performed.
Conclusion : Considering the extent of the pathology, and the intervention, the surgical repair using the ET can be performed with an acceptable mortality and morbidity. The second step should not be differed too long.