Total Thoracoabdominal Aortic Replacement in a Marfan Patient Following Heart Transplantation and Hemiarch Repair
Article Outline
Patients with Marfan syndrome were a well-known risk population to develop aortic root aneurysm and aortic dissection through entire life span. Repeated operations for ascending, descending and abdominal aortic pathology were not uncommon. We present a case report of a total thoracoabdominal aortic replacement in a Marfan patient following heart transplantation and hemiarch repair. We achieved a successful one-stage replacement of entire thoracoabdominal aorta. The peri-operative course was complicated with delayed, but reversible paraplegia. The surgical approach and strategy of spinal cord protection is discussed.
Keywords: Marfan syndrome, Heart transplantation, Type-B aortic dissection, Thoracoabdominal aneurysm
Introduction
Marfan syndrome is a relatively common heritable disorder of connective tissue that affects mostly cardiovascular systems. Here, we report a young Marfan patient who had experienced aortic root aneurysm, severe aortic regurgitation, end-stage heart failure, and dissecting thoracoabdominal aneurysm. The patient survived all these events and underwent two major cardiovascular operations within a decade: heart transplantation with hemiarch repair and total thoracoabdominal aortic replacement. Both operations involved deep hypothermia circulatory arrest.
Report
A 29-year-old man was a patient of Marfan syndrome. He had bilateral mechanical pleurodesis for repeated pneumothorax at the age of 18. Aortic root aneurysm and severe aortic regurgitation resulted in end-stage heart failure. Orthotopic heart transplantation and hemiarch repair were performed using deep hypothermic circulatory arrest at the age of 21. Till one month prior to this admission, the patient experienced extraordinary chest pain and prompted him to the emergency service. Magnetic resonance images showed huge, triple-barrel, dissecting thoracoabdominal aneurysm extending from distal arch to aortic bifurcation (Fig. 1).
Fig. 1
Triple-barrel dissecting thoracic aorta sizing 10
cm showed in (A) axial cut and (B) Sagital cut. (C) The schematic drawing shows previous hemiarch repair and current dissecting thoracoabdominal aortic aneurysm on the projection of an ordinary chest film.
On the day of surgery, a lumbar drain and an intracranial pressure monitor line were inserted through separate vertebral level. Thoracoabdominal incision was made followed by extensive left pleural adhesionolysis. Cardiopulmonary bypass was initiated after main pulmonary, left femoral and axillary artery cannulation. Core body temperature was lowered to 18°C. The aneurysm was incised after circulatory arrest. Meanwhile, cerebrospinal fluid (CSF) was allowed to drain freely. A 20-Fr Foley catheter was inserted into ascending aorta to deliver cardioplegic solution. The proximal anastomosis was constructed using a 22-mm Dacron graft. Extensive aneurysm resection was carried out. Celiac trunk, superior mesenteric artery, and both renal arteries were revascularized using a 4-branch graft. In addition, four pairs of intercostal and one pair of lumbar arteries were incorporated in the graft. (Fig. 2, Panel A & B) The cardiopulmonary bypass lasted for 5 hours. After completion of operation, CSF pressure was kept within 5∼10cm H2O. Consciousness and free limb movement were regained 6 hours after the operation. Although there was no hypotensive episode, paraplegia developed 12 hours later. CSF drainage protocol was changed from maintaining a pressure below 10cm H2O to free drainage. High dose steroids were given. The patient had gradual recovery of lower extremity muscle power. The CSF draining catheter was removed on the fifth post-operative day. After aggressive rehabilitation, the patient started to walk without assistance 5 weeks later. The overall hospital stay was 58 days. During this period, immunosuppression using cyclosporine was continued. Post-operative magnetic resonance images showed complete exclusion of aneurysm and occluded intercostal and lumbar grafts (Fig. 2, Panel C).
Fig. 2
(A) The intraoperative photo shows complex revascularization. I: intercostal graft; L: lumbar graft, R: the graft to left renal artery; V: the graft to celiac trunk, superior mesenteric and right renal arteries. (B) The schematic drawing simplifies the operative procedures. (C) Postoperative magnetic resonance image shows the reconstructed result. Notify the occluded intercostal graft remnant.
Discussion
The introduction of composite graft repair of aortic root aneurysm by Hugh Bentall in 1968 promised Marfan patients a normal life expectancy. Aortic size is important in predicting complications, and a measurement of relative aortic size allows for risk stratification, enabling appropriate surgical decision-making.1 Althought no strong evidence shows myocardium is affected in Marfan patients, end-stage heart failure usually occurs after long-exiting valvular insufficiency. Vascular complications of Marfan syndrome may diminish the anticipated results after heart transplantation. Therefore, the reluctance to place these patients on heart transplant donor waiting lists can be justified.2 There were only limited cases underwent heart transplantation, and even fewer for combined heart transplant and resection of the dissecting aneurysm.3 When pain recurs in Marfan patients with chronic dissection, acute on chronic dissection or three-channeled dissection should be suspected and subsequent surgery is required.
In this patient, recently aggravated chest pain warranted aggressive and urgent treatment. The complexity of dissecting lumens and visceral blood supply prohibited the possibility of endovascular approach. The extent of dissecting aneurysm from the arch to bifurcation also made limited resection impractical.
Hypothermic cardiopulmonary bypass with circulatory arrest provides safe and substantial protection against paralysis and renal, cardiac, and visceral organ system failure.4 The technique was used due to the size of the aneurysm precluded placement of clamps, anticipated time-consuming reconstruction for extensive aortic disease, and the risk of spinal cord ischemia. Peri-operative CSF drainage was used to ensure spinal cord protection.5 Our patient still developed delayed paraplegia. Several managements including aggressive CSF drainage, elevated perfusion pressure, and mega-dose steroid possibly contributed to the recovery of delayed paraplegia. The different approach we applied in this patient was free drainage of CSF which allowed CSF pressure at 3∼5cm H2O. The celiac trunk, superior mesenteric and right renal arteries were repaired as a single patch because close anatomical vicinity of these vessels in this patient and technical convenience. However, it is advisable not to use large patches at the site of visceral or cervical vessel anastomosis as Marfan patients usually develop aneurismal dilatations at these sites.
Thoracoabdominal aortic surgery in Marfan patient is not uncommon, but total thoracoabdominal aortic replacement in a Marfan patient following heart transplant and hemiarch repair has not been reported. Using contemporary techniques, complex Marfan patients can undergo thoracoabdominal aortic aneurysm repair with good clinical outcome, despite the extensive nature of the disease.
References
- Novel measurement of relative aortic size predicts rupture of thoracic aortic aneurysms. Ann Thorac Surg. 2006;81:169–177
- Heart transplantation in patients with Marfan's syndrome: a survey of attitudes and results. J Heart Lung Transplant. 1994;13:899–904
- Combined heart transplantation and resection of dissecting aneurysm of ascending aorta and aortic arch: a case report. Ann Thorac Cardiovasc Surg. 2000;6:61–64
- . Hypothermic cardiopulmonary bypass and circulatory arrest for operations on the descending thoracic and thoracoabdominal aorta. Ann Thorac Surg. 2002;74:S1885–S1887
- . Cerebrospinal fluid drainage reduces paraplegia after thoracoabdominal aortic aneurysm repair: results of a randomized clinical trial. J Vasc Surg. 2002;35:631–639
PII: S1533-3167(06)00081-1
doi:10.1016/j.ejvsextra.2006.10.001
© 2006 Elsevier Ltd. All rights reserved.
Refers to article:
- Total Thoracoabdominal Aortic Replacement in a Marfan Patient Following Heart Transplantation and Hemiarch Repair , 17 November 2006
