Asian Cardiovasc Thorac Ann 2003;11:337-341
© 2003 Asia Publishing EXchange Ltd
Extensive Aortic Surgery in Marfan Syndrome: 16-Year Experience
Kaan Kirali, MD,
Vedat Erentu
, MD,
Murat B Rabu
, MD,
Akin 
zgi, MD1,
Nilgün U Bozbua, MD,
Korhan Erkanl
, MD,
Esat Aknc, MD,
Cevat Yakut, MD
Department of Cardiovascular Surgery
1 Department of Cardiology, Kouyolu Heart and Research Hospital, Istanbul, Turkey
For reprint information contact: Kaan Kirali, MD Tel: 90 216 326 6969 Fax: 90 216 339 0441 email: imkkirali{at}yahoo.com Department of Cardiovascular Surgery, Kouyolu Heart and Research Hospital, Kadiköy 81020, Istanbul, Turkey.
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ABSTRACT
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The aim of this study was to evaluate the clinical outcome of surgical treatment in patients with Marfan syndrome. Between 1985 and November 2001, 33 patients with Marfan syndrome were operated for chronic aneurysm of the aortic root with involvement of the ascending aorta in 20 patients and type A dissection in 13 patients. The patients comprised 24 males and 9 females with a mean age of 31.9 ± 9.7 years (range, 18 to 54 years). The mean diameter of the ascending aorta was 6.6 ± 1.6 cm and that of the aortic root was 5.4 ± 1.2 cm. Hemodynamic instability was observed in 11 patients. The aortic arch was replaced in 7 patients. There was no hospital mortality. Late mortality was 6%, involving 2 patients who had aortic valve replacement. Actuarial freedom from death was 92.3% ± 7.4% at 12 years and from late aortic complications was 86.4% ± 9.4% at 13 years. Aortic aneurysm was a significant univariate adverse factor for late aortic complications. Aortic surgery can be performed in Marfan patients with low morbidity and mortality. Aggressive surgical intervention does not impair surgical outcome while it decreases reoperation risk.
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INTRODUCTION
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In the last decade, the etiopathogenesis and genetic abnormality of Marfan syndrome have become well established.1 This disorder affects various connective tissue systems (skeletal, ocular, cardiovascular), but the first symptoms are usually related to aortic aneurysm or acute aortic dissection.2,3 These complications are the main causes of early or late morbidity and mortality, and they reduce life expectancy if they are not surgically corrected.4 Multiple segments of the aorta can be involved, necessitating staged replacement of the entire aorta.5 Most patients would not survive beyond 30 to 40 years of age before the advent of modern cardiovascular surgery, but life expectancy has risen to the 70s in the last decade,2,5–7 thanks mainly to improvement in operative techniques, anesthetic care, perioperative management, and surgical repair of aortic pathology.
This study is a review of our experience with different surgical approaches involving the ascending aorta and, in some cases, the aortic arch in patients with Marfan syndrome.
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PATIENTS AND METHODS
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From 1985 to November 2001, 33 patients with Marfan syndrome, diagnosed according to the revised diagnostic criteria for the disease,8 underwent surgical reconstruction of the ascending aorta and, in some cases, the aortic arch. Four patients requiring cardiopulmonary resuscitation, who were transferred from other centers to our hospital, were excluded from the study because they died either in the intensive care unit or after emergency operation. The study group consisted of 24 males (72.7%) and 9 females (27.3%) with a mean age of 31.9 ± 9.7 years (range, 18 to 54 years). Two patients were siblings. The most common symptoms were dyspnea (42.4%), palpitation (39.4%), and chest pain (27.3%). Hemodynamic instability was observed in 11 patients (33.3%) with aortic dissection. At presentation, 11 patients were in New York Heart Association functional class III or IV, 18 were in class II, and 4 in class I. Diagnosis was made by means of a combination of transthoracic echocardiography (78.8%), transesophageal echocardiography (22.2%), computed tomography (15.1%), and magnetic resonance imaging (12.1%). The echocardiographic results are listed in Table 1
. Five patients (15.2%) over 45 years of age underwent coronary angiographic and aortographic evaluation for coronary artery disease. Surgical indications were chronic aneurysm of the aortic root with involvement of the ascending aorta in 20 patients (60.6%) and type A dissection in 13 patients (39.4%). The operation was performed electively in 30 patients (90.9%) and emergently in 3 (9.1%).
A standard median sternotomy was made. For cardiopulmonary bypass (CPB), femoral arterial cannulation was employed in patients with aortic dissection and/or aneurysm extending to within 2 cm of the innominate artery; in the other patients, the distal ascending aorta was cannulated. Venous return was accomplished with a two-stage right atrial–inferior vena caval cannulation, or bicaval cannulation when retrograde cerebral perfusion (RCP) was used. When the posterior portion of the sternum was intimately involved in the anterior wall of the pseudoaneurysmal sac resulting from dissection, and hence there was a risk of incision into the aorta upon sternotomy, femorofemoral cannulation and systemic cooling were instituted before sternotomy. Myocardial protection was achieved with antegrade cold crystalloid cardioplegia and topical hypothermia, which was replaced by continuous retrograde isothermic blood cardioplegia after 1993.
Extensive reconstruction included replacement of the aortic root or the arch or both. After the ascending aorta was completely transected at the sinotubular junction and distally 2 cm from the crossclamp, the aortic valve and annulus were examined to see if the valve could be preserved. Six patients (18.2%) with normal aortic cusps underwent isolated ascending aorta replacement with a supravalvular tube graft, and the proximal anastomosis of the graft was reinforced with continuous suture buttressed by an outer band of Teflon felt.9 In cases where the aortic root was to be replaced with a composite graft, the flanged technique that we modified from the Bentall procedure was used.10 If the ascending aorta was cannulated in isolated ascending aortic aneurysm, we performed closed anastomosis of the composite graft to the distal ascending aorta without removing the crossclamp and with the patient under deep hypothermic circulatory arrest (DHCA). If the ascending aortic aneurysm extended to the aortic arch and femoral arterial cannulation had to be employed, we performed open anastomosis of the composite graft to the proximal aortic arch under DHCA after completing ascending aorta replacement.
The extent of aortic replacement was principally determined by the location of the entry tear in aortic dissection and the extent of the dissection. Seven patients (21.2%) underwent aortic arch replacement. Where aortic dissection extended to the arch, femoral arterial cannulation with DHCA was used. During cooling, the ascending aorta was resected and prepared for replacement. When the patient’s body temperature fell below 18°C, CPB was suspended and the crossclamp was opened to inspect the inside of the aortic arch and descending aorta for a second or extended intimal tear. Patients with arch dissection underwent graft replacement of all of the arch together with an island of aortic tissue containing the brachiocephalic vessels, after distal anastomosis of an elephant trunk graft to the proximal descending aorta had been performed.11 After the arch had been replaced with a tubular graft, an arterial cannula was inserted into the graft and a crossclamp was applied to reestablish antegrade arterial perfusion. Patients with only the proximal segment of the lesser curvature of the arch affected had transection of the arch at the level of the innominate artery along the lesser curvature to permit a hemiarch replacement. In hemiarch replacement, the arch tissue usually would be strengthened with continuous over-and-over suture before the graft was anastomosed to the arch via a Teflon felt reinforcement on the suture line. RCP was established during DHCA via the superior vena caval cannula at a flow rate of 300 mL•min-1 or less and with the proximal venous pressure at approximately 25 mm Hg to prevent cerebral edema.12
The surgical procedures performed are given in Table 2. Mean aortic crossclamp time was 90.4 ± 40.0 minutes (range, 42 to 203 minutes), mean CPB time was 165.9 ± 42.2 minutes (range, 100 to 290 minutes), and mean operation time was 4.8 ± 1.2 hours (range, 3 to 8.5 hours). The degree of hypothermia applied depended on the surgical procedure. Mean body temperature during hypothermia, which was measured with a rectal probe, was 21.6°C ± 3.8°C (range, 16°C to 30°C) in all the study patients, 22.4°C ± 3.8°C (range, 17°C to 30°C) in patients who received only ascending aorta replacement, and 18.7°C ± 1.6°C (range, 16°C to 21°C) in patients who had arch repair. Mean RCP time was 44.2 ± 32.5 minutes (range, 6 to 87 minutes).
Postoperatively, all patients who underwent aortic valve replacement received anticoagulation with warfarin to maintain an international normalized ratio of 2.5 to 3.5. Doppler echocardiography and computed tomography were done in all patients annually.
Follow-up data were collected by interviewing the patients and from hospital records. Follow-up was completed in all the patients. The patients had been followed up for 4.5 ± 3.5 years (range, 0.4 to 14.5 years), totaling 153.5 patient-years.
Continuous data are expressed as mean ± standard deviation, and categorical variables as percentages. Statistical analyses were computed using SPSS PC version 10.0 (SPSS, Inc., Chicago, IL, USA) on several variables: age, sex, aortic pathology (aneurysm or dissection), urgency of operation (elective or nonelective), hemodynamic instability (need for inotropic and vasodilator support to maintain hemodynamics), associated diseases (chronic obstructive pulmonary disease, hypertension, mitral insufficiency), concomitant procedures (replacement of the aortic valve, aortic arch, or mitral valve), aortic crossclamp time, CPB time, use of RCP, postoperative complications (renal, pulmonary, abnormal heart rhythm, infection), late aortic complications, and mitral valve reoperation. Univariate and multivariate analyses were used to assess risk factors as independent predictors of early and late events. Cox proportional hazards regression analysis was used to assess risk factors as independent predictors of patient survival and late aortic complications. Differences between categorical variables and between continuous variables were tested using the chi-squared test and the Student’s t test, respectively. Survival curves were constructed by the Kaplan-Meier method. Results are calculated as mean ± standard error. The log rank test for independent groups was used to test the significance of differences. Statistical significance is defined as p < 0.05.
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RESULTS
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Early mortality or morbidity is defined as death or complications occurring within 30 days of operation. There was no early mortality, but 11 patients (33.3%) had 1 or more significant hospital complications. The complications were fever in 5 patients, arrhythmia in 4, atelectasis in 2, pleural effusion in 3, transient renal insufficiency in 2, transient cerebral events in 2, low cardiac output syndrome in 1, pericardial effusion requiring drainage in 1, and deep sternal wound infection in 1 patient. Permanent complete heart block occurred in 2 patients, and 1 of them received a permanent pacemaker.
The late mortality rate was 6%, involving 2 patients who had aortic valve replacement. One patient died from congestive heart failure 5 years after the operation, and the other died 13 years after the operation, probably due to rupture of a thoracic dissecting aneurysm. There were no significant univariate and multivariate adverse predictors of survival. Cumulative survival was 92.3% ± 7.4% at 12 years and 46.2% ± 32.8% at 13 years (Figure 1). There were no significant differences in survival rates between patient subgroups when we compared patients with type A dissection against those with chronic aneurysm (12-year survival, 100% and 83.3% ± 15.2%, respectively); patients with stable hemodynamics against those with unstable hemodynamics (8-year survival, 87.5% ± 11.7% and 100%); patients who had aortic valve replacement against those who did not (8-year survival, 88.9% ± 10.5% and 100%); patients who had arch replacement against those who did not (8-year survival, 100% and 87.5% ± 11.7%); and patients who had mitral valve surgery against those who did not have mitral valve disease (6-year survival, 100% and 91.7% ± 8.0%).
There was no reoperation for aortic pathology, but 1 patient was reoperated for severe mitral insufficiency 6 months after the first operation, which had involved bilateral Kay annuloplasty. During reoperation, annular dilatation was found to have recurred; early postoperative echocardiography had shown mild mitral regurgitation. The mitral valve was resected and replaced with a mechanical prosthesis. Actuarial freedom from mitral valve intervention in this series was 96.0% ± 3.9% at 13.6 years.
The only late valve-related complication was infective endocarditis in a male patient 2 years postoperatively. He healed with antibiotic therapy and no reoperation was needed. Late aortic complications occurred in 2 patients. One patient, who had undergone ascending aorta replacement to treat an aneurysm, developed descending aorta aneurysm. The other patient, who had undergone replacement of the aortic valve and root because of acute type A aortic dissection, had a pseudoaneurysm on the dissected aortic arch and descending aorta. Freedom from late aortic complications in this series was 95.0% ± 4.9% at 4 years and 86.4% ± 9.4% at 13 years. Ascending aortic aneurysm was a univariate adverse predictor for the development of late aortic complications (p < 0.03), but Cox proportional hazards regression analysis was not significant for this variable.
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DISCUSSION
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The choice of cardiac surgical treatment is based on the pathology of the aortic root, the severity of aortic regurgitation, and the left ventricular function. The same is largely true for treatment of mitral valve disease in Marfan patients.13 Standard surgical treatment in these patients is total aortic root replacement with a composite valve graft. In dissection, conservative replacement of only as much of the aorta as is deemed necessary must be performed. However, conservative therapy with glue repair or valve resuspension is unacceptable because failure to exclude the aortic sinuses results in aneurysmal dilatation and the need for early reoperation. The combined effect of aortic root pathology and histologic changes can cause aortic valve disease. Increasing aortic root dilatation and elastic modulus to simulate Marfan syndrome in experiments significantly increased leaflet stress and strain and reduced coaptation in an otherwise normal aortic valve.14 If Marfan patients with borderline dilated ascending aorta are diagnosed early and are asymptomatic, they should be scheduled for an elective prophylactic operation, particularly because surgery is all curative and valve-sparing procedures are available.15–19 Valve-sparing surgery is popular, but caution is warranted because of the potential for progressive annular dilatation and aortic valve degeneration in Marfan patients. In patients with advancing root dilatation, the aortic valve leaflets might not be normal and may not benefit from valve-sparing surgery. We usually perform a supracommissural graft interposition when a Marfan patient is found to have a normal aortic root and type A aortic dissection without involvement of the aortic root. We have not observed any late aortic root complications in these patients during follow-up, but these patients are being followed closely for such complications.
Our series included arch replacement as a concomitant procedure in 21.2% of patients, compared with reported rates of between 1.3% and 4.6%.6,19 This higher incidence may be due to the higher number of patients with acute aortic dissection. About 40% of the patients in our series suffered acute dissection, while the incidence reported in the studies noted above was 23% to 30.7%. Nonelective surgical intervention is the rule in Marfan patients if dissection develops. In spite of hemodynamic instability being the major cause of pre- and postoperative complications in patients with aortic dissection, which could impair early outcome, none of these patients experienced worse early or late outcome compared with the other patients in this study. Successful combined replacement of the ascending aorta and the arch is considered to be the most important factor responsible for the dramatic improvement in the prognosis of these patients. We believe that concomitant arch replacement and the use of RCP produce better outcome in patients with arch dissection.9–11
Aortic surgery can be performed in Marfan patients with low morbidity and mortality. Early mortality ranges between 0% and 2.6% for Marfan patients undergoing elective surgery, but it is much higher (5.6% to 11%) in patients requiring emergency operation.6,20 Our results for elective surgery were comparable to those of other studies. In our study, 11 patients with type A aortic dissection had unstable hemodynamics, but none of them died and the preoperative emergency situation did not have any adverse effect on late outcome. Late mortality was mostly due to late cardiovascular complications, especially new aneurysm or dissection in the rest of the thoracic aorta.
We conclude that aggressive surgical intervention does not impair surgical outcome while decreasing reoperation risk in Marfan patients. We believe that surgical success is dependent on early diagnosis and surgical repair before serious complications develop.
Presented at the 10th Annual Meeting of the Asian Society for Cardiovascular Surgery, Jeju Island, Korea, April 17–19, 2002, and the 8th Aortic Surgery Symposium, New York, USA, May 2–3, 2002.
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ABSTRACT
INTRODUCTION
