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Ann Thorac Surg 2001;72:1502-1507
© 2001 The Society of Thoracic Surgeons
a Department of Cardiac Surgery, The General Hospital, Southampton, United Kingdom
Accepted for publication June 11, 2001.
* Address reprint requests to Mr Livesey, Department of Cardiac Surgery, The General Hospital, Tremona Rd, Southampton SO16 6YD, UK
e-mail: steve.livesey{at}suht.swest.nhs.uk
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Abstract |
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Methods. A total of 65 consecutive patients with Marfan’s syndrome (mean age 41.7 ± 10.7 years, range 15 to 76 years) undergoing ARR between 1972 and 1998 in Southampton were studied. Of the patients, 45 had a chronic aneurysm of the ascending aorta and 20 had a type A dissection (16 acute and 4 chronic). The operations were elective in 38 and nonelective in 27 cases (emergency in 22 and urgent in 5). Mean size of the ascending aorta was 6.3 ± 1.4 cm (3.8 to 12 cm). A Bentall procedure was performed in 62 and a homograft root replacement in 3 patients. Mean follow-up was 8 ± 4.1 years (0 to 22.9 years).
Results. Operative mortality was 6.1% (4 deaths) (for the elective vs nonelective procedures it was 2.6% vs 11%, p = 0.2). The 10-year freedom from thromboembolism, hemorrhage, and endocarditis was 88%, 89.8%, and 98.4% (0.9%, 0.9%, and 0.2% per patient-year) and from late aortic events it was 86.3% (1.3% per patient-year). Aortic root replacement for dissection was an independent predictor of occurrence of late aortic events (p = 0.01). Five patients had a reoperation with one early death. The 10-year freedom from reoperation was 89.2% (1.1% per patient year) (for elective and nonelective procedures, 90.8% vs 84.6%, p = 0.6). The 10-year survival, including operative mortality, was 72.7% (for elective and nonelective procedures, 78% vs 66.5%, p = 0.6). Late aortic events was an independent adverse predictor of survival (p = 0.02).
Conclusions. In patients with Marfan’s syndrome, elective ARR, usually for chronic aneurysm, is associated with a low mortality, low rate of aortic complications, and good late survival. Nonelective ARR, mostly for dissection, has a greater operative risk and a significantly higher incidence of late catastrophic aortic events. Early prophylactic surgery in these patients is therefore recommended. Long-term clinical and radiologic follow-up to prevent or to treat late aortic events is highly desirable.
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Introduction |
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The prognosis of Marfan’s patients before the introduction of the technique for replacement of the aortic root with a composite graft–valve conduit by Bentall and DeBono in 1968 [8] was disappointing, and premature death was common [3, 7, 9]. The adoption of this technique, the appreciation of the hemodynamic benefits of the life-long administration of ß-blockers, and refinements in the quality as well as increased better availability of diagnostic imaging modalities have transformed the outlook for these patients. In 1972 their median life expectancy was 45 years; by 1995 it had risen to 72 years [3, 7, 9, 10].
In this article we describe our 26-year experience with surgical treatment of patients with Marfan’s syndrome involving the aortic root and ascending aorta. Emphasis is placed in delineating the impact of the degree of urgency of the operation and of aortic pathology on the early and late outcome.
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Material and methods |
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Definitions
Nonelective procedures include both emergency and urgent operations on the basis of the similar clinical outcome of these patients. In particular, a procedure was defined as emergency if an aortic root replacement was performed within 24 hours after an acute hospital admission. If it was done within the same hospital admission but after the first 24 hours, the procedure was defined as urgent.
Operative mortality includes death within 30 days of the operation or during the same hospital admission. Complications occurring more than 30 days postoperatively and after patient discharge from the hospital are defined as late events. "Late aortic events" include the development of aortic dissection, aortic rupture, or formation of a fistula between the aorta and another organ.
Diagnosis, clinical, and pathologic features
Diagnosis was made by means of a combination of echocardiography, aortography, and computed tomographic and magnetic resonance imaging, depending on the degree of urgency and local availability. In 7 patients who presented with acute dissection the diagnosis was based solely on the preoperative echocardiography. At presentation 31 patients were in New York Heart Association (NYHA) functional class III or IV, 58 had aortic regurgitation, and 22 had impaired left ventricular function on angiography or echocardiography. Indications for surgery were chronic aneurysm in 45 and type A aortic dissection in 20 patients (acute in 16 and chronic in 4). The mean size of the ascending aorta was 6.6 ± 1.4 cm (range 3.8 to 12 cm). For patients with a dissection this was 6 ± 1.3 and for those with a chronic aneurysm it was 6.5 ± 1.3 (p = 0.4). Three patients had undergone previous aortic valve replacement (1 of them in our unit) and 2 patients had undergone aortic root replacement elsewhere. The operations were elective in 38 and non-elective in 27 cases (emergency in 22 and urgent in 5).
Operative technique
Access was obtained through a median sternotomy. The femoral artery was cannulated in 56 and the ascending aorta in 9 patients. Venous return was accomplished with a two-stage right atrial–inferior vena caval or bicaval cannulation in 60 and femoral venous cannulation in 5 patients. Moderate hypothermia (28°C) was used in 60 and total hypothermic circulatory arrest (18°C) in 5 patients. Hypothermic temperatures were measured with a nasopharyngeal probe. Myocardial protection was provided with cold crystalloid and, more recently, with blood cardioplegia and topical hypothermia.
A modified Bentall procedure (aortic valve and root replacement with a composite valve–graft conduit and reimplantation of the coronary arteries using the button technique) was performed in 56 patients and an original Bentall procedure [8] in 6 patients. During the 1970s and 1980s plain, blood-preclotted, and albumin-baked Dacron (C. R. Bard, Haverhill, PA) conduits were used, often loosely wrapped with the aneurysmal sac to facilitate hemostasis. More recently the use of gelatin-impregnated woven polyester Dacron composite graft valves became our standard practice.
A Dacron conduit with a prosthesis of choice was used in 44 patients and a woven polyester ultra–low porosity conduit (Carboseal; Sulzer Carbomedics, Inc, Austin, TX) in 18 patients. Valves included the single-disk Björk-Shiley in 36, the bileaflet Carbomedics in 18, and St. Jude Medical in 3, and the cage-ball Starr-Edwards in 5 patients. Homograft aortic root replacement with coronary ostial reimplantation was performed in 3 patients in whom the administration of anticoagulants was contraindicated.
Gelatin-resorcin-formaldehyde tissue glue was used in 21 patients. We now use tissue glues in all patients with acute aortic dissection to facilitate apposition of layers of the dissected aorta at the distal anastomosis and in other cases to cover the anastomotic suture lines. Aprotinin was used in 13 patients. Teflon felt for reconstruction of the aorta or the aorto-coronary anastomosis was used according to the individual surgeon’s preference. A cell-saving device was used intermittently over the study period. Currently it is increasingly employed as a means of reducing the need for perioperative blood transfusion.
The mean aortic cross clamp time was 91.3 ± 18 minutes (range 57 to 152 minutes) and the mean bypass time was 120.3 ± 27 minutes (range 65 to 298 minutes). Additional procedures were performed in 8 patients: aortic arch replacement in 3, coronary artery bypass grafting in 2, mitral valve replacement in 1, mitral valve repair in 1, and resection of a retrosternal goiter in 1 patient. Postoperatively, all patients with the exception of those having a homograft root replacement, received anticoagulation treatment with warfarin. The targeted levels of International Normalized Ratio varied over the study period. Our recommended rate is currently between 3 and 4.5.
Follow-up
Patients were seen in the outpatient clinics regularly. Clinical examination, chest radiography, electrocardiography, and more recently echocardiography were routinely done. Before 1995 the use of computed tomography or magnetic resonance imaging was at the discretion of the attending physician. Since 1995 all Marfan’s patients have had a magnetic resonance imaging scan 3, 6, and 18 months postoperatively and either annually or biannually thereafter. Data was obtained through a detailed review of the hospital medical records. Additional information was sought from the referring physicians, family doctors, and patients’ families as appropriate. The mean follow up was 8 ± 4.1 years (range 0 to 22.9 years), with a total of 519 patient-years, and was complete within 1 year of the end of the study period.
Statistical analysis
Continuous data are presented as means (± standard deviation) and categorical variables as percentages. A total of 26 variables1 were tested with univariate analysis, with end points being operative mortality, late valve-related events (thromboembolism, bleeding, endocarditis), late aortic events, reoperation, and survival. Continuous variables were screened with logistic regression. Means were compared with the student t test and proportions with 
2 or Fisher’s exact test as appropriate. The prediction of freedom from late events (± standard error from the mean) was estimated with the Kaplan-Meier product limit method and the resulting curves compared with the log-rank test. The variables that attained a p value of less than or equal to 1.0 on univariate analysis were entered into multiple logistic stepwise regression analysis and Cox proportional hazards regression models. A p value of less than 0.05 was considered significant. Statistical analysis was done using the statistical package SPSS PC, version 8.0 (SPSS, Chicago, IL).
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Results |
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Fourteen patients (21.5%) sustained significant early postoperative complications. These were pneumonia in 2, respiratory failure in 2, bleeding requiring reopening in 4, pericardial effusion treated with open drainage in 2, recurrent left laryngeal nerve palsy in 1 patient who underwent concomitant thyroidectomy for retrosternal goiter, complete heart block necessitating the insertion of a permanent pacemaker in 1 patient, sternal dehiscence in 1, and deep sternal wound infection in 1. The mean blood loss was 697 ± 293 mL (range 100 to 2100 mL). The mean intensive care unit stay was 1.98 ± 1.18 days (range 1 to 17 days) and the mean hospital stay was 13.6 ± 5.8 days (range 1 to 70 days).
Late complications
Late valve or anticoagulation treatment-related complications occurred in 11 patients. Five patients, all of whom had received a single-disk (Björk-Shiley) valve, experienced thromboembolic events. Four of these patients had transient ischemic attacks, and 1 had a stroke resulting in left-sided weakness from which he recovered. Four patients were admitted to the hospital with hematuria and 1 patient with idiopathic thrombocytopenic purpura developed extensive gastric erosions and fatal gastric bleeding. Prosthetic valve endocarditis occurred in 1 patient and was managed successfully with homograft aortic root replacement.
Kaplan-Meier 10-year freedom from thromboembolism, bleeding, and prosthetic valve endocarditis were 88% ± 5.1%, 89.8% ± 4.8%, and 98.4% ± 1.6% (linearized rates 0.9%, 0.9%, and 0.2% per patient-year). Kaplan-Meier 10-year freedom from any valve-related complication (thromboembolism, bleeding, and endocarditis) was 81.2% ± 6.1% (2.1% per patient-year) (Fig 1). Poor preoperative left ventricular function was a significant factor for thromboembolism on univariate (p < 0.0001) and multivariate (p = 0.03) analysis. The differences in the actuarial estimates of thromboembolism between the patients receiving a Björk-Shiley valve versus the remaining patients (10-year freedom 83.3% ± 6.8% vs 100%, linearized rate 1.1% vs 0% per patient-year) were not significant (p = 0.09).
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Kaplan-Meier survival, including operative mortality, at 5 and 10 years was 85.5% ± 4.5% and 72.7% ± 6.5% (Fig 4). Kaplan-Meier survival free of life-threatening complications (thromboembolism, major bleeding, endocarditis, and aortic events) at 5 and 10 years was 80.5% ± 5.1% and 60% ± 7.6% (Fig 4). Ten-year survival for the patients undergoing elective versus nonelective procedures was 78% ± 8.3% vs 66.3% ± 10% (p = 0.3) and for the patients having chronic aneurysm vs type A dissection it was 79.5% ± 7.4% vs 60% ± 12.1% (p = 0.4) (Fig 5).
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Comment |
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TopAbstractIntroductionMaterial and methodsResultsCommentFootnotesAcknowledgmentsReferences |
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The 2.6% operative mortality for elective aortic root replacement (1 death due to cerebral bleed) in this series was low, comparable with 30-day mortality rates of 0% and 1.5% previously quoted for elective ARR for Marfan’s syndrome [9, 12]. Operative mortality was higher after nonelective surgery, mostly for aortic dissection, at 11%. This is in agreement with previous reports [9, 12], and it is quite acceptable considering that these patients without surgical intervention would face an almost certain death [2, 7, 15].
Bleeding episodes and thromboembolism were uncommon and were caused by difficulties in maintaining the desired level of international normalized ratio. The linearized rate of thromboembolism, 0.9% per patient-year, was low and compares favorably with a linearized rate of 1.6% for a large group of patients undergoing isolated aortic valve replacement in this unit [16]. Gott and colleagues [9] described a similar experience following ARR in Marfan’s patients—a thromboembolic rate of 0.62%, which was about half of that after isolated aortic valve replacement at Johns Hopkins Hospital. When combined these findings indicate that patients receiving composite grafts may be less at risk for thromboembolism than those with isolated aortic valves, possibly due to exclusion of valve sutures and pledgets from the circulation in the composite graft group [9]. We have not observed thromboembolic events among the patients with a composite graft and bileaflet valve over the last 8 years of this study. This suggests that thromboembolism with modern composite conduits may be even lower. In a combined study from Southampton and Birmingham, there has been no thromboembolic event among 143 patients undergoing ARR for a variety of disease etiologies with a composite (Carboseal; Sulzer Carbomedics Inc, Austin, TX) conduit, at a mean follow-up of 24 months [17].
The prevalence of prosthetic endocarditis, 0.2% per patient-year (10-year freedom of 98%) was gratifyingly low. The only patient affected was treated successfully with homograft aortic root replacement, the procedure of our choice for infected composite grafts [18]. These figures compare well with a 10-year freedom from endocarditis of 91% and 95% reported in other studies [9, 12].
The freedom from reoperation (89.2% at 10 years, 5 patients) and the accompanying operative risk, 1 death in a patient with extensive dissection of his thoracoabdominal aorta, were acceptable. Three other patients, however, died from late aortic complications before they were offered surgery. Late aortic complications were more prevalent among patients with aortic dissection at the original operation than in those with a chronic aneurysm (probability of their presence at 10 years 26% vs 8%). These complications had a major negative impact on the outcome, increasing significantly the likelihood of reoperation and late death. Finkbohner and colleagues [19], in a long-term follow-up study found that more than half of their patients required further aortic operations. Their experience and ours remind us of the need for long-term clinical and radiologic follow-up of these patients.
Late survival for the patients undergoing elective repair for chronic aneurysm was good, 79.5% at 10 years. Survival in the dissection group was somewhat lower, 60% at 10 years, but still satisfactory; natural history studies have shown that more than 90% of patients with Marfan’s syndrome will have died within weeks after they have sustained an aortic dissection if left untreated [15]. Similar survival rates for elective and nonelective ARR have also been reported in a multicenter study involving 10 North American and European units with expertise in the management of Marfan’s syndrome [12].
Aortic valve sparing procedures [20, 21] have emerged as an alternative to composite valve-graft aortic root replacement, and proponents emphasize their merits in avoiding the need for anticoagulation and late valve-related complications. Birks and colleagues, Harringer and colleagues, and David [22–24] reported good early or late results in series of patients undergoing such operations, although in some of these series there was a considerable incidence of mild–moderate aortic regurgitation and some patients needed an aortic valve replacement for progressive regurgitation. Despite these good results, there remain concerns regarding the late fate of a structurally abnormal aortic valve, that is defective in fibrillin [4–6, 25] and suspended within a synthetic graft. The reliability and reproducibility of the Bentall procedure and the low complication rate observed with the modern prosthetic devices make the composite valve-graft root replacement the most preferred option [12, 26]. This is the operation of choice for the management of Marfan’s patients in our unit. We appreciate that certain subsets of patients—namely children, women of childbearing age, and patients in whom administration of anticoagulants is contraindicated—may benefit from a valve-sparing procedure, although homograft root replacement would be another option.
The decision as to whom should be offered prophylactic surgery remains a difficult problem. The current recommendation is to operate when the size of an aortic root is about 5.5 to 6 cm [12], and is based mainly on work done with non-Marfan’s patients [27]. It is, however, common knowledge that some patients experience catastrophic aortic events even though they have aortic aneurysm sizes of less than 5 cm in diameter. Four of the patients who presented with aortic dissection or rupture in our series had aneurysms less than 5 cm, the smallest being 3.8 cm. Ongoing work aiming to construct a computerized model, to more accurately quantify the risk of subsequent rupture or dissection in Marfan’s patients and to better guide the decision-making process as to their management, is certainly of great interest [28].
In agreement with others [29], we offer prophylactic surgery to all Marfan’s patients having an aneurysm of 5 cm in diameter, and consider surgery at even smaller sizes if there are additional risk factors. These include a progressive dilatation of the size of the aneurysm, a family history of aortic dissection or rupture and aortic regurgitation. We administer life-long ß-blockers due to their beneficial effects in reducing the aortic wall stress and the cardiac workload [30], and, from 1995, follow-up these patients for indefinitely with serial magnetic resonance imaging scans.
In conclusion, in patients with Marfan’s syndrome elective aortic root replacement, usually for chronic aneurysm, is associated with a low operative mortality, low rate of aortic complications, and good late survival. Nonelective aortic root replacement, mostly for aortic dissection, has a greater operative risk and a significantly higher incidence of late catastrophic aortic events. Early prophylactic surgery in these patients is therefore recommended. Long-term clinical and radiologic follow-up to prevent or treat late aortic events is highly desirable.
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