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Eur J Cardiothorac Surg 1998;13:416-423
© 1998 Elsevier Science NL

Long-term prognosis of surgically-treated aortic aneurysms and dissections in patients with and without Marfan syndrome

^a Department of Cardiac Surgery, University Hospital Großhadern, Ludwig-Maximilian-University, Marchioninistraße 15, 81366 Munich, Germany
^b Department of Clinical Chemistry, University Hospital Großhadern, Ludwig-Maximilian-University, Marchioninistraße 15, 81366 Munich, Germany

Received 25 August 1997; received in revised form 2 February 1998; accepted 10 February 1998.

Corresponding author. Present address: Department of Thoracic and Cardiovascular Surgery, Johann Wolfgang Goethe University, Theodor Stern-Kai 7, 60590 Frankfurt, Germany. Tel.: +49 69 63015850; fax.+49 69 63015849.

	Abstract

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Objective: Aortic aneurysms and dissections are the leading causes of premature death in Marfan syndrome (MfS). This study aims to compare long-term results of surgically treated aortic aneurysms and dissections in patients with and without MfS in respect to early and late prognosis. Methods: From March 1975 to August 1994, 33 patients with classic MfS (group A, age 34.2±9 years) and 298 patients with non-fibrillinopathic aortic disease (group B, age 54±13 years) underwent aortic surgery. Acute dissections occurred in 57.6 (A) versus 37.9% (B). A total of 54.6% of patients in group A were treated with a composite graft versus 16.4% in B. The aortic arch and the descending aorta was replaced in 30.4% of MfS patients and 24.9% of patients without MfS. Results: We observed 7 (25.0%, A) versus 35 (14.2%, B) late deaths among the 28 (A) versus 247 (B) early survivors. In 5 patients (17.9%) of A and 8 patients (3.2%) of B, late death was caused by redissection or recurrent aneurysm (P<0.001). Long-term survival after 5, 10 and 15 years in group A was 82±7, 60±11 and 30±22%, and 75±3, 69±3 and 64±4% in group B. A total of 22 reoperations were performed in 11 MfS patients, 17 reoperations were due to recurrent aortic diseases. Three of the 8 patients underwent reoperation after Wheat procedure because of sinus valsalva aneurysm. None of the patients with composite graft replacement needed reoperation in this segment, but 3 patients suffered from redissection at the proximal aortic arch. In group B, reoperations were significantly less frequent (10.7%) compared to MfS patients (66.7%; P<0.001). Conclusions: Surgical treatment of aortic disease in MfS patients is associated with a high risk of redissection and recurrent aneurysm. If the ascending aorta needs to be replaced, we recommend the composite graft technique and a more aggressive approach to reduce the frequency of distal reoperations. In order to reduce the high reoperation rate in MfS patients, frequent clinical follow-up may contribute to improve life expectancy in MfS patients.

Key Words: Marfan syndrome • Aortic aneurysm • Redissection • Fibrillin • Connective tissue

	Introduction

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Marfan syndrome (MfS) is an autosomal dominant inherited connective tissue disorder with variable phenotypic expression of cardiovascular, ocular and musculoskeletal manifestations [1]. The estimated prevalence of MfS is one in 5000, of which at least one third occur in the absence of a family history and are thought to be due to sporadic mutations of the MfS-linked gene locus. MfS is caused by abnormalities in the production of fibrillin, a 350 kD glycoprotein, which represents the major structural component of connective tissue microfibrils [2]. Using the positional cloning approach, genetic linkage studies [3] [4] [5] and chromosomal in situ-hybridization experiments [6] [7] led to identification of a gene locus on chromosome 15 [8], encompassing the gene for fibrillin 1 (FBN1). Up to now, more than 70 mutations in the FBN 1 gene have been described in association with MfS.

Among the multiple clinical manifestations of MfS, involvement of the cardiovascular system such as dilatation, rupture and dissection of the aorta are the leading cause of premature death in these patients [1] [10]. The average life expectancy of patients with MfS without surgical treatment is approximately 32 years [11]. Operative therapy of thoracic aortic aneurysms and dissections are still representing a major surgical challenge associated with a high perioperative mortality. Due to the progress of the dissection or aneurysmal dilatation, which is frequently associated with aortic rupture, the late mortality in these patients is high, even after surgical treatment of aortic dissection [12].

The current study evaluates long-term results of surgical treatment of aortic aneurysms and dissections in 331 patients, considering the particular situation encountered in MfS.

	Materials and methods

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Between March 1975 and August 1994, 331 patients were operated on for aneurysms or dissections of the thoracic aorta at the Department of Cardiac Surgery at the University Hospital Großhadern, Munich, Germany.

The clinical phenotype based on standard diagnostic criteria [13] and pedigree analysis were applied to identify 33 patients with classical features of MfS (group A). The majority of patients (n=298, group B) presented with non-fibrillinopathic etiologies of aortic disease, among which cystic medianecrosis represented the most common (58%) and atherosclerosis the second most common finding (38%), whereas aortitis (0.7%), lues (1.3%) and trauma (2%) were less frequently diagnosed.

The surgical records were retrospectively reviewed. Survival and follow-up information was obtained by telephone interview or correspondence with the patients and their family practitioners, followed by a detailed examination in the hospital. Information concerning aortic dissection or dilatation was obtained from preoperative and postoperative aortic imaging studies. All living Marfan patients were seen at least annually between 1994 and 1997 in order to review their current status. The follow-up included a clinical examination, transesophageal echocardiography (TEE), spiral computed tomography (Spiral-CT) or magnetic resonance imaging (MRI). Follow-up data were available in all patients, representing 199 patient years in group A and 1726 patient years in group B. The mean follow-up time in group A was 6.0±4.4 (range 0–16.6) years, in group B 5.8±4.9 (0–20.2) years.

Preoperative characteristics
Out of the 33 patients with MfS, 23 were male and 10 female. There was no difference in gender distribution between MfS patients and not MfS related patients (220 male and 78 female). The mean age at the time of first surgical intervention in MfS was 34.2±9 years (range 19–54), which is significantly lower compared to not MfS related cases with a mean age of 54±13 years (range 9–76; P=0.0001)

The preoperative New York Heart Association (NYHA) functional class was 3.4±0.8 in A and 3.1±0.9 in B. All patients with acute dissections were classified as NYHA III or IV. Patients with NYHA I were offered surgery because of an increased risk for aortic rupture.

A total of 22 MfS patients had to undergo surgery due to acute (57.6%) or chronic (9.1%) aortic dissections. Aortic aneurysms were present in 11 MfS patients (33.3%). In contrast, there was no difference between the incidence of aneurysms versus dissections in group B (Table 1). Of MfS patients, 33.3% were classified as DeBakey type I, 24.2% as type II and 9.1% as type III. In group B 26.5% were categorized as type I, 21.5% as type II and 2.7% as type III dissections. Two MfS patients (6.1%) and 17 patients (5.7%) of group B presented with aortic rupture. Thus, involvement of the ascending aorta was the most frequent indication for surgery in both groups (A, 84.9%; B, 81.2%). Five MfS patients (15.2%) and 50 patients (16.8%) of group B presented with aortic arch involvement.

The average diameter of the aorta immediately before surgery, measured by echocardiography or angiography, was 7.5±1.7 cm (range 5–12 cm) in group A and 6.9±2.1 (range 3–20 cm) in group B.

Operative and postoperative management
Three MfS patients (9.1%) and 101 patients (33.9%) in group B underwent elective surgery. Altogether, 11 patients (33.3%) with MfS and 62 not MfS-related patients (20.8%) were urgent, while 19 MfS patients (57.6%) and 135 group B patients (45.3%) had to undergo emergency surgical intervention.

During the past 20 years, three different methods of myocardial protection were employed: Between 1975 and 1977, induced ventricular fibrillation with moderate systemic hypothermia (26–28°C) was used. After 1978, induced ventricular fibrillation with intermittent cold crystalloid cardioplegia (Kirklin) and more recently, blood cardioplegia in cases with reduced ventricular function and coronary heart disease was administered after cross-clamping of the aorta. Topical application of cold saline solution (4°C) was used for myocardial protection. Localized aneurysms of the ascending aorta were removed on cardiopulmonary bypass and moderate hypothermia (26–28°C). For aneurysms extending into the aortic arch, deep hypothermia and circulatory arrest (18–20°C) was employed. Over the past 4 years, in cases of acute type I or II dissections, we preferred an open distal anastomosis without cross-clamping of the aorta.

All patients, who received aortic valve replacement or a composite aortic graft with mechanical prostheses were continued on anticoagulation with phenprocoumon (Marcumar®). After 1994, postoperative prophylactic ß-adrenergic blockade was used in all MfS patients, in order to reduce the progression of aortic dilatation and to prevent the development of aortic complications [14].

Operative techniques
Various operative techniques were used between 1975 and 1994. In MfS, replacement of the ascending aorta as the primary surgical intervention was performed in 28 cases (84.9%). Of these, 18 patients (54.6%) received a composite graft as described by Bentall and De Bono [15]. Composite grafts were constructed during surgery by sewing a Bjork-Shiley or a bileaflet valve in a Dacron tube graft. For graft insertion, the open technique was used. In 7 patients (21.2%) and another patient undergoing reoperation, we used a supracoronary graft with separate aortic valve replacement as described by Wheat [16]. In one patient, vascular graft replacement was combined with valve resuspension. In another patient, the aortic valve showed no evidence of regurgitation and an isolated graft replacement was sufficient for treatment. In 1975, one patient was treated with the wrapping technique. In 5 patients (15.2%), surgery was extended into the aortic arch, utilizing deep hypothermic circulatory arrest as described above. Five patients (15.2%) received a graft replacement of the descending aorta.

In group B, the majority of patients underwent Wheat’s operation (Table 2). In the 1970s, aortic repair with resection of the aneurysmatic aortic segment and reconstruction by direct suture or patch interposition was preferentially used. In MfS patients, we did not use any repair because of the fragile aortic tissue.

A total of 29 patients in group B and 3 patients in the MfS group underwent concomitant operative procedures. In group B, the most common concomitant procedure was a coronary artery bypass graft in 27 patients (9.1%), 2 patients had mitral valve replacement. One patient in group A received a coronary artery bypass graft, 2 patients a mitral valve replacement.

Statistics
Continuous data were analysed using the Mann–Whitney U-test, categorial data using ²-test. Data other than Kaplan–Meier curves were expressed as the mean±S.D. The analysis of long-term survival and freedom from reoperation were calculated by the Kaplan–Meier method [17]. These data were expressed as the mean±S.E. of the mean. Univariate analysis of the relation between late mortality as well as overall survival and several predictive variables was carried out by means of log-rank statistic, followed by a multivariate analysis using the Cox regression model. Variables evaluated were patient age, sex, NYHA class, study group (Marfan patients versus non Marfan patients), time of operation, type of dissection (DeBakey I,II or III, acute or chronic dissection or chronic aneurysm), different aortic locations, emergency operation, cardiac tamponade, bypass time, different methods of myocardial protection, operative techniques (composite graft versus non-composite graft surgery), arch replacement, aortic valve regurgitation, additional coronary artery disease, reoperations and recidives. Probability values (P) of less than 0.05 were considered significant. Statistical analysis was performed by SPSS statistical software for Windows 95 (Version 7.0, 1996).

	Results

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Early mortality
Five Marfan patients (15.2%) and 51 patients of group B (17.1%) died within the first 30 days after operation of the thoracic aorta. We recorded no statistically significant difference between the early mortality in the MfS group and group B. There were no deaths in group A and 7 deaths (6.9%) in group B patients, who underwent elective surgery. Early mortality rate was significantly higher in patients who had aortic dissection (18.2% in MfS versus 26.5% in B), when compared to patients with aortic aneurysms (9.1% in MfS versus 7.5% in B). The causes of early death, as shown in Table 3, were not different in both groups. Two MfS patients died in the operation room of uncontrollable bleeding due to the fragile aortic tissue. One patient, presenting with acute dissection, suffered from redissection with ischemia of the mesenteric vessels 2 days after graft replacement and 2 other patients died from multiorgan failure. Various causes of death were observed in group B, most of the patients suffered from deteriorating organ function.

View larger version (19K): [in this window] [in a new window]   Fig. 1. Long-term survival (Kaplan–Meier) of patients with Marfan syndrome (squares; group A) and patients with non-fibrillinopathic etiologies of aortic disease (crosses; group B).

View larger version (23K): [in this window] [in a new window]   Fig. 2. Long-term survival (Kaplan–Meier) according to type of diagnosis: patients with aortic aneurysms (crosses), chronic (squares) and acute dissections (circles).

View larger version (19K): [in this window] [in a new window]   Fig. 3. Long-term survival (Kaplan–Meier) in relation to the surgical technique used: composite graft (crosses) versus other procedures (squares).

In group B, reoperations were significantly less frequent (10.7%) compared to MfS patients (66.7%; P<0.001). The causes of reoperation are shown in Table 5.

The freedom from reoperation was 65±11% at 5 years, 49±13% at 10, and 25±19% at 14 years in group A, and 91±2% at 5, 82±3% at 10, and 79±4% at 15 years in group B (P<0.001; Fig. 4 ). The indication for primary operation (aneurysm versus acute versus chronic dissection) demonstrated a significantly lower freedom from reoperation for acute dissection compared to aortic aneurysms (P<0.05), whereas the type of dissection (DeBakey I, II or III) did not have any effect on the freedom from reoperation.

View larger version (20K): [in this window] [in a new window]   Fig. 4. Freedom from reoperation (Kaplan–Meier) of patients with Marfan Syndrome (squares; group A) and patients with non-fibrillinopathic etiologies of aortic disease (crosses; group B).

	Discussion

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Marsele et al. were able to demonstrate improved survival in patients, who received more extensive surgery at an earlier point of time, using composite graft replacement of the ascending aorta [29]. Kouchoukos reported good results in 127 patients who had composite graft insertion. Using this technique, the incidence of early and late pseudoaneurysms was markedly reduced [30]. Using Bentall’s procedure, Gott et al. also succeeded in improving long-term results in 100 MfS-patients, even considering the fact that in this study, only 7 patients suffered from acute dissection. We used composite graft replacement in 18 patients without any complication in this segment. Three of these patients, however, who had DeBakey type I or II-dissection, had redissection at the proximal aortic arch, probably due to a secondary intimal tear of a persisting false lumen and the fragility of the aortic tissue. Thus, we now use the technique of deep hypothermia and circulatory arrest for an open distal anastomosis in MfS patients with acute dissection of the ascending aorta, regardless if there is an involvement of the aortic arch or not. Applying this technique, the aortic arch can be examined for additional intimal tears in order to include that part of the vessel in the resection. Increasing experience and the development of improved techniques like deep hypothermic circulatory arrest has been shown to be a safe and risk lowering method for aortic arch surgery [30] [31] [32] [33].

Despite the higher incidence of aortic dissection and limited preoperative functional status (NYHA class) in Marfan patients, the early mortality of these patients was similar to that in patients without MfS [18] [37]. Thus, MfS was not a risk factor for early mortality. This is presumably caused by the better health status and the significantly lower age of these patients, which may nullify the higher surgical risk associated with the more fragile aorta of MfS patients. The highest early mortality rate was noticed in patients with acute dissection and without MfS, due to their advanced age and the higher morbidity with multisystemic involvement. Advanced NYHA class, emergency operation, cardiac tamponade, prolonged bypass time and acute type I dissection with inclusion of the transverse arch in the repair were significant independent predictors for early mortality and overall survival.

The present study demonstrates that reoperation and recidives were considerably more frequent in MfS compared to patients with non-fibrillinopathic etiologies of aortic disease. Multiple aortic operations at different sections of the aorta are characteristic in MfS patients, an observation which has been described earlier [34] [35]. Since the recidive rate strongly affects late survival as indicated in the univariate and multivariate analysis, the prognosis in MfS patients is primarily determined by the number of recurrent aneurysms or redissections leading to a further surgical intervention [18] [21]. A more radical operation may therefore reduce the high rate of aortic recidives as well as the need for distal reoperations and lead to a decrease in late deaths [21] [22] [23] [24] [26] [27] [28]. Crawford and coworkers demonstrated that 70% of surviving patients with DeBakey type I dissection were free from aortic reoperation for aneurysmal dilation of the distal false channel, but none out of 9 patients with an intimal tear in the transverse arch, which was included in the resection, required reoperation [27].

MfS predisposes to aortic disease, which is associated with a high risk of premature death. MfS patients suffering from acute aortic dissection more likely required reoperation compared to patients with aortic aneurysm. In addition, patients with acute dissection showed a significantly lower overall survival as well as a higher early mortality rate. Also an emergency operation was a significant predictor for overall survival in the multivariate analysis. To improve long-term prognosis in these patients, efforts must be made to decrease the incidence of aortic dissection and redissection, leading to further operations.

Since aortic dissection occurs in aortic dilatation, it seems reasonable to replace a dilated aorta as early as possible. Crawford recommended surgical treatment, if the external diameter exceeded 5 cm [22]. The Johns Hopkins group has suggested 6 cm as a cut-off for elective replacement of the ascending aorta [19] [20], presenting excellent long-term results by using composite graft repair for MfS-related aneurysms of the ascending aorta. Svensson recommended an intervention as soon as the aorta reaches twice the diameter as the unaffected distal part of the aorta [24]. In contrast, Pyeritz demonstrated that even in aortas with a diameter of less than 5 cm, dissections may occur [25]. Yet, the major problem remains the rapid development and progression of aneurysmal dilatation.

In conclusion, the surgical treatment of aneurysms of the thoracic aorta in MfS-patients is associated with a considerably higher risk of redissection and recurrent aneurysm compared to other etiologies of aortic disease. If the ascending aorta has to be replaced, we recommend the composite graft technique and a more aggressive approach to reduce the prevalence of distal reoperations. In order to improve the prognosis in these patients, the incidence of acute aortic dissection and redissection must be substantially reduced. Since aortic dilatation frequently leads to dissection, early diagnosis and preventive surgical treatment must be a major goal in MfS patients. To date, the best predictor is the dynamics of aortic root dilatation [36].

Considering the very high reoperation rate in our MfS patients and the rapid development and progression of aneurysmal dilatation, we require clinical follow-up by monitoring of the entire aorta at least twice a year. If the diameter has reached or exceeded 4 cm, we perform follow-up examination every 3 months. If the aorta exceeds 5 cm or significant aortic regurgitation develops, we recommend prophylactic surgery, even if the patient is asymtomatic. According to the observation that ß-blockers may reduce the progression of aortic dilatation, all patients with MfS should receive prophylactic ß-adrenergic blockade.

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Christian Detter Helmut Mair Armin Welz Bruno Reichart Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Detter, C. Articles by Reichart, B. Search for Related Content PubMed PubMed Citation Articles by Detter, C. Articles by Reichart, B.