The Marfan syndrome and cardiac surgery Of 3/ patients with Marfan's syndrome and cardiovascular complications, 25 had ascending aortic aneurysms, five with aortic dissection; 26 had aortic regurgitation, two with aortic stenosis; and eight had mitral regurgitation, five with aortic regurgitation. Surgery included prosthetic aortic valve replacement in 24 patients and aortic valvular bicuspidization in two; /9 had resection of aneurysm with Dacron tube replacement, three had lateral aneurysmorrhaphy, and two had circumferential strip excision with end-to-end anastomosis. Four patients underwent mitral valve replacement. Operative complications occurred in /0 patients. There were nine (29 percent) hospital deaths, but only one death occurred in /2 patients operated upon since /970. Late complications included prosthetic leak in six patients (23 percent) with reoperation in five; all survived. Five late deaths occurred (/6 percent); one was unrelated and one was of unknown cause. Although risk of cardiac surgery remains high, our recent results support an aggressive surgical approach, particularly in patients with advanced or deteriorating cardiovascular complications. Zev Davis, M.D" James R, Pluth, M,D" and Emilio R. Giuliani, M.D" Rochester, Minn. The cardiovascular complications of the Marfan syndrome are primarily responsible for the reduced life expectancy in patients with this hereditary disorder. 1 Surgical therapy in the past has been reserved for pa- tients with advanced cardiovascular disease whose condition continued to deteriorate on a medical regi- men. This approach, although often lifesaving, has been associated with a high mortality rate, This retrospective study was undertaken to evaluate (I) the early and late results of surgical intervention using extracorporeal circulation and (2) the surgical complications in patients with the cardiovascular man- ifestations of Marfan's disease. Patients and methods From 1960 through June, 1976, 31 patients with Marfan's syndrome underwent surgical repair at the Mayo Clinic with extracorporeal circulation. There were 27 men and four women with an age distribution of 17to 67 years and a mean age of 39 years at the time of operation. The diagnosis of Marfan's syndrome was based on a variable combination of family history, habitus, len- From the Mayo Clinic and Mayo Foundation, Rochester, Minn. 55901 Read at the Third Annual Meeting of The Samson Thoracic Surgical Society, Colorado Springs, Colorado, June 4-7, 1977. Address for reprints: Dr. Z. Davis, c/o Section of Publications, Mayo Clinic, 200 First Street S.W., Rochester, Minn. 55901. 0022-5223/78/0475-0505$00.50/0 © 1978 The C. V. Mosby Co. ticular dislocation, cardiovascular manifestations of aortic dilatation or dissecting aneurysm, and histo- pathological examination, Six patients had a positive family history. Twenty-two patients (71 percent) had the picture of arachnodactyly, high-arched palate, chest deformity, kyphosis, and an abnormal height-arm span ratio; nine of these patients also had dislocation of the lens. In 25 patients an ascending aortic aneurysm was demonstrated, and five of these had concomitant aortic dissection. The valvular lesions are given in Table I. Four patients had concomitant coronary artery dis- ease. Congenital anomalies were noted in six patients: bilateral superior vena cava in one patient, ventricular septal defect in two patients, bicuspid aortic valve in two patients (one with a fistulous connection between the aortic sinus of Valsalva and the right atrium and the other with aortic coarctation), and bullous emphysema in an additional patient. Four patients had subacute bac- terial endocarditis preoperatively. Nineteen patients were in Functional Class II (NYHA) preoperatively, II were in Class III, and one patient was in Class IV, Surgery Twenty-six patients underwent surgery of the aorta or the aortic valve (or both) with extracorporeal circu- lation; 19 of these patients had aneurysm resection with replacement by a Dacron tube graft (Table II), Three 505
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The Marfan syndrome and cardiac surgeryThe Marfan syndrome and cardiac surgery
Of 3/ patients with Marfan's syndrome and cardiovascular complications, 25 had ascending aortic aneurysms, five with aortic dissection; 26 had aortic regurgitation, two with aortic stenosis; and eight had mitral regurgitation, five with aortic regurgitation. Surgery included prosthetic aortic valve replacement in 24 patients and aortic valvular bicuspidization in two; /9 had resection of aneurysm with Dacron tube replacement, three had lateral aneurysmorrhaphy, and two had circumferential strip excision with end-to-end anastomosis. Four patients underwent mitral valve replacement. Operative complications occurred in /0 patients. There were nine (29 percent) hospital deaths, but only one death occurred in /2 patients operated upon since /970. Late complications included prosthetic leak in six patients (23 percent) with reoperation in five; all survived. Five late deaths occurred (/6 percent); one was unrelated and one was of unknown cause. Although risk of cardiac surgery remains high, our recent results support an aggressive surgical approach, particularly in patients with advanced or deteriorating cardiovascular complications.
Zev Davis, M.D" James R, Pluth, M,D" and Emilio R. Giuliani, M.D" Rochester, Minn.
The cardiovascular complications of the Marfan syndrome are primarily responsible for the reduced life expectancy in patients with this hereditary disorder. 1
Surgical therapy in the past has been reserved for pa- tients with advanced cardiovascular disease whose condition continued to deteriorate on a medical regi- men. This approach, although often lifesaving, has been associated with a high mortality rate,
This retrospective study was undertaken to evaluate (I) the early and late results of surgical intervention using extracorporeal circulation and (2) the surgical complications in patients with the cardiovascular man- ifestations of Marfan's disease.
Patients and methods
From 1960 through June, 1976, 31 patients with Marfan's syndrome underwent surgical repair at the Mayo Clinic with extracorporeal circulation. There were 27 men and four women with an age distribution of 17 to 67 years and a mean age of 39 years at the time of operation.
The diagnosis of Marfan's syndrome was based on a variable combination of family history, habitus, len-
From the Mayo Clinic and Mayo Foundation, Rochester, Minn. 55901
Read at the Third Annual Meeting of The Samson Thoracic Surgical Society, Colorado Springs, Colorado, June 4-7, 1977.
Address for reprints: Dr. Z. Davis, c/o Section of Publications, Mayo Clinic, 200 First Street S.W., Rochester, Minn. 55901.
0022-5223/78/0475-0505$00.50/0 © 1978 The C. V. Mosby Co.
ticular dislocation, cardiovascular manifestations of aortic dilatation or dissecting aneurysm, and histo- pathological examination, Six patients had a positive family history. Twenty-two patients (71 percent) had the picture of arachnodactyly, high-arched palate, chest deformity, kyphosis, and an abnormal height-arm span ratio; nine of these patients also had dislocation of the lens.
In 25 patients an ascending aortic aneurysm was demonstrated, and five of these had concomitant aortic dissection. The valvular lesions are given in Table I.
Four patients had concomitant coronary artery dis- ease. Congenital anomalies were noted in six patients: bilateral superior vena cava in one patient, ventricular septal defect in two patients, bicuspid aortic valve in two patients (one with a fistulous connection between the aortic sinus of Valsalva and the right atrium and the other with aortic coarctation), and bullous emphysema in an additional patient. Four patients had subacute bac- terial endocarditis preoperatively.
Nineteen patients were in Functional Class II (NYHA) preoperatively, II were in Class III, and one patient was in Class IV,
Surgery
Twenty-six patients underwent surgery of the aorta or the aortic valve (or both) with extracorporeal circu- lation; 19 of these patients had aneurysm resection with replacement by a Dacron tube graft (Table II), Three
505
Thoracic and Cardiovascular Surgery
Table I. Cardiovascular complications in 3J patients with Mar/an's syndrome
Complication I No. ofpatients
patients underwent lateral aneurysmorrhaphy, and two patients had excision of a narrow circumferential aortic strip, at aneurysm level, with end-to-end anastomosis. Prosthetic valves were required in 24 of the 26 patients: 19 Starr-Edwards, four Braunwald-Cutter, and one Hancock xenograft valve. The two other patients had aortic valvular bicuspidization procedures.
Four patients had mitral valve replacement, for which three Starr-Edwards valves and one Hancock xenograft valve were used.
Additional procedures performed included closure of a ventricular septal defect and an aortico-atrial com- munication in one patient, closure of a ruptured sinus of Valsalva aneurysm in a second patient, and an aortico-saphenous vein bypass graft in a third patient.
Direct coronary artery perfusion durinq bypass was performed in all patients who had aortic or aortic valve surgery.
Results
Single or multiple perioperative complications oc- curred in 10 of the 31 patients: low cardiac output states necessitating pharmacologic support in two, dys- rhythmias in six, and hemorrhage in six. Four patients were reoperated upon within 24 hours, all because of
Aortic aneurysm With aortic dissection
Aortic insufficiency Aortic stenosis and insufficiency Aortic and mitral insufficiency Mitral insufficiency
Table II. Surgical treatment
with end-to-end anastomosis Aortic valve
Replacement Bicuspidization
24 2
4* 3
significant bleeding. One patient had an acute myocar- dial infarction in the second postoperative week and another a transient seizure of unknown origin.
There were nine (29 percent) hospital deaths. Four patients died of low cardiac output, three of dysrhyth- mia, and two of severe aortic regurgitation. Included in the last-named two patients was one patient who had had only mitral valve replacement performed and an- other patient in whom severe aortic regurgitation de- veloped 3 days after a bicuspidization procedure of the aortic valve had been performed. Eight of the nine hospital deaths occurred from 1960 through 1969 (Table III).
All patients were followed until June, 1976, or to the time of death. Mean follow-up was 4.8 years, with the longest follow-up being 10 years. Late complications are presented in Table IV.
Reoperation was necessary in five patients-four be- cause of significant prosthetic leak and one in a patient who had an acute dissection 5 years after partial exci- sion of an ascending aneurysm with end-to-end anas- tomosis. All patients survived reoperation.
There were five late deaths (16 percent) (Table IV). Two patients died of severe mitral insufficiency (3 months and 3 years after operation), one of heart failure (7 years after), one of causes unrelated to the Marfan syndrome, and one of unknown cause. Functional class was improved in 71 percent of the survivors.
Discussion
Cardiovascular complications of the Marfan syn- drome occur in 30 to 60 percent of cases." Most com- mon are aneurysmal dilatation of the aortic root, with or without dissection of the aorta, and aortic regurgita- tion, which was seen in 26 of 31 (84 percent) patients in this series. Variable difficulties arise which reflect in- volvement of the atrioventricular valves (eight patients with mitral insufficiency in our series), coronary ostial deformations (typically, abnormally high origin of the coronary arteries, not noted in our series), aneurysms of the sinus of Valsalva (in two patients in this series), and coarctation of the aorta (one case). Any combina- tion of these cardiac lesions may cause the reduced life expectancy described in this syndrome. Cardiac prob- lems led to 52 of the 56 deaths of known cause in the series of patients with Marfan's disease in the report by Murdoch and associates': aortic dilatation and its com- plications (dissection, rupture, aortic and mitral valve involvement) accounted for 80 percent of these.
Although the disease was originally described by Marfarr' 1896, it is only since the reports of Bahnson
Volume 75
Number 4
Table IV. Late complications of surgical treatment
Complication I No. ofpatients
No·1 % No·1 % No·1 %Date ofoperation patients
patients had severe mitral insufficiency and the third had severe left ventricular failure. Although late reop- eration was necessary in five patients in our series (four for prosthetic leaks and one for distal dissection), all survived this additional procedure. There was a sig- nificant improvement not only in relation to the ex- pected natural prognosis and survivorship of this select patient population! but also in the functional class in the majority of survivors.
Much less common is mitral valve involvement in Marfan's syndrome, as first described by Salle."! Suc- cessful mitral valve replacement for mitral regurgita- tion in Marfan's syndrome was initially reported by Dietzman and associates'", and Nelson and Vaughn'" reported successful correction of aortic and mitral valve regurgitation by double valve replacement. Eight pa- tients in our series had mitral regurgitation, five of them having combined aortic and mitral valve lesions.
Although the risk of cardiac surgery in patients with Marfan's syndrome is high, our recent results and those of others support an aggressive surgical approach. We attribute the improvement in surgical results to the elimination of tailoring procedures of the aortic root. A higher mortality rate in that group of patients in the early experience from 1960 to 1969 suggests that this is a major factor to which improved survivorship can be attributed.
We would like to thank Drs. R. B. Wallace, D. C. McGoon, G. K. Danielson, and D. A. Bamhorst for the opportunity to include their patients in this review.
2
6 2 3
8 42 4 21 12 63 I 8 I 8 2 17
9 29 5 16 14 45
19 12
Residual aortic insufficiency Subacute bacterial endocarditis Mitral insufficiency
Cerebrovascular accident
Totals
and Nelson" and Muller and associates" that a surgi- cal approach has been considered for progressive or acutely deteriorating cardiovascular symptoms.
As recently as 1970, however, Symbas and col- leagues" reviewed the world literature and, including their own case, found only 19 reported cases of Mar- fan's syndrome with aortic aneurysm and aortic regur- gitation that were treated surgically. The over-all hospi- tal mortality rate in those 19 patients was 31.6 percent. Statistics such as these have further supported the prev- alent apprehension that surgical intervention is hazar- dous in this multisystem connective tissue disorder, often being followed by disruption of a suture line or of a prosthetic valve from its annular mooring and by poor wound healing.
Recent reports, however, based on improved prosthe- ses and surgical techniques, have demonstrated that these difficulties can be overcome and that an accept- able morbidity and mortality can be achieved. Crosby and associates' successfully treated with surgery three patients with Marfan' s disease and two with forme fruste aortic lesions. There were no early deaths, and follow-up has extended 10 months to 2V2 years. Nasral- lah and co-workers" repaired or replaced a regurgitant aortic valve and ascending aortic aneurysm in 30 pa- tients with Marfan's syndrome, and the hospital mor- tality rate was 20 percent (6 of 30). The 24 survivors, all of whom were operated upon before 1968, lived from 5 weeks to 9 years, and 43 percent of patients in that series survived 5 years or more. However, 10 pa- tients (42 percent) died of late complications, with only three of these 10 patients dying of unrelated noncardiac causes. Ross and associates" reported that 86 percent of 14 patients undergoing aortic valve replacement with graft replacement of the aneurysm survived and were doing well 6 months to 4V2 years after surgery.
Our results lend further support to these recent re- ports. Nine of the 31 patients (29 percent) reported in our series died in the immediate perioperative period. All of these deaths except one, however, occurred in the 19 patients operated upon before 1969. Among 12 patients operated upon since 1970, there has been only one early death (a 19-year-old patient with aortic valve replacement and graft who died of low cardiac output). Furthermore, the long-range follow-up, extending up to 10 years, has demonstrated only five late deaths (16 percent) in our series. This compares favorably with the results in patients suffering from annulo-aortic ectasia with cystic medial necrosis of non-Marfan cause.l? Of the five late deaths, only three could with certainty be related to the underlying cardiovascular disease: Two
508 Davis, Pluth, Giuliani
REFERENCES
Murdoch n., Walker BA, Halpern BL, Kuzma rw, McKusick VA: Life expectancy and causes of death in the Marfan syndrome. N Engl 1 Med 286:804-808, 1972
2 Goyette EM, Palmer PW: Cardiovascular lesions in arachnodactyly. Circulation 7:373-379. 1953
3 Marfan MA-B: Un cas de deformation congenitale des quatre membres, plus prononcee aux extremities carac- terisee par I'allongement des os avec un certain degre d'amincissement. Bull Mem Soc Med Hop Paris 13: 220-226, 1896
4 Bahnson HT, Nelson AR: Cystic medial necrosis as a cause of localized aortic aneurysms amenable to surgical treatment. Ann Surg 144:519-529, 1956
5 Muller WH lr, Dammann lF lr, Warren WD: Surgical correction for cardiovascular deformities in Marfan' s syndrome. Ann Surg 152:506-516, 1960
6 Symbas PN, Baldwin Bl, Silverman ME, Galambos JT: Marfans syndrome with aneurysm of ascending aorta and aortic regurgitation. Surgical treatment and new his- tochemical observations. Am 1 CardioI25:483-489, 1970
7 Crosby IK, Ashcraft WC, Reed WA: Surgery of proximal aorta in Marfans syndrome. 1 THoRAc CARDIOVASC SURG 66:75-81, 1973
8 Nasrallah AT, Cooley DA, Goussous Y, Hallman GL, Lufschanowski R, Leachman RD: Surgical experience in patients with Marfan ' s syndrome, ascending aortic aneu- rysm and aortic regurgitation. Am 1 Cardiol 36:338-341, 1975
9 Ross DN, Frazier TG, Gonzalez-Lavin L: Surgery of Marfans syndrome and related conditions of the aortic root (annulo-aortic ectasia). Thorax 27:52-57, 1972
10 Borer lS: Early surgery recommended in cystic medial necrosis of aorta. Hosp Pract 11:32, 1976
II Salle V: Uber einen Fall von angeborene abnormer Grosse der Extrernitaten mit einem Akromegalie erin- nemden Symptomenkomplex. lahrb Kinderh 75:540- 550, 1912
12 Dietzman RH, Peter ET, Wang Y, Lillehei RC: Mitral insufficiency in Marfan's syndrome. Chest 51:650-653, 1967
13 Nelson RM, Vaughn CC: Double valve replacement in Marfans syndrome. 1 THoRAc CARDIOVASC SURG 57: 732-737, 1969
Discussion
DR. GEORGE E. MILLER, lR. Sacramento, Calif.
Dr. Davis has described a history of improvement in their treatment of the complications of Marfan's aorta. We have also noted improvement in our results. However, in review- ing their problems and our failures, we still find need for improvement in obtaining a rapid, secure, and hemostatic anastomosis to the remaining aorta. Especially in the dissect- ing aorta, intraoperative bleeding and postoperative redissec- tion occur all too frequently.
The Journal of
Thoracic and Cardiovascular
Surgery
Two years ago in the laboratory we began trying to look at this problem, especially that of the dissecting aorta, in a somewhat different manner. We felt that improvement could be attained if we could (I) increase the tensile strength of the remaining aorta so that it would be more suturable and (2) obliterate the false lumen so that we would have a single- layered, firm aorta to which to suture the graft.
First, in an attempt to provide both answers, we used cer- tain adhesives. We found we could obliterate the false lumen and improve tensile strength. However, the resultant one- walled aorta was stiff, noncompliant, and did not suture well.
We then tried using various polymers. Certain commercial polymers retain the rubber flexible characteristics even after being cured. We found that we could obtain excellent tissue support and obliterate the false lumen, but the adherence was frequently not satisfactory.
We have not been able to find a material that will both support the tissue and obliterate the false lumen.
Looking at a means of increasing tissue tensile strength, we confirmed that treatment of the canine and cadaver aorta for 20 minutes with I percent glutaraldehyde would increase the tensile strength of the artery wall three times. We then studied the 6 month effect of in vivo, in situ treated canine artery and found that the tissue could' be tanned. It became acellular. Neither the treated portion nor the junction between the un- treated artery and the treated artery became dilated, nor did significant stenosis develop. However, at 6 months intimal calcification regularly appeared.
This method does produce a marked increase in tissue ten- sile strength. It is easily applied, but the long-term results are questionable.
Finally, although this is not what we were looking for, we did find that we could increase the tensile strength rather remarkably by simply removing 2 to 3 cm. of the dissected intima and then folding the adventitia over this intimal edge. This procedure provides three layers, two of them being the stronger layers of the aortic wall.
We have used these methods only experimentally. How- ever, having evaluated them for 2 years, I would say that, at least conceptually, they hold some promise.
The improvements in results of treatment of this problem are due to changes in techniques and materials. I would like to ask Dr. Davis about their current method with regard to both technique and materials for managing a Type I dissection.
DR. lAMES B. D. MARK Stanford, Calif.
We have found that patients with Marfari's syndrome have a high incidence of spontaneous pneumothorax and bleb dis- ease with giant cystic changes in the lung. We have had to operate upon several of these patients, The ones with straightforward pneumothorax have apical blebs much like those in any patient with spontaneous pneumothorax, and we have been unable to identify any unique changes on micro- scopic examination.
Dr. Davis, has spontaneous pneumothorax occurred in any
Volume 75
Number 4
April, 1978
of your patients? It is particularly likely to occur in the postoperative period if respiratory support with positive pressure is necessary.
DR. NORMAN E. SHUMWAY Palo Alto, Calif.
I cannot remember replacing the aortic valve and not per- forming concomitant aortic resection in the Marfan syn- drome. What is the fate of the ascending aorta in these pa- tients later on, and what kind of prosthesis was utilized for the aortic valve replacement?
DR. 0 A V I S (Closing) I would like to thank the discussers for their comments. We all recognize that this is a serious problem, predomi-
nantly because of the poor connective tissue and because of the late complications that ensue even if the operation has been performed without technical fault.
In respect to Dr. Miller's comments, the concept of using tissue adhesives is extremely intriguing. Overlapping the aor- tic adventitia to create an internal and external layer to strengthen the distal suture line is a very interesting approach. I hope that we will have more experimental data to support this concept in the future.
As to technique, the approach at the Mayo Clinic varies among the cardiac surgeons practicing at the present time, and it certainly has evolved over the years.
I would like to mention a recent technique for which, un- fortunately, there is no long-range follow-up-the use of a Hancock conduit containing a xenograft valve.
Femoral arterial cannulation is performed, and a single
Marfan syndrome 5°9
venous cannula is placed through the right atrial appendage. We have not used cardioplegic solution to date. The coronary arteries are perfused, and the heart is kept beating throughout the procedure.
If the valve is to be excised, as is often the case, we resect the aneurysmal portion of the ascending aorta completely. We feel it is safer to assure careful hemostasis at both suture lines rather than to rely on the tamponade effect of closing the native remaining aortic wall over the anastomosis and possibly compressing the coronaries and the outflow of the left ventricle.
The Hancock conduit is inserted proximally (in two layers of either Ethiflex or Prolene with Teflon felt), and then the distal anastomosis is completed. The coronary orifices are anastomosed to the graft subsequently.
Dr. Mark, I do not recall any instances of spontaneous pneumothorax in the postoperative period, nor have there been any unusual pulmonary problems.
Dr. Shumway, prior to the use of the Hancock valve, we have used the Starr-Edwards valve. There are a number of surgeons who still use the Starr-Edwards valve.
DR. SHUMWAY: After…
Of 3/ patients with Marfan's syndrome and cardiovascular complications, 25 had ascending aortic aneurysms, five with aortic dissection; 26 had aortic regurgitation, two with aortic stenosis; and eight had mitral regurgitation, five with aortic regurgitation. Surgery included prosthetic aortic valve replacement in 24 patients and aortic valvular bicuspidization in two; /9 had resection of aneurysm with Dacron tube replacement, three had lateral aneurysmorrhaphy, and two had circumferential strip excision with end-to-end anastomosis. Four patients underwent mitral valve replacement. Operative complications occurred in /0 patients. There were nine (29 percent) hospital deaths, but only one death occurred in /2 patients operated upon since /970. Late complications included prosthetic leak in six patients (23 percent) with reoperation in five; all survived. Five late deaths occurred (/6 percent); one was unrelated and one was of unknown cause. Although risk of cardiac surgery remains high, our recent results support an aggressive surgical approach, particularly in patients with advanced or deteriorating cardiovascular complications.
Zev Davis, M.D" James R, Pluth, M,D" and Emilio R. Giuliani, M.D" Rochester, Minn.
The cardiovascular complications of the Marfan syndrome are primarily responsible for the reduced life expectancy in patients with this hereditary disorder. 1
Surgical therapy in the past has been reserved for pa- tients with advanced cardiovascular disease whose condition continued to deteriorate on a medical regi- men. This approach, although often lifesaving, has been associated with a high mortality rate,
This retrospective study was undertaken to evaluate (I) the early and late results of surgical intervention using extracorporeal circulation and (2) the surgical complications in patients with the cardiovascular man- ifestations of Marfan's disease.
Patients and methods
From 1960 through June, 1976, 31 patients with Marfan's syndrome underwent surgical repair at the Mayo Clinic with extracorporeal circulation. There were 27 men and four women with an age distribution of 17 to 67 years and a mean age of 39 years at the time of operation.
The diagnosis of Marfan's syndrome was based on a variable combination of family history, habitus, len-
From the Mayo Clinic and Mayo Foundation, Rochester, Minn. 55901
Read at the Third Annual Meeting of The Samson Thoracic Surgical Society, Colorado Springs, Colorado, June 4-7, 1977.
Address for reprints: Dr. Z. Davis, c/o Section of Publications, Mayo Clinic, 200 First Street S.W., Rochester, Minn. 55901.
0022-5223/78/0475-0505$00.50/0 © 1978 The C. V. Mosby Co.
ticular dislocation, cardiovascular manifestations of aortic dilatation or dissecting aneurysm, and histo- pathological examination, Six patients had a positive family history. Twenty-two patients (71 percent) had the picture of arachnodactyly, high-arched palate, chest deformity, kyphosis, and an abnormal height-arm span ratio; nine of these patients also had dislocation of the lens.
In 25 patients an ascending aortic aneurysm was demonstrated, and five of these had concomitant aortic dissection. The valvular lesions are given in Table I.
Four patients had concomitant coronary artery dis- ease. Congenital anomalies were noted in six patients: bilateral superior vena cava in one patient, ventricular septal defect in two patients, bicuspid aortic valve in two patients (one with a fistulous connection between the aortic sinus of Valsalva and the right atrium and the other with aortic coarctation), and bullous emphysema in an additional patient. Four patients had subacute bac- terial endocarditis preoperatively.
Nineteen patients were in Functional Class II (NYHA) preoperatively, II were in Class III, and one patient was in Class IV,
Surgery
Twenty-six patients underwent surgery of the aorta or the aortic valve (or both) with extracorporeal circu- lation; 19 of these patients had aneurysm resection with replacement by a Dacron tube graft (Table II), Three
505
Thoracic and Cardiovascular Surgery
Table I. Cardiovascular complications in 3J patients with Mar/an's syndrome
Complication I No. ofpatients
patients underwent lateral aneurysmorrhaphy, and two patients had excision of a narrow circumferential aortic strip, at aneurysm level, with end-to-end anastomosis. Prosthetic valves were required in 24 of the 26 patients: 19 Starr-Edwards, four Braunwald-Cutter, and one Hancock xenograft valve. The two other patients had aortic valvular bicuspidization procedures.
Four patients had mitral valve replacement, for which three Starr-Edwards valves and one Hancock xenograft valve were used.
Additional procedures performed included closure of a ventricular septal defect and an aortico-atrial com- munication in one patient, closure of a ruptured sinus of Valsalva aneurysm in a second patient, and an aortico-saphenous vein bypass graft in a third patient.
Direct coronary artery perfusion durinq bypass was performed in all patients who had aortic or aortic valve surgery.
Results
Single or multiple perioperative complications oc- curred in 10 of the 31 patients: low cardiac output states necessitating pharmacologic support in two, dys- rhythmias in six, and hemorrhage in six. Four patients were reoperated upon within 24 hours, all because of
Aortic aneurysm With aortic dissection
Aortic insufficiency Aortic stenosis and insufficiency Aortic and mitral insufficiency Mitral insufficiency
Table II. Surgical treatment
with end-to-end anastomosis Aortic valve
Replacement Bicuspidization
24 2
4* 3
significant bleeding. One patient had an acute myocar- dial infarction in the second postoperative week and another a transient seizure of unknown origin.
There were nine (29 percent) hospital deaths. Four patients died of low cardiac output, three of dysrhyth- mia, and two of severe aortic regurgitation. Included in the last-named two patients was one patient who had had only mitral valve replacement performed and an- other patient in whom severe aortic regurgitation de- veloped 3 days after a bicuspidization procedure of the aortic valve had been performed. Eight of the nine hospital deaths occurred from 1960 through 1969 (Table III).
All patients were followed until June, 1976, or to the time of death. Mean follow-up was 4.8 years, with the longest follow-up being 10 years. Late complications are presented in Table IV.
Reoperation was necessary in five patients-four be- cause of significant prosthetic leak and one in a patient who had an acute dissection 5 years after partial exci- sion of an ascending aneurysm with end-to-end anas- tomosis. All patients survived reoperation.
There were five late deaths (16 percent) (Table IV). Two patients died of severe mitral insufficiency (3 months and 3 years after operation), one of heart failure (7 years after), one of causes unrelated to the Marfan syndrome, and one of unknown cause. Functional class was improved in 71 percent of the survivors.
Discussion
Cardiovascular complications of the Marfan syn- drome occur in 30 to 60 percent of cases." Most com- mon are aneurysmal dilatation of the aortic root, with or without dissection of the aorta, and aortic regurgita- tion, which was seen in 26 of 31 (84 percent) patients in this series. Variable difficulties arise which reflect in- volvement of the atrioventricular valves (eight patients with mitral insufficiency in our series), coronary ostial deformations (typically, abnormally high origin of the coronary arteries, not noted in our series), aneurysms of the sinus of Valsalva (in two patients in this series), and coarctation of the aorta (one case). Any combina- tion of these cardiac lesions may cause the reduced life expectancy described in this syndrome. Cardiac prob- lems led to 52 of the 56 deaths of known cause in the series of patients with Marfan's disease in the report by Murdoch and associates': aortic dilatation and its com- plications (dissection, rupture, aortic and mitral valve involvement) accounted for 80 percent of these.
Although the disease was originally described by Marfarr' 1896, it is only since the reports of Bahnson
Volume 75
Number 4
Table IV. Late complications of surgical treatment
Complication I No. ofpatients
No·1 % No·1 % No·1 %Date ofoperation patients
patients had severe mitral insufficiency and the third had severe left ventricular failure. Although late reop- eration was necessary in five patients in our series (four for prosthetic leaks and one for distal dissection), all survived this additional procedure. There was a sig- nificant improvement not only in relation to the ex- pected natural prognosis and survivorship of this select patient population! but also in the functional class in the majority of survivors.
Much less common is mitral valve involvement in Marfan's syndrome, as first described by Salle."! Suc- cessful mitral valve replacement for mitral regurgita- tion in Marfan's syndrome was initially reported by Dietzman and associates'", and Nelson and Vaughn'" reported successful correction of aortic and mitral valve regurgitation by double valve replacement. Eight pa- tients in our series had mitral regurgitation, five of them having combined aortic and mitral valve lesions.
Although the risk of cardiac surgery in patients with Marfan's syndrome is high, our recent results and those of others support an aggressive surgical approach. We attribute the improvement in surgical results to the elimination of tailoring procedures of the aortic root. A higher mortality rate in that group of patients in the early experience from 1960 to 1969 suggests that this is a major factor to which improved survivorship can be attributed.
We would like to thank Drs. R. B. Wallace, D. C. McGoon, G. K. Danielson, and D. A. Bamhorst for the opportunity to include their patients in this review.
2
6 2 3
8 42 4 21 12 63 I 8 I 8 2 17
9 29 5 16 14 45
19 12
Residual aortic insufficiency Subacute bacterial endocarditis Mitral insufficiency
Cerebrovascular accident
Totals
and Nelson" and Muller and associates" that a surgi- cal approach has been considered for progressive or acutely deteriorating cardiovascular symptoms.
As recently as 1970, however, Symbas and col- leagues" reviewed the world literature and, including their own case, found only 19 reported cases of Mar- fan's syndrome with aortic aneurysm and aortic regur- gitation that were treated surgically. The over-all hospi- tal mortality rate in those 19 patients was 31.6 percent. Statistics such as these have further supported the prev- alent apprehension that surgical intervention is hazar- dous in this multisystem connective tissue disorder, often being followed by disruption of a suture line or of a prosthetic valve from its annular mooring and by poor wound healing.
Recent reports, however, based on improved prosthe- ses and surgical techniques, have demonstrated that these difficulties can be overcome and that an accept- able morbidity and mortality can be achieved. Crosby and associates' successfully treated with surgery three patients with Marfan' s disease and two with forme fruste aortic lesions. There were no early deaths, and follow-up has extended 10 months to 2V2 years. Nasral- lah and co-workers" repaired or replaced a regurgitant aortic valve and ascending aortic aneurysm in 30 pa- tients with Marfan's syndrome, and the hospital mor- tality rate was 20 percent (6 of 30). The 24 survivors, all of whom were operated upon before 1968, lived from 5 weeks to 9 years, and 43 percent of patients in that series survived 5 years or more. However, 10 pa- tients (42 percent) died of late complications, with only three of these 10 patients dying of unrelated noncardiac causes. Ross and associates" reported that 86 percent of 14 patients undergoing aortic valve replacement with graft replacement of the aneurysm survived and were doing well 6 months to 4V2 years after surgery.
Our results lend further support to these recent re- ports. Nine of the 31 patients (29 percent) reported in our series died in the immediate perioperative period. All of these deaths except one, however, occurred in the 19 patients operated upon before 1969. Among 12 patients operated upon since 1970, there has been only one early death (a 19-year-old patient with aortic valve replacement and graft who died of low cardiac output). Furthermore, the long-range follow-up, extending up to 10 years, has demonstrated only five late deaths (16 percent) in our series. This compares favorably with the results in patients suffering from annulo-aortic ectasia with cystic medial necrosis of non-Marfan cause.l? Of the five late deaths, only three could with certainty be related to the underlying cardiovascular disease: Two
508 Davis, Pluth, Giuliani
REFERENCES
Murdoch n., Walker BA, Halpern BL, Kuzma rw, McKusick VA: Life expectancy and causes of death in the Marfan syndrome. N Engl 1 Med 286:804-808, 1972
2 Goyette EM, Palmer PW: Cardiovascular lesions in arachnodactyly. Circulation 7:373-379. 1953
3 Marfan MA-B: Un cas de deformation congenitale des quatre membres, plus prononcee aux extremities carac- terisee par I'allongement des os avec un certain degre d'amincissement. Bull Mem Soc Med Hop Paris 13: 220-226, 1896
4 Bahnson HT, Nelson AR: Cystic medial necrosis as a cause of localized aortic aneurysms amenable to surgical treatment. Ann Surg 144:519-529, 1956
5 Muller WH lr, Dammann lF lr, Warren WD: Surgical correction for cardiovascular deformities in Marfan' s syndrome. Ann Surg 152:506-516, 1960
6 Symbas PN, Baldwin Bl, Silverman ME, Galambos JT: Marfans syndrome with aneurysm of ascending aorta and aortic regurgitation. Surgical treatment and new his- tochemical observations. Am 1 CardioI25:483-489, 1970
7 Crosby IK, Ashcraft WC, Reed WA: Surgery of proximal aorta in Marfans syndrome. 1 THoRAc CARDIOVASC SURG 66:75-81, 1973
8 Nasrallah AT, Cooley DA, Goussous Y, Hallman GL, Lufschanowski R, Leachman RD: Surgical experience in patients with Marfan ' s syndrome, ascending aortic aneu- rysm and aortic regurgitation. Am 1 Cardiol 36:338-341, 1975
9 Ross DN, Frazier TG, Gonzalez-Lavin L: Surgery of Marfans syndrome and related conditions of the aortic root (annulo-aortic ectasia). Thorax 27:52-57, 1972
10 Borer lS: Early surgery recommended in cystic medial necrosis of aorta. Hosp Pract 11:32, 1976
II Salle V: Uber einen Fall von angeborene abnormer Grosse der Extrernitaten mit einem Akromegalie erin- nemden Symptomenkomplex. lahrb Kinderh 75:540- 550, 1912
12 Dietzman RH, Peter ET, Wang Y, Lillehei RC: Mitral insufficiency in Marfan's syndrome. Chest 51:650-653, 1967
13 Nelson RM, Vaughn CC: Double valve replacement in Marfans syndrome. 1 THoRAc CARDIOVASC SURG 57: 732-737, 1969
Discussion
DR. GEORGE E. MILLER, lR. Sacramento, Calif.
Dr. Davis has described a history of improvement in their treatment of the complications of Marfan's aorta. We have also noted improvement in our results. However, in review- ing their problems and our failures, we still find need for improvement in obtaining a rapid, secure, and hemostatic anastomosis to the remaining aorta. Especially in the dissect- ing aorta, intraoperative bleeding and postoperative redissec- tion occur all too frequently.
The Journal of
Thoracic and Cardiovascular
Surgery
Two years ago in the laboratory we began trying to look at this problem, especially that of the dissecting aorta, in a somewhat different manner. We felt that improvement could be attained if we could (I) increase the tensile strength of the remaining aorta so that it would be more suturable and (2) obliterate the false lumen so that we would have a single- layered, firm aorta to which to suture the graft.
First, in an attempt to provide both answers, we used cer- tain adhesives. We found we could obliterate the false lumen and improve tensile strength. However, the resultant one- walled aorta was stiff, noncompliant, and did not suture well.
We then tried using various polymers. Certain commercial polymers retain the rubber flexible characteristics even after being cured. We found that we could obtain excellent tissue support and obliterate the false lumen, but the adherence was frequently not satisfactory.
We have not been able to find a material that will both support the tissue and obliterate the false lumen.
Looking at a means of increasing tissue tensile strength, we confirmed that treatment of the canine and cadaver aorta for 20 minutes with I percent glutaraldehyde would increase the tensile strength of the artery wall three times. We then studied the 6 month effect of in vivo, in situ treated canine artery and found that the tissue could' be tanned. It became acellular. Neither the treated portion nor the junction between the un- treated artery and the treated artery became dilated, nor did significant stenosis develop. However, at 6 months intimal calcification regularly appeared.
This method does produce a marked increase in tissue ten- sile strength. It is easily applied, but the long-term results are questionable.
Finally, although this is not what we were looking for, we did find that we could increase the tensile strength rather remarkably by simply removing 2 to 3 cm. of the dissected intima and then folding the adventitia over this intimal edge. This procedure provides three layers, two of them being the stronger layers of the aortic wall.
We have used these methods only experimentally. How- ever, having evaluated them for 2 years, I would say that, at least conceptually, they hold some promise.
The improvements in results of treatment of this problem are due to changes in techniques and materials. I would like to ask Dr. Davis about their current method with regard to both technique and materials for managing a Type I dissection.
DR. lAMES B. D. MARK Stanford, Calif.
We have found that patients with Marfari's syndrome have a high incidence of spontaneous pneumothorax and bleb dis- ease with giant cystic changes in the lung. We have had to operate upon several of these patients, The ones with straightforward pneumothorax have apical blebs much like those in any patient with spontaneous pneumothorax, and we have been unable to identify any unique changes on micro- scopic examination.
Dr. Davis, has spontaneous pneumothorax occurred in any
Volume 75
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April, 1978
of your patients? It is particularly likely to occur in the postoperative period if respiratory support with positive pressure is necessary.
DR. NORMAN E. SHUMWAY Palo Alto, Calif.
I cannot remember replacing the aortic valve and not per- forming concomitant aortic resection in the Marfan syn- drome. What is the fate of the ascending aorta in these pa- tients later on, and what kind of prosthesis was utilized for the aortic valve replacement?
DR. 0 A V I S (Closing) I would like to thank the discussers for their comments. We all recognize that this is a serious problem, predomi-
nantly because of the poor connective tissue and because of the late complications that ensue even if the operation has been performed without technical fault.
In respect to Dr. Miller's comments, the concept of using tissue adhesives is extremely intriguing. Overlapping the aor- tic adventitia to create an internal and external layer to strengthen the distal suture line is a very interesting approach. I hope that we will have more experimental data to support this concept in the future.
As to technique, the approach at the Mayo Clinic varies among the cardiac surgeons practicing at the present time, and it certainly has evolved over the years.
I would like to mention a recent technique for which, un- fortunately, there is no long-range follow-up-the use of a Hancock conduit containing a xenograft valve.
Femoral arterial cannulation is performed, and a single
Marfan syndrome 5°9
venous cannula is placed through the right atrial appendage. We have not used cardioplegic solution to date. The coronary arteries are perfused, and the heart is kept beating throughout the procedure.
If the valve is to be excised, as is often the case, we resect the aneurysmal portion of the ascending aorta completely. We feel it is safer to assure careful hemostasis at both suture lines rather than to rely on the tamponade effect of closing the native remaining aortic wall over the anastomosis and possibly compressing the coronaries and the outflow of the left ventricle.
The Hancock conduit is inserted proximally (in two layers of either Ethiflex or Prolene with Teflon felt), and then the distal anastomosis is completed. The coronary orifices are anastomosed to the graft subsequently.
Dr. Mark, I do not recall any instances of spontaneous pneumothorax in the postoperative period, nor have there been any unusual pulmonary problems.
Dr. Shumway, prior to the use of the Hancock valve, we have used the Starr-Edwards valve. There are a number of surgeons who still use the Starr-Edwards valve.
DR. SHUMWAY: After…
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