7horax (1972), 27, 52.

Surgery of Marfan's syndrome and related conditionsof the aortic root (annulo-aortic ectasia)DONALD N. ROSS, THOMAS G. FRAZIER, and

LORENZO GONZALEZ-LAVIN'Departments of Surgery, Institute of Cardiology, National Heart Hospital, and Guy's Hospital, Londotn

The surgical treatment of 14 patients with annulo-aortic ectasia and aortic valve regurgitationsecondary to cystic medial necrosis is presented. Resection of the aneurysm and replacement witha tubular graft, and aortic valve replacement with a rigid prosthesis, are advocated for definitivetreatment. Eighty-six per cent of the patients survive and are doing well six months to fourand a half years after surgery.

As early as 1896, Marfan's syndrome had becomea disease entity of interest to the clinician(Marfan, 1896) and, following the description ofErdheim in 1930 (Erdheim, 1930), it became im-portant for the pathologist. Several reports fol-lowed relating cardiovascular pathology toMarfan's syndrome (Baer, Taussig, andOppenheimer, 1943; Schorr, Braun, and Wildman,1951 ; Sloper and Storey, 1953; Marvel andGenovese, 1951 ; Gore, 1952; McKusick, 1955;Macleod and Williams, 1956). It was not until1956 and after the report of Bahnson and Nelson(1956), however, that this syndrome acquiredsurgical interest. Important advances in the fieldof cardiovascular surgery, and increasing under-standing of the anatomy and physiopathology ofthis disease, have made possible the developmentof surgical therapy for patients with Marfan'ssyndrome and especially for those with annulo-aortic ectasia.The purpose of this presentation is to report

our views and surgical experience in the surgicaltreatment of patients with this entity.

CLINICAL MATERIAL

Fourteen patients operated upon between October1965 and July 1970 presented with annulo-aorticectasia secondary to cystic medial necrosis and allhad aortic valve regurgitation. Based on the criteriaof skeletal (Fig. 1), ocular, and cardiovascular defects,it was possible to divide these cases into two definitegroups nine patients with Marfan's syndrome andfive with the 'form fruste. Twelve patients were malesand two were females; their ages ranged from I 1 to59 with an average of 40 years.

All patients were or had been in left ventricularfailure secondary to aortic regurgitation at the timeiReprint requests to Lorenzo Gonzalez-Lavin, M.D., Institute ofCardiolog. 35 Wimpole Street, London WIM SEX.

of surgery. Two were operated upon on an emergencybasis because of rapid deterioration. Eight patientsgave a history of angina pectoris. Radiographic (Fig.2a) and electrocardiographic evidence of severe leftventricular enlargement and hypertrophy were presentin all patients. Twelve patients underwent angio-graphic studies prior to surgery (Fig. 2b).

FIG. 1. Radiograph of the hands with a metacarpal inidexof 8-4 (Sutton, 1969): a characteristic finding in patienttswith Marfan's syndromiie.

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[: |~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~%FIG. 2 (a). Postero-anterior chest radiograph with evidence of leftventricular enlargement and an ascending aortic aneurysm. (b) Retrogradeaortogram outlining a large aneurysm of the annular level and severeaortic regurgitation.

SURGICAL APPROACH

Excellent exposure of the heart and ascending aortais obtained through a midline sternotomy. Theaneurysm usually occupies most of the anterior partof the pericardial cavity and the heart appears to bedisplaced towards the left (Fig. 3). The arterial lineis placed in the aortic arch distal to the innominateartery; for venous return to the pump a single lineis placed in the right atrium. Normothermic high-flowcardiopulmonary bypass is established using a bubbleoxygenator and haemodilution.The ascending aorta is cross-clamped just proximal

to the innominate artery, leaving enough cuff ofaorta for the distal anastomosis. A vent is placed inthe left ventricle and the aortic aneurysm is thenincised longitudinally. A previously selected Dacrontubular graft is preclotted and the coronary lines arepassed through the graft. Exposure of both coronaryostia is obtained and cannulation is performed afterplacing a pursestring suture to maintain the cannulaein place. The myocardium is perfused continuouslythroughout the procedure. Dissection of the aneurysmfrom the pulmonary artery and right and left atriais easily accomplished. It is then excised proximallyto the aortic annulus and distally to 1 cm from theaortic clamp near the base of the innominate artery.

Attention is then directed to the aortic valve. It FIG. 2 (b).

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Donald N. Ross, Thomas G. Frazier, and Lorenzo Gonzalez-Lavin

may be completely normal but rendered regurgitantdue to dilatation of the annulus (Fig. 4). Multipleinterrupted sutures are used for insertion of theprosthetic valve (Fig. 5), taking substantial bites witheach stitch in both the valve sewing ring and theaortic annulus.The Dacron tubular graft is tailored; the proximal

suture line is placed with a running suture of 3-0Mersilene; the distal suture line is placed in thesame manner, leaving a small opening to exclude theair after removing the coronary cannulae. The distal,and sometimes the proximal, suture lines are rein-forced with a strip of Teflon felt.

After the air has been excluded from the heart,cardiopulmonary bypass is gradually discontinued,and the heart is allowed to resume the load ofcirculation. When systemic and venous pressures areadequate, decannulation of the heart is performed.Atrial and ventricular pacing wires are placed and,after securing haemostasis and draining the peri-cardium, the chest is closed in layers. It is ofparticular importance to achieve haemostasis; theuse of topical thrombin applied over a steri-spongeis helpful in controlling a persistent ooze along thesuture line.Should a coronary artery, and, in particular, the

right coronary artery, emerge from the aneurysmitself, the coronary ostia with a circumference of

FIG. 3. Typical appearance of aneurysm of the aorticannulus. The right coronary (arrow) arises high withinthe aneurysm.

A....

FIG. 4. Aortic valve regurgitation due to dilatation ofthe annulus. All three cusps appear normal.

FIG. 5. Diagram showing a prosthetic aortic valve inplace. The coronary perfusion lines are passed throughthe Dacron tubular graft, so coronary perfusion is con-tinuous throughout the procedure.

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aortic wall is excised and reimplanted into theDacron graft with a running suture of 4-0 Mersilene(Fig. 6). Wheat, Wilson, and Bartley (1964) havedescribed an alternative: a tongue of aortic wallfrom the aortic annulus to the coronary ostia ispreserved and a slit is then made in the prostheticgraft incorporating the tongue of aortic wall into thegraft (Fig. 7).

ANALYSIS OF SURGERY

Thirteen patients had large fusiform ascendingaortic aneurysms of the 'inverted' type; most ofthem had very thin walls. A double aortic lumen

FIG. 6. When the right coronary emerges from theaneurysm itself, the coronary ostia with a small circum-ference of aortic wall is excised and re-implanted intothe Dacron graft.

typical of chronic dissection was found in sixpatients (Fig. 8). In only one patient was theascending aorta slightly dilated without dissectionor aneurysm. All 14 patients had typical findingsof cystic medial necrosis of the aortic wall (Fig.9), even the patient without a large aneurysm.The aneurysms all tended to extend down to theaortic annulus.Of the entire group, five patients had bicuspid

valves. All the valves were attenuated. Histo-pathology was consistent with Marfan's syndrome

FIG. 7. In some cases a tongue of aortic wall from theaortic annulus to the coronary ostia is preserved andincorporated in the Dacron graft (Wheat et al., 1964).

cm5

Jr s

4 -

3-

2

FIG. 8. Double aortic lumen, a typical finding of chronicdissection found at surgery in six of our patients.

in onlyhave areplace

three; most of the valves were found tonormal architecture. It was necessary tothe valve in 13 instances. Starr-Edwards

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Donald N. Ross, Thomlas G. Fr-azier, and Lo-enzo Gonzalez-Lavin

ball valves were inserted in 10 patients. A homo-graft was placed in one patient; it became re-gurgitant six months postoperatively and was sub-sequently replaced with a Starr-Edwards valve.Two patients underwent supported fascia lataaortic valve replacements. The other patient ex-hibited only minimal aortic regurgitation and thevalve was left in place.

.si - .-

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FIG. 9. A mild aortic lesion. Photomicrograph of ascendingarch of aorta stained to show elastic tissue (black). lntima(above) shows m1inimal thickening only. Adventitia (largelyabsent) was normal elsewhere. Greater part of section ishere formed by media. Elastica of its outer third is normal.As lumen is approached, progressively larger breaks inelastica appear; these lacunae proved to be filled bysmooth muscle and acid muncopolysaccharide ground-suibstance-both locally in excess. Verhoeff/van Giesonx 20.

Resection of the ascending aorta to the in-nominate artery and replacement with a Dacrontubular graft was carried out in 10 instances, fourof which were accompanied by right coronaryartery re-implantation. In three patients a repairof the aorta was carried out with a Dacron patchafter oversewing the double lumen of the aorta,and in one case, with only slight dilatation of theaorta, no resection was necessary.

RESULTS

Among the 14 patients there was one operativedeath, an operative mortality of 70,',. This oc-curred in the first patient, who died with apersistent ventricular fibrillation.

Only one late death occurred, 45 days aftersurgery (700o). This patient ran a persistentlyfebrile course with arrhythmias to which he suc-cumbed. Necropsy revealed a massive infarctionof 'the right ventricle but no thrombosis of thepreviously implanted right coronary artery.

All the survivors are doing well six months tofour and a half years after surgery. Only onepatient has evidence of aortic regurgitation-thepatient in whom the aortic valve was left in placeat the time of surgery: it has not shown any in-crease in two and a half years.

DISCUSSION

Cystic medial necrosis is characterized by an ab-normal increase of mucopolysaccharides in thetunica media of the arterial tree. This materialaccumulates and forms cystic areas which separateand compress the normal elastic fibres. The fibresbecome thinner and fragmentation frequentlyoccurs with the end result of a weakened arterialwall. As the ascending aorta is the segment ex-posed to the distending forces of the left ven-tricular stroke and subjected to the highestpressure (Laplace Law), it is not surprising thatit is the most common location of aneurysm inpatients with cystic medial necrosis. Theseaneurysms are localized, as seen in 13 patients inthis series, and therefore amenable to surgicalresection. Only occasionally do they extendbeyond the innominate artery (Bloodwell,Hallman, and Cooley, 1966). Excision and re-placement with a tubular prosthetic graft is man-datory to avoid further distension of the arterialwall.Changes in the connective tissue usually result

from the aortic annulus with separation of thetunica media from the annulus fibrosus (Edwards,Burchell, and Christensen, 1956). Weakness occursin this area and progressive dilatation may followwhich renders even a normal aortic valve severelyregurgitant. The aortic valve may be completelynormal or exhibit changes characteristic of thisdisease entity. Three valves in our series hadtypical cystic degeneration. Bicuspid valves maybe found in 2000/ of patients with cystic medialnecrosis (Tung and Liebow, 1952), or perhaps ineven a higher incidence; five of our 14 patients

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had bicuspid valves (35 7%). When replacing theaortic valve, a rigid prosthesis is indicated to avoidfurther dilatation of the aortic annulus. In thepresent series, a homograft valve became regurgi-tant six months after surgery and was successfullyreplaced with a prosthesis. The homograft valvewas well preserved but rendered regurgitant be-cause of progressive annular dilatation. We doubtthe wisdom of using autologous fascia lata be-cause of the systemic alterations to connectivetissue in this disease.The right coronary artery, located slightly

higher than the left in relation to the aorticannulus, is frequently involved in the aneurysmalprocess, as noted in four of our patients. Thisproblem was approached by excision of thecoronary ostia with a patch of aortic wall andfree re-implantation into the prosthetic tubulargraft. One of us has used the method of Wheatand colleagues in two cases, not included here,with excellent results.

In the light of our experience and that of others(Bloodwell et al., 1966; Muller, Dammann, andWarren, 1960; Ellis, Cooley, and De Bakey, 1961;Blanchard, Felder, Culligan, and MacLean, 1963;Groves, Effler, Hawk, and Gulati, 1964;Chapman et al., 1965; Merendino, Winterscheid,and Dillard, 1967), the surgical treatment of patientswith annulo-aortic ectasia and aortic regurgitationsecondary to cystic medial necrosis should con-sist of resection of the aneurysm and replacementwith a tubular prosthetic graft, and aortic valvereplacement with a rigid prosthesis. The techniqueof free implantation of the right coronary arteryinto the prosthetic graft is recommended whenthis artery arises from the aneurysm. This surgicalapproach has given us good results; 8577% ofthe patients in this series of 14 survive, and aredoing well, six months to four and a half yearsafter surgery.

We are grateful to Dr. G. A. K. Missen, Departmentof Pathology, Guy's Hospital, London, for selectingand preparing the photomicrograph in Fig. 9, andto Miss Julie Brunner, S.R.N. for the diagrams used.

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Congenital aneurysmal dilatation of the aorta associatedwith arachnodactyly. Bull. Johns Hopk. Hosp., 72, 309.

Bahnson, H. T., and Nelson, A. R. (1956). Cystic medialnecrosis as a cause of localized aortic aneurysmsamenable to surgical treatment. Ann. Suirg., 144, 519.

Blanchard, R. J. W., Felder, D. A., Culligan, J. A., andMacLean, L. D. (1963). Cardiovascular surgery inMarfan's syndrome. Ann. Surg., 158, 990.

Bloodwell, R. D., Hallman, G. L., and Cooley, D. A. (1966).Aneurysm of the ascending aorta with aortic valvularinsufficiency. Arch. Surg., 92, 588.

Chapman, D. W., Beazley, H. L., Peterson, P. K., Webb,J. A., and Cooley, D. A. (1965). Annulo-aortic ectasiawith cystic medial necrosis. Diagnosis and surgicaltreatment. Amer. J. Cardiol., 16, 679.

Edwards, J. E., Burchell, H. B., and Christensen, N. A.(1956). Specimen exhibiting the essential lesion inaneurysm of the aortic sinus. Proc. Maylo Clin., 31, 407.

Ellis, P. R., Cooley, D. A., and De Bakey, M. E. (1961).Clinical considerations and surgical treatment ofannulo-aortic ectasia. J. thorac. cardiovasc. Surg.,42, 363.

Erdheim, J. (1930). Medionecrosis aortae idiopathica cystica.Virchows Arch. f. path. Anat., 276, 187.

Gore, 1. (1952). Pathogenesis of dissecting aneurysm of theaorta. Arch. Path., 53, 142.

Groves, L. K., Effler, D. B., Hawk, W. A., and Gulati, K.(1964). Aortic insufficiency secondary to aneurysmatchanges in the ascending aorta: Surgical management.J. thorac. cardiovasc. Surg., 48, 362.

Macleod, M., and Williams, A. W. (1956). The cardiovascu-lar lesions in Marfan's syndrome Arch. Path., 61, 143.

McKusick, V. A. (1955). The cardiovascular aspects ofMarfan's syndrome: A heritable disorder of connectivetissue. Circulation, 11, 321.

Marfan, A. B. (1896). Un cas de deformation congenitaledes quatre membres, plus prononcee aux extremites,characterisee par I'allongement des os avec un certaindegre d'amincissement. Bull. Soc. Med. Hop. Paris, 3eser., 13, 220.

Marvel, R. J., and Genovese, P. D. (1951). Cardiovasculardisease in Marfan's syndrome. Amer. Heart J., 42, 814.

Merendino, K. A., Winterscheid, L. C., and Dillard, D. H.(1 967). Cystic medial necrosis with and without Marfan'ssyndrome. Surgical experience with 20 patients and a noteabout the modified bicuspidization operation. Surg. Clin.N. Amer., 47, 1403.

Muller, W. H., Jr., Dammann, J. F., Jr., and Warren, W. D.(1960). Surgical correction of cardiovascular deformitiesin Marfan's syndrome. Ann. Surg., 152, 506.

Schorr, S., Braun, K., and Wildman, J. (1951). Congenitalaneurysmal dilatation of the ascending aorta associatedwith arachnodactyly: an angiographic study. Amer.Heart J., 42, 610.

Sloper, J. C., and Storey, G. (1953). Aneurysms of the as-cending aorta due to medial degeneration associatedwith arachnodactyly (Marfan's disease). J. dlin. Path.,6, 299.

Sutton, D. (ed.) ( 1969). A Textbook ofRadiology. Livingstone:Edinburgh.

Tung, H. L., and Liebow, A. A. (1952). Marfan's syndrome:Observations at necropsy: with special reference tomedionecrosis of the great vessels. Lab. Invest., 1, 382.

Wheat, M. W., Jr., Wilson, J. R., and Bartley, T. D. (1964).Successful replacement of the entire ascending aortaand aortic valve. J. Amer. med. Ass., 188, 717.

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