The Marfan Syndrome: Surgical Technique and Follow-up in 50 Patients

Transcription

1 The Marfan Syndrome: Surgical Technique and Follow-up in 50 Patients Roberto Gallotti, M.D., and Donald N. Ross, F.R.C.S. ABSTRACT Fifty patients with Marfan s syndrome underwent operation at the National Heart Hospital because of cardiovascular complications. Fortysix had an aneurysm of the ascending aorta, 13 had chronic dissection, and 6 had acute dissection of the aortic wall. Forty-three aortic valves were incompetent, and five were stenotic and incompetent. One mitral valve had minor regurgitation. The Starr- Edwards prosthesis was used in 36 patients, homograft valves in 4, fascia lata valves in 2, and xenograft valves in 6. The ascending aorta was replaced with a Dacron tube in 40 patients and with an aortic homograft in 2. Three patients required Dacron patches over the aneurysm, and 1 patient had plication of the aortic wall. Early mortality totaled 12% (6 patients). Only 1 of these patients died in the last five years. Reoperations for homograft incompetence, periprosthetic leak, and acute dissection of an unreplaced aorta resulted in 1 hospital death (33%). Forty-three patients have been followed for up to 8.5 years (mean, 3.5 years), with 7 late deaths (16.2%). The improvement in recent surgical results with decreased operative mortality supports an aggressive surgical approach to Marfan s syndrome in view of the poor prognosis for the natural history of this disease. Marfan s syndrome was described in 1896 as a hereditary disorder of connective tissue with skeletal, optical, and cardiovascular lesions The cardiovascular lesions are more dramatic since they can be the cause of early deaths [15, 16, 181. Cases in which all these lesions are present are classified as typical Marfan s syndrome; those with only cardiovascular lesions are classified as forme fruste [lo, 26,273. The cardiovascular lesion is characterized by a dilatation of the proximal ascending aorta, From the National Heart Hospital, Middlesex Hospital, and Cardiothoracic Institute, London W1, England. Accepted for publication Sept 12, Address reprint requests to Dr. Gallotti, Department of Surgery, Cardiothoracic Institute, 2 Beaumont St, London W1N ZDX, England. which assumes a flask shape. This aortic dilatation includes the valve annulus and usually results in aortic incompetence 111, 22, 231. Aortic wall dissection is a grave complication and can cause rupture, sudden occlusion, or aortic insufficiency if it dissects proximally or there may be distal dissection with rupture or occlusion of branch vessels 111, 171. Marfan s syndrome also can involve the mitral valve, with prolapse of one or both cusps, but mitral incompetence is infrequent; when it occurs, it is usually due to ruptured chordae [2, 4, 21, 241. The first corrective operations in the early 1960s consisted of replacement of the ascending aorta together with repair or replacement of the aortic valve. The results of these operations were not encouraging because of recurrent aortic regurgitation, frequent periprosthetic leaks, and hemorrhage from suture lines 18, 17, 261. Material Between October, 1965, and April, 1978, in the National Heart Hospital 50 patients with Marfan s syndrome underwent operation. There were 42 male and 8 female patients ranging in age from 11 to 64 years (mean, 40.7 years). Skeletal, optical, and cardiovascular lesions typical of Marfan s were present in 29 patients. Family history was positive in only 6. Twentyone had forme fruste. Of all these patients, 18 (36%) had a previous history of angina. Fortythree (86%) had sustained one or more episodes of cardiac failure due to aortic regurgitation. Specification by Functional Class was as follows: 3 patients (6%) belonged to New York Heart Association (NYHA) Class 11, 28 (56%) belonged to Class 111, and 19 (38%) belonged to Class IV. Nine patients (18%) required emergency operation, and 6 of them had symptoms of acute dissection. In all patients the clinical diagnosis was confirmed by radiology and angiography. Prolapse of the anterior leaflet of the mitral valve was detected angiographically in 10 patients and by Roberto Gallotti

2 429 Gallotti and Ross: The Marfan Syndrome was doubtful in another 3. Echocardiography, which was carried out in 15 patients, confirmed the diagnosis, with particular reference to the characteristics of the mitral valve, the aortic dilatation, and the existence of a double lumen when there was a dissection. All patients had electrocardiographic manifestations of left ventricular overload. In all except 4 patients there was evidence at operation of the characteristic aneurysmal dilatation of the ascending aorta. In 27 patients, there was an aneurysm without aortic dissection; in 13, an aneurysm with chronic aortic dissection; and in 6, an aneurysm with acute aortic dissection. In 13 patients, there was additional evidence of a chronic dissection of the aorta with endothelialization of the false lumen. Six patients were seen with an acute dissection of the aorta. This was localized near the valve and in the region of the coronary ostia. In 4 patients, the intima was circumferentially dissected. In 1 patient, the right coronary ostium also was involved, but was not obstructed. The aortic valve cusps were usually thin and attenuated. In 43 patients, the aortic valve was grossly regurgitant due to dilatation of the aortic annulus. Only 1 of these patients had mitral incompetence as well. In 5 patients, however, there were both aortic stenosis and incompetence due to calcium in the cusps, which appeared thick and fibrotic. In 8 patients, the aortic valve was bicuspid. In 1 of them the valve lesion was accompanied by subaortic stenosis, and in another, there was evidence of a previous repair of aortic coarctation. Two patients had normal aortic valves. Exploration of the mitral valve in 10 patients showed a prolapse of the anterior leaflet, but in only 1 was there evidence of minor mitral regurgitation. In 6 patients, the right coronary 0stium was at some distance from the aortic valve due to stretching of the anterior aortic wall. This phenomenon involved the left coronary ostium only once. Surgical Techniques Hypothermic cardiopulmonary bypass was used in all patients. When possible, the aortic arch was used for cannulation at the level of the innominate artery. When this was difficult either because of the size of the aneurysm or the presence of a dissection, the femoral artery was cannulated. Uninterrupted perfusion of the coronary ostium with pump blood at 30" to 37 C was used routinely to protect the myocardium. The aortic valve was replaced in all but 2 patients who had minimal or absent regurgitation at the time of angiography. In 36 patients, a Starr-Edwards prosthesis was inserted and in the remaining 12 patients, a biological valve: 4, an aortic homograft; 2, a stented fascia lata graft; and 6, a Carpentier-Edwards xenograft. The ascending aorta was resected and replaced with a Dacron tube in 40 patients. In 3 patients, the aortic wall was repaired with a Dacron patch after the double lumen of the dissection had been oversewn. In 2 patients, the aortic root was replaced with a length of aortic homograft. In 1 of these patients, it was reinforced with a tubular length of externally applied Dacron. One patient had only a wide elliptical excision of the aorta, and 4 others had no aorta resected because there was only modest dilatation. The Dacron conduit was inserted using five different techniques. In the first 7 patients, it was anastomosed to the aortic root just above the coronary ostia where the aorta had been transected because the aortic root itself did not appear to be dilated. In 6 patients, the right coronary orifice was displaced distally from stretching of the anterior aneurysmal wall. Therefore, the coronary ostium was circumscribed with a disc of aortic wall and reimplanted into the side of the Dacron prosthesis. In another 6 patients, the Dacron prosthesis was presewn directly to the ring of the artificial valve and both coronary arteries were reimplanted into the Dacron tube. In 2 of these 6 patients, the right coronary-artery conduit continuity was achieved with an interposed saphenous vein graft, in 1 because the dissection involved the right coronary ostium and in the other because it was necessary to sacrifice the origin of the right coronary artery to control bleeding. On five occasions a tongue of aortic wall was preserved to include the coronary ostia and was attached to the Dacron tube, which was appropriately tailored to fit without kinking or traction [28].

3 430 The Annals of Thoracic Surgery Vol 29 No 5 May 1980 Surgical technique used in repair of Marfan s syndrome. In 16 patients we used the technique proposed by Bentall and De Bono [l, 251 and popularized by Edwards and Kerr [91. The aneurysm was opened longitudinally, and coronary cannulae were inserted (Figure, A). An appropriately sized mechanical prosthesis or xenograft was sewn into a preclotted Dacron prosthesis of the same diameter as the aortic arch with a continuous suture of Prolene (Figure, B). The aortic cusps were excised (Figure, C), and the valved conduit was sutured to the cusp remnant with a continuous 2-0 Prolene suture, interrupted at several points, plus some additional sutures (Figure, D). At this stage, the suture line was tested with the heart beating, the conduit was clamped, and the left ventricular vent was closed temporarily. Two holes about 1 cm in diameter were excised from the distended Dacron tube opposite the coronary ostia. The coronary cannulae then were repositioned through the conduit and out the holes. The internal wall of the aneurysm around the coronary ostium was sutured to the windows with a continuous 5-0 Prolene suture (Figure, E). The distal anastomosis to the aorta was completed after the coronary cannulae were removed (Figure, F). The aneurysmal sac, freed from adhesions and after tailoring, was then wrapped around the prosthesis and sewn with a loose mattress suture (Figure, G). The suture was tightened as soon as coagulation seemed satisfactory so as to avoid the formation of a hematoma between the sac and prosthesis. In 6 patients with acute dissecting aneurysms involving the arch at the level of the innominate artery, the false lumen was obliterated distally with a whip stitch, sometimes reinforced with a strip of Teflon felt, before the prosthesis was anastomosed to the aortic arch. In the patient with calcific aortic valve disease and a muscular subvalvular stenosis, an additional vertical myotomy was done on the ventricular septum. Results There were 6 (12%) hospital deaths within 45 days of operation (Table). The first patient died of intractable ventricular fibrillation at the end of the operation. One patient had sudden bleeding from the proximal suture line at the end of the procedure, followed by hypotension, cardiac arrest, and serious cerebral damage, which resulted in death several days later. One patient died of neurological and pulmonary complications 25 days after the orotracheal tube

4 431 Gallotti and Ross: The Marfan Syndrome Deaths in 50 Patients with Marfan's Syndrome Treated Surgically Deaths No. of Patients Percent Hospital mortality after op eration Hospital mortality after reoperation Hospital mortality in the last 5 yr (22 patients) Late mortality (43 patients available for follow-up) over 8.5 yr (avg, 3.5 yr) accidentally became kinked and caused cardiac arrest on the first postoperative night. The patient who had an additional myotomy for subaortic muscular stenosis had three emergency reoperations within the first forty-eight hours because of bleeding and cardiac arrest due to atrioventricular block. He died of progressive cardiovascular deterioration after 5 days. One patient died 45 days postoperatively of a right ventricular infarct incurred after operation and complicated by arrhythmias. The reimplanted right coronary artery was open at postmortem examination. Another patient died in coma 15 days postoperatively and, at postmortem examination, the cause of the cerebral damage was attributed to further dissection of the aorta. Postoperative bleeding necessitated emergency reopening of the chest on three occasions. In 2 patients, the bleeding was from the apex of the left ventricle and in 1, from a dehiscence of the proximal suture line. Atrial and ventricular ectopia secondary to atrioventricular dissociation and rapidly followed by return of sinus rhythm occurred in 8 patients in the first forty-eight hours after operation. One patient had two episodes of rapid ventricular tachycardia associated with a myocardial infarct on the fifth and sixth postoperative days. This patient was successfully converted to sinus rhythm. Three patients were discharged in atrial fibrillation. Atrioventricular dissociation developed in 1 patient at the time of reoperation for bleeding, and a pacemaker was implanted. Three patients had late reoperation. The first had a second operation 6 months after aortic homograft replacement. There was progressive dilatation of the valvular annulus and tearing of a commissure, which led to a sudden regurgitation. The incompetent homograft was replaced with a Dacron conduit bearing a Starr- Edwards valve. This patient is alive 6 years after reoperation. The patient who had an aortic valve replacement with a Starr-Edwards prosthesis but preservation of the apparently normal ascending aorta had an emergency reoperation 4 years 10 months postoperatively for acute dissection of the ascending aorta. The ascending aorta was replaced with a Dacron tube, and his condition appeared totally satisfactory 1 year after the second operation. The third patient was reoperated on a year after surgical repair with a Dacron tube bearing an aortic Starr-Edwards valve. There was a periprosthetic leak caused by a staphylococcal infection. The valve was replaced with an aortic homograft, but the patient died of myocardial infarction 2 days postoperatively with irreversible dysrhythmias (see Table). There was a total of 7 late deaths among the 43 patients followed-up (16.2%) (see Table). One patient is living abroad, and follow-up data were not available. Two died of myocardial infarction 9 months and 35 months after operation. Postmortem examination of the first revealed that a hematoma between the conduit and the external aortic wall caused compression of the origin of the coronary artery. Another patient died of infective endocarditis on the prosthesis 4 years 10 months after operation. Postmortem examination showed many vegetations on the prosthesis causing malfunction and a large abscess behind the sternum originating at the base of the tubular Dacron prosthesis. One patient died of carcinoma in the left lower lobe 37 months after repair. One patient died of a myocardial infarction 2 days following reoperation, and a further 2 patients died of unknown cause 45 and 20 months postoperatively. Both had been asymptomatic. The remaining 36 patients have been followed for 6 months to 8% years (average, 3% years). One patient with an aortic Starr- Edwards prosthesis had an embolic episode with residual hemianopsia 2 months postoperatively. One with a homograft valve has a diastolic murmur, and a new hemodynamic study is planned. The mitral valve, which was

5 432 The Annals of Thoracic Surgery Vol29 No 5 May 1980 known to be prolapsing but competent at exploration, has become moderately incompetent in 1 patient during the subsequent 3 years. In the patient who had a demand pacemaker inserted for atrioventricular block, the pacemaker was found to be pacing only sporadically at 6 months after operation but the circulatory state was good. The remaining 32 patients are in a satisfactory condition and a good hemodynamic state. In 70% of them, heart volume has been reduced significantly, and the Functional Class has improved substantially in 65%. Comment Cardiovascular lesions in patients with Marfan s syndrome are the chief cause of the poor life expectancy. They are present in 30 to 60% of patients [4,11,15,21]. They were recognized as the cause of death in 92.8% of patients with Marfan s syndrome studied by Murdoch and co-workers [MI. The most common cause of death is left ventricular failure due to aortic incompetence; sudden deaths are usually due to aortic dissection but are not frequent [ll]. The average age of patients with Marfan s syndrome who did not undergo operation in the different series ranges from 30 to 45 years [3, 12, 181. Unsuccessful surgical therapy in a patient with Marfan s syndrome was reported in 1957 by Dimond and associates [B], who used a Hufnagel valve positioned in the descending aorta. In 1960, Muller and co-workers [17] successfully replaced the aneurysmal ascending aorta and carried out bicuspidization of the valve in three patients with Marfan s disease. In a full revision of the literature in 1970, Symbas and colleagues [26] reported a mortality of 31.6% in 9 patients operated on up to that time. All the unsuccessful operations were attributed to the poor coaptation of the defective inelastic connective tissue to the graft. In the last 10 years, improvements in diagnostic methods and especially refinements of surgical techniques have provided encouraging results. The method proposed by Bentall and De Bono [l] in 1968 offers a simplified and more rapid operation with less risk of bleeding, which is borne out by subsequent surgical series. Against a hospital mortality of 20% and a survival of 42.8% in 30 patients operated on before 1969, Nasrallah and associates [191 re- ported a hospital mortality of 13% in the patients having operation in the following 5 years. Crosby and co-workers 151 in 1973 referred to three patients operated on successfully and living 2% years postoperatively. A group from the Mayo Clinic [61 reported that among 31 patients operated on for Marfan s syndrome up to June, 1976, total hospital deaths were 29% but that 8 of the 9 deaths happened before or during Their follow-up for the first 10 years (mean, 4.8 years) showed a late mortality of 16%. In 7 patients with Marfan s syndrome operated on by the Bentall and De Bono procedure, Koizumi and colleagues [131 reported a hospital mortality of 14.2% and a late mortality of 16.6% after a mean follow-up of 2 years. In our series, only 1 hospital death (4.5%) occurred in the last 5 years out of 22 patients operated on mainly with the Bentall and De Bono procedure (see Table). Late mortality was 16.2%. A possible disadvantage of the procedure of Bentall and De Bono is the creation of a hematoma between the tubular prosthesis and the original aortic wall. This can cause compression of the origin at the coronary artery and lead to death, as in 1 of our patients, or it can cause supravalvular aortic stenosis, as reported in a patient operated on by Crosby and coworkers [51. On the other hand, the method has the advantage of containing the bleeding from a leaking conduit. This is a safety factor since 1 of our patients was successfully reoperated on for a late leak at the lower suture line at 12 months. The mitral valve was successfully replaced in a patient with Marfan s syndrome by Dietzman and colleagues [7] in Two years later, Simpson and co-workers [24] replaced the mitral valve in a 4-year-old girl with Marfan s syndrome and in gross failure due to ruptured chordae. Nelson and Vaughn [20] in 1968 carried out both aortic and mitral valve replacements in a patient with Marfan s syndrome. They emphasized the advantages of replacing the defective mitral valve at the first operation to try to avoid a second operation for this inevitably progressive disease. The mitral valve was replaced in only 4 of the 31 patients at the Mayo Clinic [61. In one echocardiographic study of Marfan s disease, Brown and colleagues [4] found the existence of mitral prolapse in 91% of the pa-

6 433 Callotti and Ross: The Marfan Syndrome tients examined. They observed that the incidence of mitral prolapse was the same in adults and children of the same sex and deduced that the mitral prolapse did not seem to increase with age and produce mitral incompetence. In our series of patients who underwent operation for Marfan s syndrome, the mitral valve was never replaced in spite of cineangiographic and echocardiographic evidence of prolapse of one or both leaflets in more than 20% of the patients studied. Modest clinical mitral incompetence was present in only 1 patient and was not corrected. The regurgitant mitral murmur has become increasingly obvious in the 3 following years but the regurgitation is tolerated well. In conclusion, because the natural history of these patients offers a grave prognosis and since recent surgical results are acceptable, it now seems justifiable to offer surgical treatment in the management of patients with Marfan s syndrome. References 1. Bentall HH, De Bono A: A technique for complete replacement of the ascending aorta. Thorax 23:338, Bowden DH, Pavara BE, Donahue JL: Marfan s syndrome accelerated course in childhood associated with lesions of mitral valve and pulmonary artery. Am Heart J 69:96, Bowers D, Lim DW: Subacute bacterial endocarditis and Marfan s syndrome. Can Med Assoc J 86:455, Brown OR, Demots H, Kloster FE, et al: Aortic root dilatation and mitral valve prolapse in Marfan s syndrome. Circulation 52:651, Crosby IK, Ashcroft WC, Reed WA: Surgery of proximal aorta in Marfan s syndrome. J Thorac Cardiovasc Surg 66:75, Davis Z, Pluth JR, Giuliani ER: The Marfan syndrome and cardiac surgery. J Thorac Cardiovasc Surg 75:505, Dietzman RA, Peter ET, Wang Y: Mitral insufficiency in Marfan s syndrome. Dis Chest 51:650, Dimond EG, Larsen WE, Johnson WB, et al: Post traumatic aortic insufficiency occurring in Marfan s syndrome, with attempted repair with a plastic valve. N Engl J Med 256:8011, Edwards WS, Kerr AR: A safer technique for replacement of entire ascending aorta and aortic valve. J Thorac Cardiovasc Surg , Golden RL, Lackin H: The forme fruste in Marfan s syndrome. N Engl J Med 260:797, Goyette EM, Palmer PW: Cardiovascular le- sions in arachnodactyly. Circulation 7:373, Hirst AE, Johns VJ, Kime SW: Dissecting aneurysms of the aorta: a review of 505 cases. Medicine (Baltimore) 37:217, Koizumi S, Mohri H, Kagana Y, et al: Surgical treatment of annuloaortic ectasia: experience in 7 consecutive patients. Ann Thorac Surg 25:425, Marfan AB: Un cas de deformation congenitale des quatres membres, plus prononcee aux extrkmites, caractkride par l allongement des 0s avec un certain degre d amincissement. Bull Mem SOC Med Hop Paris 13:220, McKusick VA: The cardiovascular aspects of Marfan s syndrome: a heritable disorder of connective tissue. Circulation 11:321, McKusick VA: Heritable Disorders of Connective Tissue. Third edition. St. Louis, Mosby, 1966, pp Muller WH, Damman JF, Warren WD: Surgical correction of cardiovascular deformities in Marfan s syndrome. Ann Surg 152:506, Murdoch JL, Walker BA, Halpem BL, et al: Life expectancy and causes of death in the Marfan s syndrome. N Engl J Med , Nasrallah AT, Cooley DA, Goussous Y, et al: Surgical experience in patients with Marfan s syndrome: ascending aortic aneurysm and aortic regurgitation. Am J Cardiol 36:338, Nelson RM, Vaughn CC: Double valve replacement in Marfan s syndrome. J Thorac Cardiovasc Surg 57:732, Phornphutkul C, Rosenthal A, Nadas AS: Cardiac manifestations of Marfan s syndrome in infancy and childhood. Circulation 47:587, Prokop EK, Palmer RF, Wheat MW Jr: Hydrodynamic forces in dissecting aneurysms. Circulation 38:Suppl 6:158, Ross DN, Frazier TG, Gonzalez-Lavin L: Surgery of Marfan s syndrome and related conditions of the aortic root (annulo-aortic ectasia). Thorax 27:52, Simpson JW, Nora JJ, McNamara DG: Marfan s syndrome and mitral valve disease; acute surgical emergencies. Am Heart J 77:96, Singh MP, Bentall HH: Complete replacement of the ascending aorta and the aortic valve for treatment of aortic aneurysm. J Thorac Cardiovasc Surg 63:218, Symbas PN, Baldwin FBJ, Silverman ME, et al: Marfan s syndrome with aneurysm of ascending aorta and aortic regurgitation. Am J Cardiol 25:483, Weaver WF, Edwards JE, Brandeburg RO: Idiopathic dilatation of the aorta with aortic valvular insufficiency: a possible forme fruste of Marfan s syndrome. Proc Staff Meet Mayo Clin 34:518, Wheat MW Jr, Wilson JR, Bartley TD: Successful replacement of entire ascending aorta and aortic valve. JAMA 188:99, 1964