Pharmacologically induced preconditioning with diazoxide: a novel approach to brain protection.

BACKGROUND: Ischemic preconditioning is an endogenous mechanism whereby brief periods of ischemia render neurons resistant to subsequent lethal insults. This protection appears to alter cellular apoptosis and can be induced by potassium channel openers acting on the inner membrane of the mitochondria (mitoK(ATP)). To test the hypothesis that pharmacologic preconditioning could provide neuroprotection, the mitoK(ATP) opener diazoxide was used in a canine model of brain injury induced by hypothermic circulatory arrest (HCA). METHODS: Seventeen dogs were placed on cardiopulmonary bypass (CPB) and cooled to 18 degrees C. After 2 hours of HCA, animals were rewarmed and weaned from CPB. Six dogs received intravenous diazoxide (2.5 mg/kg bolus 15 minutes prior to CPB, then 0.5 mg/min until circulatory arrest, then restarted for the first hour of rewarming). Six animals received vehicle only. Five received diazoxide and the mitoK(ATP) blocker 5-hydroxydecanoate (5-HD). Using a modified Pittsburgh Canine Neurological Scoring System (0 = normal, 500 = brain death), animals were evaluated every 24 hours for 3 days. The brains were removed and histologic sections of four regions characteristically injured in this model were scored (0 = no injury, 4 = infarction) by a neuropathologist in a blinded fashion. RESULTS: Clinical scoring showed marked improvement in the diazoxide group at 48 hours (101 +/- 10.5 vs 165 +/- 14.8, p < 0.01) and 72 hours (54 +/- 9.3 vs 137 +/- 12.1, p < 0.01). This neuroprotection was attenuated when 5-HD was concomitantly administered. Three of four brain regions typically injured in this model (cortex, hippocampus, and entorhinal cortex) had significant neuron preservation in the diazoxide group. Likewise, combined region scores were significantly improved in the treatment group (1.18 +/- 0.2 vs 2.46 +/- 0.2, p < 0.01). CONCLUSIONS: Pretreatment with diazoxide resulted in significant improvement in both clinical neurologic scores and histopathology in our model of HCA. This suggests that pharmacologic preconditioning with the mitoK(ATP) channel opener diazoxide may offer effective neuroprotection during HCA.

Serendipity and the development of heparin and carbon surfaces.

In the early 1960s, bileaflet valves fabricated with polymer housings routinely thrombosed within a few hours after implantation in the canine heart. In a serendipitous series of events, the authors found a way to bond heparin to these bileaflet valves using a coating of graphite-carbon and benzalkonium chloride. Over the ensuing 30 years, improved heparin coatings have been developed by other investigators for bonding to various biomedical devices; currently, about 25% of oxygenators used in this country utilize heparin coatings to minimize surface activation of clotting factors. Also, and somewhat serendipitously, a pyrolytic carbon material developed in the 1960s as a coating for nuclear fuel rods was submitted to the authors' laboratory for possible coating with benzalkonium and heparin. This carbon coating, developed at Gulf General Atomic, Inc, would not bond heparin, but it proved to be the best rigid material available for prosthetic valve construction; more than one million pyrolytic carbon valves have been clinically implanted over the last 29 years.

Operative management of Marfan syndrome: The Johns Hopkins experience.

BACKGROUND: Doctor Antoine Marfan described the first case of Marfan syndrome in 1896. It was over 50 years later that the development of aortic aneurysms and subsequent rupture was appreciated as the most life-threatening component of the syndrome. METHODS: Doctor Vincent Gott, at our institution, performed the first Bentall procedure for an aneurysm of the ascending aorta in 1976. Since that time, the aortic root has been replaced in 231 Marfan patients. Of this group, 218 patients had a composite graft repair, 11 had an aortic root replacement with a homograft, and 2 patients had valve sparing procedures. There were 168 males and 63 females. Of the total 231 patients, 150 were operated on by Dr Gott. The remaining 81 patients were operated on by 10 other Hopkins surgeons. The average diameter of the ascending aorta was 6.8 cm, with a range from 4.5 to 10. The average aortic diameter of 43 patients who had an ascending aortic dissection was 7.3 cm. Fourteen of these patients had dissection with an aortic diameter of 6.5 cm or less. RESULTS: Among the 198 patients who underwent elective repair, there was no 30-day mortality. Thirty-three patients underwent urgent repair with 2 deaths, yielding a 30-day mortality of 6.1%. The mortality for the entire group of patients was 0.9%. Complications associated with this series of patients included 8 with endocarditis, 7 with thromboembolism, and 4 late coronary dehiscences. Actuarial survival was 88% at 5 years, 81% at 10 years, and 75% at 20 years. Multivariate analysis revealed New York Heart Association classification, male gender and urgent surgery as independent risk factors for mortality. CONCLUSION: Marfan patients with aortic aneurysms can undergo elective surgery with a low operative risk and excellent long-term survival with low morbidity. We feel that elective resection of an aneurysm in a Marfan patient should occur when it approaches a diameter of 5.5 cm. It is essential that a timely diagnosis be made in this group of young patients.

Replacement of the aortic root in patients with Marfan's syndrome.

BACKGROUND: Replacement of the aortic root with a prosthetic graft and valve in patients with Marfan's syndrome may prevent premature death from rupture of an aneurysm or aortic dissection. We reviewed the results of this surgical procedure at 10 experienced surgical centers. METHODS: A total of 675 patients with Marfan's syndrome underwent replacement of the aortic root. Survival and morbidity-free survival curves were calculated, and risk factors were determined from a multivariable regression analysis. RESULTS: The 30-day mortality rate was 1.5 percent among the 455 patients who underwent elective repair, 2.6 percent among the 117 patients who underwent urgent repair (within 7 days after a surgical consultation), and 11.7 percent among the 103 patients who underwent emergency repair (within 24 hours after a surgical consultation). Of the 675 patients, 202 (30 percent) had aortic dissection involving the ascending aorta. Forty-six percent of the 158 adult patients with aortic dissection and a documented aortic diameter had an aneurysm with a diameter of 6.5 cm or less. There were 114 late deaths (more than 30 days after surgery); dissection or rupture of the residual aorta (22 patients) and arrhythmia (21 patients) were the principal causes of late death. The risk of death was greatest within the first 60 days after surgery, then rapidly decreased to a constant level by the end of the first year. CONCLUSIONS: Elective aortic-root replacement has a low operative mortality. In contrast, emergency repair, usually for acute aortic dissection, is associated with a much higher early mortality. Because nearly half the adult patients with aortic dissection had an aortic-root diameter of 6.5 cm or less at the time of operation, it may be prudent to undertake prophylactic repair of aortic aneurysms in patients with Marfan's syndrome when the diameter of the aorta is well below that size.

Antoine Marfan and his syndrome: one hundred years later.

In 1896, in the Bulletin of the Medical Society of Paris, Antoine Marfan described a five-year-old girl with arachnodactyly. It took almost 50 years to fully elucidate this syndrome including aneurysm of the ascending aorta. It is critical to make an early diagnosis of Marfan aneurysm because there is a high frequency of dissection and rupture once the aortic diameter reaches 6 cm. Before the availability of the Bentall composite graft procedure in 1968, the operative results were very poor. The Bentall operation now carries a 30-day mortality rate of less than 5% at major cardiac surgical centers. Two hundred and thirty-one Marfan patients underwent aortic root replacement at The Johns Hopkins Hospital between September 1976 and December 1997. The 30-day mortality for 198 patients undergoing elective aortic root replacement was 0%. Two of 33 patients undergoing urgent surgery died in the first 30 days after surgery; both of these patients arrived in the operating room with ruptured aortas. The actuarial survival for the 231 patients undergoing aortic root replacement was 88% at five years, 81% at ten years, and 75% at 20 years. Clearly, the outlook for Marfan patients undergoing elective aortic root replacement has been excellent. Accompanying the steadily improving surgical results have been spectacular developments in understanding the genetic role in Marfan families. Since 1991, over 150 mutations have been discovered in the gene that is critical in the production of the structural protein fibrillin. The identification of mutations in the fibrillin gene has enabled the diagnosis of Marfan disease in some patients before they become symptomatic; prenatal diagnosis has been achieved in some patients. The ultimate hope for Marfan families is to eliminate the disease by genetic manipulation; however, this may be years away.

Cardiac operations in children with Marfan's syndrome: indications and results.

BACKGROUND: The development of new screening techniques for the early detection of Marfan's syndrome has prompted evaluation of the results of cardiac operations in children with this syndrome. The purpose of this study was to determine the surgical indications, operative results, and need for reoperation in children with Marfan's syndrome. METHODS: From 1980 to 1996, 245 patients underwent cardiac operations for complications of Marfan's syndrome; 26 (11%) were less than 18 years of age. The mean age at the time of operation was 10.3 +/- 1 years (range, 8 months to 17 years); 18 of the patients were male. Indications for operation were aortic root dilatation (15 patients), mitral regurgitation (4 patients), aortic root dilatation and mitral regurgitation (6 patients), and aortic arch aneurysm (1 patient). Operations included aortic root replacement (15 patients), aortic root replacement and mitral repair (5 patients), aortic root replacement and mitral replacement (1 patient), mitral repair (3 patients), mitral replacement (1 patient), and arch aneurysm repair (1 patient). The mean aortic root diameter in patients undergoing aortic root replacement was 6.2 +/- 0.2 cm. Only 1 patient underwent ascending aortic dissection. RESULTS. There were no operative deaths. At a mean follow-up of 67.1 +/- 10.2 months, 8 patients required a second cardiac procedure (41% +/- 17% 10-year freedom from reoperation). Indications for further operations were distal aortic pathology (3 patients), aortic root dilatation after initial mitral operation (3 patients), failed mitral repair (1 patient), and homograft degeneration (1 patient). Risk factors for a second cardiac procedure were age less than 10 years at the time of the first operation (p < 0.003) and mitral regurgitation (p < 0.04). Overall, 25 (96%) of 26 patients have undergone aortic root replacement and 11 (42%) patients have undergone a mitral procedure. There have been 4 late deaths, all of presumed cardiac origin. The 10-year survival rate is 79% +/- 10%. All surviving patients are in New York Heart Association functional class I or II. CONCLUSIONS: We conclude that (1) aortic root dilatation is the most common surgical indication in children with Marfan's syndrome, (2) mitral regurgitation is the second most common indication, (3) aortic dissection is unusual in children with Marfan's syndrome, and (4) careful follow-up is necessary, particularly in younger children, because more than half of all children with Marfan's syndrome require repeated cardiac operations within 10 years.

The Marfan syndrome and the cardiovascular surgeon.

The authors present the current status of surgery for the cardiovascular manifestations of the Marfan syndrome. In addition, a brief review of current Marfan genetic research is presented. Data on all Marfan patients undergoing aortic root replacement at the Johns Hopkins Hospital (September 1976-June 1995) were analyzed. Survival and event-free curves were calculated and risk factors for early and late death were determined by univariate and multivariate analysis. Two hundred twelve Marfan patients underwent aortic root replacement using composite graft (202), homograft (8) or valve-sparing procedures (2). One hundred eighty-five patients underwent elective repair with no 30-day mortality. Twenty-seven patients underwent urgent surgery, primarily for acute dissection; two patients with aortic rupture died in the operating room. Actuarial survival of the 212 patients was 88% at 5 years, 78% at 10 years and 71% at 14 years. By multivariate analysis, only poor NYHA class, male gender and urgent surgery emerged as significant independent predictors of early or late mortality. Histologic examination of excised Marfan aortic leaflets by immunofluorescent staining for fibrillin showed fragmentation of elastin-associated microfibrils. These studies suggest cautious use of valve-sparing procedures in Marfan patients. Over the last 5 years significant progress has been made in identifying mutant genes that code for defective fibrillin microfibrils in Marfan patients. Attempts are underway to develop animal models of Marfan disease for study of possible gene therapy. Aortic root replacement can be performed in Marfan patients with operative risk under 5%. Long-term results are gratifying. At present, valve-sparing procedures should be used cautiously in Marfan patients because of fibrillin abnormalities in the preserved aortic valve leaflets.

Aortic root replacement. Risk factor analysis of a seventeen-year experience with 270 patients.

Between September 1976 and September 1993, 270 patients underwent aortic root replacement at our institution. Two hundred fifty-two patients underwent a Bentall composite graft repair and 18 patients received a cryopreserved homograft aortic root. One hundred eighty-seven patients had a Marfan aneurysm of the ascending aorta (41 with dissection) and 53 patients had an aneurysm resulting from nonspecific medial degeneration (17 with dissection). These 240 patients were considered to have annuloaortic ectasia. Thirty patients were operated on for miscellaneous lesions of the aortic root. Thirty-day mortality for the overall series of 270 patients was 4.8% (13/270). There was no 30-day mortality among 182 patients undergoing elective root replacement for annuloaortic ectasia without dissection. Thirty-six of the 270 patients having root replacement also had mitral valve operations. There was no hospital mortality for aortic root replacement in these 36 patients, but there were seven late deaths. Twenty-two patients received a cryopreserved homograft aortic root; 18 of these were primary root replacements and four were repeat root replacements for late endocarditis. One early death and two late deaths occurred in this group. Actuarial survival for the overall group of 270 patients was 73% at 10 years. In a multivariate analysis, only poor New Year Heart Association class (III and IV), non-Marfan status, preoperative dissection, and male gender emerged as significant predictors of early or late death. Endocarditis was the most common late complication (14 of 256 hospital survivors) and was optimally treated by root replacement with a cryopreserved aortic homograft. Late problems with the part of the aorta not operated on occur with moderate frequency; careful follow-up of the distal aorta is critical to long-term survival.

Composite graft repair of Marfan aneurysm of the ascending aorta: results in 150 patients.

One hundred fifty consecutive Marfan patients undergoing composite graft repair of an ascending aorta aneurysm are reported. Twenty-six of the 150 patients had a preoperative dissection of the ascending aorta. There were no early deaths among 138 patients undergoing elective composite graft repair. There was one early death among 12 patients undergoing urgent operation; this patient arrived at the hospital with a rupturing aneurysm. Twenty-four of the 150 patients had mitral procedures; there were no early deaths in this group. There have been 14 late deaths among the 149 hospital survivors (9%). Actuarial survival of 150 patients at 1, 5, 10, and 14 years was 93%, 92%, 81%, and 73% respectively. Risk factors for early or late death were identified by multivariate analysis and only New York Heart Association class (III or IV) and male gender emerged as significant independent predictors of mortality. Late complications directly related to the composite graft have been gratifyingly low; only 2 patients had coronary dehiscence and 3 had thromboembolic events. Endocarditis emerged as an important late complication in 8 patients (5%). Two patients were successfully treated with antibiotics, 3 died before widespread availability of cryopreserved homografts, and 3 patients treated with antibiotics and homograft root replacement have had no evidence of recurrent infection. Seven patients with dissection in this series had aortic diameters of 6.5 cm or less. This experience supports the concept that composite graft repair in Marfan patients is mandated when the aneurysm reaches 5.5 to 6 cm, even in the asymptomatic patient.(ABSTRACT TRUNCATED AT 250 WORDS)

Mitral valve operation in patients with the Marfan syndrome.

Optimal surgical treatment of mitral regurgitation in the Marfan syndrome (valve repair versus replacement) is controversial because the underlying connective tissue defect theoretically might compromise repair durability. To examine the results of mitral valve repair in these patients, we did a retrospective review of 160 patients with the Marfan syndrome who had cardiac surgical procedures between January 1983 and January 1993. Thirty-six patients had mitral procedures, 29 of which were repairs. Mitral valve replacement was necessary in seven patients because of extensive annular calcification and/or severe anterior leaflet abnormalities. The 18 men and 11 women undergoing mitral valve repair had a mean age of 26.5 +/- 2.6 years (range 9 months to 54 years); seven patients were less than 18 years of age. Twenty-four of the 29 patients had concomitant aortic root replacement because of aortic dilation or valvular insufficiency. All 29 repairs included annuloplasty, and 11 patients also required leaflet resection. There were no operative deaths. At mean follow-up of 26.6 +/- 4.8 months, there have been three late deaths, two caused by arrhythmia and one by complications of type III aortic dissection. All survivors are in New York Heart Association class I or II. In three patients recurrent mitral regurgitation developed (grade III or IV); 5-year actuarial freedom from significant mitral regurgitation was 88.3%. One patient required repeat mitral annuloplasty after endocarditis of the composite aortic graft spread to the mitral valve. No patient required late mitral valve replacement. These results demonstrate that (1) 22% of patients with the Marfan syndrome who undergo cardiac operation require a mitral valve procedure, (2) most can be treated by mitral repair rather than replacement, and (3) at early follow-up, results of mitral repair in this population are satisfactory.

Single-stage repair of distal aortic arch and thoracoabdominal dissecting aneurysms using aortic tailoring and circulatory arrest.

The adjuncts of profound hypothermic circulatory arrest and aortic tailoring are combined with standard graft replacement of the noncritical portions of the aorta. This combination permits single-stage replacement of the arch, thoracic, and abdominal aorta in patients with postdissection aneurysm. Reconstruction is begun with repair of the mid to distal aortic arch and Dacron graft replacement of the proximal descending thoracic aorta, usually under profound hypothermic circulatory arrest. The proximal graft is then cannulated and the patient partially warmed while the middle segment is "tailored." Aortic tailoring consists of longitudinal aortotomy and removal of the dissection membrane(s) from the distal third of the descending thoracic aorta to below the renal arteries. The aorta is then closed creating a single channel 2 to 3 cm in diameter and containing the origins of the important intercostal, lumbar, and visceral arteries. A Dacron graft is used to replace the infrarenal segment. Five patients have successfully undergone single-stage, total repair of the aorta including the arch, thoracic, and abdominal segments. All survival without paraplegia or significant visceral ischemia. Follow-up has not shown dilatation of the tailored segment. We believe that reduction in diameter of the tailored aortic segment and thus wall tension, growth of neointima on all luminal aortic surfaces, and active collagen deposition contribute to the success of this procedure.

Current diagnosis and prescription for the Marfan syndrome: aortic root and valve replacement.

Since 1976, the procedure of choice for patients with Marfan aneurysm of the ascending aorta at this institution has been the Bentall procedure or its modification. One hundred seventy-four Marfan patients have had ascending aortic aneurysm repair with a prosthetic composite graft. An additional four children and two adults have had aortic root replacement with a cryopreserved homograft. Thirty-seven (21%) patients came to operation with an acute or chronic dissection involving the ascending aorta. Thirty-one (17%) patients having aortic root replacement also had mitral valve repair or replacement. The first 86 patients in the series had a hemostatic Bentall wrap constructed; however, in the last 88 patients, the ascending aorta has been transected completely and the aortic wall tacked loosely over the composite graft. It is our preference to construct a direct end-to-side anastomosis of each coronary ostium to the side of the composite graft. In the past, we used the Cabrol interposed graft for low-lying coronary ostia, but currently favor the "button technique" for this procedure. One hundred fifty-six of 180 patients underwent elective root replacement with no hospital mortality. Twenty-four patients underwent urgent or emergency surgery; 17 of these patients with acute dissection or rupture underwent emergency surgery. Two of these patients with rupture died intraoperatively. Therefore, overall hospital mortality among the 180 patients was 1.1%. There have been 14 late deaths among 178 patients discharged from the hospital (7.9%). Five of these late deaths occurred among our first 11 patients operated on between 1976 and 1979. Since 1980, there have been nine late deaths among 167 patients (5.4%).(ABSTRACT TRUNCATED AT 250 WORDS)

Centrifugal ventricular assist device for support of the failing heart after cardiac surgery.

OBJECTIVE: To determine the morbidity and mortality associated with use of centrifugal ventricular assist devices for postcardiotomy cardiogenic shock and to determine factors that might influence outcome and thus, aid in patient selection. DESIGN: A retrospective study. SETTING: Surgical intensive care unit in a university hospital. PATIENTS: During a 6-yr period, a total of 7,385 adult patients underwent cardiac operations requiring cardiopulmonary bypass. Myocardial protection consisted of single-dose cold crystalloid cardioplegia and continuous topical hypothermia by saline lavage. A total of 72 (1%) patients developed postcardiotomy cardiogenic shock. Of 72 patients, 28 met the institutional criteria and were placed on centrifugal ventricular assist devices. INTERVENTIONS: Twenty-eight adult patients with postcardiotomy cardiogenic shock were supported with centrifugal ventricular assist devices. MEASUREMENTS AND MAIN RESULTS: A total of 15 patients received left ventricular assist devices, five received right ventricular assist devices, and eight received both right and left ventricular assist devices. Mean age of ventricular assistance patients was 50.8 +/- 12.9 yrs (range 22 to 72), and mean duration of ventricular assistance was 2.8 +/- 2.5 days (range 4 hrs to 10 days; median 2 days). Twenty-five complications occurred in 16 patients and included bleeding (13), tamponade (2), systemic embolism (6), seizures (2), and sepsis (2). Nine patients required reexploration for bleeding or tamponade. Nine (32%) of 28 patients were discharged from the hospital. Ventricular assistance for cardiac failure after transplantation was associated with improved survival (p < .10), while age > 50 yrs and postoperative tamponade each showed trends toward association with mortality (p = .10). Survival was not predicted by gender, weight, time on cardiopulmonary bypass, aortic cross-clamp time, urgency of operation, or preoperative congestive heart failure. At 27 +/- 20 months follow-up, all survivors were alive and New York Heart Association functional class I or II. CONCLUSIONS: These results document a low incidence of ventricular assist device use in a surgical practice that employs a relatively simple method of myocardial protection. When postcardiotomy ventricular assistance was necessary, a centrifugal pump was used and successful outcome and satisfactory long-term results were possible in nearly one third of patients. Ventricular assistance for cardiac failure after transplantation was associated with improved survival. Older age is a relative contraindication to mechanical ventricular assistance.

Biomechanical study of sternal closure techniques.

Median sternotomy is the most commonly used incision in cardiothoracic surgery. Closure of this incision is usually performed with parasternal wires, but alternate techniques have been proposed to improve closure stability. This study compares biomechanical stability of standard wire (No. 5 stainless steel) with that of three types of band closure: 5-mm Mersilene ribbon, 5-mm stainless steel band, and 5-mm plastic band. Eight bisected cadaver sterna were reapproximated using each method of sternal fixation and tested for biomechanical stability using an MTS Bionix 858 Biomechanical Tester. Loads of 50, 100, 150, and 200 Newtons (1 Newton = 1 kg.m/s2) were applied as a distracting force across the closure. A linear regression line of displacement as a function of increasing load was determined for each closure method; the slope of this line is inversely proportional to fixation stability. Displacement and load correlated linearly for each closure (r = 0.99). Mean slopes were 0.012 mm/Newton (95% confidence limits, 0.0098 to 0.0142 mm/Newton) for No. 5 stainless steel wire, 0.014 mm/Newton (95% confidence limits, 0.0118 to 0.0162 mm/Newton) for plastic band, 0.017 mm/Newton (95% confidence limits, 0.0148 to 0.0192 mm/Newton) for Mersilene ribbon, and 0.017 mm/Newton (95% confidence limits, 0.0148 to 0.0192 mm/Newton) for 5-mm steel band. No. 5 stainless steel wire provided the most stable closure, although statistical significance was achieved only in comparison with Mersilene ribbon and stainless steel band (p < 0.05). The superior stability of stainless steel wire closure may be due to tightening of the wires by twisting, which results in more tension across the reapproximated sternal halves than with other methods.

Valvular disease in the elderly: influence on surgical results.

Aortic valve disease in the elderly is primarily calcific stenosis with preservation of left ventricular function. In contrast, mitral valve disease in the elderly often is ischemic in nature with damage occurring to both valve and myocardium. The present study was undertaken to compare results of aortic (AVR) and mitral valve replacement (MVR) in the elderly and to ascertain predictors of poor outcome. Because patients who had concomitant coronary artery bypass grafting (CABG) are included (51% for AVR, 55% for MVR), patients who had isolated CABG were used as a comparison group. Between January 1, 1984, and June 30, 1991, 1,386 patients aged 70 years and older underwent CABG (n = 1,043), AVR (n = 245), or MVR (n = 98). The operative mortality rates were 5.3% for AVR, 20.4% for MVR, and 5.8% for CABG. Late follow-up of patients undergoing operation in 1984 and 1985 was available for 98% (231/237). Overall survival was comparable for all three groups through the first 5 years of follow-up (AVR, 68% +/- 8%; MVR, 73% +/- 8%; CABG, 78% +/- 3%). After 5 years, survival for patients having AVR and MVR was less than that for those having CABG. Patient age, sex, New York Heart Association functional class, concomitant CABG, prosthetic valve type, native valve pathology, and preoperative catheterization data were examined as possible predictors of outcome by multivariate logistic regression.(ABSTRACT TRUNCATED AT 250 WORDS)

Composite aortic valve replacement and graft replacement of the ascending aorta plus coronary ostial reimplantation: how I do it.

Aortic root replacement in the patient with Marfan syndrome is indicated in the presence of aortic root dilatation more than 6 cm, ascending aortic dissection, or 3 to 4+ aortic valve insufficiency. The objective of operation is to prevent catastrophic aortic rupture or dissection and to treat or prevent aortic insufficiency with consequent left ventricular dysfunction. In over 170 patients undergoing aortic root replacement, operative mortality has been 1%. At mean follow-up of 5 years, the survival rate was 75%. The most frequent serious late postoperative complication has been graft endocarditis (4%). The technique described above carries a low operative risk and late complication rate. We believe it is an important surgical arm in the treatment of cardiovascular complications of the Marfan syndrome.