Aortic repair in Marfan's syndrome: prevention of mechanical leaflets damage

Aortic incompetence in Marfan's syndrome results from distortion or dilatation of the sinuses of Valsalva, annuloaortic ectasia or a combination of these problems. Valve leaflets in these patients are macroscopically normal in spite of aortic insufficiency. Replacement of the ascending aorta, root and aortic valve with a composite graft was, for a long time, the treatment of choice for Marfan patients. Valve-preserving procedures (remodeling or reimplantation) provide the advantages of avoiding the shortcomings of standard surgical techniques, and maintaining the functional integrity of the left ventricular (LV) outflow tract, aortic root and ascending aorta. We developed a modified valve-sparing reimplantation technique for avoiding leaflet damage. This was achieved by leaving a 'cushion' of aortic wall (8--10 mm) that, sewn on the Dacron graft, works as a 'damper' and prevents leaflets injury during the systolic opening of the valve. For final judgment of this operative method long-term results are necessary.     

Aortic root reconstruction with preservation of native aortic valve and sinuses in aortic root dilatation with aortic regurgitation.

BACKGROUND: We assessed the results of a modified technique for aortic root reconstruction including preservation of the native aortic valve and sinuses. METHODS: A modified technique for reconstruction of the aortic root was devised in which the native aortic sinuses are preserved and remodeled, the diameter of the sinotubular junction is reduced, the ventriculoaortic junction is reinforced with a Dacron prosthesis, and the coronary ostia are reimplanted. Since January 1995, this modified operative technique was performed in 13 patients with a mean age of 54 +/- 21 years. The median grade of aortic regurgitation was 3; in 10 patients it was caused by dilatation of the sinotubular junction, and 3 had additional annuloaortic ectasia. RESULTS: The aortic crossclamping time was 61 +/- 18 minutes. In-hospital mortality was 2 of 13 (15. 3%) patients, both deaths being related to complications of aortic dissection. In 1 patient aortic regurgitation increased to grade 3, necessitating aortic valve replacement. At a mean follow-up of 2.1 years, the remaining 10 patients had stable aortic valve function with a median grade of regurgitation of 1. The mean New York Heart Association functional class was 1.2. CONCLUSIONS: Aortic root reconstruction with preservation of the native aortic valve and sinuses allows symmetric reconstruction of the aortic sinuses and adaptation of the diameters of the sinotubular and ventriculoaortic junctions, thus optimizing aortic valve function. Moreover, it prevents contact of the aortic valve leaflets with the Dacron graft, which may enhance the durability of the repair.     

An aortic valve-sparing operation for patients with aortic incompetence and aneurysm of the ascending aorta.

A number of patients who require an operation for complications of annuloaortic ectasia, such as aortic incompetence or aneurysm of the aortic root (or both), have normal aortic valve leaflets. We have treated these patients by excising the aneurysmal portion of the ascending aorta and sinuses of Valsalva but by leaving the aortic valve leaflets and some arterial wall attached to the left ventricular outflow tract. The aortic valve is reimplanted inside a collagen-impregnated tubular Dacron graft, similar to what is done for implantation of an aortic valve homograft. The coronary arteries are also reimplanted. This operation was performed in 10 patients. All patients had annuloaortic ectasia and five had the stigmata of Marfan syndrome. Four patients had acute aortic dissection. There were no operative deaths, but one patient required composite replacement of the aortic valve and ascending aorta because of persistent aortic incompetence. Postoperative Doppler echocardiography revealed normal aortic valve function in six patients and mild incompetence in three. The preliminary results of this new operation are encouraging. Further investigation is necessary to establish the best size, shape, and type of material that should be used to replace the aortic root while preserving the aortic valve.     

One-staged surgery for coarctation of the aorta associated with annuloaortic ectasia--a case report

A 37-year-old man was admitted to St. Marianna University Hospital with an episode of severe anterior chest pain. Contrast enhanced CT scan and digital subtraction angiography disclosed an aneurysmal dilatation of the aortic root and almost completely obstructed aortic isthmus. Modified Bentall operation was performed with simultaneous graft bypass using a woven Dacron prosthesis between the ascending aorta and the abdominal aorta through the median thoracoabdominal incision. Histologically the aortic wall showed cystic medial necrosis, and the aortic valve showed myxomatous degeneration. Only 9 cases with coarctation of the aorta associated with annuloaortic ectasia have been reported in the Japanese literature, and this is believed to be the first report that one-staged surgery has successfully been done dealing simultaneously both the coarctation and the annuloaortic ectasia.     

Flow patterns in the aortic root and the aorta studied with time-resolved, 3-dimensional, phase-contrast magnetic resonance imaging: implications for aortic valve-sparing surgery

OBJECTIVE: Sparing the aortic valve has become a surgical option for patients who require repair of aortic root ectasia and have normal valve leaflets. Surgical approaches to valve sparing differ with regard to preservation of the native sinuses of Valsalva. The role of the sinuses and the importance of maintaining them remain controversial. METHODS: By using a time-resolved, 3-dimensional, phase-contrast magnetic resonance imaging technique, aortic root and aortic blood velocity data were acquired from 2 patients with Marfan syndrome 6 months after aortic valve-sparing surgery with straight Dacron grafts and contrasted with data from 6 normal volunteers. RESULTS: In normal aortas vortical blood flow became apparent in the individual sinuses after peak systole. The vortices filled the available space behind the valve leaflets and persisted until diastole, expanding and moving inward during aortic valve closure. In contrast, no vortices were observed in the postoperative patients with Marfan syndrome with negligible sinuses. CONCLUSIONS: Changes in supravalvular flow accompany loss of sinus architecture. Whether the presence, size, and velocity of supravalvular vortices affects the function or durability of the preserved aortic valve remains to be studied.     

Replacement of ascending aorta with aortic valve reimplantation: midterm results

Objective: Aneurysms of the aortic root lead to aortic valve incompetence due to dilatation of the sinotubular junction and annuloaortic ectasia. Reimplantation of the native, structurally intact aortic valve within a Dacron tube graft corrects annular ectasia as well as dilatation of sinotubular junction and aortic sinuses. Durability of this valve repair with respect to increased mechanical stress on valve cusps is discussed controversially and is yet unknown. Methods: Since 7/93, replacement of the ascending aorta with repair of the aortic valve was performed in 48 patients (34 male, 14 female; 47±20 years) with aortic insufficiency and aneurysm of the aortic root. Fifteen patients (31%) had Marfan's syndrome and five patients (10%) had an aortic dissection type A (two acute, three chronic). In 11 patients (23%), concomitant replacement of the aortic arch was necessary utilizing elephant trunk technique in two patients. Additionally, one patient required mitral valve repair and two other patients coronary artery bypass grafts. Clinical and echocardiographic follow-up was performed in 6–12 month intervals for a cumulative study period of 100 patient years. Results: There were no operative deaths. Two patients (4%) died 5 and 20 months postoperatively. One additional patient experienced a TIA within the first postoperative week. Three patients (6%) with an early postoperative aortic insufficiency (AI)>1 required aortic valve replacement after 9, 11, and 14 months due to progressive AI. In these patients, distortion of the aortic root geometry led to valve incompetence. All other patients have no or mild aortic insufficiency. The repair now remains stable for up to 63 months (mean 25±18 months). Other valve related complications did not occur. Conclusions: Our results demonstrate that this type of aortic valve repair achieves excellent results in selected patients. Perfect coaptation of valve cusps during the repair with no or only trace AI at initial echocardiography seems to be essential for durability.     

Valsalva prosthesis in aortic valve-sparing operations

Aortic valve-sparing operations have provided very good clinical outcomes. However, the absence of the sinuses of Valsalva might limit valve durability. The Gelweave Valsalva prosthesis, which presents pre-fashioned neo-sinuses, has been designed in order to avoid early leaflets deterioration. We report our results in 63 patients who underwent valve-sparing operations (reimplantation technique) using the Gelweave Valsalva graft. The main indication was ascending aorta aneurysm or annuloaortic ectasia, with or without aortic insufficiency. The operation was performed also in cases of Marfan syndrome, Bicuspid Aortic Valve (BAV), and acute Type A dissection. In-hospital mortality was of 4.7%, and two thirds were acute Type A dissection patients (P=0.01). There were no late deaths. Three years freedom from grade 3-4 AI and freedom from late aortic valve replacement were 91.7+/-4.3% and 93.8+/-5.1%, respectively. Aortic valve-sparing operations show good results in patients electively operated for aortic root ectasia. Aortic cusps repair may lead to late failure. Even if the Gelweave Valsalva prosthesis is easy to implant and it also reproduces pseudosinuses, a long-term follow up is necessary to determine if this graft may reduce leaflets deterioration.     

Bentall Operation for a Child with Marfan Syndrome: A Case Report

A bstract Children with Marfan syndrome rarely undergo surgery for annuloaortic ectasia and aortic regurgitation in the first decade. A 7-year-old girl presented with congestive heart failure due to severe aortic regurgitation associated with annuloaortic ectasia (6 cm). She also had funnel chest. She underwent a Bentall operation and sternal turn-over with a satisfactory result. Since the aortic valve cusps had rolled edges, the aortic valve was not spared. Histology of the aortic valve cusps showed myxoid degeneration and fragmentation of elastic fibers.     

The management of aortic root replacement using the Top-Hat/Gelweave composite graft

Composite graft replacement of the aortic root has become a routine procedure for annuloaortic ectasia (AAE) and aortic valve insufficiency (AR) with aortic dissection and the results have improved. We treated six cases of aortic root reconstruction using the Carrel patch method in 1998. The Top-Hat/Gelweave Composite graft fit together well and the procedure is technically similar to standard valve replacement. Upon measuring the valve size a Gelweave graft 1 mm larger than the valve size should be selected. There were no incidence of hemorrhage or postoperative hemolysis. Further long-term follow-up is necessary.     

Long-term results of modified Bentall procedure using flanged composite aortic prosthesis and separately interposed coronary graft technique

We have selected the flanged composite aortic prosthesis and separately interposed coronary graft technique for the aortic root replacement over seven years. We sought to evaluate the long-term results of aortic root replacement with this technique. Between April 1996 and September 2003, 71 patients (mean age 46.1+/-12.9 years, 67.6% males) underwent aortic root replacement with this technique. Sixty-two patients had annuloaortic ectasia, and seven patients acute type A aortic dissection. Marfan syndrome was recognized in 35 patients. Two separate 8-10 mm knitted Dacron grafts were interposed between a valved composite graft and both coronary ostia to avoid kinking of coronary arteries. The early mortality rate was 4.2%. The actuarial survival rate was 93.9+/-3.0% at 5 years. The freedom from operation related complications was 86.7+/-4.1% at 5 years. No patients had anticoagulant-related hemorrhage, valve thrombosis, reoperation, graft thrombosis, or coronary pseudoaneurysm. The separately interposed coronary graft and the flanged composite graft technique is predictable and safe. Coronary pseudoaneurysm and graft thrombosis have been eliminated.     

One-staged operation for mitral regurgitation and annuloaortic ectasia without aortic regurgitation with Marfan syndrome; report of a case

A 32-year-old man with Marfan syndrome was admitted to our hospital for detail examination of congestive heart failure. Doppler echocardiography showed severe mitral regurgitation due to prolapse of posterior mitral leaflet. Annuloaortic ectasia without aortic regurgitation was also detected by aortography. Considering the future operative need for aortic root and ascending aorta, we performed mitral valve replacement with a mechanical valve and preventive concomitant aortic root replacement with a composite valve graft. His postoperative course was uneventful. Optimal surgical treatment of mitral regurgitation and annuloaortic ectasia in Marfan syndrome is controversial because the underlying connective tissue defect theoretically might compromise repair durability. Several surgical options for mitral regurgitation and annuloaortic ectasia in Marfan syndrome are discussed.     

A simple modification of 'David-V' aortic root reimplantation.

Valve-sparing aortic root reimplantation with creation of Dacron graft pseudosinuses is a popular and promising surgical choice for annuloaortic ectasia or ascending aortic aneurysm. We have developed a simple modification of the David-V technique, which facilitates free adjustment of the size of the new aortic annulus and the pseudosinus of each patient, as well as the creation of pseudosinuses of excellent shape, with only one Dacron graft.     

Valve-preserving aortic replacement: does the additional repair of leaflet prolapse adversely affect the results?

OBJECTIVE: Valve-preserving aortic replacement has evolved into an accepted therapeutic option for aortic ectasia with morphologically intact leaflets. Some patients, however, exhibit additional leaflet prolapse. We compared the results of established valve-preserving techniques with those of the combination of valve-preserving aortic surgery and additional repair of leaflet prolapse. METHODS: Between October 1995 and March 2000, 99 patients underwent valve-preserving root replacement by means of root remodeling or valve reimplantation for acute dissection (n = 25), chronic dissection (n = 4), or aneurysm (n = 70). In group A (63 patients) either root remodeling (n = 49) or valve reimplantation (n = 14) was performed with a standard technique. In group B (36 patients) valvepreserving aortic replacement (remodeling, n = 31; reimplantation, n = 5) was combined with repair of leaflet prolapse in the presence of bicuspid (n = 24) or tricuspid (n = 12) valve anatomy. Additional replacement of the aortic arch was required more frequently in group A (group A, n = 43; group B, n = 14; P =.006); otherwise, the groups were comparable. RESULTS: Cardiopulmonary bypass (group A, 133 +/- 31 minutes; group B, 117 +/- 30 minutes; P =.006) and myocardial ischemia times (group A, 96 +/- 25 minutes; group B, 88 +/- 20 minutes; P =.05) were significantly longer in group A. Mortality was not significantly different between groups (group A, 4.8%; group B, 0%). One patient in each group underwent secondary valve replacement, and all other patients had stable valve function. Freedom from aortic regurgitation of grade 2 or greater after 48 months was 93.0% in both groups. CONCLUSION: Repair of leaflet prolapse in conjunction with valve-preserving root replacement leads to midterm results that are equal to those of valve-preserving root replacement for morphologically intact leaflets.     

A case of combined operation of aortic valve slicing and root remodeling for ascending aortic aneurysm with aortic regurgitation]

A 56-year-old female admitted with severe back pain, and her chest computed tomography demonstrated non-dissecting sclerotic aneurysm of the ascending aorta. Aortography and echocardiography showed marked dilatation of the ascending aorta and the Valsalva sinuses resulting in disappearance of the sinotubular junction. Aortic regurgitation of grade three was, also, recognized. A combined operation of aortic valve slicing of the right and the left coronary cusps and aortic root remodeling (Yacoub's method) was successfully performed. A woven Dacron double-veloured graft (Hemashield) of 22 mm in diameter was used for reconstruction of the ascending aorta and its root. Postoperative aortography figured the new sinotubular junction and the new Valsalva-like sinus composed by the graft, and aortic regurgitation was controlled to grade one.     

A case report of Bentall procedure for annulo-aortic ectasia associated with right coronary artery dissection

A 30-year-old man who had annuloaortic ectasia associated with aortic insufficiency owing to marked annular dilatation was treated by replacement of the ascending aorta and aortic valve with a composite graft. It was necessary to transpose the origin of the coronary artery because of the development of dissection to right coronary ostia. We have applied the Bentall procedure with aorta coronary bypass between the right coronary artery and the aortic prosthesis with the use of saphenous vein graft. His post operative course is uneventful and engaging full work 18 month after operation. This technique is useful for the case of annuloaortic ectasia associated with difficulty coronary anastomosis such as coronary artery dissection, obstruction or dislocation.     

Surgical treatment with the rifampicin-soaked vascular prosthesis for mycotic pseudoaneurysm after aortic root replacement

A 59-year-old man had undergone aortic root replacement for annuloaortic ectasia (AAE) and aortic regurgitation (AR) 18 years before. The computed tomography (CT) showed a pseudoaneurysm of ascending aorta 4 years after the 1st operation. Re-aortic root replacement was done 15 years after the 1st operation because of the pseudoaneurysm. However, 3 years after the 2nd operation, the patient suffered from fever and another pseudoaneurysm was revealed by CT. Echocardiography showed a vegetation attached to the aortic valve. The 3rd operation was successfully performed using rifampicin-soaked vascular prosthesis. The postoperative course was uneventful. Thus, graft infection was effectively treated with a rifampicin-soaked vascular prosthesis.     

Valved stentless composite graft: clinical outcomes and hemodynamic characteristics

BACKGROUND: The valved stentless composite graft has become well established in our hospital for replacement of the ascending aorta and aortic valve in elderly patients and those with contraindication for lifelong anticoagulation. This study was conducted to evaluate the postoperative hemodynamic characteristics and clinical outcomes after implantation of this device. METHODS: Between November 1998 and February 2001, 45 consecutive patients with a mean age of 69 years underwent implantation of a composite graft using a stentless valve prosthesis (Toronto SPV) incorporated in a collagen-coated Dacron tube (InterGard). The indication for surgery was aortic valve disease with an accompanying true aneurysm of the ascending aorta in 42 patients and a dissection of the aortic wall in 3 patients. Postoperative echocardiographic examinations were performed before discharge from the hospital and at the time of the follow-up. Mean follow-up duration was 18 months (range 3 to 30 months). RESULTS: There was no perioperative mortality. During follow-up, there were two noncardiac, nonvalve-related deaths. Echocardiographic evaluation before discharge and at follow-up demonstrated favorable hemodynamics of the valve prosthesis with mean transvalvular gradients of 8.5 +/- 2.9 mmHg and 8.0 +/- 3.1 mmHg, respectively. No regurgitation across the valve and no contact of the cusps with the Dacron tube were seen in any case. CONCLUSIONS: A stentless composite graft for replacement of the aortic valve and ascending aorta offers excellent hemodynamic results and is a suitable device for patients in whom anticoagulation should be avoided.     

Concomitant aortic valve-sparing operation and closed biatrial radiofrequency ablation for annuloaortic ectasia and paroxysmal atrial fibrillation

A 53-year-old woman was admitted for annuloaortic ectasia with moderate aortic valve insufficiency (AI) and paroxysmal atrial fibrillation. Concomitant aortic root replacement with a valve-sparing technique and closed biatrial procedure using bipolar radiofrequency ablation was performed successfully. Postoperative echocardiography showed trivial AI with regular sinus rhythm, which meant she could avoid anticoagulation therapy.     

Bentall procedure with a stentless valve and a new aortic root prosthesis

We describe a technique to replace the aortic root by means of a stentless valve and a new aortic root Dacron graft (Gelweave Valsalva, Sulzer Vascutek, Renfrewshire, Scotland) that allows an anatomical reconstruction of the sinuses of Valsalva.     

One-year appraisal of a new aortic root conduit with sinuses of Valsalva.

OBJECTIVES: We evaluate the clinical results 1 year after an anatomic reconstruction of the aortic root in which we used a specifically designed aortic root prosthesis that incorporates the sinuses of Valsalva. MATERIAL AND METHODS: The new aortic Dacron prosthesis has a proximal portion in the Dacron conduit that expands on implantation, creating pseudosinuses. During a 12-month period, 28 patients (mean age 59 +/- 14 years) underwent a Bentall operation (12 cases), a remodeling procedure (7 cases), and a reimplantation procedure (9 cases) with the use of a new aortic root conduit. All patients had aortic root aneurysm with an anatomically normal (for the valve-sparing procedure) or diseased aortic valve (for the Bentall operation). Five patients had aortic dissection and 5 had Marfan disease. The mean follow-up was 6 +/- 3 months. All patients underwent postoperative transesophageal and transthoracic echocardiographic studies. RESULTS: All patients survived and were in good clinical condition at the latest follow-up. Postoperative echocardiography showed a marked reduction in ventricular volumes in all patient groups (P <.0005). In the Bentall group the new prosthesis appeared to reduce the tension on the coronary ostial sutures. In patients undergoing both types of valve-sparing procedures a similar normal anatomy of the aortic root was reconstructed. In the reimplantation group the anulus was smaller than in the remodeling group (P =.01). Patients undergoing the reimplantation procedure had less bleeding and a lower incidence of residual valve insufficiency. CONCLUSIONS: The new aortic root prosthesis allowed the reconstruction of the aortic root anatomy in all types of surgical techniques with low postoperative morbidity.