Remodeling of the aortic root and reconstruction of the bicuspid aortic valve

BACKGROUND: Currently, isolated reconstruction of a regurgitant bicuspid aortic valve can be performed with adequate early results. Dilatation of the proximal aorta is known to be associated with this valve anomaly and may be partially responsible for the development of primary regurgitation or secondary failure of valve repair. We have used repair of the bicuspid valve with remodeling of the aortic root as an alternative to insertion of a composite graft. METHODS: Between October 1995 and May 1999, 16 patients (12 men, 4 women, aged 35 to 73 years) were seen with a regurgitant bicuspid aortic valve and dilatation of the proximal aorta of more than 50 mm. All patients underwent repair of the valve using either coapting sutures alone (n = 12) or in combination with triangular resection of a median raphe (n = 4). Using a Dacron graft, the aortic root was remodeled and the ascending aorta (n = 16) and proximal arch (n = 4) replaced. RESULTS: No patient died. The postoperative degree of aortic regurgitation was less than grade II in all patients. Valve function has remained stable in all patients between 2 and 43 months postoperatively. CONCLUSIONS: Reconstruction of the regurgitant bicuspid valve in the presence of proximal aortic dilatation is feasible with good results by combining the root remodeling technique with valve repair.     

A case of ascending aortic dissection after aortic valve replacement in congenital bicuspid aortic valve]

Congenital bicuspid aortic valve is a risk factor of aortic dissection, but the case is rare in Japan. Several reports described ascending aortic aneurysm after aortic valve replacement. In these reports, most of aneurysms were false aneurysm, but the cases of ascending aortic dissection were rare. In this case, dissecting aneurysm of the ascending aorta occurred 4 years after aortic valve replacement, which was performed with mechanical prosthesis because of infective endocarditis, and it was repaired successfully by the modified Cabrol's method. This case was congenital bicuspid aortic valve, and had already been complicated with moderate aortic dilatation in the ascending aorta. In patients of congenital bicuspid aortic valve with aortic dilatation, consideration of complete replacement of the ascending aorta with aortic valve replacement is important.     

Valve-sparing replacement of the noncoronary sinus of Valsalva in patients with a bicuspid aortic valve

Progressive dilatation of the aortic root and ascending aorta is frequent in patients with bicuspid aortic valve, and isolated dilatation of the noncoronary sinus has been reported. We describe our technique for the selective replacement of the noncoronary sinus of Valsalva in patients with bicuspid aortic valve.     

Aortic valve sparing operations: an update

Background. Aortic valve sparing operations in patients with ascending aorta and/or aortic root aneurysms have been performed for a decade in our institution. Initially only patients with normal aortic valve leaflets had these operations, but more recently we utilized them in patients with prolapse of a single leaflet and in those with a bicuspid aortic valve. This article is an update on the clinical results of these operations.

Methods. From May 1988 to December 1997, 126 patients with ascending aorta and/or aortic root aneurysms and aortic insufficiency underwent replacement of the ascending aorta with reconstruction of the aortic root and preservation of the native aortic valve. There were 85 men and 41 women, with a mean age of 54 years (range, 14 to 84). Thirty-two patients had the Marfan syndrome; 17 patients had acute and 10 had chronic type A aortic dissection; 23 had a transverse arch aneurysm; 26 had coronary artery disease, and 8 had mitral regurgitation. The aortic valve sparing operation consisted of simple adjustment of the sinotubular junction in 33 patients, adjustment of the sinotubular junction and replacement of one or more aortic sinuses in 60, and reimplantation of the aortic valve in a tubular Dacron (C.R. Bard, Haverhill, PA) graft in 33. Fifteen patients also had repair of aortic leaflet prolapse. Only 4 patients had a bicuspid aortic valve.

Results. There were 3 operative deaths due to cardiac failure. Patients were followed from 2 to 117 months, with a mean of 31. There were 11 late deaths: 7 cardiovascular and 4 from unrelated causes. The actuarial survival was 72 ± 8% at 7 years. Two patients required aortic valve replacement; the freedom from aortic valve replacement was 97 ± 2% at 7 years. Doppler echocardiography revealed absent, trivial or mild aortic insufficiency in most patients; only 9 patients had moderate aortic insufficiency.

Conclusions. Aortic valve sparing operations are feasible in most patients with ascending aorta and/or aortic root aneurysms who have normal or near normal aortic leaflets. The functional results of the repaired aortic valve are excellent, and the repair appears to be durable.     

Post-stenotic aortic dilatation

Aortic stenosis is the most common valvular heart disease affecting up to 4% of the elderly population. It can be associated with dilatation of the ascending aorta and subsequent dissection. Post-stenotic dilatation is seen in patients with AS and/or aortic regurgitation, patients with a haemodynamically normal bicuspid aortic valve and following aortic valve replacement. Controversy exists as to whether to replace the aortic root and ascending aorta at the time of aortic valve replacement, an operation that potentially carries a higher morbidity and mortality.     

升主动脉根部瘤的外科治疗

目的总结升主动脉根部瘤的外科治疗经验。方法101例升主动脉根部瘤患者（年龄14～72岁，平均年龄42．7岁）的主要病因为马方综合征（Marfan syndrome，58例），主动脉瓣环扩张症（34例），主动脉瓣二叶瓣畸形（5例），大动脉炎（4例）；术前有主动脉瓣关闭不全96例，主动脉瓣狭窄4例，术前合并有A型夹层26例，急性左心衰竭（5例）。手术类型：Wheat手术4例，传统或改良Cabrol手术13例，David手术1例，Bentall手术83例。同期行主动脉半弓置换术或降主动脉腔内支架植入术16例，全弓置换术或降主动脉腔内支架植入术4例，二尖瓣置换术或成形术14例，冠状动脉旁路移植术8例。结果全组手术死亡率为6．9％（7／101），2000年后降至3．6％（3／83）；术后主要并发症为低心排血量10例，呼吸功能不全9例，肾功能不全9例。术后随访94例，随访期间死亡1例，5例马方综合征患者术后出现B型夹层。结论Bentall手术是治疗升主动脉根部瘤的首选手术方法，术前左心功能及手术技术是影响手术效果的关键因素。     

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.     

Aortic insufficiency secondary to aortic root aneurysm or dissection.

Twenty-four patients with aortic regurgitation secondary to aortic root aneurysm (13 patients) or dissection (11 patients) were operated on, utilizing a variety of surgical procedures to cope with the varied pathological findings. These ranged from primary repair of the ascending aorta without any prostheses in patients with acute aortic dissection to replacement of the valve and the entire ascending aorta for aortic root aneurysm. In four patients with Marfan syndrome the right coronary artery was transplanted to the ascending aortic graft, allowing an extension of the graft to the valve anulus and excision of the entire aneurysmal aorta. The immediate and late results have been most encouraging.     

Thoracic aortic aneurysm associated with congenital bicuspid aortic valve.

Congenital bicuspid aortic valve is a relatively rare malformation. It is reported that the presence of this anomaly predisposes the patient to development of true aortic aneurysms or dissecting aortic aneurysms. Between 1981 and August 1997, 25 patients with an aneurysm of the thoracic aorta associated with congenital bicuspid aortic valve underwent surgical treatment at the authors' institution. There were 20 males and five females. The age of the patients ranged from 27 to 74 years (mean 53 years). There were 18 patients with true ascending aortic aneurysms (of which 10 presented with annulo-aortic ectasia) and seven with dissecting aortic aneurysms (four with DeBakey type I dissection, two with type II and one with type IIIb). These 25 patients constituted 2.6% (25/973) of all cases of surgical operations for aneurysms in the thoracic aorta. Aortic valve dysfunction was noted in 20 patients. The authors performed a valved conduit operation in nine patients, aortic valve replacement and wrapping of the ascending aorta in six, graft replacement of the ascending aorta in five, graft replacement of the ascending aorta and aortic arch in four, and graft replacement of the descending aorta in one. No hospital deaths occurred in the authors' patients. Pathological examination of surgical specimens of the aortic wall showed cystic medial necrosis in 11 patients and mucoid degeneration in nine. In patients with congenital bicuspid aortic valve, attention should be paid to aneurysmal dilatation and aortic dissection as complications in addition to valve dysfunction.     

Aortic root replacement after operation for the ascending aorta and/or aortic valve

We reviewed ten cases who underwent aortic root replacement after operation for the ascending aorta and/or aortic valve. As initial operation, aortic valve replacement (AVR) was performed in five patients, replacement of the ascending aorta in two, original Bentall operation in two, and entry closure and suspension of the aortic valve in one. At reoperation, three patients were diagnosed as aneurysm of the ascending aorta, two were annulo-aortic ectasia, and one was acute aortic dissection, chronic dissecting aneusym, pseudoaneurysm of the ascending aorta, prosthetic valve endocarditis, and massive aortic regurgitation. Aortic root replacement was performed using mechanical valved composite graft in all cases. One patient who underwent repeat aortic root replacement for prosthetic valve endocarditis was died of septemia and ventricular fibrillation. Five patients had nine complications (two low output syndrome, respiratory failure and cerebral infarction, one gastrointestinal bleeding, septemia and ventricular fibrillation). In conclusion, aortic root replacement after operation for the ascending aorta and/or aortic valve was performed with acceptable morbidity and mortality.     

Mid-term results of aortoplasty for dilated ascending aorta associated with aortic valve disease.

BACKGROUNDS: Dilatation of the ascending aorta concomitant with aortic valve disease is occasionally associated with progressive enlargement of the ascending aorta or acute aortic dissection (AAD). However, surgical procedure of choice for the aorta and its indication are controversial. PATIENTS AND METHODS: From July 1995 to August 2001, 10 patients with a moderately dilated ascending aorta (mean diameter, 52+/-4.8 mm) underwent concurrent aortic valve replacement (AVR) and aortoplasty. The aortic valve was bicuspid in eight patients. To tailor the ascending aorta 30-35 mm in diameter, the aortic wall was partially resected along the aortotomy, and the aorta was directly closed. RESULTS: Operation time and most of other perioperative variables were comparable to those of patients who underwent isolated AVR. The aortic diameter was reduced to 36.1+/-4.1 mm. Nine patients survived to hospital discharge uneventfully, but one patient developed disruption of the suture line in the aorta and died. During follow-up, no patient suffered AAD but redilatation was observed in one patient. In the two problematic patients, the ascending aorta was larger than 55 mm, and its media was histologically abnormal. CONCLUSION: In patients with dilated ascending aorta less than 55 mm in diameter, aortoplasty can be a procedure of choice. However, a prosthetic graft replacement is recommended when the diameter of the ascending aorta is larger than 55 mm.     

Reoperations and late adverse outcome in Marfan patients following cardiovascular surgery.

OBJECTIVES: Anulo-aortic ectasia represents the most common cardiovascular manifestation requiring surgery in Marfan patients. Aim of this report was to analyze the type of presentation and the incidence of cardiovascular lesions and the clinical follow-up after initial surgery, mainly aortic root repair or replacement. METHODS: Between 1/1990 and 6/2003 a total of 71 patients (mean age 29+/-17 years, 8-65 years) received first surgical treatment at our institution. 69 received root repair or replacement. 22 patients presented with acute aortic dissection (31%), out of them, 3 pregnant females and 1 just after delivery. Composite graft replacement was performed in the majority of patients (61/71, 85%). Aortic valve sparing root repair was performed in 7 patients, supra-coronary graft with refixation of the aortic valve in 2 and replacement of the descending aorta in 1 patient. All patients underwent close clinical and imaging follow-up in a specialized outpatient consultation. RESULTS: During a mean follow-up interval of 5.2+/-1.8 years, 14 patients (20.5%) underwent a total of 27 aortic reoperations. Seven patients had one and 6 patients had up to 4 reoperations; 13/14 patients had chronic aortic dissection. There was no hospital mortality and no major cardiac or neurologic morbidity following reoperation. During follow-up, 2 patients suffered from acute type B dissection following aortic root surgery and 3 patients surprisingly died: 2 from a rupture of a normal-sized descending aorta and one from intracranial hemorrhage. These 5 patients had had uncomplicated primary aortic root operation. CONCLUSIONS: The incidence of reoperations is significantly higher in patients who presented initially with acute type A aortic dissection than in those with dilatation only. In addition, this survey demonstrates that unexpected fatal outcome may appear in the remaining native aorta following uncomplicated elective aortic root surgery, even if the aorta is normal-sized. A close follow-up of all Marfan patients is necessary to detect asymptomatic changes requiring surgery because complex elective redo-operations can be performed with a very low perioperative risk.     

Aneurysmen der Aorta ascendens und des Aortenbogens

Background

The treatment of patients with aneurysms of the ascending aorta and aortic arch often represents a challenge due to their complexity. The clinical picture is characterized by further progression of the disease and severe complications, such as acute aortic dissection or even aortic rupture are not uncommon.

Objectives

This article gives a review of definitions, etiology and indications for operative treatment of patients with aneurysms of the ascending aorta and aortic arch.

Results and discussion

An aortic aneurysm of the ascending aorta is defined as a dilatation of all wall layers of the aorta of more than 50?% in comparison to the normal diameter. This is dependent on age, sex, body surface area and the underlying diseases. In most cases the cause is the presence of atherosclerosis. Connective tissue diseases, bicuspid or monocuspid aortic valves and aortitis represent additional risk factors. Surgical treatment of the ascending aorta, aortic root and aortic arch is carried out in patients without connective tissue diseases and a diameter of ≥?55 mm and for patients with Marfan syndrome and bicuspid aortic valves with a diameter of ≥?50 mm. Earlier indications for surgery are present with an aortic diameter of ≥?45 mm in patients with the following risk factors: familial disposition for aortic dissection (often unrecognized as sudden cardiac death), annual growth rate of the aortic diameter of >?2 mm, comorbid severe aortic valve or mitral valve insufficiency and female patients with a desire to have children. The standard surgical procedures include aortic root replacement, aortic valve reconstruction with aortic root or ascending aorta replacement and partial or total aortic arch replacement depending on the extent of the aneurysm. The so-called hybrid procedures for aortic arch surgery include frozen elephant trunk (ET) techniques and debranching procedures. Good perioperative and postoperative results can be achieved by early diagnostics and an assessment of the indications depending on the individual etiology. Individual and valve-preserving treatment strategies have a favorable influence on the operative outcome.     

Aortic root remodeling in a patient with Turner syndrome using the reference curves of aortic diameters in children

We report the successful surgical treatment of aortic regurgitation in a 27-year-old woman with Turner syndrome (TS) who was admitted with exacerbation of dyspnea on exertion. Echocardiography showed a bicuspid aortic valve with severe aortic regurgitation and computed tomography showed dilatation of the ascending aorta and aortic root. Due to the patient’s low body surface area (due to TS), standard determination of aortic size was not possible; therefore, we used the reference curves of aortic diameters in children. Because of the possibility of fatal ascending aortic dissection and rupture, we performed concomitant aortic root remodeling and aortic valve repair.     

A single-stage operation for bicuspid aortic valve, annulo-aortic ectasia, hypoplastic aortic arch, and coarctation of the aorta: A case report.

The patient was an 18-year-old man who had been diagnosed as having a bicuspid aortic valve and dilatation of the ascending aorta six years previously. As he grew up, aneurysmal change of the ascending aorta and hypertension in the upper body gradually progressed. Preoperative evaluation showed annulo-aortic ectasia and the following congenital abnormalities: bicuspid aortic valve, hypoplastic aortic arch, and coarctation of the aorta. Composite graft replacement and extended total aortic arch replacement were carried out.     

Ross procedure for ascending aortic replacement

Background. Patients with aortic valve disease and aneurysm or dilatation of the ascending aorta require both aortic valve replacement and treatment of their ascending aortic disease. In children and young adults, the Ross operation is preferred when the aortic valve requires replacement, but the efficacy of extending this operation to include replacement of the ascending aorta or reduction of the dilated aorta has not been tested.

Methods. We reviewed the medical records of 18 (5.9%) patients with aortic valve disease and an ascending aortic aneurysm and 26 (8.5%) patients with dilation of the ascending aorta, subgroups of 307 patients who had a Ross operation between August 1986 and February 1998. We examined operative and midterm results, including recent echocardiographic assessment of autograft valve function and ability of the autograft root and ascending aortic repair or replacement to maintain normal structural integrity.

Results. There was one operative death (2%) related to a perioperative stroke. Forty-two of 43 survivors have normal autograft valve function, with trace to mild autograft valve insufficiency, and one patient has moderate insufficiency at the most recent echocardiographic evaluation. None of the patients has dilatation of the autograft root or of the replaced or reduced ascending aorta.

Conclusions. Early results with extension of the Ross operation to include replacement of an ascending aortic aneurysm or vertical aortoplasty for reduction of a dilated ascending aorta are excellent, with autograft valve function equal to that seen in similar patients without ascending aortic disease.     

Surgical implication of aortic dissection on long-term outcome in marfan patients

We herein review our 17-year surgical experience for the treatment of ascending aortic aneurysm in patients with Marfan syndrome to clarify the risks of increased mortality and reoperation. The subjects consisted of 15 patients who had all undergone surgery for the aortic root and ascending aorta at Niigata University Hospital between July 1978 and January 1995. Aortic valve replacement and ascending aortic wrapping were performed in 5 patients, Bentall or Cabrol operation in 6, and combined aortic arch reconstruction and Carbrol operation in 2, as the initial surgery. Patients who had an aortic dissection (Stanford type A) at initial surgery were assigned to group I (n=7), while those with an aortic root aneurysm were assigned to group II (n=8). In group I, 3 patients required a second operation for the remaining aortic arch aneurysm, and 1 died due to a late rupture of the distal aneurysm. In group II, no patient needed a reoperation; however, 1 died due to an intracranial hemorrhage and another due to composite valve graft failure and distal dissection. The results thus indicate that aortic dissection seems to affect long-term outcome, and therefore the combined repair of the aortic root and transverse arch is recommended in Marfan patients with aortic dissection involving the transverse aortic arch.     

Medial Necrosis in Aortic Root Aneurysm after Repair of Tetralogy of Fallot

Abstract Aneurysmal dilatation of the aortic root occurs in some patients with tetralogy of Fallot (TOF) late after repair. It can lead to aortic valve insufficiency and rarely to aortic rupture or dissection. Aortic valve or aortic surgery is rarely performed in this group of patients. We present a case of aneurysmal dilatation of the ascending aorta treated with aortic valve sparing root replacement 43 years after the TOF repair. Histological examination of the aortic wall revealed medial necrosis. The implications of those finding and the timing of surgery are discussed. (J Card Surg 2010;25:230‐232)     

Single-stage surgical repair of type II acute aortic dissection associated with coarctation of the aorta

A 36-year-old man, who was referred for severe chest pains, was found to have acute type II aortic dissection associated with a dilated ascending aorta, aortic coarctation, and congenitally bicuspid valve. A single-stage surgical repair consisting of valve-sparing aortic root implantation and graft replacement of the ascending aorta, arch, and the coarctation segment was successful.     

Reduction of the sinotubular junction in patients undergoing ascending aortic replacement with coexisting aortic insufficiency

Abstract Background and Aim of the Study: Dilatation of the STJ may cause consequent aortic insufficiency (AI) in patients with normal aortic valve, in patients with ascending aortic aneurysm. In this study, we analyzed the results of ascending aorta replacement with STJ diameter reduction to correct consequent AI in patients with ascending aortic aneurysm. Methods: Forty‐five consecutive patients who had ascending aortic aneurysm underwent replacement of ascending aorta with reduction of the STJ diameter to correct AI. Mean age of the patients was 61.3 ± 5.2. Twenty‐six (57.8%) were female. Six patients had arch aneurysm. Postoperative echocardiographic studies were performed at discharge and annually thereafter. The mean duration of follow‐up was 4.6 ± 2.9 years. Results: Hospital mortality rate was 4.9% (n = 2). Three patients died during follow‐up. Three patients had late recurrence of AI that was caused by aortic root dilatation. One of these patients required aortic valve replacement because of severe aortic insufficiency. The five‐year survival and survival free from aortic insufficiency were 91.4%± 5.0% and 91.2%± 5.1%, respectively. Conclusions: Reduction of the diameter of STJ can be used to treat AI in patients with ascending aortic aneurysm with nearly normal aortic cusps. Midterm results of this procedure are encouraging. (J Card Surg 2011;26:88‐91)