Partial aortic root remodelling for fistula between the non-coronary sinus and the right atrium

This report describes a rare case of the fistula between the non-coronary sinus and the right atrium (RA) after ascending aortic replacement for chronic aortic dissection. A 67-year-old lady had been suddenly suffering from severe dyspnoea with general fatigue for a couple of days. Trans-thoracic echocardiogram in the emergency room demonstrated massive shunt flow from the non-coronary sinus to the RA with remarkable dilatation of the RA, right ventricle (RV) and inferior vena cava, similar to the rupture of sinus of Valsalva (Konno-type IV). The fistula was successfully treated by partial remodelling of the aortic root in an emergency basis because of her life-threatening illness. Some remaining diseased aortic root, which may be related to initial dissection or inappropriate use of gelatin-resorcin-formalin glue at the previous ascending aortic replacement, may cause this kind of serious events. Modified aortic root remodelling method with only diseased sinus resected was successfully applied to the localised aortic root disorder.     

Surgical techniques. Ascending aorta

Aneurysm and dissection are the most common diseases affecting the ascending aorta. Graft replacement of the ascending aorta is a straightforward cardiovascular procedure with excellent early and late results. When aneurysm or dissection extends into the aortic sinuses or arch, management becomes more complex and may entail replacement of the aortic root, aortic valve, or a portion of the aortic arch using hypothermic circulatory arrest. The optimal root prosthesis depends on several patient- and procedure-related variables. Valve-sparing procedures confer many long-term advantages and should be considered in all cases where the aortic valve leaflets are normal. The Ross procedure, although ideally suited for isolated aortic valve disease in young patients, may be applicable to some patients with combined aortic valve and ascending aortic disease, unless there is evidence of a systemic connective tissue disorder.     

Diagnostic and surgical management of patients with aneurysms of the thoracic aorta with various causes. Echocardiography and contrast enhanced computed tomography in prophylactic replacement of the ascending aorta

Sixty eight patients with aneurysms of the thoracic aorta were studied. Forty one had aortic dissection, 24 had dilatation only, and three had transverse aortic rupture. Sixteen had Marfan's syndrome; 17 had hypertension; and in eight there were other causal factors. In 17 the cause of the aneurysm was unknown. Histological examination did not help to establish the cause of aneurysm. Echocardiography failed to detect dissection of the ascending aorta in four (21%) out of 19 cases studied. The mortality rate in the whole series was 26%. Early (operative and hospital) and late deaths occurred in 20% and 6% of patients respectively. The early mortality rate was 40% in the 24 emergency cases of dissection of the ascending aorta, 9% in patients operated on for dilatation of the ascending and transverse aorta without dissection, and 8% in patients with chronic dissection of the ascending aorta who had elective operation. Early and late mortality rates were no higher in patients with Marfan's disease than in any of the other groups. It is suggested that contrast enhanced computer tomography should be performed in all patients with pronounced aortic root dilatation and in patients with Marfan's disease with symptoms which suggest dissection, even if they have only slight aortic root dilatation. Preventive replacement of the ascending aorta should be considered in more patients to reduce the number of emergency operations, in which the mortality rate is high. There is no definite limit of aortic root dilatation above which preventive replacement of the ascending aorta should be routinely considered.     

Long-term follow-up after separate replacement of the aortic valve and ascending aorta.

Between May 1974 and November 1991, 28 patients underwent a separate replacement of the aortic valve and the ascending aorta (20 male, eight female; 32 to 71 years old, x = 52 years). 23 patients were operated for ascending aortic aneurysm, three for chronic and two for acute aortic dissection type A. 17/18 patients living at the beginning of this study were re-investigated after a mean follow-up interval of 8.5 years postoperatively with DSA, thoraco-abdominal CT and echocardiography. 1/8 biological aortic valves and 1/20 mechanical valves had to be replaced (four years and two months postoperatively) for valve degeneration and paravalvular leakage respectively. Three patients developed a sinus of Valsalva aneurysm and were reoperated five, 9.2 and 9.3 years after primary repair. In all three patients histological signs of idiopathic degenerative media disease of the aorta were found. Two other patients presented with a perfused perigraft channel and therefore had to be reoperated. Patients with chronic aortic dissection type A and/or ascending aortic aneurysms presenting clinical or intraoperative signs of degenerative media disease of the aorta should undergo composite graft replacement to preclude formation of sinus Valsalva aneurysms.     

Diagnostic and surgical management of patients with aneurysms of the thoracic aorta with various causes. Echocardiography and contrast enhanced computed tomography in prophylactic replacement of the ascending aorta.

Sixty eight patients with aneurysms of the thoracic aorta were studied. Forty one had aortic dissection, 24 had dilatation only, and three had transverse aortic rupture. Sixteen had Marfan's syndrome; 17 had hypertension; and in eight there were other causal factors. In 17 the cause of the aneurysm was unknown. Histological examination did not help to establish the cause of aneurysm. Echocardiography failed to detect dissection of the ascending aorta in four (21%) out of 19 cases studied. The mortality rate in the whole series was 26%. Early (operative and hospital) and late deaths occurred in 20% and 6% of patients respectively. The early mortality rate was 40% in the 24 emergency cases of dissection of the ascending aorta, 9% in patients operated on for dilatation of the ascending and transverse aorta without dissection, and 8% in patients with chronic dissection of the ascending aorta who had elective operation. Early and late mortality rates were no higher in patients with Marfan's disease than in any of the other groups. It is suggested that contrast enhanced computer tomography should be performed in all patients with pronounced aortic root dilatation and in patients with Marfan's disease with symptoms which suggest dissection, even if they have only slight aortic root dilatation. Preventive replacement of the ascending aorta should be considered in more patients to reduce the number of emergency operations, in which the mortality rate is high. There is no definite limit of aortic root dilatation above which preventive replacement of the ascending aorta should be routinely considered.     

Supravalvular aortic stenosis: repair with the Yacoub procedure

Preservation and restoration of the aortic valve and root, as well as relief of the stenosis, is of paramount importance during surgical treatment of supravalvular aortic stenosis. A 23-year-old woman with a history of medically treated endocarditis presented with progressive fatigue and dyspnea on exertion. A diagnostic work-up revealed multiple stenoses and a small saccular aneurysm involving the diffusely hypoplastic ascending aorta, in addition to severe mitral regurgitation. The aortic root and ascending aorta were replaced using the Yacoub remodeling technique. Hemiarch replacement was performed under hypothermic circulatory arrest, and the mitral valve replaced. By using a remodeling technique, it was possible to augment the left sinus of Valsalva, to obtain the largest diameter at the sinotubular junction, and to adjust the spatial relationship of commissures that renders good leaflet coaptation. Although extended aortoplasty has been used widely for supravalvular aortic stenosis, the Yacoub procedure is a good option in diffuse type hypoplasia with multiple stenosis of the ascending aorta.     

Annulo-ectasing disease of the aorta: apropos of 10 cases

Ten patients were investigated and operated for severe aortic regurgitation due to dystrophic aortic dilatation. This is the third commonest cause of pure aortic regurgitation (18 p. 100) operated at Necker Hospital during the same period. This condition, comprising aneurysm of the ascending aorta, dilatation of the aortic ring and dystrophic aortic valves, is often responsible for severe aortic regurgitation and is noteworthy because of the associated risk of aortic dissection. Cardiovascular surgery is indicated and usually includes replacement of the ascending thoracic aorta with aortic valve replacement.     

Turner's syndrome and cardiovascular anomalies: a case report and review of the literature

Turner's syndrome is a genetic disease in which many cardiovascular anomalies have been reported, coarctation of the aorta being the most frequent. The most serious complication that can arise from these abnormalities is aortic dissection. The authors present an unusual case of Turner's syndrome with an aortic sinus aneurysm and severe aortic insufficiency in the absence of coarctation of the aorta. The various cardiovascular anomalies seen in Turner's syndrome, such as coarctation of the aorta, bicuspid aortic valve, aortic dissection, aortic sinus aneurysm and ascending aorta aneurysm, can best be understood on a common basis of congenital structural abnormalities involving the aorta and the aortic valve. The only evidence available for such an abnormality is the presence of cystic medial necrosis in the affected vascular tissues.     

83例主动脉根部疾病经典和非经典新术式外科治疗的临床经验和体会

目的:探讨主动脉根部病变外科治疗的经典和非经典术式的适应证和前景,总结经验教训。方法:2003年至2006年在8例新术式的基础上,我们对2000年至2004年北京安贞医院75例连续经典Bentall手术临床资料进行回顾性分析,根据保留瓣膜的主动脉根部成形术或重建术的适应证原则,即瓣环直径<28mm且瓣叶无明显病变,计算其潜在的应用百分率。结果:75例Bentall术死亡3例,死亡率为4.0%。4例改良Bentall术和4例保留瓣膜的主动脉根部成形术无手术和住院死亡,1例Yacoub术后第8个月因继发主动脉瓣返流而行2次瓣膜置换术。75例Bentall术中理论上适合进行瓣膜保留的主动脉根部成形术者有18例(24.0%)。结论:经典Bentall术依然是治疗主动脉根部病变和升主动脉扩张的标准术式,改良Bentall术理论上可能延长机械或生物瓣膜的使用寿命,更适合儿童马方综合征患者;尽管Yacoub术和David术存在主动脉瓣关闭不全(AI)复发的缺点,但是对主动脉瓣正常的根部病变是一种理想术式,并具有良好的应用前景。     

Long-term results of the bentall operation versus separate replacement of the ascending aorta and aortic valve

BACKGROUND AND AIMS OF THE STUDY: Aortic valve disease associated with ascending aorta dilatation can be treated either by separate replacement of the aortic valve and ascending aorta, or by a composite valved graft. METHODS: Between 1974 and 1999, 117 patients underwent a Bentall operation (BP), and 63 a separate replacement procedure (SP) of the ascending aorta and aortic valve. Anatomic lesions were dystrophic aneurysm in 79 patients, annuloectasia in 65, chronic dissection in 14, acute dissection in 18, and other etiology in four. Mean follow up was 3.45+/-3.47 and 8.75+/-6.8 years in the BP and SP groups, respectively. RESULTS: Early mortality was 7.7% in the BP group versus 11% in the SP group (p = NS). Actuarial survival at 10 years postoperatively in these groups was respectively 77.7+/-5.6% versus 75.8+/-6.9% (p = NS). However, freedom from late complication of the ascending aorta was significantly different (97.3+/-1.9% versus 68.3+/-9.0% at 10 years postoperatively). SP was identified as a risk factor for late complication of the ascending aorta by multivariate analysis (p = 0.01; odds ratio = 9). No statistical difference was observed on late reoperation rates. CONCLUSION: Separate replacement of the ascending aorta and aortic valve carries a higher complication rate for the remaining ascending aorta on long-term follow up when compared with the Bentall procedure. However, there were no differences in terms of late mortality.     

Clinical and hemodynamic follow-up of a patient after operation for dissection of an ascending aortic aneurysm secondary to coarctation of the aorta

We present clinical follow-up of a 20-year-old male with an aortic aneurysm secondary to aortic coarctation. The diagnosis of aortic aneurysm secondary to aortic coarctation was made in 1997. The patient did not agree to undergo any invasive or therapeutic procedures at that time. He presented to an emergency unit with severe chest pain after chest trauma obtained during judo exercises in 1998. Two-dimensional echocardiography showed bicuspid aortic valves, an ascending aortic aneurysm 6 cm in diameter with an intimal flap and false lumen, aortic coarctation distal to the left subclavian artery, and aortic insufficiency secondary to annular dilatation. Type II aortic dissection was confirmed by transesophageal echocardiography, which showed the dissection was confined to the ascending aorta. The dissection extended to the beginning of the arcus aorta. Following stabilization of the patient's clinical condition, balloon coarctation angioplasty was performed to reduce afterload and hypertension and to facilitate femoral artery cannulation for cardiopulmonary bypass. Surgical procedures included resection of the aortic valve and prosthetic valve implantation, resection of the ascending aorta, and interposition of a 22 mm Hamashied tubular vascular graft. At a follow-up visit 6 years later, the patient reported being easily fatigued and having palpitations. He had been suffering from hemolytic anemia and mild renal function impairment. Cardiac catheterisation and angiography showed a 40 mmHg gradient due to kinking of the aortic graft and no gradient at the coarctation site. We postulated the kinking of the aortic vascular graft may be related to an inappropriate vascular graft length. We also thought that the severe hemolysis was attributable to the disturbance of blood flow by a jet of blood at the site of the kinking aortic vascular graft. A second operation was performed because the renal function of the patient had decreased progressively and hemolysis symptoms increased. After the second operation, hemolysis on peripheral blood smears had disappeared and renal function had shown progressive improvements.     

Endarterectomy and external prosthetic grafting of the ascending and transverse aorta under hypothermic circulatory arrest

A 78-year-old woman presented with acute pulmonary edema, a blood pressure of 250/160 mmHg, and a 4/6 diastolic murmur of probable aortic origin. Aortography revealed 4+ aortic regurgitation, left ventricular dysfunction, a right coronary artery with good distal run-off but complete proximal occlusion, a fusiform aneurysm of the ascending and transverse aorta (with a transverse dissection in the left anterolateral wall of the upper ascending aorta, but no evidence of intramural lumen), and milder, isolated dilatation of the descending thoracic aorta. Upon operation, on 8 September 1987, I discovered an incompetent aortic valve, advanced atherosclerosis in the ascending and transverse aorta, and a loose intimal flap--but no false lumen--in the upper ascending aorta. After valve replacement and construction of a vein graft to the distal right coronary artery, I decided against replacement of the diseased segment of the ascending and transverse aorta and chose, instead, aortic endarterectomy reinforced by external grafting, as a simpler, quicker, and safer procedure for this patient. Safety was further enhanced by use of profound hypothermia (16 degrees C) to induce total circulatory arrest during the brief period (15 minutes) required for endarterectomy of the arch and approximation of the flap. The patient was discharged 19 days after surgery and continues well and asymptomatic to the present, 21 months after surgery; her milder dilatation of the descending thoracic aorta, which was not treated, is stable and is being monitored.     

Asymmetric medial degeneration of the intrapericardial aorta in aortic valve disease.

BACKGROUND: A geometrical and functional asymmetry in the normal aortic root has been recently demonstrated. Whether the distribution of medial degeneration (MD) within the wall of the dilated ascending aorta in adult patients reflects such asymmetry, still has to be assessed. METHODS: Nineteen patients with fibrocalcific aortic valve disease and dilatation of the intrapericardial aorta, without clinical signs and familiar history of primary elastic connective tissue disorders, underwent surgery. Biopsies (57 specimens) were taken in each patient from three areas of the ascending aortic wall distal to the three sinuses of Valsalva. MD lesions found at histology in each specimen were classified in three degrees. Comparisons were made between the three sites as to distribution of the three degrees and between one site and the other two as to incidence of the highest degree. RESULTS: A mild degree of MD was found in 26 specimens (45.6%), moderate in 14 (24.6%), severe in 17 (29.8%). The distribution of the three degrees of MD changes was significantly different between one of the three studied wall areas and the other two (P<0.001): a significantly greater incidence of the highest degree of involvement in the aortic wall distal to the non-coronary sinus than in the wall areas corresponding to the coronary sinuses was found (P<0.001). CONCLUSIONS: MD lesions in dilated intrapericardial aorta are more severe in the wall area distal to the non-coronary sinus, likely due to haemodynamic stress asymmetry.     

Aortic root dilatation in tetralogy of Fallot long-term after repair--histology of the aorta in tetralogy of Fallot: evidence of intrinsic aortopathy

The ascending aorta or pulmonary trunk in congenital heart disease may dilate out of proportion to hemodynamic or morphogenetic expectations, may become aneurysmal, and may rupture. A bicuspid aortic valve and/or coarctation of the aorta are consistently associated with ascending aortic and para-coarctation medial abnormalities. Congenital heart diseases such as single ventricle, truncus arteriosus, transposition of the great arteries and tetralogy of Fallot are also associated with aortic medial abnormalities. Aortic regurgitation in unrepaired tetralogy of Fallot imposes volume overload on both ventricles. A significant subset of adults late after repair of tetralogy of Fallot exhibits progressive aortic root dilatation which may lead to regurgitation and predispose to dissection and rupture which can be fatal, and necessitating aortic valve and aortic root surgery. The aortic dilatation relates medial abnormalities coupled with previous long-standing volume overload of the ascending aorta. Risk factors for aortic dilatation and regurgitation in tetralogy of Fallot relate to specific hemodynamic abnormalities such as pulmonary atresia, right aortic arch and a history of an aorto-pulmonary shunt, and patient demographics such as male sex and the association of chromosome 22q11 deletion. There is no current consensus on beta-blocker administration for limiting progressive dilatation of the aortic root in patients with congenital heart disease and repaired tetralogy of Fallot. Aortic root surgery should be considered for these patients and address aortic regurgitation and or prevent the risk of aortic dissection. Meticulous follow-up of the aortic root after repair, tetralogy of Fallot are thus recommended.     

Relation of aortic wall alterations and ascending aorta diameter in patients with bicuspid aortic valve

Patients with a bicuspid aortic valve tend to develop local enlargement in the convexity of the ascending aorta. There is controversial discussion as to whether this is a dilatation caused by abnormal hemodynamic stress or an aneurysm due to a common developmental defect of the aortic valve and the aortic media. Systematic studies of aortic wall specimens from patients with different diameters of the ascending aorta have not been available until now. We investigated histologically (HE, E.v.G., alpha-Actin, Alcian blue/van Gieson) aortic wall tissue obtained from 107 patients (31 female, 76 male, mean age 60.9+/-12.8 years) with a bicuspid aortic valve. According to the preoperative measurement of the ascending aorta diameter, which was done by CT scan, angiography or echocardiography, the patients were divided into three groups [Group 1: ascending aorta diameter <3.8 cm; Group 2: diameter >/=3.8-4.9 cm and Group 3: diameter >/=5.0 cm]. We looked for histological signs of dilatation or more severe structural changes. All patients of group 1 showed normal findings in histological examination. In 23 of 43 patients in group 2 (65.1%), we found histological signs of dilatation. Histological signs of dilatation were present in all patients in group 3. We conclude that in patients with a bicuspid aortic valve histological signs of dilatation were more frequently found with increasing diameter of the ascending aorta. More severe histological changes, such as cystic media necrosis, were not present.     

Aortic coarctation and aneurysms of the ascending aorta

Dissection and rupture of the aorta account for 20% of death in the natural history of aortic coarctation. We describe here in four patients in whom an ascending aortic aneurysm was associated with aortic coarctation. Three patients had aortic valve incompetence. In two cases there was a dissection. This had not been recognized preoperatively in one, in whom the intimal tear was small; in the other patient, with dissection and shock, the associated mild coarctation was demonstrated only at autopsy. Surgical treatment of patients with aortic coarctation and associated aortic aneurysm must include resection of both the stenotic isthmus and dilated section of the aorta, because of a documented poor prognosis. Furtherly, when evaluating patients with aortic dissection a coexisting coarctation although infrequent must be ruled out. If such an association is identified in emergency cases the dissected aorta should be repaired first, employing a suitable technique (double arterial cannulation, above and below the isthmus) in order to ensure adequate perfusion. When there is no acute dissection, repair of the coarctation should be undertaken first.     

Bentall operation in a patient with an anomalous left circumflex artery: Case report and review

Anomalous origin of a left circumflex artery from the right coronary sinus represents a technical challenge in patients who require aortic valve/root procedures. This case report describes a patient who presented with bicuspid aortic valve, anomalous origin of the circumflex artery, severe aortic regurgitation, and aneurysm of the ascending aorta as well as aortic root that was safely managed following the Bentall procedure with the combined button technique.     

Spontaneous rupture of the ascending aorta. A surgically treated case

Rupture of the ascending aorta may follow thoracic trauma or complicate an aortic aneurysm or acute dissection. It is otherwise extremely rare. The authors report a case of spontaneous rupture of the ascending aorta occurring in a patient with a pre-existing incomplete rupture of the ascending aorta, and treated surgically. The clinical presentation was of acute dissection with pericardial effusion. This diagnosis was excluded by aortography with multiple views which showed abnormalities of the aortic wall: an abnormal notch, continuity of the internal wall and extravasation of the contrast medium. These abnormalities are often minimal but should be recognised and surgery proposed as this is the only chance of a favourable outcome.     

Surgical Treatment of Ascending Aorta and Aortic Root Aneurysms

Aneurysms of the aortic root and ascending aorta are often due to degenerative disease of media. Aneurysm of the aortic root often affects patients in their second to fourth decades of life, whereas aneurysm of the ascending aorta occurs mostly in the fifth to seventh decades of life. These aneurysms can cause aortic insufficiency, dissection, and/or rupture. Current guidelines recommend surgical treatment when the diameter of the aneurysm exceeds 50 mm. In patients with family history of aortic dissection or with Loyes-Dietz syndrome (a more severe form of Marfan syndrome), surgery should be considered when they are even smaller. Composite replacement of the aortic valve and ascending aorta used to be the standard treatment for patients with aortic root aneurysms. During the past 2 decades, a conservative procedure whereby the aneurysm is replaced with Dacron graft and the aortic valve is preserved has gained widespread use, and the results have been excellent in experienced hands.     

The ascending aorta in aortic stenosis.

Dilatation of the ascending aorta, a frequently reported sign of aortic stenosis, was assessed in 47 patients for whom aortic angiograms has been recorded. Twenty-eight of the patients had rheumatic valvar disease and 19 had congenital aortic stenosis. A simple ratio between the maximal width of the mid-ascending aorta and the width of the aortic root was calculated. Patients with congenital aortic stenosis had significantly greater supravalvar aortic dilatation than did those with rheumatic aortic valve involvement. There was no correlation between the pressure difference across the aortic valve and degree of dilatation of the ascending aorta.