Outcome of aortic valve repair in children with congenital aortic valve insufficiency

OBJECTIVE: Surgical aortic valvotomy has a long history of providing excellent palliation for aortic stenosis in infancy and childhood. The fate of aortic valve repairs for dominant aortic regurgitation in this same age group is considerably less clear. METHODS: From 1990 to 2000, a total of 21 patients underwent aortic valve repair for aortic regurgitation at our institution. Seventeen patients were younger than 17 years at the time of repair (3-17 years, mean 8.1 +/- 3.7 years). Of these 17 children, 6 (35%) had bicuspid valves and 11 (65%) had tricuspid valves. Type of repair varied with valve type, but repair generally consisted of commissure resuspension, partial commissure closure, triangular resection of redundant leaflets, or some combination. RESULTS: There were no deaths. Follow-up ranged from 1 to 11 years (mean 5.3 +/- 2.4 years). At present 3 of 17 (17.6%) have mild aortic regurgitation according to echocardiography and 6 (35.2%) have moderate aortic regurgitation. In 8 of 17 cases (47.1%) the repair clearly failed, requiring reoperation from 0.5 to 73 months after the original operation (mean 18.9 months). Reoperation consisted of 6 Ross procedures and 2 mechanical aortic valve replacements. There were no deaths at the secondary operation. CONCLUSION: Aortic valve repair in children with a dominant feature of aortic insufficiency tended to fail progressively and at a high rate. Leaflet thickening was associated with higher risk of repair failure in this series. The threshold for aortic valve replacement should remain low.     

Pericardial patch augmentation for repair of incompetent bicuspid aortic valves at midterm

Objective: Reoperation rates after repair of bicuspid aortic valves are higher than for mitral valve reconstruction. Satisfactory results have been reported for patch augmentation for tricuspid aortic valves. We have applied this technique for the repair of bicuspid aortic valves. Methods: Autologous pericardium is sutured to the free edge of the prolapsing bicuspid leaflet. A large coaptation surface is created and competence of the bicuspid valve is achieved. Forty patients underwent reconstruction of their bicuspid aortic valves by pericardial patch augmentation. Patients were followed up at regular intervals by echocardiography in yearly intervals. Results: There were no perioperative deaths. One year postoperatively, one patient died due to endocarditis. Seven patients (17.5%) had aortic regurgitation grade I, and the other 33 patients had non or trivial aortic regurgitation at discharge. At 4.2 ± 3.1 years postoperatively, only four patients (10%) had aortic regurgitation grade I. There were no cases of progression of regurgitation. Planimetric effective orifice areas ranged above 2 cm². Mean aortic gradients dropped from 8.2 ± 4.8 mmHg at discharge to 3.8 ± 3 at four years and the mean height of coaptation surface from 14.7 ± 2 mm to 12.3 ± 4, respectively. Conclusions: The pericardial patch augmentation technique increases coaptation surface, and thus provides reliable early and midterm competence of reconstructed bicuspid aortic valves.     

Aortic valve reconstruction using self-developed aortic valve plasty system in aortic valve disease

Aortic valve disease is usually treated by prosthetic valve replacement. We have performed aortic valve plasty (AVP) using glutaraldehyde-treated autologous pericardium. AVP was performed for 88 patients from April 2007 through August 2009. Sixty-five patients had aortic stenosis, and 23 patients had aortic regurgitation (AR). Twenty-one patients showed bicuspid aortic valves, and one patient showed quadricuspid valve. There were 43 males and 45 females. Their mean age was 70.6±10.5 years old. First, diseased leaflets excised. Then, the distance between each commissure was measured. The new leaflet were trimmed with an original template from a glutaraldehyde-treated autologous pericardium sample. Finally, the annular margin of the pericardial leaflet was running sutured to each annulus. There was no operative mortality or embolic event. Postoperative echocardiography revealed a mean peak pressure gradient (PG) of 19.0±9.1 mmHg one week after surgery. Thirty-two patients had echocardiography one year after surgery. The peak PG became 12.9±5.8 mmHg. Ten patients showed no AR, 20 patients showed trivial AR, and two patients showed mild AR. Freedom from reoperation is 100% at three years follow-up.     

Outcomes of aortic valve repair according to valve morphology and surgical techniques

OBJECTIVES The aim of this study was to assess the impact of aortic valve morphology and different surgical aortic valve repair techniques on long-term clinical outcomes. METHODS Between February 2003 and May 2010, 216 patients with aortic insufficiency underwent aortic valve repair in our institution. Ages ranged between 26 and 82 years (mean 53?±?15 years). Aortic valve dysfunctions, according to functional classification, were: type I in 55 patients (25.5%), type II in 126 (58.3%) and type III in 35 (16.2%). Sixty-six patients (27.7%) had a bicuspid valve. Aortic valve repair techniques included sub-commissural plasty in 138 patients, plication in 84, free-edge reinforcement in 80, resection of raphe plus re-suturing in 40 and the chordae technique in 52. Concomitant surgical procedures were CABG in 22 (10%) patients, mitral valve repair in 12 (5.5%), aortic valve-sparing re-implantation in 78 (36%) and ascending aorta replacement in 69 (32%). Mean follow-up was 42?±?16 months and was 100% complete. RESULTS There were six early deaths (2.7%). Overall late survival was 91.5% (18 late deaths). There were 15 (6.9%) late cardiac-related deaths. NYHA functional class was ≤II in all patients. At follow-up, 28 (14.5%) patients had recurrent aortic insufficiency?≥?grade II. The freedom from valve-related events was significantly different between bicuspid and tricuspid valve implantation (P?相似文献   

Morphometric analysis of aortic media in patients with bicuspid and tricuspid aortic valve

BACKGROUND: Patients with bicuspid aortic valves tend to develop dilatation of the ascending aorta. The aim of this study was to analyze whether or not there is any histologic difference in the aortic media of patients with a bicuspid aortic valve or a tricuspid aortic valve. METHODS: A morphometric analysis of the wall of the ascending aorta was performed in 107 patients with bicuspid aortic valves undergoing aortic valve operations. The thickness of the elastic lamellae of the aortic media and the distances between the elastic lamellae were measured with the use of an image analysis system. The histologic specimens of the ascending aorta from 61 surgical patients with tricuspid aortic valve disease served as a control. RESULTS: The patients with bicuspid aortic valves had thinner elastic lamellae of the aortic media (2.71 +/- 0.23 microm) of the ascending aortic wall than the patients with tricuspid aortic valve disease (2.83 +/- 0.23 microm) (p = 0.006). The patients with bicuspid aortic valves also had greater distances between the elastic lamellae (27.21 +/- 8.69 microm) of the ascending aortic wall in comparison with the patients with tricuspid aortic valve disease (24.34 +/- 5.32 microm) (p = 0.033). There was no difference in the total thickness of the aortic media between the groups (p = 0.62). CONCLUSIONS: Patients with a bicuspid aortic valve had thinner elastic lamellae of the aortic media and greater distances between the elastic lamellae than patients with a tricuspid aortic valve.     

An operative case of left ventricular-right atrial communication complicated with aortic valve stenosis and regurgitation

A 70-year-old woman suffering from aortic valve stenosis and regurgitation and tricuspid valve regurgitation was admitted to our hospital. At the operation, the right atrium and both ventricles were dilated, and a thrill was felt over the aortic root and right atrium. The aortic valve was bicuspid and heavily calcified. After aortic leaflets were resected, a defect was seen in the left ventricle. Then the right atrium was opened. The defect was located in the atrioventricular membranous septum just above the septal leaflet of the tricuspid valve. The defect was diagnosed to be a left ventricular-right atrial communication (group I according to Riemenschneider's classification). The defect was repaired directly and then the aortic valve was replaced with a prosthesis. After the operation, the heart murmur disappeared. Eleven days after the operation, the patient developed a transient complete AV block for a month.     

Bicuspid aortic valve.

The bicuspid aortic valve is a common congenital cardiac anomaly, having an incidence in the general population of 0.9% to 2.0% and a frequency of 54% in all patients aged >15 years with valvular aortic stenosis. In most cases it remains undetected until infective endocarditis or calcification supervenes. The bicuspid aortic valve may function normally throughout life, may develop progressive calcification and stenosis or may develop regurgitation with or without infection. The association of the bicuspid aortic valve with dissection of the aorta is also common. The recognition of the bicuspid valve in patients with aortic valve disease remains an important challenge to the clinician, whereas preoperative knowledge of valve morphology would be helpful in planning the surgery. Antibiotic prophylaxis is also recommended in such patients, since these valves are likely to become the most important intrinsic cardiac predisposition for infective endocarditis with the virtual disappearance of rheumatic fever in developed countries.     

Right ventricular reduction as an adjunct procedure in tricuspid valve repair

Functional tricuspid regurgitation secondary to mitral valve disease can not be attributed to the dilatation of the tricuspid annulus alone. Furthermore, geometrical changes of the right ventricle lead to tethering of the tricuspid valve leaflets and thereby to an incomplete leaflet coaptation. With this pathologic entity, conventional isolated tricuspid valve annuloplasty will presumably result in significant residual tricuspid regurgitation. The surgical goal should be the reduction of tricuspid annulus dilatation and annihilation of tethering forces on the tricuspid leaflets. In combination with conventional tricuspid valve annuloplasty, right ventricular reduction surgery, as demonstrated, may be effective in reaching these goals and hereby avoiding residual tricuspid regurgitation in this patient population.     

Ebstein's anomaly of the tricuspid valve: the cone repair

Ebstein's anomaly is a cardiac malformation affecting the tricuspid valve and right ventricle with a wide range of anatomic and pathophysiologic presentations. The cone repair technique is described in detail, as applied to several types of this complex congenital heart disease. The importance of extensive mobilization of displaced and tethered tricuspid leaflets to permit a good leaflet-to-leaflet coaptation after the cone construction is highlighted. This technique was performed in 100 patients with a hospital mortality rate of 3.0%, good clinical outcome, and no need for tricuspid valve replacement. Echocardiograph results showed good anatomic and functional tricuspid valves at immediate and long-term postoperative follow-up.     

Surgical pathology of aortic valve disease. A study based on 602 specimens

A consecutive series of 602 surgically excised aortic valves was evaluated by means of macroscopic and histological study. Pure aortic stenosis was diagnosed in 140 patients, pure incompetence in 254 and combined dysfunction in 208. Of the cases with pure aortic stenosis, 38% were rheumatic, 34% were calcified bicuspid valves and 23% showed dystrophic calcification. Half the patients with pure aortic regurgitation showed aortic root dilatation. Most cases of combined aortic stenosis and regurgitation were the sequelae of rheumatic fever. A male prevalence was detectable in each group (mean male: female ratio = 2.6), and was highest in infective endocarditis and aortic root dilatation. Infective endocarditis was a frequent complication of congenitally bicuspid valves. In conclusion, rheumatic disease is still a frequent cause for surgical replacement of the aortic valve. At least half the explanted aortic valves have degenerative or congenital diseases which are often the site of a superimposed infective endocarditis.     

Valvuloplasty for aortic valve regurgitation resulting from cusp prolapse

Three adults, 2 with tricuspid aortic valve and 1 with bicuspid valve, underwent valvuloplasty for aortic valve regurgitation resulting from cusp prolapse. Surgical procedures consisted of combined cusp plication by triangular cusp resection and subcommissural annuloplasty. Doppler echocardiography revealed trivial aortic valve regurgitation intraoperatively and less than I/IV at discharge in all cases. After mean follow-up of 15 months, 2 tricuspid aortic valve patients remain I/IV regurgitation and II/IV in the bicuspid patient. Although long-term results remain unclear, our results show that this procedure is feasible and beneficial in patients with aortic valve regurgitation due to cusp prolapse.     

Pure aortic regurgitation due to congenital bicuspid valve: a case report

A rare case of pure severe aortic regurgitation (AR) accompanied with congenital bicuspid aortic valve is presented. A 36-year-old female was admitted with rapid development of congestive heart failure. Examinations clarified severe AR with congenital bicuspid valve. Either calcification or vegetation on the valve was completely denied. At operation, both leaflets were apart from each other with a distance of 7 mm at the anterior commissura. A mechanical valve (Bj?rk-Shiley 23A) was sutured and she has been well. Pathological study of the resected leaflets revealed neither calcium deposit nor finding of infection but collagenous fibrous tissue with focal mucinous degeneration. A few cases of AR with bicuspid aortic valve have been reported in patients of annuloaortic ectasia. The partial annular ectasia was suspected as the cause of solitary aortic regurgitation with bicuspid aortic valve in our case.     

Bicuspid aortic valve disease and pulmonary autograft root dilatation after the Ross procedure: a clinicopathologic study.

OBJECTIVE: Bicuspid aortic valve disease has been associated with histologic abnormalities of the aortic root. Recent reports have suggested similar alterations may exist in the pulmonary artery of patients with bicuspid aortic valve. The present study was undertaken to define the histologic condition of the aortic and pulmonary artery root in bicuspid aortic valve disease and the relationship with pulmonary autograft root dilatation after the Ross procedure. METHODS: In 17 patients undergoing aortic root replacement with the pulmonary autograft, biopsy specimens of the aortic root and pulmonary artery trunk were collected. Clinical and histologic findings of patients with bicuspid aortic valves were compared with those with tricuspid aortic valves. RESULTS: There were 9 patients (8 male, 1 female) with bicuspid aortic valve (group 1) and 8 (all male) with tricuspid aortic valve (group 2). Mean age was comparable (24.4 +/- 9.8 vs 23.6 +/- 10.8 years, P =.9). Aortic insufficiency as an indication for operation was more common in group 1 (9/9 vs 5/8, P =.007), whereas preoperative aortic root dilatation was equally prevalent (4/9 vs 1/8, P =.1). Prior aortic valve repair had been performed in 2 patients (1/9 vs 1/8, P =.9). Prevalence of cystic medionecrosis of the aortic wall was similar in the 2 groups (4/9 vs 3/8, P =.6). Cystic medionecrosis of the pulmonary artery trunk was found only in 1 patient with tricuspid aortic valve (0/9 vs 1/8, P =.3). During a mean follow-up of 26.5 +/- 12.2 months (32.1 +/- 12.7 vs 20.1 +/- 7.4 months, P =.04), prevalence of pulmonary autograft root dilatation (greater than 4.0 cm) was equally represented in patients with native bicuspid or tricuspid aortic valve (3/9 vs 2/8, P =.6). CONCLUSIONS: Histologic abnormalities of the pulmonary artery root are rare and equally prevalent in young patients with bicuspid and tricuspid aortic valves. On the contrary, root dilatation is relatively common late after autograft root replacement but appears unrelated to bicuspid aortic valve disease or to pre-existing degenerative changes of the pulmonary artery root.     

Successful valve repair for congenital bicuspid aortic valve and degenerated prolapsed mitral valve

A 54-year-old man with congenital bicuspid aortic valve underwent simultaneous valve repair for aortic and mitral regurgitation. Surgical technique consisted of plication of redundant aortic valve repair and mitral annuloplasty with chordal replacement. One-year follow-up transthoracic echocardiography showed no valve regurgitation. Valve repair for both bicuspid aortic valve and mitral valve regurgitation should be the first option in this subset of patients.     

Vascular matrix remodeling in patients with bicuspid aortic valve malformations: implications for aortic dilatation

BACKGROUND: Patients with bicuspid aortic valve malformations are at an increased risk of aortic dilatation, aneurysm formation, and dissection. Vascular tissues with deficient fibrillin-1 microfibrils release matrix metalloproteinases, enzymes that weaken the vessel wall by degrading elastic matrix components. In bicuspid aortic valve disease a deficiency of fibrillin-1 and increased matrix metalloproteinase matrix degradation might result in aortic degeneration and dilatation. METHODS: Samples of the pulmonary artery and aorta were obtained from surgical patients with bicuspid aortic valves (n = 21) and tricuspid aortic valves (n = 16). RESULTS: Fibrillin-1 content was reduced in bicuspid aortic valve aortas compared with that seen in tricuspid aortic valve aortas (P =.001), whereas the associated matrix components, elastin and collagen, were unchanged (P =.51 and P =.21). Reductions of aortic fibrillin-1 content were independent of valve function and patient age. Compared with tricuspid aortic valve aorta, matrix metalloproteinase 2 activity was increased more than 2-fold in bicuspid aortic valve aortas (P =.04) and correlated positively with aortic diameter (r = 0.74, P =.05). Matrix metalloproteinase 9 activity was not significantly different. Fibrillin-1 content was also reduced in the pulmonary arteries of patients with bicuspid aortic valves (P =.06), suggesting a systemic deficiency of fibrillin-1. Promatrix metalloproteinase 2 was increased (P =.04), reflecting an increased production of matrix metalloproteinase 2 in these fibrillin-1-deficient tissues, whereas active matrix metalloproteinase 2 and matrix metalloproteinase 9 species were unchanged, and correspondingly, the pulmonary arteries were not dilated. CONCLUSIONS: Deficient fibrillin-1 content in the vasculature of patients with bicuspid aortic valves might trigger matrix metalloproteinase production, leading to matrix disruption and dilatation. This process of vascular matrix remodeling in patients with bicuspid aortic valves offers novel therapeutic targets to prevent the aortic degeneration and dilatation characteristic of this disease.     

Tricuspid valve involvement in combined mitral and aortic valve surgery.

BACKGROUND: Combined mitral and aortic valve disease requiring surgery may involve the tricuspid valve as well. Our treatment policy is conservative especially for tricuspid regurgitation which is operated on when severe only. METHODS: A retrospective study was performed at a tertiary and secondary referral center for cardiovascular disease. Over a 15-year period, 65 consecutive patients underwent aortic and mitral surgery with concomitant tricuspid regurgitation and/or stenosis. Fifty-five/65 (85%) patients were in NYHA class III-IV. Fifty-eight/65 (89%) patients had tricuspid regurgitation secondary to right chamber dilatation and 7/65 (11%) had tricuspid stenosis and/or regurgitation because of previous endocarditis. Twenty-two/65 (34%) tricuspid valves were operated on: 18/22 (82%) de Vega annuloplasty, 2/22 (9%) commissurotomies and 2/22 (9%) prosthetic valves. Mortality and complications were recorded during a mean follow-up of 5.3 yrs (range, 6 months-15.3 yrs). Event-free survivals were determined using the Kaplan-Meier method. RESULTS: Hospital mortality was 6.2% (4/65) and the complication rate was 18.5% (12/65). The freedom from late valve related mortality and morbidity at 5, 10 and 15 years was 86+/-5.5%, 81.9+/-6.8%, and 81.9+/-6.8% respectively. One valve related complication was due to the tricuspid valve. At last follow-up, 87% (47/54) of the survivors were in NYHA class I-II. CONCLUSIONS: With a conservative policy of tricuspid valve intervention, functional results of this patient population are good and long-term valve related morbidity and mortality are mainly related to the mitral and aortic valve procedures.     

Correction of leaflet prolapse in valve-preserving aortic replacement: pushing the limits?

BACKGROUND: For aortic dilatation with morphologically intact leaflets, valve-preserving aortic replacement has become an accepted treatment modality. Leaflet prolapse, however, may be present, making composite replacement the most frequent choice. Alternatively, valve preservation may be combined with correction of leaflet prolapse. The results of this approach should be comparable with those of valve-preserving aortic surgery in the presence of normal leaflets. METHODS: Between 1995 and 2002, 156 patients were treated by valve-preserving surgery. The aortic valve was bicuspid in 46, and tricuspid in 110 instances. In 88 aortic valves, apparently normal leaflet coaptation (normal, 12 bicuspid and 76 tricuspid), and in 68 instances, prolapse of one or more leaflets, was observed. Root remodeling (n = 133) or aortic replacement with valve reimplantation (n = 23) were performed. Leaflet prolapse was corrected by triangular resection (n = 16) or plicating sutures (n = 59), mostly placed in the central portion of the leaflet. RESULTS: Neither operative mortality nor 5-year survival were influenced by the additional correction of prolapse. Freedom from reoperation at 1 year (normal, 98.8%; prolapse, 96.5%) and 5 years (normal, 97.3%; prolapse, 96.5%) were comparable in both cohorts, as was freedom from aortic regurgitation > or = II at 1 year (normal, 98.8%; prolapse, 94.2%) and 5 years (94.4%). CONCLUSIONS: Surgical correction of leaflet prolapse in combination with proximal aortic replacement is feasible with good results. Midterm results are identical with those known for morphologically normal leaflets. Repair of prolapse allows for preservation of the native valve in most patients with aortic regurgitation and aortic pathology, and thus appears a beneficial addition to valve-preserving surgery.     

Repair of flail leaflet of the tricuspid valve by a simple cusp remodeling technique

BACKGROUND AND AIM: The approach of repairing tricuspid valve insufficiency caused by congenital lack of chordae or traumatic rupture of chordae is often complicated and difficult. We try to present an alternative method and midterm results. METHODS: Between April 1997 and December 2004, eight patients (5 males, 3 females; mean age 23.9 +/- 5.8 years; range: 8 to 57 years) with severe tricuspid regurgitation (congenital lack of chordae in 5 cases and traumatic rupture of chordae in 3 cases) underwent surgical repair at Fu Wai Hospital. Four patients were in NYHA (New York Heart Association) class III, and 4 in class IV. Eight flail anterior leaflets and one flail septal leaflet of the tricuspid valve with massive tricuspid regurgitation were identified by echocardiography and the spaces of the free edges of the flail leaflets ranged from 20 to 30 mm. Tricuspid repair was performed under hypothermic cardiopulmonary bypass. The free edge of the affected cusp segment was sutured in folio, the segment of annulus devoid of leaflet was plicated, and the neo-annulus was fixed with a flexible annuloplasty ring. RESULTS: All patients survived and recovered after the operation. Echocardiography showed good coaptation with no regurgitation of the tricuspid valve in five patients and a mild residual tricuspid regurgitation in three patients. A remarkable decrease in the diameter of the right ventricle was observed, from a mean of 42.6 +/- 12.5 mm to a mean of 23.6 +/- 5.3mm (p < 0.01). Mean follow up was 50 +/- 42.9 months. Six patients were in NYHA class I, and two in class II and III. Except for one patient who had a mild-to-moderate increase in tricuspid regurgitation a year later, all the other patients were doing well. CONCLUSION: The procedure provided a simple and valuable option for repair of flail leaflet of tricuspid valve caused by congenital lack of chordae or traumatic rupture of chordae.     

Surgery of aortic valvular disease with congenital bicuspid or quadricuspid aortic valve

Twelve patients (age 18 to 69 years old) with surgical treatment for congenital bicuspid aortic valve and one (age 26 years old) for quadricuspid aortic valve were reported. These 13 patients occupied 12.3% of the total number of patients with aortic valve replacement during the same period. Among the patients with bicuspid aortic valve, 10 patients were male (83%) and 2 were female (17%). Seven patients exhibited aortic stenosis and 5 showed predominant aortic regurgitation. The mean age of patients with aortic stenosis was higher than that of patients with aortic regurgitation. (63.3 years vs 32.0 years old). Infective endocarditis occurred in 2 patients (17%). Three patients had pure aortic regurgitation without evidence of endocarditis. One 26-year-old female patient who had a quadricuspid aortic valve presented with aortic regurgitation associated with PDA. All patients underwent aortic valve replacement with no early and late deaths.     

三尖瓣脱垂的外科治疗

目的探索应用三尖瓣脱垂瓣缘折叠缝合技术治疗三尖瓣关闭不全的外科方法和疗效。方法1997年4月至2006年3月为6例先天性三尖瓣前叶腱索缺如和3例外伤性腱索断裂的患者实施了外科矫治手术,其中男性6例,女性3例,年龄8～57岁。术前9例患者均有三尖瓣重度关闭不全,右心室前后径均值为(43.6±4.2)mm。5例患者心功能为Ⅲ级,4例为Ⅳ级。连续对折缝合脱垂的三尖瓣瓣缘,折叠缝合脱垂瓣叶相对应的瓣环,并用成形环固定成形后三尖瓣瓣环。结果9例患者术后恢复顺利,无死亡。术后超声心动图检查示:6例患者三尖瓣对合良好无反流,3例患者有少量反流。所有患者术后右心室前后径均显著减小,术后均值为(24.0±1.8)mm,与术前相比差异有统计学意义(P<0.01)。3例房颤心律的患者均转为窦性心律。患者随访1～109个月,除1例患者外,其他8例患者三尖瓣成形效果稳定。8例患者心功能为Ⅰ～Ⅱ级,1例为Ⅲ级。结论应用三尖瓣脱垂瓣叶及其相对应的瓣环折叠技术,可有效修复先天性三尖瓣部分腱索缺如和胸外伤后三尖瓣腱索断裂所致的三尖瓣重度关闭不全。