二叶主动脉瓣的超声分析

目的 探讨二叶主动脉瓣的超声心动图特征。方法 采用回顾性方法分析了26例先天性二叶主动脉瓣,其中主动脉瓣狭窄17例（有或无主动脉瓣关闭不全）和单纯主动脉瓣关闭不全9例。结果 在主动脉瓣狭窄组、轻度狭窄14例（82％）;二叶主动脉瓣感染性心内膜炎的发生率为42％;主动脉瓣狭窄组与单纯主动脉瓣反流组相比,主动脉瓣增厚钙化有极显性差异（P＜0．00005）,结果表明,二叶主动脉瓣狭窄的发生与主动脉瓣的增厚钙化有关,且主动脉瓣狭窄多为轻度狭窄。结论：二叶主动脉瓣狭窄的发生与主动脉瓣的增厚钙化有关,且主动脉瓣狭窄多为轻度狭窄。二叶主动脉瓣感染性心内膜炎的发生率较高。彩色多普勒二维超声心动图对二叶主动脉瓣的诊断具有重要价值。     

Congenitally bicuspid aortic valve causing severe,pure aortic regurgitation without superimposed infective endocarditis: Analysis of 13 patients requiring aortic valve replacement

Although stenosis and infective endocarditis are commonly appreciated complications of the congenitally bicuspid aortic valve, pure severe aortic regurgitation complicating this congenital malformation, unassociated with either stenosis or infection, is not well recognized. Among 189 patients who had aortic valve replacement at the National Heart, Lung, and Blood institute because of isolated pure aortic regurgitation, the congenitally bicuspid aortic valve, never the site of infective endocarditis, was responsible for the aortic regurgitation in 13 (7 percent). This report describes certain clinical and morphologic findings in 13 men, aged 26 to 65 years (mean 43), who required aortic valve replacement because of severe aortic regurgitation secondary to a noninfected, nonstenotic congenitally bicuspid aortic valve. Although not generally recognized, the noninfected congenitally bicuspid aortic valve is an important cause of pure aortic regurgitation severe enough to warrant aortic valve replacement.     

Feasibility of the ross procedure: its relationship with the bicuspid aortic valve

BACKGROUND AND AIM OF THE STUDY: The feasibility of the Ross procedure, and which patients benefit most from its performance, have not yet been fully established. The study aim was to analyze the relationship between the etiology of aortic valve disease, the feasibility of performing the Ross procedure, and late pulmonary autograft performance. METHODS: Between June 1995 and June 2001, 117 patients (77 males, 40 females; mean age 37+/-12 years) underwent the Ross procedure at the authors' institution. Of these patients, 53 (45.3%) had severe aortic stenosis, 53 (45.3%) had significant aortic insufficiency, four (3.4%) had active endocarditis, two (1.7%) had subaortic stenosis, and five (4.3%) had prosthesis dysfunction. Eighty-one patients (69%) had a bicuspid aortic valve. Pulmonary autograft dysfunction was defined as regurgitation grade > or =2, as registered by Doppler echocardiography. RESULTS: The Ross procedure was successful in 100 patients (85.5%); hospital mortality was 2.6% (n = 3). The procedure was not feasible in 17 patients (14.5%); of these, seven had bicuspid pulmonary valve, six had >3 mm multiple pulmonary valve fenestrations, three had severe pulmonary insufficiency, and one patient had dissection-related pulmonary valve injury. Twelve of 16 patients presenting with pulmonary valve defects had bicuspid aortic valve (p = 0.04). At six-year follow up, the probability of not requiring reoperation was 93% (confidence interval 86-100%). During follow up (30+/-14 months; range: 2-72 months), six patients presented with grade 2 pulmonary autograft insufficiency, three with grade 3, and two with grade 4. Six of the latter 11 patients (p = 0.03) had a history of bicuspid aortic valve with aortic regurgitation. Freedom from autograft dysfunction was 87% (confidence interval 82-92%). Patients with bicuspid aortic valve and aortic valve regurgitation had a higher tendency towards autograft dysfunction than those with bicuspid aortic valve and aortic stenosis (65% versus 100%, p = 0.004). CONCLUSION: The feasibility of performing the Ross procedure is high, unless there is presence of bicuspid aortic valve. Patients with bicuspid aortic valve and a history of aortic insufficiency tend to develop moderate autograft dysfunction during long-term follow up.     

Presence of a congenitally bicuspid aortic valve among patients having combined mitral and aortic valve replacement

Although bicuspid aortic valve occurs in an estimated 1% of adults and mitral valve prolapse in an estimated 5% of adults, occurrence of the 2 in the same patient is infrequent. During examination of operatively excised aortic and mitral valves because of dysfunction (stenosis and/or regurgitation), we encountered 16 patients who had congenitally bicuspid aortic valves associated with various types of dysfunctioning mitral valves. Eleven of the 16 patients had aortic stenosis (AS): 5 of them also had mitral stenosis, of rheumatic origin in 4 and secondary to mitral annular calcium in 1; the other 6 with aortic stenosis had pure mitral regurgitation (MR) secondary to mitral valve prolapse in 3, to ischemia in 2, and to unclear origin in 1. Of the 5 patients with pure aortic regurgitation, each also had pure mitral regurgitation: in 1 secondary to mitral valve prolapse and in 4 secondary to infective endocarditis. In conclusion, various types of mitral dysfunction severe enough to warrant mitral valve replacement occur in patients with bicuspid aortic valves. A proper search for mitral valve dysfunction in patients with bicuspid aortic valves appears warranted.     

Quadricuspid aortic valve as a cause of severe aortic regurgitation.

Quadricuspid aortic valves (QAVs) constitute a rare congenital malformation, with an incidence ranging from 0.008 to 0.048%. We report a case of severe aortic regurgitation associated with a QAV, which was diagnosed intraoperatively using transesophageal echocardiography. Since the first case described in 1862, 186 QAVs have been reported. In most cases, QAVs are associated with valve regurgitation, with a concurrent stenosis in some patients, while only a small number of QAVs are functionally normal. Once the diagnosis has been made, echocardiographic follow-up is recommended, as progression to severe valve regurgitation is common. Antibiotic prophylaxis is advisable for dental, and "dirty" surgical procedures, to minimize the risk of infective endocarditis.     

Ultrasonic aortic valve decalcification: serial Doppler echocardiographic follow-up

Serial two-dimensional and Doppler echocardiography was performed on 61 patients who had surgical ultrasonic aortic valve decalcification for calcific aortic stenosis. The mean patient age at the time of operation was 77.4 +/- 7.0 years; 93% had moderate to severe preoperative symptomatic limitation. Compared with preoperative studies, Doppler echocardiographic evaluation before hospital discharge revealed a significant reduction in the mean aortic valve pressure gradient (45.3 +/- 16.2 to 14.4 +/- 6.5 mm Hg, p less than 0.0001) and improvement in aortic valve area (0.62 +/- 0.17 to 1.33 +/- 0.33 cm2, p less than 0.0001). There was no initial change in aortic regurgitation grade. Follow-up Doppler echocardiographic evaluation was possible in 43 patients alive at 9.3 +/- 3.9 months. A small but statistically significant trend toward aortic restenosis was found; only one patient had severe restenosis. Severe aortic regurgitation had developed in 26% of patients and moderate aortic regurgitation in 37%. Aortic valve replacement was performed in six patients (14%) with severe symptomatic aortic regurgitation. Significant deficiency in central coaptation as a result of cusp scarification and retraction appeared to be the mechanism of postdecalcification regurgitation. Attempted salvage of the native aortic valve in severe calcific stenosis by ultrasonic decalcification adequately relieves stenosis but leads to an unacceptable incidence of significant aortic regurgitation at follow-up study.     

The natural history of a non-stenotic bicuspid aortic valve.

Forty-one patients in whom the diagnosis of a non-stenotic bicuspid aortic valve had been established by noninvasive techniques were followed up for a mean of 10.9 years. During this period, 2 patients required aortic valve replacement because of the development of calcific aortic valve stenosis at the ages of 52 and 64 and 5 others developed evidence of mild aortic valve stenosis. The appearance of calcium in a bicuspid aortic valve suggests the possibility of subsequent calcific aortic stenosis, and patients with this feature should be carefully followed up. Bacterial endocarditis on the aortic valve occurred in 3 patients, one of whom developed severe aortic regurgitation and subsequently died. Patients with a bicuspid aortic valve are at definite risk from bacterial endocarditis and should receive appropriate antibiotic prophylaxis. In 26/41 (63%) patients there was no clinical change during the follow-up period, including 7 of the patients over the age of 50.     

The ross procedure for replacement of the bicuspid aortic valve: mid-term results from 55 consecutive cases

BACKGROUND AND AIM OF THE STUDY: A congenitally dysfunctioning bicuspid aortic valve may require surgical treatment in children and young adults. This retrospective study evaluated mid-term clinical results obtained with the Ross procedure in this patient group. METHODS: Between July 1994 and December 2000, 55 patients (48 males, seven females; mean age 27+/-10 years; range: 7-49 years) underwent replacement of a diseased bicuspid aortic valve (stenosis in six cases (11%); insufficiency in 36 (65%); mixed lesion in 13 (24%)) with a pulmonary autograft. Mean NYHA functional class was 1.7. Five patients (9%) had healed endocarditis and six (11%) had previous cardiac surgery. The autograft was inserted as a subcoronary implant in two cases (4%), as a root in 40 (73%), and as a cylinder in 13 (23%). The right ventricular outflow tract was reconstructed with a cryopreserved pulmonary homograft in all cases. Mean cardiopulmonary bypass and aortic cross-clamp times were 207+/-34 min and 162+/-18 min, respectively. RESULTS: No early or late deaths had occurred at a mean follow up of 31+/-19 months. Two patients (4%) were re-explored for bleeding. Four patients (7%) experienced intraoperative coronary complications which resolved without sequelae. Two-dimensional echocardiographic evaluation of neoaortic valve competence at six months revealed no evidence of aortic valve regurgitation in 46 patients (84%), trivial regurgitation in seven (13%), mild regurgitation in one patient (2%), and moderate regurgitation in one patient. The latter patient (subcoronary implant) required reoperation. At six months, the degree of regression of left ventricular mass compared (versus preoperative) was 34+/-13% (p <0.05). Three patients (5%) showed mild dilatation (>4 cm) of the neoaortic root after two years follow up. All patients are currently asymptomatic, in NYHA class I, and enjoy a normal social lifestyle. CONCLUSION: The Ross procedure may be offered as a low-risk alternative in adolescents and young adults with a bicuspid aortic valve. Although the inclusion cylinder technique might help to prevent root dilatation, continued patient evaluation with regard to root sizing, evidence of neoaortic valve degeneration and homograft dysfunction is required in the long term.     

Clinical characteristics of bicuspid aortic valves in surgical patients

Of 413 patients undergoing surgery for aortic valve disease, 42 were confirmed to have congenital bicuspid aortic valve. There were 24 males and 18 females, with a mean age of 54.9 +/- 14.6 years. The etiology of the surgical indications in patients with bicuspid valves were degeneration, rheumatic disease, and infectious endocarditis. Thirty-two patients had aortic stenosis and 10 had aortic regurgitation. Infective endocarditis was seen mainly in patients < or =50 years of age at the time of surgery, while degenerative aortic stenosis occurred more often in older patients. The annular diameter measured during surgery was significantly narrower in the 42 patients with bicuspid valves compared to 371 patients with tricuspid valves who underwent aortic valve replacement during the same period. It is important to prevent infectious endocarditis in younger patients diagnosed with bicuspid aortic valve, whereas careful attention should be paid to decalcification during surgery in older patients because the vast majority suffer from degenerative aortic stenosis.     

Heart valve involvement in systemic lupus erythematosus: an echocardiographic study]

The purpose of this study was to evaluate the spectrum of morphologic and functional cardiac involvement in a selected population of patients with systemic lupus erythematosus (SLE) by means of echocardiography. Thirteen patients (2 male and 11 female) affected by SLE, mean age 41.9 years (range, 21-64), underwent M-Mode, two-dimensional and Doppler echocardiography. Eleven patients had renal disease and 3 of them were undergoing dialysis. One patient had findings of active disease. Six patients had systemic hypertension. None had a history suggestive of rheumatic fever or infective endocarditis. At echocardiographic study nine patients demonstrated findings of valvular involvement. These alterations were defined, according to the echocardiographic features, in two types: vegetation (verrucous Libman-Sacks endocarditis) and thickening. Vegetations were present in 6 patients, involving the mitral valve in all six and the aortic valve in three. The mitral valve vegetations were more frequent on the subannular portion of the posterior leaflet. Seven patients had valvular thickening: involvement of both mitral and aortic valve was present in five, and isolated mitral or aortic valve lesions in the remaining two patients. Combined valvular vegetation and thickening were observed in 4 patients. Eight patients had mild valvular dysfunction on Doppler examination: five isolated mitral regurgitation, two combined mitral and aortic regurgitation and one combined mitral stenosis and regurgitation. In agreement with previous reports, our study shows that valvular involvement in SLE is relatively frequent. Echocardiography can identify additional patterns of valvular lesions different from the known "verrucous Libman-Sacks endocarditis". The degree of valvular dysfunction is not important.     

Acute aortic regurgitation due to spontaneous rupture of a bicuspid aortic valve: detection by echocardiography

A 42-year-old man was admitted with acute severe aortic regurgitation. There were no signs of a systemic infection. M-mode and two-dimensional echocardiography revealed bicuspid aortic valve and echocardiographic features consistent with aortic leaflet rupture. The diagnosis was confirmed at surgery. This report illustrates that spontaneous rupture of a bicuspid aortic valve should be considered in acute aortic regurgitation without infective endocarditis.     

Pathology of aortic valve stenosis and pure aortic regurgitation a clinical morphologic assessment—part I

This two-part article examines the histologic and morphologic basis for stenotic and purely regurgitant aortic valves. Part I discusses stenotic aortic valves and Part II will discuss causes of purely regurgitant aortic valves. In over 95% of stenotic aortic valves, the etiology is one of three types: congenital (primarily bicuspid), degenerative, or rheumatic. Other rare causes of stenotic aortic valves include active infective endocarditis, homozygous type II hyperlipoproteinemia, and systemic lupus erythematosis. The causes of pure aortic regurgitation are multiple but can be separated into diseases affecting the valve (normal aorta) (infective endocarditis, congenital bicuspid, rheumatic, floppy), diseases affecting the walls of aorta (normal valve) (syphilis, Marfan's, dissection), disease affecting both aorta and valve (abnormal aorta, abnormal valve) (ankylosing spondylitis), and diseases affecting neither aorta nor valve (normal aorta, normal valve) (ventricular septal detect, systemic hypertension). Diseases affecting the aortic valve alone are the most common subgroup of conditions producing pure aortic valve regurgitation.     

Effect of aortic valve replacement on c-reactive protein in nonrheumatic aortic stenosis

Plasma levels of C-reactive protein were higher in 20 patients with bicuspid or trileaflet degenerative aortic stenosis than in 31 normal controls and in 19 patients with pure aortic regurgitation. C-reactive protein decreased from before to 6 months after aortic valve replacement for aortic stenosis. These observations suggest that aortic stenosis is an inflammatory disease.     

Brucella endocarditis of bicuspid aortic valve. Surgical treatment with successful result

The authors report a case of Brucella Melitensis endocarditis of a bicuspid aortic valve which caused rapid progression of the hemodynamic signs of aortic stenosis, and was associated with a para-aortic abscess and a pericardial effusion. Surgery resulted in correction of the hemodynamic abnormalities and cured the infection: the results were sustained 10 months after operation. This case illustrates the precision of the Doppler, echocardiographic diagnosis of the lesions, which was confirmed at surgery so that potentially dangerous cardiac catheterisation could be avoided.     

主动脉瓣四叶瓣畸形合并主动脉瓣病变的外科处理

目的 探讨主动脉瓣四叶瓣畸形对主动脉瓣功能的影响及其外科治疗.方法 2000年1月至2013年1月,我院通过经胸超声心动图和术中病理诊断主动脉瓣四叶瓣畸形13例.其中4例主动脉瓣的功能基本正常,9例合并主动脉瓣病变：重度关闭不全7例,重度关闭不全伴狭窄2例,合并二尖瓣中度关闭不全2例,三尖瓣中重度关闭不全1例.结果 13例患者中4例主动脉瓣功能正常者,继续随访中.9例合并主动脉瓣病变者,均行主动脉瓣置换术,同期行二尖瓣成形术2例、三尖瓣成形术1例.围术期无严重并发症及早期死亡,均康复出院.术后平均随访（6.34±5.17）年,心彩超提示主动脉瓣功能良好,无远期死亡.结论 主动脉瓣四叶瓣畸形是一种少见的先天性畸形,超声心动图有助于早期诊断.主动脉瓣功能正常时,可以随访;当合并主动脉瓣功能障碍时,应及时行主动脉瓣置换或修复,手术后可获得良好的远期效果.     

Association of left dominant coronary arterial system with congenital bicuspid aortic valve

Clinical angiographic studies have documented an association of left dominance of the coronary arteries with aortic stenosis and congenital bicuspid aortic valve. The postmortem arteriograms of 973 autopsy patients were reviewed for pattern of coronary dominance and the hearts examined for the nature of any aortic valve disease. There were 673 hearts (70 percent) with a right dominant pattern, 198 (20 percent) with equal dominance and 102 (10 percent) with left dominance. Of 34 hearts with congenital bicuspid aortic valve, 10 (29 percent) had left dominance, a difference significant at the 0.005 level. Of 44 hearts with calcific aortic stenosis, an acquired valve lesion, 9 (20 percent) had left dominance. Rheumatic aortic valve disease (47 cases) and aortic regurgitation (27 cases) had no apparent relation to the coronary arterial pattern. The results confirm the association of left coronary arterial dominance with congenital bicuspid aortic valve. It is suggested that a left dominant coronary system may arise as a consequence of disproportionately decreased blood flow in the left heart chambers, one cause of which is aortic valve stenosis, during early cardiogenesis.     

Incidence and prognosis of congenital aortic valve stenosis in Liverpool (1960-1990).

OBJECTIVE--To determine the incidence and prognosis of congenital aortic valve stenosis in the five Health Districts of Liverpool that make up the Merseyside area. DESIGN--The records of the Liverpool Congenital Malformations Registry and the Royal Liverpool Children's Hospital identified 239 patients (155 male, 84 female) born with aortic valve stenosis between 1960 and 1990. Patients were traced to assess the severity of stenosis at follow up. Information on the severity at presentation and all subsequent events was obtained. RESULTS--Congenital aortic valve stenosis occurred in 5.7% of patients with congenital heart disease born in the Merseyside area. The median age at presentation was 16 months (range 0-20 years). Stenosis was mild at presentation in 145 patients, moderate in 33, severe in one and critical in 21 and 39 had a bicuspid valve without stenosis. Additional cardiac lesions were significantly more common in children presenting under one year of age and in those with critical stenosis. The median duration of follow up was 9.2 years (range 1-28 years) and seven patients were lost to follow up. 81 operations were performed in 60 patients. The reoperation rate was 28.3% after a median duration of 8.7 years (range 2.5-18 years). 15% of patients who presented with mild stenosis subsequently required operation compared with 67% of those with moderate stenosis. There were no sudden unexpected deaths and no deaths after aortic valvotomy, except in those presenting with critical stenosis. Mortality was 16.7% but patients presenting with critical aortic stenosis had a much worse prognosis. Actuarial and hazard analysis showed that the survival and absence of serious events (aortic valve surgery or balloon dilatation, endocarditis, or death) were significantly better in patients who presented with mild aortic stenosis than in those who presented with moderate aortic stenosis. 75% of patients presenting with mild stenosis had not progressed to moderate stenosis after 10 years of follow up. CONCLUSIONS--Congenital aortic valve stenosis may be progressive even when it is mild at presentation. Patients presenting with mild stenosis, however, have a significantly better prognosis than those presenting with moderate stenosis. An accurate clinical and echocardiographic assessment of the severity of aortic valve stenosis at presentation provides a good guide to prognosis into early adult life.     

Retinal embolization of bicuspid aortic valve calcification

We report the case of a 36-year-old man with calcified bicuspid aorta. Aortic disease was diagnosed after retinal embolism, which caused loss of vision affecting the left eye. Doppler examination did not identify carotid stenosis. Transthoracic echocardiography showed thickness and calcification of two leaflets, moderate aortic regurgitation, high normal left ventricular diastolic dimensions, and high normal interventricular septum thickness. Parasternal long-axis view in the systolic frame showed dilated left ventricular outflow tract, dilated ascending aorta, and calcification and thickening of aortic leaflets, causing an echo-lucent area, in the diastolic frame. It also showed the eccentric position of the closed valve leaflets, short axis in the diastolic frame, and the absence of the typical "Y" letter sign, tracked by the three leaflets (septal, right coronary, and left coronary) in the normal tricuspid aortic valve. On long-axis parasternal acquisition, we note high normal interventricular septum thickness and high normal diastolic diameter. Color Doppler analysis from the apical five-chamber view showed aortic regurgitation; regurgitant jet area was about 44% of the outflow tract, so aortic regurgitation was classified as moderate. Pulsed Doppler showed a high normal value of peak aortic velocity. Transesophageal echocardiography was performed, but no further findings were identified. We think that the likely retinal embolism sources were the aortic calcified leaflets, even if the aortic valve was not stenotic and with no signs of endocarditis. However, because we cannot exclude other potential causes, we put the patient on anticoagulant therapy. Furthermore, aortic valve replacement was not advised.     

Parachute mitral valve with severe mitral regurgitation in an adult patient

Parachute mitral valve (PMV) is rarely seen in the adult population, in isolation or in association with other congenital heart defects, since most patients may have had milder lesions previously that were asymptomatic early in life, or were not detected due to lack of a comprehensive examination. We report a case of an 18-year-old woman with a history of exertional dyspnea, atypical chest pain, and cough for about 1 year. The echocardiographic examination identified a PMV with severe mitral regurgitation associated with bicuspid aortic valve and coarctation of the aorta. Most patients present with mitral stenosis of varying degree of severity, and rarely present with severe mitral regurgitation as seen in our patient.     

Ultrasonic decalcification of the aortic valve. Initial experience]

We report our initial experience in 2 patients with degenerative calcific aortic stenosis who underwent ultrasonic debridement of the aortic valve. Compared with preoperative studies, doppler echocardiographic and hemodynamic evaluation before hospital discharge revealed a reduction in the mean aortic valve pressure gradient (80 and 65 mmHg to less than 10 mmHg). There was no change in aortic regurgitation grade. Follow-up doppler echocardiographic evaluation at four and six months showed no changes in gradient or regurgitation in the comparison to the postoperative data. Long-term results will show the convenience to attempt or not ultrasonic salvage of the native aortic valve in severe calcific stenosis.