Prevalence and clinical significance of aortic valve prolapse

The prevalence and clinical significance of aortic valve prolapse were determined prospectively in 2000 consecutive patients undergoing routine clinical cross sectional echocardiography. Two hundred and twelve patients were excluded because the aortic cusps were not adequately visualised. Aortic valve prolapse was defined as downward displacement of cuspal material below a line joining the points of attachment of the aortic valve leaflets. Twenty four cases of aortic valve prolapse (1.2%) were identified. The patients were aged 12-64 years and nine were women. All had underlying valvar heart disease and the commonest lesion (in 11 cases) was prolapse of the larger cusp in bicuspid valves. Aortic valve prolapse was seen in four patients with mitral valve prolapse (two with severe regurgitation), one of whom had marfanoid aortic root dilatation. The remaining examples of aortic prolapse were seen in patients with various disorders including one with pulmonary atresia, two with aortic root disease (one with dissection and one with idiopathic dilatation), and one case of severe mitral regurgitation. Valves destroyed by infective endocarditis were seen in two cases. Aortic valve prolapse may be detected in various cardiac disorders and does not imply the presence of aortic regurgitation, but when bicuspid aortic valves are present it may well be important in producing such regurgitation. Although aortic valve prolapse may be associated with severe forms of mitral valve prolapse, these patients rarely have aortic regurgitation.     

Prevalence and clinical significance of aortic valve prolapse.

The prevalence and clinical significance of aortic valve prolapse were determined prospectively in 2000 consecutive patients undergoing routine clinical cross sectional echocardiography. Two hundred and twelve patients were excluded because the aortic cusps were not adequately visualised. Aortic valve prolapse was defined as downward displacement of cuspal material below a line joining the points of attachment of the aortic valve leaflets. Twenty four cases of aortic valve prolapse (1.2%) were identified. The patients were aged 12-64 years and nine were women. All had underlying valvar heart disease and the commonest lesion (in 11 cases) was prolapse of the larger cusp in bicuspid valves. Aortic valve prolapse was seen in four patients with mitral valve prolapse (two with severe regurgitation), one of whom had marfanoid aortic root dilatation. The remaining examples of aortic prolapse were seen in patients with various disorders including one with pulmonary atresia, two with aortic root disease (one with dissection and one with idiopathic dilatation), and one case of severe mitral regurgitation. Valves destroyed by infective endocarditis were seen in two cases. Aortic valve prolapse may be detected in various cardiac disorders and does not imply the presence of aortic regurgitation, but when bicuspid aortic valves are present it may well be important in producing such regurgitation. Although aortic valve prolapse may be associated with severe forms of mitral valve prolapse, these patients rarely have aortic regurgitation.     

Morphologic features of the normal and abnormal mitral valve

Anatomic and functional features of the normal and abnormal mitral valve are reviewed. Of 1,010 personally studied necropsy patients with severe (functional class III or IV, New York Heart Association) cardiac dysfunction from primary valvular heart disease, 434 (43%) had mitral stenosis (MS) with or without mitral regurgitation (MR): unassociated with aortic valve stenosis or regurgitation or with tricuspid valve stenosis in 189 (44%) patients, and associated with aortic stenosis in 152 (35%), with pure (no element of stenosis) aortic regurgitation in 65 (15%) patients, and with tricuspid valve stenosis with or without aortic valve stenosis in 28 (6%) patients. The origin of MS was rheumatic in all 434 patients. Of the 1,010 necropsy patients, 165 (16%) had pure MR (papillary muscle dysfunction excluded): unassociated with aortic valve stenosis or regurgitation or with tricuspid valve stenosis in 97 (59%) patients, and associated with pure aortic regurgitation in 45 (27%) and with aortic valve stenosis in 23 (14%) patients. When associated with dysfunction of the aortic valve, pure MR was usually rheumatic in origin, but when unassociated with aortic valve dysfunction it was usually nonrheumatic in origin. Review of operatively excised mitral valves in patients with pure MR unassociated with aortic valve dysfunction disclosed mitral valve prolapse (most likely an inherent congenital defect) as the most common cause of MR. Excluding the patients with MR from coronary heart disease (papillary muscle dysfunction), mitral prolapse was the cause of MR in 60 (88%) of the other 68 patients, and a rheumatic origin was responsible in only 3 of the 68 patients, all 68 of whom were greater than 30 years of age. Mitral anular calcification in persons aged greater than 65 years is usually associated with calcific deposits in the aortic valve cusps and in the coronary arteries. Because calcium in each of these 3 sites is common in older individuals residing in the Western World, it is most reasonable to view mitral anular calcification in older individuals as a manifestation of atherosclerosis. Mitral anular calcium appears to be extremely uncommon in persons with total serum cholesterol levels less than 150 mg/dl. Mitral anular calcium may produce mild MR and, if the deposits are heavy enough, MS.     

Reversal of dominance of the coronary arterial system in isolated aortic stenosis and bicuspid aortic valve.

The dominance of the coronary arterial system was determined angiographically in 75 adult patients with isolated aortic stenosis with or without insufficiency, 25 adult patients with pure aortic insufficiency, 51 adult patients with combined aortic stenosis and mitral valve disease, and 44 children with bicuspid aortic valves. There was an unusually high incidence of left dominance in adults with isolated aortic stenosis (36%) and in children with bicuspid aortic valves (56.8%). These findings suggest the existence of a variant developmental complex consisting of a bicuspid aortic valve and reversal of dominance of the coronary arterial system. Furthermore, it adds support to the concept that progressive stenosis of a bicuspid aortic valve is frequently the etiology of isolated aortic stenosis in adults.     

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.     

Clinical and necropsy observations early after simultaneous replacement of the mitral and aortic valves

Clinical and necropsy findings are described in 54 patients, aged 25 to 83 years (mean 53), who died within 60 days of simultaneous replacements of both mitral and aortic valves. The patients were separated into 4 groups on the basis of the presence of stenosis (with or without associated regurgitation) or pure regurgitation of each valve: 30 patients (56%) had combined mitral and aortic valve stenosis; 12 patients (22%) had mitral stenosis and pure aortic regurgitation; 8 patients (15%) had pure regurgitation of both valves; and 4 patients (7%) had pure aortic regurgitation and mitral stenosis. Necropsy examination in the 54 patients disclosed a high frequency (48%) of anatomic evidence of interference to poppet or disc movement in either the mitral or aortic valve position or both. Anatomic evidence of interference to movement of a poppet or disc in the aortic valve position was twice as common as anatomic evidence of interference to poppet or disc movement in the mitral position. Interference to poppet movement is attributable to the prosthesis's being too large for the ascending aorta or left ventricular cavity in which it resided. The ascending aorta is infrequently enlarged in patients with combined mitral and aortic valve dysfunction irrespective of whether the aortic valve is stenotic or purely regurgitant. Likewise, the left ventricular cavity is usually not dilated in patients with combined mitral and aortic valve stenosis, the most common indication for replacement of both left-sided cardiac valves. Of the 54 patients, 12 (22%) had 1 mechanical and 1 bioprosthesis inserted. It is recommended that both substitute valves should be mechanical prostheses or both should be bioprostheses.     

Aortic valve regurgitation and the congenitally bicuspid aortic valve: a clinico-pathological correlation.

OBJECTIVE--To investigate the morphology of congenitally bicuspid aortic valves causing pure valve regurgitation. DESIGN--A case series collected over five years. SETTING--An academic hospital. PATIENTS AND METHODS--One hundred and forty eight excised congenitally bicuspid aortic valves. The morphological findings were correlated with sex, age, clinical history, and data on haemodynamic function before operation. Pure valve regurgitation was defined as grade 3-4/4 with a gradient less than 30 mm Hg. Aortic root dilatation was evaluated angiographically or echocardiographically or both. RESULTS--Three types were recognised: valves that were purely bicuspid (23%), bicuspid valves with a raphe (34%), and valves with an additional indentation of the free edge of the conjoined cusp (43%). In 14 cases pure valve regurgitation was present. Dilatation of the aortic root was present in 47 cases. The relative risk for regurgitation when the aortic root was dilated (compared with no dilatation) was 3.99. The relative risk for valve regurgitation when there was indentation of the conjoined cusp (compared with no indentation) was 4.95. The mean age at operation in patients with pure regurgitation was 56 years, which is significantly younger (p = 0.0008) than that of patients with a congenitally bicuspid valve with combined valve stenosis and regurgitation (64.7 years). CONCLUSIONS--Congenitally bicuspid aortic valves with a central indentation of the free edge of the conjoined cusp seem particularly likely to develop pure aortic valve regurgitation.     

Aortic valve regurgitation and the congenitally bicuspid aortic valve: a clinico-pathological correlation.

OBJECTIVE--To investigate the morphology of congenitally bicuspid aortic valves causing pure valve regurgitation. DESIGN--A case series collected over five years. SETTING--An academic hospital. PATIENTS AND METHODS--One hundred and forty eight excised congenitally bicuspid aortic valves. The morphological findings were correlated with sex, age, clinical history, and data on haemodynamic function before operation. Pure valve regurgitation was defined as grade 3-4/4 with a gradient less than 30 mm Hg. Aortic root dilatation was evaluated angiographically or echocardiographically or both. RESULTS--Three types were recognised: valves that were purely bicuspid (23%), bicuspid valves with a raphe (34%), and valves with an additional indentation of the free edge of the conjoined cusp (43%). In 14 cases pure valve regurgitation was present. Dilatation of the aortic root was present in 47 cases. The relative risk for regurgitation when the aortic root was dilated (compared with no dilatation) was 3.99. The relative risk for valve regurgitation when there was indentation of the conjoined cusp (compared with no indentation) was 4.95. The mean age at operation in patients with pure regurgitation was 56 years, which is significantly younger (p = 0.0008) than that of patients with a congenitally bicuspid valve with combined valve stenosis and regurgitation (64.7 years). CONCLUSIONS--Congenitally bicuspid aortic valves with a central indentation of the free edge of the conjoined cusp seem particularly likely to develop pure aortic valve regurgitation.     

Cardiovascular abnormalities associated with adult polycystic kidney disease

In a combined retrospective and prospective study, 11 patients with adult polycystic kidney disease were found to have one or more cardiac or aortic lesions. Seven patients had primary dilatation of the aortic root and annulus with aortic regurgitation. The severity of the aortic regurgitation necessitated aortic valve replacement in 2. Mitral regurgitation was present in 3 patients, of whom 2 had documented redundant mitral leaflets and ruptured chordae tendinae, and the third had mitral valve prolapse. Histologic analysis of available aortic and mitral valve tissue from these acquired lesions showed myxomatous degeneration with loss and disruption of collagen. Four patients had congenital bicuspid aortic valves with aortic regurgitation; 1 of these patients also had mild valvular stenosis, and 1 had coarctation of the aorta.     

Clinical and morphologic observations after simultaneous replacement of the tricuspid, mitral and aortic valves

Clinical and morphologic observations are described in 12 patients who underwent simultaneous replacement of the tricuspid, mitral and aortic valves. All 12 patients had mitral stenosis, 10 aortic valve stenosis and 2 pure aortic valve regurgitation; 5 had tricuspid valve stenosis and 7 pure tricuspid valve regurgitation. Of the 10 patients who died within 60 days of triple valve replacement, 7 had the low cardiac output syndrome, which in 4, and possibly 5, of the 7 was attributed to prosthetic aortic valve stenosis. In none of the 12 patients was the ascending aorta dilated, and in the 4 (possibly 5) patients with low cardiac output, the space between the surface of the caged poppet (4 patients) or margins of the tilting disc (1 patient) in the aortic valve position and the aortic endothelium appeared inadequate to allow unobstructed flow despite small-sized prostheses in all but 1 patient. Thus, aortic valve replacement in the setting of triple valve dysfunction is hazardous or potentially so. The relative small sizes of the hearts in these patients also make valve replacement more difficult (and hazardous) compared to hearts with larger ventricles and aortas.     

Progression of aortic valve dysfunction in 51 adult patients with congenital bicuspid aortic valve: assessment and follow up by Doppler echocardiography.

OBJECTIVE--To assess the pattern and progression of aortic valve dysfunction by serial Doppler echocardiographic examinations in ambulatory adult patients with congenital bicuspid aortic valve. DESIGN AND SETTING--Retrospective analysis of patients referred for Doppler echocardiography over a four year period. SUBJECTS--Fifty one adult patients with echocardiographic diagnosis of congenital bicuspid aortic valve had serial Doppler echocardiographic studies at least six months apart. There were 40 men and 11 women with a mean age of 36 years. MAIN OUTCOME MEASURE--Doppler echocardiographic values of aortic valve dysfunction. Cardiac events including endocarditis and aortic valve replacement were also evaluated. RESULT--Coarctation was present in five patients. 31 (61%) patients had a functionally normal bicuspid aortic valve defined as a mean gradient < 25 mm Hg and mild regurgitation. Significant aortic regurgitation was present in 15 patients (moderate in 12 and severe in three). Three patients had isolated aortic stenosis and two patients had combined aortic valve dysfunction. At a median follow up of 21 months (range six to 46 months), six patients had aortic valve surgery (one for aortic stenosis, three for aortic regurgitation, and two for endocarditis). Only 22 patients (43%) continued to have a functionally normal aortic valve. CONCLUSION--In this cohort of fairly young patients, aortic regurgitation is more common than aortic stenosis. Progression of aortic valve dysfunction occurs in patients with pre-existing valve dysfunction and even in those with normal aortic valve function at the initial echocardiographic examination.     

Accessory mitral valve tissue in association with bicuspid aortic valve and aortic coarctation

Accessory mitral valve tissue (AMVT) is a rare congenital malformation of the heart. The first case of AMVT was described in 1842. The first surgical treatment of this lesion was published in 1963 and the first echocardiography (ECHO) diagnosis of AMVT was performed in 1985.A 50-year-old male with medical history of surgery of aortic coarctation 39 years ago was accepted to the hospital because of dyspnoea and repeated pain in the left thorax. ECHO revealed bicuspid aortic valve including progressive dilatation of an aortic root and ascending aorta with mild aortic regurgitation. Dilatation of mitral annulus with moderate regurgitation (without stenosis) and accessory mitral valve tissue combined with trivial obstruction of left ventricle outflow tract (LVOT) was also present. AMVT with chordae was excised and mitral annuloplasty has been accomplished. Aortic root remodelling (Yacoub procedure) combined with an aortic valve repair and extra-aortic ring implantation has been performed subsequently. Control ECHO one year following surgery showed no regurgitation on either aortic or mitral valve.Symptomatology of the patient depends on the presence of AMVT associated with LVOT obstruction, moderate affection of aortic and mitral valves and on concomitant cardiovascular malformations. Echocardiography plays a principal role in the diagnosis, follow-up and indication of surgery of patients with this congenital lesion. Clinically silent AMVT requires follow-up, but it is not an indication for surgery itself. On the other hand AMVT associated with LVOT obstruction or mitral stenosis is an indication for surgery, especially combined with other cardiac malformations.     

Etiology of aortic valve disease and recent changes in Japan:a study of 600 valve replacement cases

BACKGROUND: Recent studies on the etiology of aortic valve disease in the US showed a decrease in rheumatic valve disease and an increase in age-related degenerative disease. The purpose of this study was to describe the etiology of aortic valve disease and its temporal changes in Japan, based on a large number of cases. METHODS: The medical charts of all patients who underwent aortic valve replacement at our institute between 1977 and 1999 were reviewed. Among the 600 patients analyzed, 213 (36%) had pure aortic stenosis, 265 (44%) had pure aortic regurgitation, and 122 (20%) had combined stenosis and regurgitation. RESULTS: The causes were rheumatic change (49%), degenerative change (19%), bicuspid valves (18%), infective endocarditis (5%) and others (9%). Rheumatic disease continued to be the most common cause of aortic stenosis, but its frequency decreased from 100% in 1977-1979 to 37% in 1995-1999. In contrast, the frequency of degenerative change among stenotic valves increased recently from 11% in 1990-1994 to 30% in 1995-1999. Similarly, rheumatic disease remained to be the leading cause of aortic regurgitation, with a decline in frequency from 46% in 1985-1989 to 27% in 1995-1999. The percentage of degenerative change among regurgitant valves did not change appreciably. CONCLUSIONS: There was a shift in the causes of aortic valve disease, with a decrease in rheumatic disease and an increase in degenerative disease. This trend was similar to that observed in the US. These findings suggest the increasing importance of aortic valve disease due to degenerative change.     

Demographic characteristics of patients undergoing aortic valve replacement for stenosis: relation to valve morphology.

OBJECTIVE: To determine the relative importance of the different causes of isolated aortic valve stenosis in a surgical series, and to relate these to patient characteristics including the rate of insertion of bypass grafts for coronary artery disease. DESIGN: Survey of the clinical and pathological data on patients undergoing aortic valve replacement for isolated stenosis. SETTING: Tertiary care cardiothoracic surgical unit. PATIENTS AND METHODS: 465 adult patients undergoing aortic valve replacement representing a consecutive series in one surgical unit. Retrospective review of patients records and classification of cause of aortic stenosis based on pathological examination of excised valve cusps. RESULTS: 63.7% patients had calcific bicuspid valves, 26.9% tricuspid calcific valves, and 5.4% rheumatic, 2.6% mixed pathology and 1.5% unicommissural valves. The ratio of males to females for bicuspid valves was 1.85:1 and for tricuspid calcific valves 0.76:1. The mean age of patients with bicuspid valves was 64.9 years compared with 73.4 years for those with tricuspid valves. Some 22.3% of patients with bicuspid valves and 44.8% of those with tricuspid valves had sufficient coronary artery disease to necessitate insertion of coronary bypass grafts. The differential rate of insertion of coronary bypass grafts was independent of age. CONCLUSIONS: Bicuspid calcified aortic valves are the predominant cause of isolated aortic valve stenosis followed by tricuspid calcified aortic valves. The sex and age distribution of bicuspid and tricuspid calcific aortic valve stenosis is different. The higher rate of insertion of vascular grafts in tricuspid calcific aortic valves may indicate that risk factors for atherosclerosis enhance cusp calcification in these patients.     

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。     

Incidence and concomitant factors of tricuspid valve insufficiency in patients with aortic and mitral valve diseases

Invasive data about the frequency and associated factors of tricuspid regurgitation in normals and in patients with aortic and mitral valve disease are still rare. Thus, right ventricular biplane angiograms (RAO/LAO projection), the mean pulmonary artery pressure and the presence of atrial fibrillation were analyzed with regard to tricuspid regurgitation in 30 normals and 165 patients with pure mitral regurgitation, mitral stenosis, aortic regurgitation, aortic stenosis, combined mitral valve disease or combined aortic valve disease. Patients with tricuspid stenosis or coronary artery disease were excluded. In 52 of the 195 patients tricuspid regurgitation was present. Tricuspid regurgitation occurred statistically more often in patients with mitral stenosis (33%), mitral regurgitation (48%) or combined mitral valve disease (68%) than in patients with aortic regurgitation (4%) or combined aortic valve disease (3%). In patients with aortic stenosis and in normals tricuspid regurgitation was not present. In patients with combined mitral valve disease, tricuspid regurgitation was more often present than in patients with pure mitral stenosis (p less than 0.002), despite comparable values of the mean pulmonary artery pressure, the right ventricular enddiastolic and endsystolic volume indexes, the right ventricular ejection fraction and the frequency of atrial fibrillation. Only in patients with pure mitral regurgitation tricuspid regurgitation was associated with an elevated mean pulmonary artery pressure (p less than 0.02). Differences in the right ventricular size and function did not occur between normals and patients with mitral or aortic valve disease. Therefore, the mean pulmonary artery pressure, atrial fibrillation and the size and function of the right ventricle are not major determinants for the occurrence of tricuspid regurgitation.(ABSTRACT TRUNCATED AT 250 WORDS)     

Congenital quadricuspid aortic valve in children

Quadricuspid aortic valves are rare congenital anomalies, usually diagnosed in adult patients affected by severe aortic regurgitation. We have now encountered three such valves in children undergoing transthoracic echocardiography. All children were asymptomatic. The first child possessed a valve with two larger leaflets, and smaller leaflets of equal size. In the second child, 3 leaflets were of equal size, with 1 small accessory leaflet. The third child had a valve with four leaflets of approximately equal size. In one child, the aortic valve was functioning normally, and came to attention because of mitral valvar prolapse causing mild regurgitation. In the remaining two children, central mild aortic regurgitation was detected using colour flow analysis, and associated dilation of the aortic root was revealed by measurements of the cross-sectional images.     

Cardiac transplantation in adults with aortic valve disease with focus on the bicuspid aortic valve

The frequency of congenitally bicuspid aortic valves in patients having cardiac transplantation (CT) is unknown. We reviewed 243 explanted hearts in patients having CT at Baylor University Medical Center, Dallas from June 1997 through November 2011 to determine the frequency of a bicuspid aortic valve in this population. Of the 243 explanted hearts, 7 (2.9%) were found to have a congenitally bicuspid aortic valve: 3 had severe aortic valve stenosis and before CT had had the aortic valve replaced; the other 4 had normally functioning bicuspid valves and underwent CT for cardiomyopathy (ischemic in 1, idiopathic in 2, and hypertrophic in 1). Review of previously published reports of CT and aortic valve disease disclosed that 4 patients had had aortic valve replacement (AVR) from 2 to 8 years before CT, 3 had AVR or aortic valve repair of the donor heart at the time of CT, and 4 had AVR or transcatheter aortic valve implantation from 1 to 14 years after CT. Some of these aortic valve replacements, before, at the time of, or after CT were in patients with congenitally bicuspid aortic valves. In conclusion, congenitally bicuspid aortic valves were found in 7 of 243 explanted hearts in patients having CT at a single medical center in a 14-year period: 4 had functioned normally and 3 were severely stenotic. Previous reports of patients having AVR or repair before, during, and after CT were reviewed.     

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.     

Mitral valve prolapse in patients with prior rheumatic fever

It is known that rheumatic heart disease frequently results in isolated mitral regurgitation without concomitant mitral stenosis, especially in countries with a high prevalence of rheumatic fever. However, more recent surgical pathologic data also have demonstrated a high incidence of mitral valve prolapse in cases of rheumatic heart disease, which suggests that rheumatic fever may be a cause of mitral valve prolapse. To determine whether this association of mitral valve prolapse and rheumatic heart disease is present in a stable clinic population, we studied 30 patients who had an apical systolic murmur and a well-documented history of rheumatic fever with dynamic auscultation, two-dimensional echocardiography, and pulsed Doppler examinations. Twenty of the 30 patients (67%) had findings on physical examination consistent with isolated mitral regurgitation and 25 patients (84%) had mitral regurgitation by Doppler examination. Echocardiography demonstrated mitral valve prolapse in 24 patients (80%), whereas only one of the total study group had echocardiographic findings consistent with mitral stenosis. We conclude that (1) the presence of an isolated systolic murmur in patients with a history of rheumatic fever frequently represents pure mitral regurgitation secondary to mitral valve prolapse and (2) postinflammatory changes in valvular tissue resulting from rheumatic fever may be the etiology of mitral valve prolapse in these patients.