Surgical treatment of mitral-aortic incompetence and aneurysm of the ascending aorta in a child with Marfan's syndrome: case report

A 7-year-old girl with Marfan's syndrome developed severe mitral and aortic valve incompetence and aneurysmatic dilatation of the ascending aorta. She underwent successful replacement of the mitral valve, aortic valve, and ascending aorta with coronary reimplantation. After 3 months of follow-up, she continues to be asymptomatic.     

Aortic insufficiency in Takayasu's disease. Apropos of 3 surgically treated cases and review of the literature

The author report the cases of three women with aortic regurgitation associated with aneurysmal dilatation of the ascending thoracic aorta. Aortic valve replacement was carried out in the 3 cases, with resection of the aortic aneurysm in 2 cases. Histological examination of the aortic wall in all 3 cases showed non-specific aortitis in the adventitia and media, appearances comparable to those described in Takayasu's disease. The incidence of aortic regurgitation in Takayasu's disease is about 10%. These 3 cases are compared with 30 other cases with histological confirmation in the medical literature, only 9 of which have undergone aortic valve replacement. The mechanism of the aortic regurgitation is analysed: dilatation of the aortic ring, inflammation of the valve cusps, commissural dysjunction or dilatation of the ascending aorta involving the line of commissural insertion. Aortic regurgitation is usually associated with other arterial localisations of the disease, but may also be found alone when the disease seems limited to the ascending aorta. Coronary artery disease, especially ostial, is observed in 30% of patients with this type of aortic regurgitation. Coronary angiography is therefore mandatory before surgery. The arterial involvement may be investigated initially and followed-up by 2 non-invasive investigations = digitalised intravenous angiography (DIVA) and M mode and 2D echocardiography. The two investigations give comparable results with regards to the study of the aorta, but the branches of the aorta and pulmonary arteries can only be investigated by DIVA.(ABSTRACT TRUNCATED AT 250 WORDS)     

Aortic dissection after aortic valve replacement: clinical and anatomic features]

BACKGROUND. Cardiac surgery has become a possible cause of ascending aortic dissection, particularly in patients who have undergone aortic valve replacement. METHODS. From January 1979 to December 1989, 134 patients with angiographic evidence of aortic dissection, were studied in the Haemodinamic Laboratory of the University of Turin. Twelve of these patients (7 men and 5 women), mean age 51.6 years, had been previously operated for aortic valve replacement. We evaluated incidence, clinical and anatomic features of ascending aortic dissection in this group. RESULTS. A dilated ascending aorta with a mean diameter of 4.92 cm. was noted in all these patients. The interval between valve replacement and diagnosis of aortic dissection was 57 months (range 1 day to 9 years). Reoperation was performed in 11 patients. Four intraoperative deaths occurred and 2 other patients died in the early post-operative period. Survival rate at 12 months was 50%. Our incidence of ascending aortic dissection after aortic valve replacement was 0.66% (10 patients out of 1499 operated for aortic valve replacement) and it is in agreement with reports from other Authors in literature. CONCLUSIONS. Replacement of the ascending aorta must be considered if an important dilatation of aortic root is found at time of aortic valve surgery. New efforts must be undertaken to identify patients at high risk for this complication because an elevated mortality rate is observed in reoperation for aortic dissection.     

Operative results with total composite replacement of the aortic valve and ascending aorta]

From December 1980 to December 1990, ten patients, 9 male and 1 female, ranging in age from 21 to 68 years, were operated on for aortic valve insufficiency associated with an aneurysm of the ascending aorta. The surgical treatment in all cases consisted of total replacement of the ascending aorta with Bentall's procedure (n = 4), or Cabrol's procedure (n = 6). In 5 patients an uncomplicated annulo-aortic ectasia existed. Three of them had annulo-aortic ectasia with an aortic dissection. One had aortitis syndrome, and one had syphilitic aortitis. The operative mortality for the entire group was 0% (0 death). Hospital survivors revealed satisfactory clinical improvement in NYHA class (mean value: 3.2 to 1.0). Late complications developed in 2 of the 10 patients. They had a picture of pseudoaneurysm formation at the anastomoses between the graft and the right coronary 46 months and 15 months, respectively, after the initial operation. Despite the reoperation, one died of hepatic failure 30 days after the operation, and the other died of postoperative bleeding at the anastomosis sites. We, furthermore, considered the difference in aortic cross clamp time and cardiopulmonary bypass time between Bentall's procedure and Cabrol's procedure. Aortic cross clamp time and cardiopulmonary bypass time were significantly shorter in Cabrol's procedure than in Bentall's procedure, if a probability value less than 0.20 was considered to be of statistical significance. We were able to conclude that the treatment of aortic valve regurgitation associated with an aneurysm of the ascending aorta by insertion of a composite graft is a reliable method with low operative mortality and excellent long term results, especially in Cabrol's procedure.     

Takayasu's arteritis revealed by aortic regurgitation with ascending aortic aneurysm (About 2 cases)

Aortic regurgitation caused by non-specific aortitis is relatively rare, and is now considered as an important risk factor related to mortality. Aortic valve replacement surgery is the only curative treatment. Aneurismal dilatation of the ascending thoracic aorta associated with aortic regurgitation is a rare involvement in Takayasu, there are many difficult problems in surgical treatment of this lesion, because of its inflammatory nature, so steroid therapy before and after surgery is therefore vital. We report the cases of tow young Moroccans women (32-35 years-old) with an ascending aortic aneurism associated to aortic insufficiency. The subsequent evaluation of the entire aorta, demonstrated the presence of multiple steno-occlusive lesions. Aortic valve replacement was performed associated with graft replacement of the ascending aorta without coronary artery reimplantation. Histopathological examination of the ascending aorta and aortic valve, showed findings in favour Takayasu's arteritis.     

Combined mitral and aortic regurgitation in ankylosing spondylitis: Angiographic and anatomic features

Clinical and cardiac morphologic features are described in a man with combined aortic and mitral regurgitation associated with ankylosing spondylitis. Although aortic regurgitation is a recognized accompaniment of ankylosing spondylitis, the occurrence of hemodynamically-significant mitral regurgitation in this arthritic condition has not been documented previously. Histologic study disclosed changes in the anterior mitral leaflet identical to those observed in the wall of the aorta and base of the aortic valve cusps in patients with ankylosing spondylitis. Thus, ankylosing spondylitis may be associated with characteristic lesions in anterior mitral leaflet in addition to those in the ascending aorta and aortic valve. The subaortic bump at the base of the anterior mitral leaflet, the most characteristic cardiovascular lesion of ankylosing spondylitis, may be visualized during life by left ventricular angiography, and its identification allows proper etiologic diagnosis of the valvular regurgitation.     

Suitability of stentless bioprostheses for combined replacement of the aortic valve and ascending aorta.

BACKGROUND: The Edwards Prima stentless valve (EPSV) is a bioprosthesis made of the porcine aortic root which, owing to its versatility, may be implanted with different techniques depending on the underlying disease. The aim of this study was to demonstrate the usefulness of the EPSV implanted as a miniroot in patients with disease of the aortic valve and ascending aorta. METHODS: Between January 1998 and July 2002, 91 patients (mean age 67 +/- 7 years) underwent combined replacement of the aortic valve and ascending aorta with an EPSV for aortic stenosis (n = 12), incompetence (n = 45) or mixed disease (n = 26); all had aneurysmal dilation of the ascending aorta and 8 had an acute aortic dissection. The EPSV was implanted using the miniroot (or inclusion) technique and by extending the aorta with a tubular graft; aortic arch replacement was required in 5 patients. Hospital survivors underwent clinical and echocardiographic follow-up at 6 and 12 months and yearly thereafter. RESULTS: The hospital mortality was 11% (10 patients). Causes of death included stroke (n = 3), septic shock (n = 3), myocardial infarction (n = 2), and low output syndrome (n = 2). The mean follow-up of the 81 discharged patients was 16 +/- 13 months and was 100% complete; there were 6 late deaths due to non-cardiac causes. All patients presented with clinical improvement (95% are in NYHA functional class I) with low transvalvular gradients and significant regression of left ventricular hypertrophy at two-dimensional echocardiography. CONCLUSIONS: The EPSV used as a miniroot has proved to be a valid option in patients requiring simultaneous replacement of the aortic valve and ascending aorta. Due to the limited information available on the long-term fate of the porcine aortic valve and root, the use of this device as a miniroot should presently be limited to elderly patients.     

Outcome of reoperative aortic root or ascending aorta replacement after prior aortic valve replacement

BackgroundThere are limited data regarding the clinical outcomes of reoperative aortic root or ascending aorta replacement after prior aortic valve replacement (AVR). We aimed to analyze outcomes of reoperative aortic root or ascending aorta replacement after prior AVR.MethodsEighty patients with prior AVR underwent reoperative aortic root or ascending aorta replacement in our hospital. The indications were root or ascending aortic aneurysm in 36 patients, root or ascending aortic dissection in 37, root false aneurysm in 2, prosthesis valve endocarditis (PVE) with root abscess in 2, Behçet’s disease (BD) with root destruction in 3 patients. An elective surgery was performed in 63 patients and an emergent surgery in 17. The survival and freedom from aortic events during the follow-up were evaluated with the Kaplan-Meier survival curve and the log-rank test.ResultsThe operative techniques included ascending aorta replacement in 14 patients, ascending aorta replacement with AVR in 3, prosthesis-sparing root replacement (PSRR) in 35, Bentall procedure in 24, and Cabrol procedure in 4 patients. Operative mortality was 1.3% (1/80). A composite of adverse events occurred in 5 patients, including 1 operative death, 2 stroke and 3 renal failure necessitating hemodialysis. The mean follow-up was 35.5±22.1 months. Five late deaths occurred. The Kaplan-Meier survival at 1 year, 3 years and 6 years were 97.5%, 91.1% and 84.1%, respectively. Aortic events developed in 3 patients. The freedom from aortic events at 1-year, 3-year, and 6-year were 100%, 96.3% and 88.9%, respectively. There were no differences in survival and freedom from aortic events between the elective group and the emergent group.ConclusionsReoperative aortic root or ascending aorta replacement after prior AVR could be performed to treat the root or ascending pathologies after AVR, with satisfactory early and midterm outcomes.     

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.     

升主动脉成形术治疗二叶式主动脉瓣患者升主动脉扩张的效果分析

目的:探讨升主动脉成形术(reduction ascending aortoplasty,RAA)治疗二叶式主动脉瓣(bicuspid aortic valve,BAV)患者升主动脉扩张的临床效果。方法:回顾性分析2006年1月至2015年12月长海医院208例因主动脉瓣病变合并升主动脉扩张行主动脉瓣置换术(aortic valve replacement,AVR)联合RAA治疗患者的临床资料。根据患者主动脉瓣情况,分为BAV组和三叶式主动脉瓣(tricuspid aortic valve,TAV)组;在BAV组中,根据术中升主动脉的目标直径,将患者分为30 mm组和≥30 mm组。结果:BAV组与TAV组的术后死亡率、并发症发生率、二次手术比例和随访升主动脉直径等指标差异无统计学意义(P均0.05);BAV组术中升主动脉目标直径30 mm组的随访升主动脉直径、术后5年和9年升主动脉扩张程度均明显小于术中升主动脉≥30 mm组(P均0.05)。结论:对于BAV合并升主动脉扩张的患者,BAV不是行RAA的不利因素,在RAA术中将升主动脉目标直径控制在30 mm以下,中远期疗效更好。     

Aortic valve replacement for aortic regurgitation caused by aortitis

Between January 1984 and December 1998, 19 patients (16 with Takayasu's arteritis, 3 with non-Takayasu's aortitis) underwent surgical treatment for aortic regurgitation resulting from the aortitis. Of the 19 patients, 14 had aortic valve replacement (AVR) and 5 had aortic root replacement. One patient (5.3%) died of graft infection during the hospital stay. During the follow-up period, 1 (5.6%) of the 18 postoperative patients died of paravalvular leakage due to valve detachment, which also required redo-operations in 2 patients with non-Takayasu's aortitis. Both patients were operated on during the active phase of the inflammation without perioperative steroid therapy. Although transmural pledgeted sutures were used for replacement of the detached prosthetic valve in 1 of these 2 patients, disruption of the aortic wall resulted in recurrence of valve detachment. In the other patient, aortic root replacement was successfully performed with the Cabrol technique in the second operation. Perioperaitve steroid therapy plays an important role in preventing complications after AVR when the valve replacement is carried out during the active phase of the inflammation, and for patients with non-Takayasu's aortitis, aortic root replacement should be considered to reduce the tension on the suture line and the native aortic valve annulus.     

Late aortic dissection after aortic valve substitution. Considerations on 5 operative cases

This study analyses the clinical course of five patients who developed acute ascending aortic dissection 45.8 months (17-87 months) after aortic valve replacement (AVR) and underwent reoperation in our Department. All except one had aortic insufficiency with mild dilatation of the ascending aorta. No evidence of aortic wall degeneration was present at the time of AVR. Prostheses implanted were: Hancock 2, St. Jude 2, Bjork-Shiley 1. Associated procedures (coartectomy, coronary artery by-pass) were carried out in two patients. The dilated aorta was managed at the first operation by longitudinal resection in 3 of them. Emergent aortic dissection repair was performed: in 3 cases with composite graft according to the Bentall procedure, in one case with non valved dacron conduit and in the last case with teflon patch. Histological examination of the dissected aortic wall showed elastic fiber degeneration like cystic medial necrosis. Three patients survived surgical repair and are doing well at 22.4 months of mean post-operative follow-up. Surgical strategy of this complication is discussed; particular emphasis is given to the method of treatment of ascending aorta dilatation during AVR.     

Determinants of ascending aortic dimensions after aortic valve replacement with a stented bioprosthesis

BACKGROUND AND AIM OF THE STUDY: Concomitant replacement of the mildly dilated ascending aorta during aortic valve replacement (AVR) is controversial because progress of aortic dilatation is uncertain after elimination of the valvular lesion. The study aim was to determine factors influencing the aortic dimensions, to analyze their clinical impact, and to identify criteria for concomitant surgery on the aorta. METHODS: Between February 1994 and May 1999, 100 patients with tricuspid aortic valve disease received the stented porcine Mosaic prosthesis within a FDA approval study. Follow up (mean 4.8 years; range: 0.1-8.8 years; total 483.4 patient-years) included documentation of adverse events and transthoracic echocardiography with measurements of the aortic sinus, sinotubular junction, and ascending aorta. RESULTS: Baseline aortic dimensions were dependent on gender and body surface area, but independent of the type and extent of valvular lesion, patient age, and atherosclerotic risk factors. Larger baseline aortic diameters were associated with smaller postoperative annual aortic expansion rates (r = -0.47, p <0.001). In patients with baseline aortic dilatation > or =40 mm (10.2%; mean 42.5+/-2.6 mm), aortic diameter decreased during follow up (p = 0.032; expansion rate -1.9+/-2.0 mm/year). Baseline aortic dilatation did not influence postoperative morbidity and mortality. Prosthetic regurgitation was associated with increases in aortic diameter (p <0.001). Survival was reduced in patients with aortic expansion rates >3.6 mm/year (0.0% versus 68.2+/-9.7%; p <0.001). CONCLUSION: AVR without concomitant surgery on the aorta in patients with mild aortic dilatation is feasible, as aortic diameters were decreased after removal of the diseased valve. The aortic expansion rate had a strong prognostic importance, even on aortic diameters, which are considered to be within normal ranges.     

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.     

Configuration of the ascending aorta in patients with bicuspid and tricuspid aortic valve disease undergoing aortic valve replacement with or without reduction aortoplasty

BACKGROUND AND AIM OF THE STUDY: Patients with bicuspid aortic valves (BAV) tend to develop dilatation of the ascending aorta. The study aim was to analyze differences in aortic root diameter and configuration in patients with bicuspid and tricuspid aortic valve disease. METHODS: A retrospective analysis was conducted of the angiographies of 461 patients allocated to four groups with: (i) BAV disease with (n = 179) and (ii) without (n = 78) dilatation of the ascending aorta; (iii) tricuspid aortic valve disease (TAV) and dilatation of the ascending aorta (n = 154); and (iv) coronary artery disease (CAD), TAV and normal diameter of the ascending aorta (n=50). Diameters and distances in the aortic root region were measured, and the ascending aorta configuration analyzed. RESULTS: The diameter of the ascending aorta in patients with BAV and dilatation was significantly larger than in those with TAV and dilatation (26.6 +/- 5.22 versus 24.4 +/- 3.74 mm/m2, p = 0.002). Distances between aortic valve level and point of maximum diameter of the ascending aorta at the outer and inner curve of the vessel in patients with BAV without dilatation were greater than those of the CAD group (31.1 +/- 5.27 versus 28.0 +/- 4.86 mm/m2, p = 0.002 and for the indexed values 21.6 +/- 4.05 versus 20.0 +/- 2.71 mm/m2, p = 0.011). All patients with BAV and enlargement of the ascending aorta showed asymmetric dilatation of the vessel. CONCLUSION: All patients with BAV had an abnormal configuration of the ascending aorta. In cases with enlargement of the ascending aorta exclusively, asymmetric dilatation at the convexity of the vessel occurred. Patients with BAV and normal ascending aorta diameter showed an elongation of this vessel segment.     

Long-term risk of aortic events following aortic valve replacement in patients with bicuspid aortic valves

Bicuspid aortic valve (BAV) is associated with ascending aortopathy predisposing to aneurysmal dilatation and dissection, even after successful aortic valve replacement (AVR). There is, however, scant evidence on which to make recommendations for prophylactic replacement of the ascending aorta at the time of AVR. The medical records of patients who underwent AVR for BAV without aortic replacement or repair from 1960 to 1995 were reviewed. Follow-up was by review of the medical record and postal questionnaire. Among 1,286 patients, the mean age at operation was 58 ± 14 years. During the follow-up interval (median 12 years, range 0 to 38), there were 13 documented aortic dissections (1%), 11 ascending aortic replacements (0.9%), and 127 documented cases of progressive aortic enlargement (9.9%). Fifteen-year freedom from aortic dissection, enlargement, or replacement was 89% (95% confidence interval [CI] 87% to 91%) and was lower in patients with documented aortic enlargement at the time of AVR (85%, 95% CI 81% to 89%) compared to those whose aortic dimensions were normal (93%, 95% CI 90% to 96%) (p = 0.001). Multivariate predictors of aortic complications included interval (subsequent) AVR (hazard ratio [HR] 3.5, 95% CI 2.3 to 5.4, p <0.001), concomitant coronary artery bypass grafting (HR 2.6, 95% CI 1.7 to 4.0, p <0.001), enlarged aorta (HR 1.8, 95% CI 1.3 to 2.6, p = 0.001), and history of tobacco abuse (HR 1.8, 95% CI 1.2 to 2.6, p = 0.003). Aortic dilatation did not predict mortality. In conclusion, despite a true risk for aortic events after AVR for BAV, the occurrence of aortic dissection was low. Any incremental surgical risk imposed by prophylactic replacement of the ascending aorta must be equally low.     

Long-term results of aortic valve replacement with Edwards Prima Plus stentless bioprosthesis: eleven years' follow up

BACKGROUND AND AIM OF THE STUDY: The Edwards Lifesciences Prima Plus stentless valve (ELSV) is a bioprosthesis manufactured from a porcine aortic root. The study aim was to evaluate late clinical outcomes after aortic valve replacement (AVR) with ELSV implanted as a miniroot in patients with aortic valve disease. METHODS: Between 1993 and 2004, 318 patients (232 males, 86 females; mean age 69 +/- 9 years; range: 37-83 years) underwent AVR with the ELSV. Preoperatively, 102 patients (32%), 162 (51%) and 54 (17%) were in NYHA classes I/II, III and IV, respectively. Aortic stenosis, aortic regurgitation and combined lesions were present in 124 patients (39%), 114 (36%) and 41 (13%), respectively. Twenty patients (6%) were referred for an acute aortic dissection, 20 (6%) for an aortic root aneurysm, and 139 (44%) had an associated aneurysmal dilatation of the ascending aorta. The ascending aorta was replaced in 159 patients (50%); aortic arch replacement was required in 10 (3%). Coronary artery bypass graft was performed in 86 patients (27%). The follow up was based on clinical data. RESULTS: Operative mortality was 5% (n = 17). There were 49 late deaths (5.2%/pt-yr). Valve-related mortality occurred in 10 patients (1%/pt-yr). Actuarial survival at five and 10 years was 78% and 33%, respectively. Actuarial freedom from valve reoperation and structural valve deterioration at 10 years were 100% and 64%. Actuarial freedom from embolic events and endocarditis at 10 years were 84% and 81%, respectively. CONCLUSION: The ELSV, when implanted as a miniroot, provided good early and long-term results in terms of survival and freedom from major complications.     

Frequency of cardiovascular events in women with a congenitally bicuspid aortic valve in a single community and effect of pregnancy on events

Pregnancy and congenitally bicuspid aortic valve (BAV) are thought to be risk factors for aortic dissection; however, the population-based risk for patients with the 2 risk factors remains unknown. We investigated the relation between pregnancy and ascending aortic events in women with congenital BAV living in Olmsted County, Minnesota. Our institutional echocardiographic database was searched for women residing in Olmsted County with congenital BAV diagnosed from 1980 to 1999. We reviewed medical records for end points of aortic events (dilatation, dissection, or surgery) and aortic valve replacement (AVR). Obstetric history and further outcome follow-up were collected by postal survey. We identified 88 women with BAV. Median age at diagnosis was 35 years. Obstetric history totaled 216 pregnancies and 186 deliveries. There were no aortic dissections. During follow-up (median 12.3 years), 24 patients underwent AVR (n = 14), ascending aortic surgery (n = 3), or AVR and ascending aortic surgery (n = 7). Pregnancy was not associated with dilatation of the aorta, aorta surgery, or AVR. At echocardiographic diagnosis of BAV, 5 patients (6%) had aortas >40 mm in greatest diameter and 1 patient has >50 mm. Of 60 patients with serial echocardiograms for comparison (median interval 10.7 years), 21 patients (35%) had aortas measuring >40 mm in greatest dimension and 2 patients had >50 mm. In conclusion, aortic dissection in women with BAV and pregnancy is rare in the community. There is a significant rate of progressive enlargement of the aorta, warranting longitudinal follow-up.     

Aortic valve replacement in a case of Ehlers Danlos syndrome.

A case of Ehlers Danlos syndrome is described in a 54-year-old Caucasian male. He had calcific aortic valve disease and dilatation of the ascending aorta. An aortic valve replacement was performed using a Starr prosthesis.     

Aortic valve replacement in a case of Ehlers Danlos syndrome.

A case of Ehlers Danlos syndrome is described in a 54-year-old Caucasian male. He had calcific aortic valve disease and dilatation of the ascending aorta. An aortic valve replacement was performed using a Starr prosthesis.