A patient with Hodgkin's lymphoma, antiphospholipid syndrome and severe mitral insufficiency

The association between Hodgkin's lymphoma, antiphospholipid syndrome and severe mitral insufficiency is a very rare event. We report on a 25-year-old female patient suffering from Hodgkin's lymphoma and presenting with thromboembolic events and severe mitral insufficiency. The possible link between these symptoms being antiphospholipid antibodies, is discussed briefly.     

Surgical management of mitral regurgitation in patients with Marfan syndrome

BACKGROUND AND AIM OF THE STUDY: Although, in patients with Marfan syndrome, the most common cardiovascular abnormalities are aortic root dilatation and aortic valve regurgitation, mitral valve dysfunction is the most frequently accompanying cardiac lesion. METHODS: The clinical course and management is reported of nine patients (seven men, two women; mean age 29.2 +/- 2.3 years) with Marfan syndrome who required different mitral valve procedures and aortic valve replacement. Mitral regurgitation was caused by annulus dilatation in all patients, by leaflet prolapse in five patients, and by chordal rupture due to endocarditis in two. Six patients underwent mitral valve replacement; three of these were operated on using preservation of the mitral valve apparatus. Three patients underwent mitral annuloplasty and chordal shortening. RESULTS: There was no hospital mortality, nor any valve-related complications in the series. The postoperative course was uneventful after each operation. At a mean follow up of 4.5 years, all patients were in NYHA class I or II, with improved functional capacity. CONCLUSION: This experience in mitral valve procedures indicated that the connective tissue defect might compromise the results of such surgery, but that mitral valve operations could be performed satisfactorily in young Marfan syndrome patients, despite complications of left ventricular dilatation.     

Aortic insufficiency in a patient with Marfan syndrome after aortic root reconstruction with a tailored-sinus graft

A patient with Marfan syndrome underwent valve-preserving aortic root reconstruction with a Robicsek-Thubrikar graft. Intraoperative transesophageal echocardiography showed aortic insufficiency after extracorporeal circulation was discontinued. Placing 3 subcommissural annuloplasty sutures corrected the severe aortic insufficiency. Herein, we discuss the mechanism and prevention of aortic regurgitation after aortic root replacement with a new graft that contains pre-designed aortic sinuses.     

Annuloplasty in children and young adolescents with severe rheumatic mitral insufficiency

Eleven patients aged 8 to 15 years underwent measured asymmetrical annuloplasty for severe mitral regurgitation in the years 1961 through 1966. They had had a total of 20 attacks of acute rheumatic fever. The intervals between the last attack of acute rheumatic fever and operation ranged from 2 to 8 years. The criteria for surgery were congestive failure and progressive cardiac enlargement. Using the hydraulic formula of Gorlin, a mitral annuloplasty was tailored to the size of each patient so that insufficiency was eliminated without producing hemodynamically significant stenosis. In this group of 11 children there has been one death. The majority of our 11 patients reacquired murmurs of mitral regurgitation. Satisfactory results, however, are not dependent on complete hemodynamic correction. All patients have improved remarkably and have sustained this improvement up to 7 years. These results suggest that mitral annuloplasty should be the operation of choice in children with severe mitral regurgitation.     

MitraClip used for severe mitral regurgitation after surgery for type A aortic dissection combined with Marfan syndrome

Mitral regurgitation is a rare but catastrophic condition in patients after surgery for type A aortic dissection. The second thoracotomy to complete the mitral valve operation could be fatal. Here, we report a case of severe mitral regurgitation treated with MitraClip in a 53-year-old woman after surgery for type A aortic dissection combined with Marfan syndrome. She was discharged uneventfully, and a significant reduction of regurgitation of mitral valve and tricuspid valve was observed at the 6-month follow-up. MitraClip could be an alternative device for such high-risk patients.     

Sicca syndrome with severe renal insufficiency

The interstitial nephritis associated with sicca syndrome is usually symptomless or responsible for mild renal impairment. The authors report five cases in which renal failure was severe, requiring haemodialysis in two patients. The physiopathological mechanisms and the treatment of interstitial nephritis in sicca syndrome are discussed. Corticosteroids improved renal function in three of these five patients.     

Anterior leaflet augmentation with autologous pericardium for mitral repair in rheumatic valve insufficiency

BACKGROUND AND AIM OF THE STUDY: The mechanism of insufficiency in rheumatic valve disease includes annulus dilatation and restricted leaflet motion. In order to improve the treatment of restriction, the anterior mitral leaflet (AML) can be augmented with a piece of glutaraldehyde-treated autologous pericardium. METHODS: Between January 1995 and December 1999, among 274 patients referred for rheumatic mitral disease, 143 (52%) underwent a valve repair. Of these patients, 81% had pure regurgitation and 19% had significant associated stenosis. Ring annuloplasty was used in all cases. Techniques used to treat the restrictive component of the regurgitation were compared in two consecutive cohorts of patients, either with (n = 62) or without (n = 81) AML augmentation. Mean patient age was 42 +/- 3 years, and all preoperative variables were comparable except for the incidence of redo patients, who all underwent AML extension. RESULTS: In-hospital mortality was 0.7% (n = 1 with AML extension), and there was one early reoperation for pericardial patch dehiscence. After a mean follow up of 3.2 years, there was one sudden death (no AML extension). The reoperation rate was lower with (2.5%) than without (12.9%) AML augmentation (p <0.05). Echocardiography showed a lower incidence in recurrence of mitral insufficiency when AML augmentation was performed (grade 2, 9% versus grade 3, 3%) as compared to no AML augmentation (grade 2, 35% versus grade 3, 14%) (p <0.05). The mitral orifice area was larger (AML augmentation 2.2 +/- 0.3 cm versus no AML augmentation 1.8 +/- 0.4 cm2). CONCLUSION: Ring annuloplasty alone failed to correct rheumatic mitral insufficiency in all cases. AML augmentation improved the quality of the repair, and decreased the risk of reoperation.     

Mitral valve replacement in patient with absent right superior vena cava, pectus excavatum, Marfan syndrome and severe mitral regurgitation

Combination of persistent left superior vena cava (PLSVC) draining into right atrium via the coronary sinus with a normal right superior vena cava (RSVC) is the most common systemic venous anomaly in visceroatrial situs solitus. On the other hand, a PLSVC in absence of RSVC is an extremely rare anomaly. Cardiovascular abnormalities are frequently encountered in patients with Marfan's syndrome but absence of RSVC with PLSVC is not reported in these patients. We report on a 52-year-old man with Marfan syndrome, an absence of right superior vena cava, severe pectus excavatum and severe mitral regurgitation who underwent successful mitral valve replacement. CT scan chest revealed marked cardiac deviation to the left associated with severe pectus excavatum, absent right superior vena cava and persistent left superior vena cava. During surgery replacement of mitral valve was performed through midsternotomy successfully.     

Predictors of outcome of mitral valve prolapse in patients with the Marfan syndrome

Mitral valve prolapse has a prevalence of 2% to 3% in the general population, with adverse outcomes such as mitral valve regurgitation (MVR), heart failure, and endocarditis. Predictors of outcomes are used in idiopathic mitral valve prolapse for the timing of surgery, but such predictors are unknown in Marfan syndrome. Therefore, a population-based cohort study of 112 patients (49 male, 63 female; mean age 34 ± 15 years) with classic Marfan syndrome and mitral valve prolapse with moderate or less MVR at baseline was conducted. During 4.6 ± 3.6 years of follow-up, progression of MVR was observed in 41 patients and valve-related events, which comprised mitral valve endocarditis (7 events), heart failure (5 events), and mitral valve surgery (25 events), were observed in 31 patients. Multivariate Cox proportional-hazards regression analysis identified a flail mitral leaflet (hazard ratio [HR] 3.262, 95% confidence interval [CI] 1.406 to 7.566, p = 0.006) and increased indexed end-systolic left ventricular diameters (HR 1.113, 95% CI 1.043 to 1.188, p = 0.001) as independent predictors of progression of MVR. Similarly, mitral valve-related events were independently predicted by a flail mitral leaflet (HR 5.343, 95% CI 2.229 to 12.808, p <0.001), and mild (HR 14.336, 95% CI 1.873 to 109.755, p = 0.01) or moderate (HR 16.849, 95% CI 2.205 to 128.76, p = 0.006) degree of MVR. Conversely, aortic dilatation, dural ectasia, and sporadic mode of inheritance were not associated with outcome. In conclusion, the same clinical determinants that predict outcomes in idiopathic mitral valve prolapse also predict outcomes in mitral valve prolapse associated with Marfan syndrome.     

The clinical diagnosis of acute severe mitral insufficiency

The clinical and hemodynamic data of 8 patients with acute severe mitral insufficiency are presented. The natural history, physical signs, electrocardiogram, X-ray films and findings at cardiac catheterization present a characteristic picture which is distinct from the chronic form of the disease.