Diagnostic accuracy study of routine echocardiography for bicuspid aortic valve: a retrospective study and meta-analysis.

BACKGROUND: Transthoracic echocardiography (TTE) is the standard procedure to distinguish tricuspid aortic valve (TAV) from bicuspid aortic valve (BAV). Published studies assessed the accuracy of TTE for BAV under ideal conditions. Conversely, we aimed at assessing accuracy of TTE for BAV under routine conditions. METHODS: This retrospective, cross-sectional study of 216 adults included 132 men aged 62±14 years. Of these, 108 had BAV and 108 were age-matched individuals with TAV. All diagnoses were confirmed at surgery. We assessed TTE in two patient groups. First, in the (I) group of all 216 individuals, where we assessed accuracy for BAV according to the original diagnoses as documented by the primary investigators during original TTE examination. Second, we assessed accuracy for BAV according to expert re-evaluation in (II) all 158 TTE with availability of original recordings. Third, we performed a meta-analysis of published results on the accuracy of TTE for BAV according to PRISMA standards. RESULTS: Sensitivity, specificity and accuracy of (I) primary investigators was 46.3%, 97.2, and 71.8% as compared to (II) expert re-evaluation with 59.7%, 93%, and 77.8%, respectively. Sensitivity was significantly higher at re-evaluation (P<0.001). TTE at a non-tertiary care center (P=0.012), presence of aortic aneurysm (P=0.001) and presence of severe aortic valve calcification (P=0.003) predicted an inaccurate diagnosis of BAV. Conversely, meta-analysis of published TTE studies identified a pooled sensitivity of 87.7% and a pooled specificity of 88.3% for BAV. CONCLUSIONS: The current study shows that TTE yields almost ideal diagnostic accuracy when ideal investigators examine ideal patients. However, the study also shows that TTE yields suboptimal diagnostic accuracy under routine conditions. TTE in non-tertiary care settings, concomitant aortic aneurysm, and presence of severe aortic valve calcification predict an inaccurate diagnosis of BAV.

Mitral valve prolapse syndrome and MASS phenotype: Stability of aortic dilatation but progression of mitral valve prolapse.

BACKGROUND: Mitral valve prolapse syndrome (MVPS) and MASS phenotype (MASS) are Marfan-like syndromes that exhibit aortic dilatation and mitral valve prolapse. Unlike in Marfan syndrome (MFS), the presence of ectopia lentis and aortic aneurysm preclude diagnosis of MVPS and MASS. However, it is unclear whether aortic dilatation and mitral valve prolapse remain stable in MVPS or MASS or whether they progress like in MFS. METHODS: This retrospective longitudinal observational study examines clinical characteristics and long-term prognosis of 44 adults with MVPS or MASS (18 men, 26 women aged 38 ± 17 years) as compared with 81 adults with Marfan syndrome (MFS) with similar age and sex distribution. The age at final contact was 42 ± 15 years with mean follow-up of 66 ± 49 months. RESULTS: At baseline, ectopia lentis and aortic sinus aneurysm were absent in MVPS and MASS, and systemic scores defined by the revised Ghent nosology were lower than in MFS (all P < .001). Unlike in MFS, no individual with MVPS and MASS developed aortic complications (P < .001). In contrast, the incidence of endocarditis (P = .292), heart failure (P = .644), and mitral valve surgery (P = .140) was similar in all syndromes. Cox regression analysis identified increased LV end-diastolic (P = .013), moderate MVR (P = .019) and flail MV leaflet (P = .017) as independent predictors of mitral valve surgery. CONCLUSIONS: The study provides evidence that MVPS and MASS are Marfan-like syndromes with stability of aortic dilatation but with progression of mitral valve prolapse. Echocardiographic characteristics of mitral valve disease rather than the type of syndrome, predict clinical progression of mitral valve prolapse.

Thirty-day VARC-2 and performance data of a new self-expanding transcatheter aortic heart valve.

AIMS: Transcatheter aortic valve therapy has become an established procedure for patients at high risk for surgical valve replacement. The BIOVALVE-I study aims to assess the safety and performance of a novel self-expanding transcatheter heart valve. METHODS AND RESULTS: In this prospective, single-centre, first-in-human study, 13 patients with severe aortic stenosis suitable for transfemoral transcatheter aortic valve implantation were enrolled. Mean logistic EuroSCORE was 14.4±3.7% and mean STS PROM score was 4.3±1.6%. The primary endpoint, 30-day early safety composite per VARC-2, was observed in two patients (15.4%, one life-threatening bleeding and one valve-in-valve procedure). The implant was aborted in two patients due to unsuitable aortic anatomy. Overall, device success was obtained in nine patients (69.2%, two aborted implants, one valve-in-valve procedure and one patient with moderate aortic regurgitation). As determined by an independent core laboratory, all but one patient had less than moderate total aortic regurgitation at 30-day follow-up, mean aortic gradient was 6.7±2.3 mmHg and effective orifice area 1.8±0.3 cm. Pacemakers were implanted in three patients (23.1%), and no death, stroke, myocardial infarction or acute kidney failure was observed. CONCLUSIONS: In this first-in-human study, the feasibility of implantation of the BIOVALVE system and its re-sheathing functionality was demonstrated, and short-term safety data were encouraging. Larger studies are required to confirm the performance of the device.

Blood transfusion is associated with impaired outcome after transcatheter aortic valve implantation.

OBJECTIVES: We sought to evaluate the relationship of blood transfusion after transcatheter aortic valve implantation (TAVI) and mid-term outcome to improve patient selection and periprocedural treatment. BACKGROUND: Increasing evidence suggests a negative influence of blood transfusion on outcomes of patients with cardiovascular diseases. While the adverse impact of bleeding events on survival has been documented after TAVI, data on the impact of postoperative blood transfusions are scarce. METHODS: TAVI was performed in 700 consecutive patients; 14.7% of TAVI patients suffered from bleeding or access site complications and were excluded from analysis to minimize confounding. Outcomes were analyzed with emphasis on blood transfusions and according to the Valve Academic Research Consortium definitions. Median follow-up duration was 364 days. Multivariable analyses were performed to identify predictors for transfusion and 1-year mortality. RESULTS: 33.0% of patients received blood transfusions after TAVI, irrespective of access choice. Blood transfusions were associated with a higher baseline risk profile (median logistic EuroSCORE 21.0 vs. 17.0%), increased rates of postoperative complications and impaired survival (21.2 vs. 36.1% all-cause 1-year mortality). Transfusion was an independent predictor of mortality at 1 year (OR 2.78 [CI 1.59-4.86]). Low body mass index (OR 0.94 [0.89-1.0]), low baseline hemoglobin (OR 0.39 [0.33-0.47]) and combined anticoagulation/antiplatelet therapy were identified as independent predictors of blood transfusion after TAVI. CONCLUSIONS: Blood transfusions were frequently required after TAVI even in the absence of overt bleeding or access site complications and were identified as an independent predictor of impaired mid-term outcome. Optimization of baseline factors, strict blood conservation strategies, and individualized antiplatelet or anticoagulant regimens may improve outcome after TAVI.

Single-centre experience with next-generation devices for transapical aortic valve implantation.

OBJECTIVES: Transcatheter aortic valve implantation (TAVI) has become an established procedure in patients with aortic stenosis and high surgical risk. Experience with first-generation transcatheter heart valves (THVs) is broad but limitations, e.g. paravalvular regurgitation, have been demonstrated. Much hope rests on the recently Conformité Européenne mark approved next-generation devices to improve results in these patients. However, apart from the initial approval studies, clinical data with these new devices are still scarce. We aimed to assess short-term outcomes of 200 consecutive patients who underwent transapical TAVI with next-generation THV at our institution. METHODS: Transapical TAVI was performed in 200 consecutive patients 80.5±6.7 years old (38.5% female) at high surgical risk (log EuroSCORE 20.2±16.5%). Devices implanted were the Engager (Medtronic, Inc., Minneapolis, MN, USA; n=50), JenaValve (JenaValve Technology, Munich, Germany; n=88) and Symetis Acurate (Symetis SA, Ecublens, Switzerland; n=62) THV that were selected by the heart team on an individual basis. Data at baseline, during the procedure and follow-up were analysed according to standardized Valve Academic Research Consortium end points. Median follow-up was 219 days. RESULTS: Implantation was successful in 96.5% of cases. Valve function improved significantly with an increase in effective orifice area from 0.8±0.4 to 1.8±0.3 cm2 and a reduction in mean transvalvalvular gradients from 34.0±17.0 to 11.2±5.4 mmHg. Paravalvular regurgitation was none or trace in 70.3% of patients, Grade 1 in 26.1%, and Grade 2 in 3.5%. No patients developed aortic regurgitation>Grade 2. Major access site complications occurred in 6.5%, major stroke in 1.5% and stage-3 kidney injury in 2.5% of patients. A permanent pacemaker was implanted in 20.5% of patients overall and in 8.0% for a complete heart block. At 30-day follow-up 72.8% of patients were in New York Heart Association class I or II (10.5% at baseline). Overall survival was 91.5% at 30 days and 73.9% at 1 year. CONCLUSIONS: In a real-world clinical setting, next-generation transapical THV yielded positive haemodynamic results. The incidence of relevant paravalvular regurgitation was scarce in this group and clinical outcomes were encouraging during short-term follow-up. Long-term follow-up is required to investigate the durability of these new devices.

Kidney injury and mortality after transcatheter aortic valve implantation in a routine clinical cohort.

OBJECTIVES: We aimed at identifying predictors of renal impairment and its impact on long-term outcome after transcatheter aortic valve implantation (TAVI). BACKGROUND: Renal impairment is common in mostly elderly, multimorbid patients undergoing TAVI. The risk of periprocedural renal function impairment and its association with outcome is incompletely understood. METHODS: In 458 consecutive patients (mean age, 80.6 ± 7.0 years, 52.2% women) who underwent routine TAVI procedures, we assessed estimated glomerular filtration rate (eGFR) at baseline, during 72-hr postprocedure and at discharge. Over a median follow-up of 0.96 years, we observed 142 deaths. RESULTS: In multivariable-adjusted models, predictors of renal function deterioration within 72 hr were baseline eGFR (ß = 0.83, 95% confidence interval [CI] = 0.76/0.91; P < 0.0001), body mass index (ß = -1.20, 95% CI = 1.77/-0.62; P < 0.0001), and major access site complications (ß = -14.82, 95% CI = -26.52/-3.11; P = 0.013) including bleeding (ß = -11.97, 95% CI = -21.05/-2.89; P = 0.0099). Strongest renal function predictor of 1-year mortality in risk factor adjusted analyses was the change of eGFR within 72 hr (odds ratio, 0.97; 95% CI = 0.96/0.98; P < 0.0001). The addition of information on the change of eGFR increased the C-statistic of the logistic EuroSCORE (P = 0.021). CONCLUSIONS: In our routine TAVI sample, baseline eGFR, body mass index, and major access site complications mainly owing to bleeding were correlates of acute kidney injury after TAVI. Acute renal impairment was a risk factor for mortality and adverse cardiovascular events which provided risk information beyond the EuroSCORE.

The main pulmonary artery in adults: a controlled multicenter study with assessment of echocardiographic reference values, and the frequency of dilatation and aneurysm in Marfan syndrome.

BACKGROUND: Echocardiographic upper normal limits of both main pulmonary artery (MPA) diameters (MPA-d) and ratio of MPA to aortic root diameter (MPA-r) are not defined in healthy adults. Accordingly, frequency of MPA dilatation based on echocardiography remains to be assessed in adults with Marfan syndrome (MFS). METHODS: We enrolled 123 normal adults (72 men, 52 women aged 42 ± 14 years) and 98 patients with MFS (42 men, 56 women aged 39 ± 14 years) in a retrospective cross-sectional observational controlled study in four tertiary care centers. We defined outcome measures including upper normal limits of MPA-d and MPA-r as 95 quantile of normal persons, MPA dilatation as diameters > upper normal limits, MPA aneurysm as diameters >4 cm, and indication for surgery as MPA diameters >6 cm. RESULTS: MPA diameters revealed normal distribution without correlation to age, sex, body weight, body height, body mass index and body surface area. The upper normal limit was 2.6 cm (95% confidence interval (CI) =2.44-2.76 cm) for MPA-d, and 1.05 (95% CI = .86-1.24) for MPA-r. MPA dilatation presented in 6 normal persons (4.9%) and in 68 MFS patients (69.4%; P < .001), MPA aneurysm presented only in MFS (15 patients; 15.3%; P < .001), and no patient required surgery. Mean MPA-r were increased in MFS (P < .001), but ratios >1.05 were equally frequent in 7 normal persons (5%) and in 8 MFS patients (10.5%; P = .161). MPA-r related to aortic root diameters (P = .042), reduced left ventricular ejection fraction (P = .006), and increased pulmonary artery systolic pressures (P = .040). No clinical manifestations of MFS and no FBN1 mutation characteristics related to MPA diameters. CONCLUSIONS: We established 2.6 cm for MPA-d and 1.05 for MPA-r as upper normal limits. MFS exhibits a high prevalence of MPA dilatation and aneurysm. However, patients may require MPA surgery only in scarce circumstances, most likely because formation of marked MPA aneurysm may require LV dysfunction and increased PASP.

Valve-in-valve implantation of a novel and small self-expandable transcatheter heart valve in degenerated small surgical bioprostheses: the Hamburg experience.

BACKGROUND: Transcatheter valve-in-valve (VIV) implantation has emerged as a novel treatment option in patients with degenerated aortic bioprostheses and high surgical risk. However, VIV implantation in small aortic bioprostheses using first generation TAVI devices has frequently resulted in high postprocedural gradients and small effective orifice areas. Recently, an updated version of the self-expandable Medtronic CoreValve prosthesis, which is particularly suitable for small aortic annuli, has become available. We report on the feasibility and early results of VIV implantation using this novel device in a series of patients with degenerated small aortic bioprostheses. METHODS AND RESULTS: 16 patients from two Hamburg hospitals (age range 72-92 years) underwent implantation of a 23-mm CoreValve Evolut (Medtronic, Minneapolis, MN, USA) transcatheter heart valve into failing aortic bioprostheses with internal diameters below 21 mm. All patients were considered high risk for surgical aortic valve replacement after evaluation by an interdisciplinary heart team mean (logistic EuroSCORE range 9.0-88%). Implantation was successful without relevant remaining aortic regurgitation or signs of stenosis and a marked reduction in postprocedural gradientswas observed in 14 out of 16 patients. The mean gradient was reduced from from 34 mm Hg (SEM 10 mm Hg) to 14 mm Hg (SEM 6 mm Hg). No major device- or procedure-related adverse events occurred during 30-day follow up and clinical improvement was observed. CONCLUSIONS: Failing aortic bioprostheses with small internal diameters continue to be a challenging pathology for TAVI due to the risk of high residual gradients and small aortic orifice areas. This report provides first evidence that the 23-mm CoreValve Evolut, a novel self-expanding prosthesis, provides an improved treatment option in these specific patients due to its low profile which resulted in promising early results. © 2013 Wiley Periodicals, Inc.

Development of a risk score for outcome after transcatheter aortic valve implantation.

AIMS: Transcatheter aortic valve implantation (TAVI) is an increasingly common procedure in elderly and multimorbid patients with aortic stenosis. We aimed at developing a pre-procedural risk evaluation scheme beyond current surgical risk scores. METHODS: We developed a risk algorithm for 1-year mortality in two cohorts consisting of 845 patients undergoing routine TAVI procedures by commercially available devices, mean age 80.9 ± 6.5, 51 % women. Clinical variables were determined at baseline. Multivariable Cox regression related clinical data to mortality (n = 207 deaths). RESULTS: To account for variability related to age and sex and by enrolment site we forced age, sex, and cohort into the score model. Body mass index, estimated glomerular filtration rate, hemoglobin, pulmonary hypertension, mean transvalvular gradient and left ventricular ejection fraction at baseline were most strongly associated with mortality and entered the risk prediction algorithm [C-statistic 0.66, 95 % confidence interval (CI) 0.61-0.70, calibration χ (2)-statistic = 6.51; P = 0.69]. Net reclassification improvement compared to existing surgical risk predication schemes was positive. The score showed reasonable model fit and calibration in external validation in 333 patients, N = 55 deaths (C-statistic 0.60, 95 % CI 0.52-0.68; calibration χ (2)-statistic = 16.2; P = 0.06). Additional measurement of B-type natriuretic peptide and troponin I did not improve the C-statistic. Frailty increased the C-statistic to 0.71, 95 % CI 0.65-0.76. CONCLUSIONS: We present a new risk evaluation tool derived and validated in routine TAVI cohorts that predicts 1-year mortality. Biomarkers only marginally improved risk prediction. Frailty increased the discriminatory ability of the score and needs to be considered. Risk algorithms specific for TAVI may help to guide decision-making when patients are evaluated for TAVI.

The challenge of valve-in-valve procedures in degenerated Mitroflow bioprostheses and the advantage of using the JenaValve transcatheter heart valve.

AIMS: Recently, the feasibility of valve-in-valve procedures using current first-generation transcatheter heart valves (THV) in cases of structural valve degeneration has been reported as an alternative to conventional open repeat valve replacement. By design, certain biological valve xenografts carry a high risk of coronary ostia occlusion due to lateral displacement of leaflets after valve-in-valve procedures. In the present report we aimed to prove feasibility and safety of transapical valve-in-valve implantation of the JenaValve THV in two cases of degenerated Mitroflow bioprostheses. METHODS AND RESULTS: We herein report two cases of successful transapical valve-in-valve procedures using a JenaValve THV implanted in Sorin Mitroflow bioprostheses for structural valve degeneration. Both patients were alive and in good clinical condition at 30 days from the procedure. However, increased transvalvular gradients were noted in both cases. CONCLUSIONS: Transcatheter valve-in-valve implantation of a JenaValve THV is a valid alternative for patients with degenerated Mitroflow bioprostheses of sufficient size and in the presence of short distances to the coronary ostia who are too ill for conventional repeat open heart surgery. Increased pressure gradients have to be expected and weighed against the disadvantages of other treatment options when planning such a procedure.

Transapical transcatheter aortic valve implantation without prior balloon aortic valvuloplasty: feasible and safe.

OBJECTIVES: Currently, preimplant balloon aortic valvuloplasty (BAV) is considered a prerequisite for successful subsequent transapical transcatheter aortic valve implantation (TA-TAVI) using balloon-expandable devices. However, cerebral embolization has been shown to originate at least in part from BAV procedures. Omitting BAV may therefore reduce neurological events after TAVI and facilitate the procedure while yielding non-inferior haemodynamic and clinical outcomes. METHODS: From May 2011 through December 2012, a total of 50 consecutive patients were treated by TA-TAVI without preimplant BAV (TA-TAVI(-BAV), study group) using the Edwards Sapien XT device (54% male, age 78 ± 8 years, logistic European System for Cardiac Operative Risk Evaluation I 21 ± 14%). Data were prospectively entered into a dedicated database, retrospectively analysed and compared with a consecutive series of conventional TA-TAVI using the same device (control group, n = 50). Reporting of data followed Valve Academic Research Consortium definitions. RESULTS: Overall device success rate was 94% (47/50) and 86% (43/50) in study and control groups, respectively (P = 0.32). Procedure time was similar in the study group compared with the control group (88 ± 31 vs 91 ± 25 min, P = 0.60), while significantly less contrast was used (138 ± 68 vs 183 ± 78 ml, P < 0.001). Post-procedural peak and mean transvalvular gradients were 16 ± 7 and 8 ± 3 mmHg, respectively, in the study group with similar values in the control group (19 ± 9 and 9 ± 5 mmHg, P = 0.08 and P = 0.09, respectively). Residual paravalvular leakage (PVL) grade 2 was present in 2 and 8% in study and control groups, respectively (P = 0.36), with no PVL >grade 2 in any patient. Rates of 30-day mortality and periprocedural stroke were 4 and 10% (P = 0.44) and 2 and 6% (P = 0.62), respectively. CONCLUSIONS: TA-TAVI(-BAV) is feasible and safe and has become ur default technique for patients allocated to TA-TAVI with balloon-expandable devices. This approach resulted in less contrast agent used and facilitated the procedure without compromising valve performance. Possible beneficial effects of this approach on the incidence of cerebrovascular events, other periprocedural complications or haemodynamic valve performance need to be verified in larger patient numbers before general recommendations can be made.

Transapical implantation of a second-generation transcatheter heart valve in patients with noncalcified aortic regurgitation.

OBJECTIVES: This study sought to report on the feasibility and early results of transcatheter aortic valve implantation employing a second-generation device in a series of patients with pure aortic regurgitation. BACKGROUND: Efficacy and safety of transcatheter aortic valve implantation in patients with calcific aortic stenosis and high surgical risk has been demonstrated. However, experience with implantation for severe noncalcified aortic regurgitation has been limited due to increased risk for valve dislocation or annular rupture. METHODS: Five patients (mean age: 66.6 ± 7 years) underwent transapical implantation of a JenaValve (JenaValve Technology GmbH, Munich, Germany) transcatheter heart valve for moderate to severe, noncalcified aortic regurgitation. All patients were considered high risk for surgical aortic valve replacement after evaluation by an interdisciplinary heart team (logistic EuroSCORE [European System for Cardiac Operative Risk Evaluation] range 3.1% to 38.9%). Procedural and acute clinical outcomes were analyzed. RESULTS: Implantation was successful in all cases without relevant remaining aortic regurgitation or signs of stenosis in any of the patients. No major device- or procedure-related adverse events occurred and all 5 patients were alive with improved exercise tolerance at 3-month follow-up. CONCLUSIONS: Noncalcified aortic regurgitation continues to be a challenging pathology for transcatheter aortic valve implantation due to the risk for insufficient anchoring of the valve stent within the aortic annulus. This report provides first evidence that the JenaValve prosthesis may be a reasonable option in these specific patients due to its unique stent design, clipping the native aortic valve leaflets, and offering promising early results.

Predictors and outcomes after transcatheter aortic valve implantation using different approaches according to the valve academic research consortium definitions.

OBJECTIVES: We report the results of a large single-center study evaluating predictors and outcomes after transcatheter aortic valve implantation (TAVI) with different devices and access routes according to the Valve Academic Research Consortium (VARC). BACKGROUND: The widespread adoption of TAVI warrants a systematic analysis of outcomes. Only few comprehensive data exist comparing different approaches as selected by a heart team. METHODS: TAVI was performed in 326 consecutive patients (mean age 80.6 ± 7.1 years, 55.5% female) at high risk for surgery with balloon-expandable and self-expanding devices through transfemoral or transapical access. Data were analyzed according to VARC endpoints; predictors of mortality were identified. RESULTS: All-cause mortality was 10.1% (30 days) and 29.9% (1 year) overall and comparable with regard to valve or access choice (P = 0.295) despite different risk profiles at baseline. Device success and 30-day safety endpoints were achieved in 87.1 and 21.2%. Myocardial infarction [hazard ratio (HR) 6.52], stage-2 and -3 acute kidney injury (HR 2.52 and 6.80) and major access site complications (HR 1.96) were independent predictors of 1-year all-cause mortality. Device success had a protective effect (HR 0.58). Baseline predictors included body mass index <20 kg/m(2) (HR 3.20), NYHA class IV (HR 1.87), left ventricular ejection-fraction <30% (HR 2.30), higher STS-PROM scores (HR 1.05 per percent), and age (group 75-85 years, HR 0.47). CONCLUSIONS: Comparable results were achieved with different devices and access routes in a heart team approach. Baseline and perioperative predictors of all-cause mortality were identified, contributing to the refinement of patient and device selection criteria for TAVI.

Patient gender does not affect outcome after transcatheter aortic valve implantation (TAVI).

BACKGROUND AND AIM OF THE STUDY: Female gender has recently been suggested to predict a beneficial outcome and lower mortality following transcatheter aortic valve implantation (TAVI). The study aim was to address gender differences in outcome following TAVI and potentially to replicate these findings. METHODS: The present single-center prospective registry of 326 patients with severe aortic stenosis treated by TAVI between 2008 and 2011 consisted of 181 women and 145 men. RESULTS: The procedural risk was not significantly different between men and women at baseline. For all-cause mortality no difference was observed at 30 days and at 12 months after TAVI. CONCLUSION: While several baseline characteristics between men and women treated by TAVI were shown to be different among the study population, no difference was observed in survival between both genders.

Impact of patient-prosthesis mismatch after transcatheter aortic valve-in-valve implantation in degenerated bioprostheses.

OBJECTIVE: Transcatheter valve-in-valve implantation is evolving as an alternative to reoperative valve replacement in high-risk patients with degenerated bioprostheses. Nevertheless, hemodynamic performance is limited by the previously implanted xenograft. We report our experience with patient-prosthesis mismatch (PPM) after valve-in-valve implantation in the aortic position. METHODS: Eleven patients (aged 79.3 ± 6.1 years) received transapical implantation of a balloon-expandable pericardial heart valve into a degenerated bioprosthesis (size, 23.9 ± 1.6 mm; range, 21-27 mm) in the aortic position. All patients were considered high risk for surgical valve replacement (logistic European System for Cardiac Operative Risk Evaluation, 31.8% ± 24.1%). Severe PPM was defined as an indexed effective orifice area less than 0.65 cm(2)/m(2), determined by discharge echocardiography. RESULTS: Severe PPM was evident in 5 patients (group 1) and absent in 6 patients (group 2). Mean transvalvular gradients decreased from 29.2 ± 15.4 mm Hg before implantation to 21.2 ± 9.7 mm Hg at discharge (group 1) and from 28.2 ± 9.0 mm Hg before implantation to 15.2 ± 6.5 mm Hg at discharge (group 2). Indexed effective orifice area increased from 0.5 ± 0.1 cm(2)/m(2) to 0.6 ± 0.1 cm(2)/m(2) and from 0.6 ± 0.3 cm(2)/m(2) to 0.8 ± 0.3 cm(2)/m(2). Aortic regurgitation decreased from grade 2.0 ± 1.1 to 0.4 ± 0.5 overall. No differences in New York Heart Association class improvement or survival during follow-up were observed. One patient required reoperation for symptomatic PPM 426 days after implantation. CONCLUSIONS: Valve-in-valve implantation can be performed in high-risk surgical patients to avoid reoperation. However, PPM frequently occurs, making adequate patient selection crucial. Small bioprostheses (<23 mm) should be avoided. Implantation into 23-mm xenografts can be recommended only for patients with a body surface area less than 1.8 m(2). Larger prostheses seem to carry a lower risk for PPM. Although no delay in clinical improvement was seen at short-term, 1 PPM-related surgical intervention raises concern regarding long-term performance.

A heart team's perspective on interventional mitral valve repair: percutaneous clip implantation as an important adjunct to a surgical mitral valve program for treatment of high-risk patients.

OBJECTIVE: Surgical mitral valve repair carries an elevated perioperative risk in the presence of severely reduced ventricular function and relevant comorbidities. We sought to assess the feasibility of catheter-based mitral valve repair using a clip-based percutaneous edge-to-edge repair system in selected patients at high surgical risk with mitral regurgitation grade 3 or worse. METHODS: Between 2002 and January 2011, 202 consecutive patients without prior mitral valve surgery (age 75 ± 9 years; 63% were male) with symptomatic functional (65%), degenerative (27%), or mixed (8%) mitral regurgitation were treated with a percutaneous clip system for approximation of the anterior and posterior mitral leaflets. Risk for mitral valve surgery was considered high in terms of a mean logistic European System for Cardiac Operative Risk Evaluation of 44% (range, 21%-54%). Preprocedural left ventricular ejection fraction was 35% or less in 36% of patients. An interdisciplinary heart team of cardiologists and cardiac surgeons discussed all patients. RESULTS: Percutaneous clip implantation was successful in 186 patients (92%). Patients were treated with 1 clip (n = 125; 62%), 2 clips (n = 64; 32%), or 3 or more clips (n = 7; 3%). Reduction in mitral regurgitation from pre- to postprocedure was significant (P < .0001) and remained stable within the first 12 months in the majority of patients. Thirty-day mortality was 3.5% (7/202 patients). Hospital stay was 12 ± 10 days, and median intensive care unit stay was 1 day (range, 0-45 days). Eleven patients required surgical valve repair/replacement at a median of 38 days (0-468 days) after percutaneous clip implantation. CONCLUSIONS: Clip-based percutaneous mitral valve repair is a safe, low-risk, and effective therapeutic option in symptomatic patients with a high risk for surgery and does not exclude later surgical repair.

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.

Frequency and age-related course of mitral valve dysfunction in the Marfan syndrome.

Mitral valve (MV) prolapse (MVP) has a high prevalence of 2% to 3% in the general population and thus constitutes the most common cause of severe nonischemic MV regurgitation (MVR). MVP is also common in persons with the Marfan syndrome. However, to date, a large-scale population-based cohort study using modern echocardiographic techniques has not been performed, and the frequency of MVP and the relation of MV dysfunction and age have not been investigated. Therefore, we conducted a population-based cohort study of 204 patients (108 males and 96 females, aged 31.2 ± 16.4 years) with classic Marfan syndrome. We performed echocardiographic follow-up of 174 patients for a mean of 4.4 ± 4.3 years. On the initial or subsequent echocardiographic scan, MVP was present in 82 patients (40%), severe MVR in 25 (12%), and MV endocarditis in 5 patients (2.5%). At 30 years of age, the Weibull cumulative distribution was 42.6% (95% confidence interval [CI] 36% to 50%) for MVP, 56.5% (95% CI 49.3% to 64%) for MVR of any degree, 6.7% (95% CI 3.9% to 11.3%) for severe MVR, and 0.92% (95% CI 0.21% to 3.91%) for MV endocarditis. The cumulative hazard for severe MVR and MV endocarditis was estimated to increase with age. MVP was associated with dural ectasia (p = 0.01), ectopia lentis (p = 0.02), and skeletal involvement (p <0.001). Severe MVR was related to tricuspid valve prolapse (p = 0.002) and to the sporadic form of the Marfan syndrome (p = 0.006). In conclusion, MVP was comparatively frequent in patients with the Marfan syndrome and carries an increased risk of progression to severe MVR and endocarditis, especially in older adults.

Frequency of sleep apnea in adults with the Marfan syndrome.

Obstructive and central sleep apneas are treatable disorders, which contribute to cardiovascular morbidity in older adults. Younger adults with Marfan syndrome may also be at risk for sleep apnea, but the relation between cardiovascular complications and sleep apnea is unknown. We used MiniScreen8 portable monitoring devices for polygraphy in 68 consecutive adults with Marfan syndrome (33 men, 35 women, 41 +/- 14 years old) to investigate frequency of sleep apnea and its relation to cardiovascular morbidity. The apnea-hypopnea index (AHI) was 6 to 15/hour in 14 subjects (mild sleep apnea, 21%), and AHI was >15/hour in 7 subjects (moderate or severe sleep apnea, 10%). Among established risk factors for sleep apnea, only older age (Spearman rho = 0.35, p = 0.004) and body mass index (rho = 0.26, p = 0.03) were associated with increased AHI. Of all cases of apnea, 12 +/- 27 were obstructive, 11 +/- 25 central, and 3 +/- 9 mixed. AHI was associated with decreased left ventricular ejection fraction (rho = -0.33, p = 0.01), increased N-terminal pro-brain natriuretic peptide levels (rho = 0.35, p = 0.004), enlarged descending aortic diameters (rho = 0.44, p = 0.001), atrial fibrillation (phi = 0.43, p = 0.002), and mitral valve surgery (phi = 0.34, p = 0.02). Of these, left ventricular ejection fraction, N-terminal pro-brain natriuretic peptide levels, atrial fibrillation, and mitral valve surgery were associated with AHI independently of age and body mass index. We found similar associations with oxygen desaturation index. In conclusion, sleep apnea exhibits increased frequency in Marfan syndrome and is not predicted by classic risk factors. Obstructive and central sleep apneas may relate to cardiovascular disease variables.