Long-term outcome of bicuspid aortic valve disease.

AIMS: Bicuspid aortic valve (BAV) is a common congenital condition that is frequently associated with aortic stenosis (AS) and aortic regurgitation (AR), as well as aortic aneurysms, but specific outcome data are scarce. The present study sought to assess outcomes in a large cohort of consecutive patients with BAV. METHODS AND RESULTS: A total of 581 consecutive patients (median age 29 years, 157 female) with BAV were included in the study and followed prospectively in a heart valve clinic follow-up programme. The overall survival rate after 10 years was 94.5%. During follow-up, 158 patients developed an indication for surgery. Event-free survival rates were 97%, 94%, 87%, and 73% at 1, 2, 5, and 10 years, respectively. In the multivariable analysis, event rates were independently predicted by AS [subdistribution hazard ratio (SHR) 2.3 per degree of severity], AR (SHR 1.5 per degree of severity), baseline aortic dilatation ≥ 40 mm (SHR 1.9), and age (SHR 1.3) (P < 0.001). CONCLUSION: BAV disease is associated with a high rate of cardiac events, but state-of-the-art care results in good survival with low rates of infective endocarditis, aortic dissection, and sudden death. Incremental degrees of AS and regurgitation, the presence of aortic dilatation, and age are predictive of cardiac events.

Left atrial size predicts outcome in severe but asymptomatic mitral regurgitation.

Patients with severe asymptomatic primary mitral regurgitation (MR) can be safely managed with an active surveillance strategy. Left atrial (LA) size is affected by MR severity, left ventricular function and is also associated with the risk of atrial fibrillation and may be an integrative parameter for risk stratification. The present study sought to determine the predictive value of LA size in a large series of asymptomatic patients with severe MR. 280 consecutive patients (88 female, median age 58 years) with severe primary MR and no guideline-based indications for surgery were included in a follow-up program until criteria for mitral surgery were reached. Event-free survival was determined and potential predictors of outcome were assessed. Survival free of any indication for surgery was 78% at 2 years, 52% at 6 years, 35% at 10 years and 19% at 15 years, respectively. Left atrial (LA) diameter was the strongest independent echocardiographic predictor of event-free survival with incremental predictive value for the thresholds of 50, 60 and 70 mm, respectively. In a multivariable analysis that encompassed age at baseline, previous history of atrial fibrillation, left ventricular end systolic diameter), LA diameter, sPAP > 50 mmHg and year of inclusion, LA diameter was the strongest independent echocardiographic predictor of event-free survival (adjusted HR = 1.039, p < 0.001). LA size is a simple and reproducible predictor of outcome in asymptomatic severe primary MR. In particular, it may help to identify patients who may benefit from early elective valve surgery in heart valve centers of excellence.

Open Transcatheter Mitral Valve Implantation Into the Native Mitral Valve for Inoperable "Big MAC".

Because of the growing population of older adult patients, the prevalence of severe mitral annulus calcification ("big MAC") is increasing. The surgical techniques used to treat big MACs are technically demanding; despite the technical aspect, up to one-third of patients are considered too high risk for conventional surgery but are candidates for the coulisse technique, which is a procedure that implants a transcatheter valve into a native mitral annulus. The anterior leaflet is unfolded, thus reducing the risk of obstructing the left ventricular outflow tract and for paravalvular leak and avoiding valve migration. Preoperative planning, based on a computed tomography scan, is mandatory.

Isolated tricuspid valve regurgitation: old concepts, new insights and innovation.

: Tricuspid valve regurgitation is usually progressive and associated with reduced long-term survival. International guidelines recommend surgery in cases of isolated severe regurgitation associated with symptoms or progressive worsening of right ventricular function. In addition, several percutaneous strategies have been reported with promising results in the last decade. The aim of the present review is to summarize the current state of the art in the management of isolated functional or degenerative tricuspid valve insufficiency, from the preoperative assessment to the results of both surgical and transcatheter approaches and explore future directions.

Off-pump tricuspid valve repair by automated sutured tricuspid annular plication via transatrial cannulation: preclinical ex vivo and in vivo results.

OBJECTIVES: Surgical repair is considered the gold standard treatment for severe symptomatic tricuspid valve (TV) regurgitation. However, patients undergoing isolated surgical tricuspid repair face a high perioperative mortality while long-term data on interventional treatment options are currently missing. We investigated a novel, minimally invasive approach for transatrial off-pump beating-heart tricuspid annular plication based on the surgical Hetzer repair. METHODS: TV annular plication for the creation of a double-orifice valve using novel devices for automated annular suturing was performed in 10 human heart specimens in an ex vivo perfusion model under endoscopic guidance. Additionally, the technique was tested in an in vivo porcine model using the transatrial access under echocardiographic and fluoroscopic guidance. RESULTS: Endoscopically guided conduction of the procedure was successful in all 10 human heart specimens in the ex vivo perfusion model with 1 observed suture pull-through of 60 sutures placed (1.7%). TV measurements yielded significant reductions of the TV septal-lateral diameter (50.9 ± 7.3 vs 42.6 ± 7.9 mm; P = 0.015) and the TV area (1208 ± 399 vs 193 ± 122 mm2; P < 0.0001). TV plication without direct vision using device-embedded intracardiac echocardiography, epicardial echocardiography and fluoroscopy was feasible in both acute animals with no observed device-related adverse events. CONCLUSIONS: Successful plication was completed in 10 ex vivo human hearts, additionally proof-of-concept was conducted in 2 animals. We herein present encouraging early preclinical results of a novel minimally invasive technique for TV repair, which warrants further investigation.

Concomitant Transapical Transcatheter Aortic Valve Implantation and Transapical Mitral Valve Repair With NeoChord Implantation.

One-third of the patients with severe symptomatic aortic valve stenosis (sAS) present with hemodynamic relevant mitral valve insufficiency (rMI). In patients who undergo conventional surgery, the rMI never would be left untreated; however, in cases of transcatheter aortic valve implantation (TAVI), the impact of rMI is often overlooked and left untreated. The combination of transapical TAVI (TA-TAVI) and NeoChord implantation represents a novel, promising therapeutic option for high-risk-surgery patients with sAS and rMI due to a prolapsed or flailed leaflet. This case report describes 2 patients (1 male, 1 female; mean age 82 years) who underwent TA-TAVI and concomitant NeoChord implantation at our institute. Both presented with sAS and rMI due to a prolapse of the P2 segment of the mitral valve. At first, the TA-TAVI was implanted under angio-guidance, followed by three-dimensional echo-guided implantation of the NeoChords, through the same approach, which was slightly posterior and lateral to the apex. TA-TAVI using an Edwards Sapien 3 (26 mm, n = 1 and 29 mm, n = 1) and NeoChord implantation (2 in the first and 3 in the second patient) was successful in both cases. Post-intervention discharge echo indicated no paravalvular or central insufficiency after the procedure and only a trace of mitral valve insufficiency. TA-TAVI and concomitant NeoChord implantation is a feasible and promising treatment option for high-risk patients with rMI. Despite its technical demands, in experienced hands, it is a safe procedure for those not well suited for surgical intervention.

Long-Term Outcome of Active Surveillance in Severe But Asymptomatic Primary Mitral Regurgitation.

OBJECTIVES: This study sought to assess the long-term outcome of active surveillance in these patients. BACKGROUND: The optimal timing of mitral valve surgery in asymptomatic primary mitral regurgitation (MR) remains controversial. METHODS: Between 1997 and 2015, 280 consecutive patients with severe asymptomatic primary MR were enrolled in our heart valve clinic follow-up program. They were prospectively followed up every 6 months clinical and echocardiographical examinations until surgical criteria were reached. Event-free survival and overall survival as compared with the age- and gender-matched general population were assessed. RESULTS: During a median potential follow-up of 93.4 (quartiles 55.3 to 152.9) months, 161 patients developed an indication for surgery and 13 patients died. Event-free survival rates were 78.0% (95% confidence interval [CI]: 73.2% to 83.2%) at 2 years, 52.2% (95% CI: 46.3% to 59.0%) at 6 years, 35.5% (95% CI: 29.3% to 43.1%) at 10 years, and 18.7% (95% CI: 12.3% to 28.5%) at 15 years. Overall survival rate was 99.6% (95% CI: 98.9% to 100%) at 2 years, 94.6% (95% CI: 91.7% to 97.6%) at 6 years, 85.6% (95% CI: 80.3% to 91.2%) at 10 years, and 74.5% (95% CI: 66.6% to 83.4%) at 15 years. Overall survival of patients managed according to an active surveillance strategy was comparable with the expected cumulative survival and early survival rates were even better in the study population (standardized mortality ratio: 0.667; 95% CI: 0.463 to 0.963; p = 0.013). CONCLUSIONS: Patients with severe asymptomatic primary MR may remain free of indications for surgery for extensive periods of time. In such patients, active surveillance performed in experienced centers is associated with a favorable prognosis, resulting in timely referral to surgery, excellent long-term survival, and good surgical outcomes.

Prognostic relevance of mitral and tricuspid regurgitation in patients with severe aortic stenosis.

Aims: Although concomitant mitral regurgitation (MR) and tricuspid regurgitation (TR) are frequently present in patients with aortic stenosis (AS), outcome data are scarce and treatment strategies are controversial. The aim of the present study was to assess the presentation and outcome of patients with AS and coexisting MR and TR. Methods and results: Eighty-nine consecutive patients with severe AS and at least moderate MR (72 functional and 17 degenerative) were included and followed. Seventy-five patients were symptomatic at presentation. Sixty of these had severe symptoms (New York Heart Association class ≥3). Nine additional patients had an indication for valve procedures during follow-up. However, 35 patients were managed conservatively. Isolated aortic valve intervention was performed in 29 patients (22 valve replacement and 7 transcatheter aortic valve replacement) and concomitant mitral valve surgery in 20 patients. For the assessment of outcome, overall survival (i.e. time from study entry to death from any cause) was assessed: adjusted survival was significantly higher for patients undergoing any valve procedure as compared with patients managed conservatively (P = 0.032). Surgical treatment of severe concomitant MR was associated with improved survival in an unadjusted population but did not reach statistical significance after propensity adjustment. 14 of 36 patients who had concomittant moderate-to-severe tricuspid regurgitation (TR) underwent surgery of which 7 (50 %) died within 3 months postoperatively. On the other hand, only 1 of 35 (3%) with no or mild TR undergoing surgery died within 3 months post-operatively (P < 0.001). Conclusion: Presence of MR in patients with severe AS characterizes a high-risk population. Timely aortic valve intervention confers a survival benefit and concomitant mitral valve surgery should be considered according to operative risk. The additional presence of significant TR is associated with dismal outcomes, regardless of the treatment strategy.

Asymptomatic Severe Aortic Stenosis in the Elderly.

OBJECTIVES: This study sought to assess the natural history and optimal timing of surgery in elderly patients with severe asymptomatic aortic stenosis (AS). BACKGROUND: AS is increasingly diagnosed in an aging population, and large numbers of elderly patients are undergoing aortic valve procedures. However, the average age of patients represented in most natural history studies on AS is between 60 and 70 years. METHODS: A total of 103 consecutive patients >70 years of age (51 female; mean age 77 ± 5 years) with asymptomatic severe AS (peak aortic jet velocity [AV-Vel] 4.7 ± 0.6 m/s) were prospectively followed. RESULTS: During follow-up, 91 events occurred, including an indication for aortic valve replacement in 82 patients and cardiac deaths in 9, respectively. Event-free survival was 73%, 43%, 23%, and 16% at 1, 2, 3, and 4 years, respectively. Physical mobility was impaired in 29% of the patients, and symptom onset was severe (New York Heart Association functional class ≥III) in 43% of those who developed symptoms. Patients with AV-Vel ≥5.0 m/s had event-free survival rates of 21% and 6% at 2 and 4 years, respectively, compared with 57% and 23% for patients with AV-Vel <5.0 m/s (p < 0.001). Seventy-one patients underwent aortic valve replacement, and post-operative survival was 89% and 77% after 1 and 3 years, respectively. CONCLUSIONS: In elderly patients with severe but asymptomatic AS, mild symptoms may be difficult to detect, particularly when mobility is impaired and severe symptom onset is common, warranting close clinical follow-up. Furthermore, a very high event rate can be expected, and cardiac deaths are not infrequent. Thus, elective aortic valve procedures may be considered in selected elderly patients at low procedural risk.

Role of a heart valve clinic programme in the management of patients with aortic stenosis.

AIMS: We sought to assess the efficacy of a heart valve clinic (HVC) follow-up programme for patients with severe aortic stenosis (AS). METHODS AND RESULTS: Three hundred and eighty-eight consecutive patients with AS (age 71 ± 10 years; aortic-jet velocity 5.1 ± 0.6 m/s) and an indication for aortic valve replacement (AVR) were included. Of these, 290 patients presented with an indication for surgery at their first visit at the HVC and 98 asymptomatic patients who had been enrolled in an HVC monitoring programme developed indications for surgery during follow-up. Time to symptom detection was significantly longer in patients that presented with symptoms at baseline (352 ± 471 days) than in patients followed in the HVC (76 ± 75 days, P < 0.001). Despite being educated to recognize and promptly report new symptoms, 77 of the 98 patients in the HVC programme waited until the next scheduled consultation to report them. Severe symptom onset (NYHA or CCS Class ≥III) was present in 61% of patients being symptomatic at the initial visit and in 34% of patients in the HVC programme (P < 0.001). CONCLUSION: Delays in referral and symptom reporting as well as symptom denial are common in patients with AS. These findings support the concept of risk stratification to identify patients who may benefit from elective surgery. A structured HVC programme results in the detection of symptoms at an earlier and less severe stage and thus in an optimized timing of surgery.

Natural history of very severe aortic stenosis.

BACKGROUND: We sought to assess the outcome of asymptomatic patients with very severe aortic stenosis. METHODS AND RESULTS: We prospectively followed 116 consecutive asymptomatic patients (57 women; age, 67 + or - 16 years) with very severe isolated aortic stenosis defined by a peak aortic jet velocity (AV-Vel) > or = 5.0 m/s (average AV-Vel, 5.37 + or - 0.35 m/s; valve area, 0.63 + or - 0.12 cm(2)). During a median follow-up of 41 months (interquartile range, 26 to 63 months), 96 events occurred (indication for aortic valve replacement, 90; cardiac deaths, 6). Event-free survival was 64%, 36%, 25%, 12%, and 3% at 1, 2, 3, 4, and 6 years, respectively. AV-Vel but not aortic valve area was shown to independently affect event-free survival. Patients with an AV-Vel > or = 5.5 m/s had an event-free survival of 44%, 25%, 11%, and 4% at 1, 2, 3, and 4 years, respectively, compared with 76%, 43%, 33%, and 17% for patients with an AV-Vel between 5.0 and 5.5 m/s (P<0.0001). Six cardiac deaths occurred in previously asymptomatic patients (sudden death, 1; congestive heart failure, 4; myocardial infarction, 1). Patients with an initial AV-Vel > or = 5.5 m/s had a higher likelihood (52%) of severe symptom onset (New York Heart Association or Canadian Cardiovascular Society class >II) than those with an AV-Vel between 5.0 and 5.5 m/s (27%; P=0.03). CONCLUSIONS: Despite being asymptomatic, patients with very severe aortic stenosis have a poor prognosis with a high event rate and a risk of rapid functional deterioration. Early elective valve replacement surgery should therefore be considered in these patients.