Alkaptonuria is a rare inherited tyrosine metabolism disorder, resulting in homogentisic acid deposition in the connective tissues. The condition is commonly referred to as ochronosis and manifests as skin pigmentation, degenerative arthropathy, and black urine. Among the rare complications of this disease is the involvement of the cardiovascular system. We report a case of a 63-year-old woman with alkaptonuric ochronosis who had already undergone three joint replacements. She was referred to our center for aortic valve replacement after accidentally discovering severe aortic valve stenosis in the preoperative assessment prior to her fourth joint replacement. Intraoperative findings included ochronosis of a severely calcified black-pigmented aortic valve and black pigmentation of the aortic intima. Histopathological analysis and elevated homogentisic acid levels in the patient’s urine confirmed the diagnosis of alkaptonuria. However, alkaptonuria was not diagnosed until aortic valve replacement despite the previous symptoms and signs. This report aims to outline the history, etiology, pathogenesis, clinical presentation, and treatment of aortic valve ochronosis in addition to presenting the case.

BACKGROUND:The relationship between preoperative cardiac conduction abnormalities (CCA) and long-term outcomes after transcatheter aortic valve-in-valve implantation (TAVI-VIV) remains unclear. The aim of the study was to evaluate the effects of preoperative CCA on mortality and morbidity after TAVI-VIV and to estimate the impact of new-onset CCA on postoperative outcomes. METHODS:Between 2011 and 2020, 201 patients with degenerated aortic bioprostheses were qualified for TAVI-VIV procedures in two German heart centers. Cases with previously implanted permanent rhythm-controlling devices were excluded (n = 53). A total of 148 subjects met the eligibility criteria and were divided into 2 study groups according to the presence of preexisting CCA (CCA (n = 84) and non-CCA (n = 64), respectively). Early and late mortality and morbidity were evaluated. Follow-up functional status was assessed according to New York Heart Association (NYHA) classification. RESULTS:There were no procedural deaths. TAVI-VIV related new-onset CCAs were observed in 35.8% patients. The 30-day permanent pacemaker implantation rate was 1.6% in non-CCA vs 9.5% in CCA group (p = 0.045). Preexisting right bundle-branch block (OR:5.01; 95%CI, 1.05-23.84) and first-degree atrioventricular block (OR:4.55; 95%CI, 1.10-18.73) were independent predictors of new pacemaker implantation. One-year and five-year probability of survival were comparable in CCA and non-CCA groups: 90.3% vs 91.8% and 68.2% vs 74.3%, respectively. Surviving patients with preexisting and new-onset CCA had a worse functional status according to NYHA classification at follow-up. CONCLUSION:Preexisting and new-onset postoperative CCAs did not affect early and late mortality after TAVI-VIV procedures, however, they may have a negative impact on late functional status.

Background: Surgical reoperation is still a standard procedure performed for degenerated aortic bioprostheses. On the other hand femoral minimally invasive valve-in-valve implantation (femTAVI-VIV) is an intriguing alternative. This clinical study was design to compare the early and late outcomes of redo-surgery (Redo-AVR) and femTAVI-VIV procedures for failed aortic bioprostheses. Methods: We retrospectively reviewed 108 patients with degenerated aortic bioprostheses qualified for isolated Redo-AVR (n = 40) or femTAVI-VIV (n = 68) between 2003 and 2018. Both cohorts were divided into intermediate and high-risk groups according to the EuroSCORE II (4-9% and >9%). Propensity score matching selected 20 pairs in Intermediate-risk group and 10 pairs in High-risk group for the final comparison. Results: Patients qualified for femTAVI-VIV were older (79.2 vs 72.9 years, p < 0.001) and at higher risk (EuroSCORE II 10.9 vs 7.8%, p = 0.005) than Redo-AVR subjects. Overall survival in femTAVI-VIV and Redo-AVR was comparable at 30-days, 1- and 5-years, respectively (92.6% vs 92.5%, 85.2% vs 85.0% and 62.9% vs 72.5%, p = 0.287). After PSM no differences in mortality, myocardial infarction, pacemaker implantation, stroke or acute renal insufficiency were found. Transcatheter procedure was associated with shorter hospital stay, lower rate of blood products transfusions and higher incidence of mild paravalvular leaks. Conclusion: Our study supports the opinion that transcatheter approach for treatment of patients with degenerated aortic bioprostheses is a safe alternative to Redo-AVR procedures particularly for those at high-risk. (c) 2020 Elsevier B.V. All rights reserved.

Aim:To evaluate the impact of preinterventional moderate-to-severe functional tricuspid regurgitation (FTR) on early outcome after percutaneous edge-to-edge mitral valve repair (pMVR) with MitraClip procedures for functional mitral regurgitation (FMR) in patients with heart failure with reduced ejection fraction (HFrEF).Methods and results:From January 2013 to December 2017, 80 patients with HFrEF (ejection fraction 22%±5.3%) and FMR (grade 3.0±0.36) underwent successful pMVR. The 3-year actuarial survival was 58%. However, 73% (n=22) of non-survivors died of cardiac failure within 1 year. Patients were categorised into none-to-mild (n=36) and moderate-to-severe (n=44) postinterventional FTR groups according to pre-MitraClip tricuspid regurgitation grade. Cox regression analysis on 1-year survival demonstrated an impact of FTR severity (HR=1.8, 95% CI 1.01% to 3.09%, p=0.047), preoperative New York Heart Association class (HR=2.8, 95% CI 1.2% to 6.5%, p=0.015) and peripheral artery disease (HR=5.4, 95% CI 1.6 to 18, p=0.0054). Kaplan-Meier analysis showed that 1-year cardiac death was higher in the moderate-to-severe FTR group (p=0.048). In our study, 77% of pre-MitraClip moderate-to-severe FTR cannot be significantly reduced. Post-MitraClip moderate-to-severe FTR grade was related to lower survival (p<0.001).Conclusion:In patients with HFrEF treated with MitraClip for FMR, moderate-to-severe FTR was an independent predictor of cardiac death within 1 year. To improve survival, additional therapy to residual FTR should be considered in early phase after MitraClip therapy.

Abstract Aims The aim of this study was to compare the outcomes of surgical mitral valve repair or replacement (sMVR) and percutaneous edge‐to‐edge repair using MitraClip (pMVR) in patients with severe left ventricular dysfunction affected by functional mitral regurgitation (FMR). Methods and results We retrospectively identified 132 patients with left ventricular ejection fraction (LVEF) ≦ 30% submitted to sMVR (n = 47) or pMVR (n = 85) for FMR at our centre from January 2013 to December 2017. To adjust for baseline imbalances, we used a propensity score matching by age, logistic EuroSCORE, and left ventricular end‐systolic volume. After being matched, MitraClip therapy showed lower perioperative mortality and rate of complications yet increased residual mitral regurgitation (MR) grade than did surgery (0.2 ± 0.50 in sMVR vs. 1.3 ± 0.88 in pMVR, P < 0.0001). According to stratified multivariate Cox model analysis, residual MR severity was an independent risk factor for cardiac death [hazard ratio (HR), 2.81; 95% confidence interval [CI], 1.44–5.48, P = 0.0025] and re‐hospitalization for heart failure (HR, 3.07; 95% CI, 1.50–6.29, P = 0.0022) at 1 year follow‐up. Stratified multivariable Cox regression analysis at 3 years identified pMVR as risk factor for cardiac death (HR, 0.19; 95% CI, 0.040–0.86, P = 0.031) and re‐hospitalization for heart failure (HR, 0.28; 95% CI, 0.077–0.99, P = 0.048). Conclusions In patients with FMR and LVEF ≤ 30%, MitraClip therapy resulted in lower perioperative complications and mortality than sMVR. However, surgically treated patients who survived the perioperative stage had less residual MR and experienced lower rates of re‐hospitalization for heart failure at 1 year and lower cardiac mortality at 1 and 3 years of follow‐up than did patients undergoing pMVR.

Optimal treatment for residual mitral regurgitation (MR) after MitraClip failure is not clearly defined. We report our clinical experience and discuss treatment options. Between January 2013 and January 2018, 37 patients (75 ± 8.9 years, 46% male) were admitted for symptomatic MR (grade 3.1 ± 0.47) diagnosed after previous MitraClip therapy. Clinical outcome of these patients, who underwent medical therapy alone (n = 8, M-group), repeat MitraClip therapy (n = 8, reMC group), or mitral valve surgery (n = 21, S-group) for residual MR, were retrospectively analyzed. Thirty-day survival was 88% (M-group), 100% (reMC-group), and 76% (S-group). The rate of discharge to home was 88% in the reMC-group, better than 38% in the M-group (p = 0.051) and 19% in the S-group (p < 0.001). Perioperative non-survivors in the S-group had high surgical risk with median logistic EuroSCORE of 64.6% (interquartile range 57.4%–87.0%); all died from low cardiac output syndrome or multiple organ failure. The main MR pathologies resulted from leaflet tear and tethering in the M-group, tethering in the reMC-group, and degenerative valve and leaflet tear in the S-group. Kaplan–Meier analysis of overall survival at 1 year showed better outcome for patients in the reMC-group (50%, 95% CI 15.2–77.5%) and S-group (47.6%, 95% CI 25.7–66.7%), as compared to those in the M-group (12.5%, 95% CI 0.70–42.3%) (log-rank test p = 0.108 and p = 0.167, respectively). Medical therapy alone after failed MitraClip therapy resulted in poor 1-year prognosis. In patients without extremely high surgical risk, repeat MitraClip therapy, or surgical revision MIGHT BE CONSIDERED depending on valve pathology and cardiac comorbidities

Background: Patients with left ventricular ejection fraction (LVEF) ≤ 35% are at a high risk of sudden cardiac death (SCD) and benefit from implantable cardioverter-defibrillator (ICD) therapy. ICD implantation is not indicated during the first 40 days after acute myocardial infarction and Methods: This is a retrospective study conducted in the Heart Center in Cottbus. From 02.2015 through 02.2018 26 WCD patients were retrospectively analyzed and followed-up. Patient demographics, defibrillation treatments, and daily wear times were retrospectively obtained from our clinical database and LifeVest network. The patients were questioned about actual NYHA grade and implanted ICD at the end of follow-up. Results: Twenty-five patients (mean age 65, 22 men, 3 women) were treated with a WCD in response to heart failure (mean EF = 24%) after cardiac surgery (21 CABG, 1 AVR, 1 AVR + CABG, 1 AVR + MVR, MVR + CABG). Average daily use of a WCD was 22.1 (SD ± 2.7) hours which were worn for 85 days (SD ± 35). At that time 11.96 (SD ± 15) events were detected but not treated, 1 defibrillation performed and no asystole seen. At the end of follow-up (12 months, SD ± 9) 20 patients were questioned. All of the patients were alive and 5 (25%) of them were with implanted ICD. 10 (50%) patients were in NYHA grade I, 3 (12%) in NYHA grade II, 3 (12%) between grade II-III, 2 (8%) in grade III and 2 (8%) patients in NYHA grade IV. Conclusions: A WCD is an effective therapy for prevention of sudden cardiac death during the recovery period of heart function after cardiac surgery. This is treatment with high patient compliance.

OBJECTIVES Although percutaneous edge-to-edge mitral valve repair with the MitraClip system is becoming widely adopted in clinical practice, surgical experience on how to correct failed MitraClip therapy is limited. We aimed to analyse the surgical and pathological outcomes after surgical revision of the failed MitraClip therapy. METHODS Between January 2011 and January 2018, 25 patients (age 73 ± 9 years; men 48%; New York Heart Association class 3.4 ± 0.49) were admitted for severe mitral regurgitation at a median of 54 days (range 1-1496 days) after MitraClip edge-to-edge repair. Perioperative variables were analysed for their association with surgical outcomes. RESULTS All patients underwent explantation of the MitraClip system and subsequent mitral valve replacement. Perioperative mortality was as high as 28%, mainly due to pre-existing cardiogenic or septic shock. The Kaplan-Meier analysis revealed a 53% overall 1-year survival. Among preoperative variables, the logistic European System for Cardiac Operative Risk Evaluation score, left ventricular ejection fraction and liver dysfunction had a significant influence on in-hospital survival. Intraoperatively, the predominant pathology included mitral valve leaflet damage due to tear, degeneration or infection. Although leaflet tears or MitraClip detachment mainly occurred within the first 6 months after MitraClip therapy, leaflet infections and degeneration mainly occurred later during follow-up. CONCLUSIONS The surgical revision of failed MitraClip therapy is feasible but has high perioperative mortality, especially among patients with cardiogenic shock, septic shock or liver failure. Mitral regurgitation after the MitraClip therapy is mainly caused by mitral valve leaflet damage due to tear, degeneration or infection, all related to the MitraClip itself.

A device-related thrombus is a very common complication after the implantation of a Watchman device. Although anticoagulation therapy is the standard first choice therapy for a device-related thrombus, complications related to bleeding or existence of non-responders to the anticoagulation therapy are reported. Therefore, the possibility of another treatment, including surgical repair, needs to be discussed in the population unsuccessfully treated with anticoagulation therapy. We performed device-related thrombus removal and covered the orifice of the left atrial appendage with an autologous pericardial patch in 2 cases involving non-responders to anticoagulation therapy. Herein, we describe the technique and results of our experience with these 2 patients. The covering technique with an autologous pericardial patch is simple but highly efficient in preventing recurrent thrombus formation.

Device dislocation associated with left atrial appendage closure with a Watchman device is rare. Few cases of surgical treatment have been reported. Therefore, surgical options and the effects of the surgery on subsequent operations are unknown. This device was implanted in 2 patients in another hospital because of frequent brain and intestinal bleeding related to coagulation therapy for atrial fibrillation. However, routine echocardiography revealed dislocation from the left atrial appendage orifice in the early and late postoperative periods. Emergency surgical removal of the devices was performed. Perioperative device management and treatment strategies are discussed briefly.

Objectives: Recurrent mitral regurgitation (MR) post repair of mitral valve Type 1 or 2 disease is a dilemma. Re-do surgical valve replacement versus bail-out fixation of the leaflets are options, but they do not target the underlying mechanism of recurrent MR, that is, continued annular dilation and leaflet tethering. The Encor Dynamic Annuloplasty Ring allows for an adjustment of the ring via shape change, and we report safety and feasibility of the 1st generation device.

a giant aneurysm of the left main coronary artery, partially obstructing the left anterior descending and circumflex arteries (Fig. 1A).The aneurysm was reconstructed by exclusion of excessive cavity, leaving a 5 mm tunnel (Fig. 1B andC) that was found patent 8 months post-surgery (Fig. 1D).

Purpose: The purpose of this study was to investigate whether: 1. sound phenomena may be used to detect prosthetic valve dysfunction; 2. clinical and experimental data permit conclusions about alterations in the functional state of mechanical valves; 3. patients can record and pass on signals via Internet. Methods: 1. We implanted bi-leaflet valves in pigs. By gradually influencing the motion of the tilting discs prosthetic dysfunction could be generated. 2. Thrombosis and lysis of bi-leaflet valves was studied in sheep. This process was documented using echocardiography and acoustically by the Fast Fourier Transformation. 3. Thirty devices were set up and handed out to patients following mechanical valve replacement. All patients regularly sent data to the hospital via Internet, regardless of their location at the time. The data were evaluated by comparing them with the reference file. Results: Animal experiments proved that changes in prosthetic function led to a significant change in sound phenomena. In contrast to echocardiography alterations at an early stage (onset of thrombosis) could be reliably verified. The sensitivity was greater than in echo-control analysis. All patients regularly recorded and passed on their signals. Surveys revealed high acceptance and easy handling of the devices. Conclusions: Online registration of sound phenomena seems to be suitable for the detection of changes in prosthetic function. This led to the development of the first hand-held device for home monitoring of valve function. Registration of flow, frequency spectrum, and ECG envisaged at the next level opens up potential applications for Internet-based, remote monitoring of cardiac patients.

Patienten mit einem alloprothetischem Herzklappenersatz haben trotz erheblicher Fortschritte hinsichtlich des Materials und des Designs der Prothesen noch immer ein Risiko eine ernste Komplikation (Blutung, Thrombembolie, Re-Operation) zu erleiden, welches sich in den ersten 10 postoperativen Jahren je nach Klappentyp und Klappenposition auf 5–20% summiert. Dabei sind technische Defekte an mechanischen Herzklappen heute zu vernachlässigen. Vielmehr sind relevante Komplikationen im Langzeitverlauf häufig mit der korrekten Antikoagulation verbunden. Um diese zu vermeiden, erachten wir eine möglichst engmaschige Kontrolle sowohl des Gerinnungsstatus als auch der funktionellen Integrität der Herzklappenprothese als unabdingbar. Dabei kommt es wesentlich darauf an, den Patienten selbst in die Lage zu versetzen, mittels einfacher und beliebig oft in seiner alltäglichen Umwelt durchzuführender Methoden an der Risikominimierung mitzuwirken. Wir stellen eine zuverlässige Methode vor, mit der vom Patienten ohne größeren Aufwand beliebig häufig die funktionelle Integrität seiner Kunstklappe überprüft werden kann.

Despite enormous progress concerning material and design, patients with artificial heart valves still face a considerable risk of serious complications (e. g. hemorrhage, thromboemboli, redos) which sum up to 5-20% withinthe first 10 postoperative years depending on type and position of the implanted prosthetic valve. Nowadays technical defects of mechanical valves are negligible and relevant complications are most frequently the consequence of inappropriate oral anticoagulation therapy. To avoid these complications a strict control of both the anticoagulation therapy and the functional integrity of the prosthetic valve is mandatory. Therefore with the aim of risk minimization the patient should be enabled to cooperate by means of methods which are easy to handle in his everyday environment. We present a reliable method for early detection of prosthetic valve dysfunction which can be applied daily with minimal effort by the patient himself.

Patients with mild pulmonary stenosis after infancy rarely have symptoms or develop increasing obstruction. We experienced a 68-year-old woman with severe pulmonary valvar and infundibular stenosis (peak to peak pressure gradient = 80 mmHg). She had been pointed out heart disease at the age of six. Endocarditis at the age of 17 might induce calcification of valve and affect the progression of pulmonary stenosis, and moreover, which might gradually develop severe subvalvar obstruction and poststenotic aneurysm of pulmonary trunk. She refused operative intervention because of mild clinical symptoms (NYHA class II), but we recommended surgical repair due to repeated transient ischemic attacks, which were suspected paradoxical embolism through persistent foramen ovale. She underwent pulmonary valvotomy and infundibular resection and is doing well.