Huge aneurysm of the ascending aorta in a patient with adult-type Pompe's disease: histological findings mimicking fibrillinopathy

Adult-type Pompe's disease (glycogen storage disease type II) has rarely been shown to present with dilatative arteriopathy, suggesting potential smooth muscle involvement in addition to lysosomal glycogen deposits usually restricted to skeletal muscle tissue. We report the case of a middle-aged man under enzyme replacement therapy presenting with an exceedingly large thoracic aortic aneurysm. Surprisingly, the histological work-up of resected aortic tissue revealed changes mimicking those observed in patients with classic connective tissue diseases. Enzyme replacement therapy, in addition to musculoskeletal and pulmonary treatment for patients with Pompe's disease, may prolong survival and lead to patients presenting with vascular alterations that may pose surgical and potential diagnostic challenges in the future.

Transapical valve-in-valve implantation for regurgitant stented aortic bioprostheses

Transcatheter aortic valve implantation (TAVI) is rapidly evolving as an alternative treatment option for elderly patients with severe symptomatic aortic stenosis and excessive risk for surgical intervention. Transcatheter valve-in-valve implantation is an alternative approach to redo-surgery for patients with degeneration of a bioprosthetic valve. Herein are reported three cases of successful transcatheter aortic valve-in-valve implantation for severely regurgitant bioprosthetic valves with a clinical follow up of more than 12 months.

Impact of permanent pacemaker implantation on clinical outcome among patients undergoing transcatheter aortic valve implantation

This study sought to assess the impact of permanent pacemaker (PPM) implantation on clinical outcomes among patients undergoing transfemoral transcatheter aortic valve implantation (TAVI).

Atrioventricular conduction after transcatheter aortic valve implantation and surgical aortic valve replacement

Atrioventricular conduction abnormalities (AVCA) may complicate transcatheter aortic valve implantation (TAVI) and surgical aortic valve replacement (SAVR). The aim of this study was to prospectively evaluate AVCA after TAVI and SAVR.

Quality of life in high-risk patients: comparison of transcatheter aortic valve implantation with surgical aortic valve replacement

OBJECTIVES To compare health-related quality of life (QoL) in patients undergoing transcatheter aortic valve implantation via transapical access (TA TAVI) with patients undergoing surgical aortic valve replacement (SAVR). METHODS One hundred and forty-four high-risk patients referred for aortic valve replacement underwent TAVI screening and were assigned to either TA TAVI (n = 51, age 79.7 ± 9.2 years, logistic EuroSCORE 26.5 ± 16.1%, 51% males) or SAVR (n = 93, age 81.1 ± 5.3 years, logistic EuroSCORE 12.1 ± 9.3%, 42% males) by the interdisciplinary heart team. QoL was assessed using the Short Form 36 (SF-36) Health Survey Questionnaire and the Hospital Anxiety and Depression Scale. Furthermore, current living conditions and the degree of independence at home were evaluated. RESULTS Patients undergoing TA TAVI were at higher risk as assessed by EuroSCORE (26.5 ± 16 vs. 12.1 ± 9, P < 0.001) and STS score (6.7 ± 4 vs. 4.4 ± 3, P < 0.001) compared with SAVR patients. At the 30-day follow-up, the rate of mortality was similar and amounted to 7.8% for TA TAVI and 7.5% for SAVR patients and raised to 25.5% in TA TAVI and 18.3% in SAVR patients after a follow-up period of 15 ± 10 months. Assessment of QoL revealed no differences in terms of anxiety and depression between TA TAVI and SAVR patients. The SF-36 mental health metascore was similar in both groups (65.6 ± 19 vs. 68.8 ± 22, P = 0.29), while a significant difference was observed in the physical health metascore (49.7 ± 21 vs. 62.0 ± 21, P = 0.015). After adjustment for baseline characteristics, this difference disappeared. However, every added point in the preoperative risk assessment with the STS score decreased the SF-36 physical health dimension by two raw points at the follow-up assessment. CONCLUSIONS Selected high-risk patients undergoing TAVI by using a transapical access achieve similar clinical outcomes and QoL compared with patients undergoing SAVR. Increased STS scores predict worse QoL outcomes.

Aortic root dimensions among patients with severe aortic stenosis undergoing transcatheter aortic valve replacement

The aim of this study was to characterize aortic root dimensions of patients with aortic valve stenosis undergoing transcatheter aortic valve replacement (TAVR) and to evaluate sex differences.

Valve disease in chronic venous disorders: a quantitative ultrastructural analysis by transmission electron microscopy and stereology

INTRODUCTION: The ultrastructure of venous valves and walls in chronic venous disease was investigated. METHODS: Consecutive patients were categorised into one of three groups (group A: patients with C1 venous disease in accordance with CEAP (Clinical severity, Etiology, Anatomy, Pathophysiology); group B: C2 and C3; group C: C4, C5 and C6). The terminal or preterminal valve and adjacent vessel wall was harvested from the great saphenous vein. Sections were examined with a transmission electron microscope. The volumes of elastin and of collagen per unit surface area of valve were assessed, as well as the surface endothelium of valve and vessel wall. RESULTS: The study population consisted of 17 patients. The elastin ratio was analysed by means of stereology. Mean values were: in group A, 0.45 μm3/m2; in group B, 0.67 μm3/m2; in group C, 0.97 μm3/m2. The ratio was similar for collagen (A, 15.7 μm3/m2; B, 26.8 μm3/m2; C, 30.1 μm3/m2). Surface analysis of the valve endothelium and the adjacent vessel wall endothelium showed a trend towards increasing damage with more severe disease. CONCLUSIONS: With progression of venous disease, the valve elastin content, assessed morphologically, seems to increase, and the endothelium of the venous valve and the vein wall tend to show more damage.

Influence of temperature management on neurocognitive function in biological aortic valve replacement. A prospective randomized trial

Aim of this study was to elucidate if postoperative neurocognitive function after biological aortic valve replacement (AVR) can be influenced by temperature management during cardiopulmonary bypass (CPB).

Costs of transcatheter versus surgical aortic valve replacement in intermediate-risk patients

Transcatheter aortic valve replacement (TAVR) offers a new treatment option for patients with aortic stenosis, but costs may play a decisive role in decision making. Current studies are evaluating TAVR in an intermediate-risk population. We assessed the in-hospital and 1-year follow-up costs of patients undergoing TAVR and surgical aortic valve replacement (SAVR) at intermediate operative risk and identified important cost components.

Increased risk of acute kidney injury in patients undergoing tricuspid valve surgery

We aimed to determine which patients undergoing tricuspid valve (TV) surgery are at increased risk for acute kidney injury (AKI).

Experience with a gravitational valve in the management of symptomatic overdrainage in children with shunts

Symptomatic overdrainage in children with shunt-treated hydrocephalus represents one of the more difficult shunt-related diseases and may require repeated surgery. Gravity-assisted valve design has become a standard device to avoid overdrainage in many European pediatric hydrocephalus centers. However, the use of a gravitational valve for relieving symptoms associated with overdrainage has not yet been addressed. The goal of this study was to evaluate the effectiveness of a gravitational valve in the treatment of symptomatic overdrainage in children with shunts.

Clinical outcomes of patients with estimated low or intermediate surgical risk undergoing transcatheter aortic valve implantation

AimsTranscatheter aortic valve implantation (TAVI) is an established treatment alternative to surgical aortic valve replacement in high-risk and inoperable patients and outcomes among patients with estimated low or intermediate risk remain to be determined. The aim of this study was to assess clinical outcomes among patients with estimated low or intermediate surgical risk undergoing TAVI.Methods and resultsBetween August 2007 and October 2011, 389 consecutive patients underwent TAVI and were categorized according to the Society of Thoracic Surgeons (STS) score into low (STS < 3%; n = 41, 10.5%), intermediate (STS ≥3% and ≤8%, n = 254, 65.3%), and high-risk (STS > 8%; n = 94, 24.2%) groups for the purpose of this study. Significant differences were found between the groups (low risk vs. intermediate risk vs. high risk) for age (78.2 ± 6.7 vs. 82.7 ± 5.7 vs. 83.7 ± 4.9, P < 0.001), body mass index (28.1 ± 6.1 vs. 26.5 ± 4.9 vs. 24.4 ± 4.6, P < 0.001), chronic renal failure (34 vs. 67 vs. 90%, P < 0.001), all-cause mortality at 30 days (2.4 vs. 3.9 vs. 14.9%, P = 0.001), and all-cause mortality at 1 year (10.1 vs. 16.1 vs. 34.5%, P = 0.0003). No differences were observed with regards to cerebrovascular accidents and myocardial infarction during 1-year follow-up.ConclusionIn contemporary practice, TAVI is not limited to inoperable or STS-defined high-risk patients and should be guided by the decision of an interdisciplinary Heart Team. Compared with patients at calculated high risk, well-selected patients with STS-defined intermediate or low risk appear to have favourable clinical outcomes.

Atrial fibrillation and aortic stenosis: impact on clinical outcomes among patients undergoing transcatheter aortic valve implantation

Atrial fibrillation (AF) is an important risk factor for stroke and is common among elderly patients undergoing transcatheter aortic valve implantation. The aim of this study was to assess the impact of AF on clinical outcomes among patients undergoing transcatheter aortic valve implantation.