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Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease that is characterized by left ventricular hypertrophy unexplained by secondary causes. Based on international epidemiological data, around 20,000-40,000 patients are expected to be affected in Austria. Due to the wide variety of clinical and morphological manifestations the diagnosis can be difficult and the disease therefore often goes unrecognized. HCM is associated with a substantial reduction in quality of life and can lead to sudden cardiac death, especially in younger patients. Early and correct diagnosis, including genetic testing, is essential for comprehensive counselling of patients and their families and for effective treatment. The latter is especially true as an effective treatment of outflow tract obstruction has recently become available in the form of a first in class cardiac myosin ATPase inhibitor, as a noninvasive alternative to established septal reduction therapies. The aim of this Austrian consensus statement is to summarize the recommendations of international guidelines with respect to the genetic background, pathophysiology, diagnostics and management in the context of the Austrian healthcare system and resources, and to present them in easy to understand algorithms., (© 2024. The Author(s).)

Background: The Good Practice Guidelines for health information, Good Health Information Austria and Guideline Evidence-based Health Information are established resources for developing evidence-based health information in the German-speaking regions. The aim of this project was to capture challenges in applying these standards in practice, identify gaps and development needs and gain insights for their further development., Methods: In December 2020, members of the working group for patient information and involvement of the Network for Evidence-based Medicine were invited to share their experiences and needs in applying German standards for evidence-based health information through an online survey focussing on open questions (part 1: needs assessment). The feedback was analysed using qualitative content analysis and presented in a workshop at the EbM Congress 2021 with the goal of specifying the feedback from the needs assessment and discussing ideas for the further development of the standards (part 2: specification). In the final step, a second survey was conducted in February 2023 to prioritize the identified topics by the working group members (part 3: prioritization). The results were analysed descriptively., Results: Among the 41 participants, only 23% considered the standards to be sufficient, and only 55% found their application in the development of information to be easy or rather easy. The needs assessment and workshop (n=46) helped to identify various areas of action. With regard to the application of the standards, the following challenges were identified: lack of user orientation, content gaps, methods and risk communication. Gaps in the standards were identified regarding formats, content, and the connection to healthcare provision. For the advancement of the standards ideas for additional content, stakeholder involvement, and improvement of the usability of the standards were identified. In the prioritization survey, the topic areas, "presenting benefits and harms" and "content beyond treatments (in particular, diagnostics and prognosis)" were considered to be the most important (n=36)., Discussion: Among members of the working group for patient information and involvement of the German Network for Evidence-based Medicine, a high demand has been identified for the further development of standards for creating evidence-based health information. In addition to content development, the integration of existing documents and tools should also be considered, including products issued by other institutions. The success of advancing the standards also depends on improving their applicability - for example through an attractive online platform. The results are limited by the sample which only included members of the EbM Network's patient information and participation working group and a limited response rate., Conclusions: The needs assessment showed that the currently established standards and recommendations for the development of evidence-based health information in the German-speaking regions represent important cornerstones but need to be expanded to answer more practice-oriented questions. The challenges and proposed solutions stated by the participants can help further develop the standards. The prioritization can be used to set priorities for the development of the standards, guide the order of possible work packages and allocate resources., (Copyright © 2023. Published by Elsevier GmbH.)

This position statement is an update to the 2011 consensus statement of the Austrian Society of Cardiology (ÖKG) and the Austrian Society of Cardiac Surgery (ÖGTHG) for transfemoral transcatheter aortic valve implantation.Due to a number of recently published studies, broadening of indications and recommendations of medical societies and our own national developments, the ÖKG and the ÖGHTG wish to combine the 2017 ESC/EACTS guidelines for the management of valvular heart disease with a national position paper and to focus on certain details for the application in Austria. Thus, this position statement serves as a supplement and further interpretation of the international guidelines., (© 2021. Springer-Verlag GmbH, AT part of Springer Nature.)

Background: Coronary artery diseases (CAD) remains a severe socio-economic burden in the Western world. Coronary obstruction and subsequent myocardial ischemia result in progressive replacement of contractile myocardium with dysfunctional, fibrotic scar tissue. Post-infarctional remodeling is causal for the concomitant decline of left-ventricular function and the fatal syndrome of heart failure. Available neurohumoral treatment strategies aim at the improvement of symptoms. Despite extensive research, therapeutic options for myocardial regeneration, including (stem)-cell therapy, gene therapy, cellular reprogramming or tissue engineering, remain purely experimental. Thus, there is an urgent clinical need for novel treatment options for inducing myocardial regeneration and improving left-ventricular function in ischemic cardiomyopathy. Shockwave Therapy (SWT) is a well-established regenerative tool that is effective for the treatment of chronic tendonitis, long-bone non-union and wound-healing disorders. In preclinical trials, SWT regenerated ischemic myocardium via the induction of angiogenesis and the reduction of fibrotic scar tissue, resulting in improved left-ventricular function., Methods/design: In this prospective, randomized controlled, single-blind, monocentric study, 80 patients with reduced left-ventricular ejection fraction (LVEF≤ 40%) are subjected to coronary-artery bypass-graft surgery (CABG) surgery and randomized in a 1:1 ratio to receive additional cardiac SWT (intervention group; 40 patients) or CABG surgery with sham treatment (control group; 40 patients). This study aims to evaluate (1) the safety and (2) the efficacy of cardiac SWT as adjunctive treatment during CABG surgery for the regeneration of ischemic myocardium. The primary endpoints of the study represent (1) major cardiac events and (2) changes in left-ventricular function 12 months after treatment. Secondary endpoints include 6-min Walk Test distance, improvement of symptoms and assessment of quality of life., Discussion: This study aims to investigate the safety and efficacy of cardiac SWT during CABG surgery for myocardial regeneration. The induction of angiogenesis, decrease of fibrotic scar tissue formation and, thus, improvement of left-ventricular function could lead to improved quality of life and prognosis for patients with ischemic heart failure. Thus, it could become the first clinically available treatment strategy for the regeneration of ischemic myocardium alleviating the socio-economic burden of heart failure., Trial Registration: ClinicalTrials.gov, ID: NCT03859466. Registered on 1 March 2019.

Objectives: Increasing experience with minimally invasive cardiac (MIC) aortic valve (AV) replacement makes AV reoperations (rAVR) an appealing alternative to conventional redo surgery. The aim of the study was to compare the perioperative outcome after isolated MIC versus full-sternotomy (FS) rAVR., Methods: We retrospectively analyzed data of 116 patients from three centers who underwent rAVR by using a FS ( n  = 70, 60.3%) or a partial upper sternotomy approach ( n  = 46, 39.7%). Both groups were compared in terms of 30-day mortality by using binary-logistic regression models. Further the EuroSCORE II was used to adjust for preoperative conditions in a multivariable model. Perioperative times and complications were compared between the two groups., Results: There was no statistically significant difference in perioperative mortality between FS ( n  = 5, 7.1%) and MIC ( n  = 1, 2.2%) rAVR in the original population (odds ratio [OR] 3.462, 95% confidence interval [CI] 0.391-30,635, p  = 0.264) and after adjusting for EuroSCORE II (OR 2.759, 95% CI 0.298-25.567, p  = 0.372). Cardiopulmonary bypass- (115.5 minutes vs. 137.5 minutes, p  = 0.070) and cross-clamp times (69.0 minutes vs. 81.0 minutes, p  = 0.028) were reduced in the MIC group. There was a lower prevalence of postoperative renal failure requiring renal replacement therapy (RRT) in the MIC group 0 and 8.6% ( p  = 0.041), respectively. No differences were detected between the groups regarding postoperative complications., Conclusion: MIC rAVR is associated with reduced cardiopulmonary and cross-clamp times as well as the need for RRT as compared with FS. MIC-rAVR seems to be a viable option in surgical candidates for AV reoperations., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Natriuretic peptide plasma levels help to manage patients with severe aortic stenosis (AS). The role of troponin plasma levels in this patient cohort remains speculative. A consortium of 4 university hospital centers in Austria analyzed retrospectively 3,595 patients admitted for valve replacement because of severe AS since 2007. The aim was to compare the additive preprocedural value of high-sensitivity troponin T (hsTnT) to N-terminal pro brain natriuretic peptide (NT-proBNP) plasma levels in predicting postoperative long-term survival in a large cohort undergoing either surgical (57.8%) or transcatheter (42.2%) aortic valve replacement. During a median follow-up of 2.93 (1.91 to 4.92) years, 919 patients (25.6%) died, in them 556 (15.5%) due to cardiovascular causes. Both normal hsTnT (<14 ng/l) and NT-proBNP (within age- and sex-corrected normal range) plasma levels were found in 481 patients (14.3%, group 1). Normal hsTnT but elevated NT-proBNP plasma levels were found in 748 patients (22.3%, group 2). Elevated hsTnT but normal NT-proBNP plasma levels were found in 258 patients (7.7%, group 3). Both elevated hsTnT and elevated NT-proBNP plasma levels were found in 1,869 patients (55.7%, group 4). Using Log Rank tests for comparison there was a highly significant difference in both cardiovascular mortality (p <0.0001) and all-cause mortality (p <0.0001). All-cause mortality rates after 1, 3, and 5 years were 2.1%, 5.4%, 7.7% in group 1; 4.0%, 7.5%, 11.5% in group 2; 5.8%, 8.9%, 14.0% in group 3; and 12.3%, 22.6%, 28.4% in group 4. In conclusion, hsTnT adds additional impact to NT-proBNP as a routinely available biomarker for risk stratification concerning postoperative survival in patients with severe AS admitted for valve replacement. The present study supports the concept to integrate hsTnT plasma levels in the management of severe AS., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objective: The impact of paravalvular aortic regurgitation (PAR) on hemodynamic performance after transcatheter aortic valve implantation (TAVI) remains disputable. Common parameters such as the diastolic blood pressure or the blood pressure amplitude do not provide reproducible results. The aim of our study was to evaluate the impact of PAR on hemodynamics and outcome using the relative amplitude index (RAI)., Methods: PAR was prospectively evaluated by echocardiography before discharge in 110 patients. The RAI was calculated according to the formula: RAI = [(Post-TAVI BP amplitude)/(Post-TAVI SBP) - (Pre-TAVI BP amplitude)/(Pre-TAVI SBP)] × 100%, where BP is blood pressure and SBP is systolic blood pressure. Correlations of increased RAI with perioperative outcome were investigated and factors influencing mortality were isolated., Results: The incidence of moderate and severe PAR after TAVI was 9% and 1%, respectively. Diastolic pressure or post-TAVI amplitude did not correlate to perioperative outcome. RAI increased from 2 when PAR was <2+ to 7 when PAR was ≥2+ (P = .006). A cut-off value of RAI ≥14 was associated with increased perioperative mortality (29 vs 5%; P = .013) and acute renal injury requiring dialysis (71 vs 18%; P = .001). RAI ≥14 was also associated with higher follow-up mortality at 1 year (57 vs 16%; P = .007). RAI ≥14 (odds ratio [OR], 3.390; 95% confidence interval [CI], 1.6-7.194; P = .00146), PAR ≥2+ (OR, 4.717; 95% CI, 1.828-12.195; P = .00135), and perioperative renal replacement therapy (OR, 12.820; 95% CI, 5.181-31.250; P = .00031) were found to be independent predictors of mortality at 1 year., Conclusions: The RAI is a useful tool to predict perioperative and 1-year outcome in patients with PAR after TAVI., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Patients and Methods: 140 patients with aortic dissection type A were admitted for cardiac surgery. Seventy-seven patients experienced their dissection in the winter season (from November to April). We analyzed cases of ascending aortic dissection associated with alpine skiing., Results: In 17 patients we found skiing-related aortic dissections. Skiers were taller (180 (172-200) cm versus 175 (157-191) cm, P = 0.008) and heavier (90 (68-125) kg versus 80 (45-110) kg, P = 0.002) than nonskiers. An extension of aortic dissection into the aortic arch, the descending thoracic aorta, and the abdominal aorta was found in 91%, 74%, and 69%, respectively, with no significant difference between skiers and nonskiers. Skiers experienced RCA ostium dissection requiring CABG in 17.6% while this was true for 5% of nonskiers (P = 0.086). Hospital mortality of skiers was 6% versus 13% in nonskiers (P = 0.399). The skiers live at an altitude of 170 (0-853) m.a.s.l. and experience their dissection at 1602 (1185-3105; P < 0.001) m.a.s.l. In 82% symptom start was during recreational skiing without any trauma., Conclusion: Skiing associated aortic dissection type A is usually nontraumatic. The persons affected live at low altitudes and practice an outdoor sport at unusual high altitude at cold temperatures. Postoperative outcome is good.

Objective: Pregnancy has been reported to be an independent risk factor for 50% of acute aortic dissections recorded in women younger than 45 years of age. The present epidemiologic study aimed to identify whether this putative association of pregnancy and acute type A dissection could be an artifact of selective reporting., Methods: This population-based study was conducted in the City of Vienna, Austria, Europe, in an average female population of 34,1381 women in the age range of 15-45 years who were followed up between 1994 and 2004 (total of 3755.195 person-years of observation). During this study, the incidence, management, and outcome of acute type A dissection were determined., Results: Fifteen patients (mean age: 38.8 years, SD: 4.8) with acute aortic dissection were identified, and an overall incidence of 0.4 case per 100000 person-years was estimated. The prehospital mortality rate was recorded to be 53%. Six patients, including two women in late pregnancy (incidence: 0.05 cases per 100000 person-years), were treated successfully by surgical repair during deep hypothermic circulatory arrest (in-hospital mortality rate: 6.6%). Pregnancy and aortic dissection were identified as events that were not related (RR: 3.27; 95% confidence interval (CI): 0.82-12.95; P=0.14). Observation during long-term follow-up was uneventful., Conclusions: Acute aortic dissection represents a rare pathology in women younger than 45 years of age; however, it is associated with a high rate of sudden death. Pregnancy may not be a risk factor for this life-threatening vascular emergency. Immediate referral to surgery, even during pregnancy, will result in a prognosis of favorable outcome., (Copyright © 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.)

Objective: This study evaluated long-term results of thoracic endovascular aortic repair for atherosclerotic aneurysms involving descending aorta., Methods: One hundred thirteen patients underwent thoracic endovascular aortic repair for this indication from 1996 to 2009. Mean follow-up was 54 ± 38 months (5-144 months). In-hospital mortality, neurologic injury, need for rerouting, occurrence of endoleaks and their treatment, and survival were recorded., Results: In-hospital mortality was 5.3%. Transient neurologic injury rate was 2.6%. Previous rerouting was performed in 51%. Assisted early and late type I and III endoleak rates were 7.9% and 5.7%, respectively. Five percent of patients required late surgical conversion. Actuarial survivals were 86%, 60%, and 42% at 1, 5, and 10 years, respectively. Aorta-related actuarial survivals were 94%, 90%, and 83% at 1, 5, and 10 years, respectively. Cox regression analysis revealed higher number of prostheses as independent risk factor for early (hazard ratio, 5.38; 95% confidence interval, 1.68-42.37) and late (hazard ratio, 8.49; 95% confidence interval, 1.09-66.06) endoleak formation. Female sex (hazard ratio, 0.35; 95% confidence interval, 0.13-0.99), no arch involvement (hazard ratio, 0.21; 95% confidence interval, 0.05-0.08), and higher number of prostheses (hazard ratio, 7.95; 95% confidence interval, 1.36-46.58) affected survival., Conclusions: Aorta-related survival is excellent among patients undergoing thoracic endovascular aortic repair for atherosclerotic aneurysms involving the descending aorta. Life-long surveillance remains mandatory, with early and late failure uncommon but still needing consideration. Thoracic endovascular aortic repair in this group of patients remains attractive and has now proven durability., (Copyright © 2010 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Since the beginning of the University of Vienna Cardiac Transplant Program in 1984, 1086 heart transplant procedures have been performed through the end of 2007. One- and five-year survival has increased steadily over time (82% and 76%). Ten-year survival is 65%. Over the past 10 years our program has seen dramatic changes in patient selection, accepting now patients with more risk factors (Age, diabetes, elevated pulmonary resistance,..). Developments in immunosuppression have decreased incidence of infection, rejection and graft arteriosclerosis continuously. Our program continues to pursue novel strategies to improve the survival and quality of life of our heart transplant patients.

The effect of successful ventricular assist device (VAD) implantation on neurocognitive function in terminal heart failure is uncertain. Additionally, the different impact of continuous versus pulsatile blood flow devices is unknown. A total of 29 patients (mean age 53 years), surviving implantation of a ventricular assist device as bridge to transplantation were prospectively followed (continuous flow: Micromed DeBakey, n = 11; pulsatile flow: Thoratec and Novacor, n = 18). Normative data were obtained in 40 age- and sex-matched healthy subjects (mean age 54 years). Neurocognitive function was objectively measured by means of cognitive P300 auditory evoked potentials before operation (baseline), at intensive care unit (ICU) discharge, and at the 8-week and 12-week follow-up. Before implantation of the VAD, cognitive P300 evoked potentials were impaired (prolonged) compared with age- and sex-matched healthy subjects (p < 0.001). After successful VAD implantation, P300 evoked potentials markedly improved compared with before operation (ICU discharge, p = 0.007; 8-week follow-up, p = 0.022; 12-week follow-up, p < 0.0001). Importantly, there was no difference between continuous and pulsatile VADs (before operation, p = 0.676; ICU discharge, p = 0.736; 8-week follow-up, p = 0.911 and 12-week follow-up, p = 0.397; respectively). Nevertheless, P300 peak latencies did not fully normalize at 12-week follow-up compared with healthy subjects (p = 0.012). Successful VAD implantation improves neurocognitive impairment in patients with terminal heart failure. Importantly, this effect is independent of the type of VAD (pulsatile vs. continuous blood flow).

Background: The purpose of this study was to investigate the impact of successful heart transplantation in patients with refractory heart failure receiving bridging therapy on sequential plasma levels of big endothelin, norepinephrine, atrial natriuretic peptide and aldosterone., Methods: Fourteen patients (2 women, 12 men) accepted for heart transplantation were studied. All had severe chronic heart failure refractory to optimized oral therapy with angiotensin-converting enzyme inhibitors and furosemide, were in New York Heart Association functional Class IV, and had a left ventricular ejection fraction of <15%, Right heart catheterization was performed in all patients (cardiac index 1.9 +/- 0.1 liters/min. m(2), pulmonary capillary wedge pressure 30 +/- 2 mmHg, systemic vascular resistance index 2,827 +/- 253 dyn. s/cm(5). m(2)). As bridging therapy, patients received either prostaglandin E(1), prostaglandin E(1) and dobutamine or dobutamine alone as a continuous infusion. Neurohumoral variables were measured prior to bridging therapy and 3.5 months before and 7 and 10 months after successful heart transplantation., Results: Big endothelin, norepinephrine and atrial natriuretic peptide plasma levels decreased from 7.4 +/- 2.9 fmol/ml, 1112 +/- 686 pg/ml and 366 +/- 312 pg/ml to 6.0 +/- 4.5 fmol/ml, 720 +/- 503 pg/ml and 198 +/- 160 pg/ml, respectively, after bridging therapy, and further to 2.1 +/- 0.9 fmol/ml (p < 0.00001 vs baseline), 527 +/- 31 pg/ml (p < 0.02 vs baseline) and 115 +/- 70 pg/ml (p < 0.03 vs baseline), respectively, after cardiac transplantation. Aldosterone plasma levels decreased from 242 +/- 220 pg/ml to 183 +/- 142 pg/ml during bridging therapy and increased after heart transplantation to 252 +/- 189 pg/ml. Plasma creatinine levels increased from 1.2 +/- 0.4 mg/dl at baseline to 1.4 +/- 0.2 mg/dl after transplantation (NS)., Conclusions: The study suggests that excessive overproduction of big endothelin, atrial natriuretic peptide and norepinephrine is predominantly related to pump failure and, after cardiac transplantation, a moderate spillover of big endothelin persists. Its specific origin, however, remains to be elucidated. Furthermore, our data suggest a protective effect of prostaglandin E(1) on kidney function after heart transplantation.