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BackgroundThe 2022 ESC/ERS guidelines on pulmonary hypertension recommend non-invasive risk assessments based on three non-invasive clinical variables during follow-up in patients with pulmonary arterial hypertension (PAH). We set out to test whether residual risk can be captured from routinely measured non-invasive clinical variables during follow-up in PAH.MethodsWe retrospectively studied 298 incident PAH patients from a German PH centre who underwent routine non-invasive follow-up assessments including exercise testing, echocardiography, electrocardiography, pulmonary function testing and biochemistry. To select variables, we used LASSO-regularized Cox regression models. Outcome was defined as mortality or lung transplant after first follow-up assessment.ResultsTwelve non-invasive variables that were associated with outcomes in a training sub-cohort (n=208) after correction for multiple testing entered LASSO modelling. A model combining 7 variables discriminated 1-year (area under the curve [AUC] 0.83, 95%-confidence interval [95%-CI] 0.68–0.99, p=8.4×10−6) and 3-year (AUC 0.81, 95%-CI 0.70–0.92, p=2.9×10−8) outcome status in a replication sub-cohort (n=90). The model's discriminatory ability was comparable to that of the guideline approach in the replication sub-cohort. From the individual model components, World Health Organisation functional class, 6-min walking distance and the tricuspid annular plane systolic excursion to systolic pulmonary arterial pressure (TAPSE/sPAP) ratio were sensitive to treatment initiation. Addition of TAPSE/sPAP to the guideline approach numerically increased its ability to discriminate outcome status.ConclusionOur real-world data suggests that residual risk can be captured by non-invasive clinical procedures during routine follow-up assessments in patients with PAH and highlights the potential use of echocardiographic imaging to refine risk assessment.

Background and Purpose: Cardiac ultrasound to identify sources of cardioembolism is part of the diagnostic workup of acute ischemic stroke. Recommendations on whether transesophageal echocardiography (TEE) should be performed in addition to transthoracic echocardiography (TTE) are controversial. We aimed to determine the incremental diagnostic yield of TEE in addition to TTE in patients with acute ischemic stroke with undetermined cause. Methods: In a prospective, observational, pragmatic multicenter cohort study, patients with acute ischemic stroke or transient ischemic attack with undetermined cause before cardiac ultrasound were studied by TTE and TEE. The primary outcome was the rate of treatment-relevant findings in TTE and TEE as defined by a panel of experts based on current evidence. Further outcomes included the rate of changes in the assessment of stroke cause after TEE. Results: Between July 1, 2017, and June 30, 2019, we enrolled 494 patients, of whom 492 (99.6%) received TTE and 454 (91.9%) received TEE. Mean age was 64.7 years, and 204 (41.3%) were women. TEE showed a higher rate of treatment-relevant findings than TTE (86 [18.9%] versus 64 [14.1%], P P Conclusions: In patients with undetermined cause of stroke, TEE yielded a higher number of treatment-relevant findings than TTE. TEE appears especially useful in younger patients with stroke, with treatment-relevant findings in one out of seven patients ≤60 years. REGISTRATION: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT03411642.

BACKGROUND: Pregnancy may be associated with adverse outcome in women with congenital heart disease (CHD). However, data regarding the outcome of pregnancy in women with CHD who receive care in cardiac-obstetric expert units are limited. METHODS: We retrospectively analyzed baseline characteristics and outcome of pregnancy in 67 females with CHD who received medical care in our tertiary center for 61 singleton and 6 twin pregnancies between 2009 and 2018. RESULTS: According to the modified World Health Organization (mWHO) risk scale for pregnancy, CHD lesions in 39 enrolled women (58%) were classified as mWHO class I or II, and in 28 females (42%) as mWHO class III or IV. Preterm births were more frequent in mWHO classes III or IV (P=0.003). Cardiac signs and complications occurred more often in mWHO classes III or IV than in women with cardiac lesions assigned to mWHO classes I or II (42.9% vs. 7.7%, P=0.002). N-terminal pro B-type natriuretic peptide (NT-proBNP) levels during pregnancy were higher in mWHO classes III or IV than in mWHO classes I or II (median 269.0 vs. 115.5 pg/mL, P=0.019). Presence of functional NYHA class III [odds ratio (OR) per standard deviation (SD) 8.8, 95% confidence interval (CI): 2.2–57.2, P=0.008] and mWHO classes III/IV (OR per SD 3.4, 95% CI: 1.2–9.9, P=0.018) prior to pregnancy were identified as independent predictors of adverse cardiac outcome of pregnancy. CONCLUSIONS: Adverse cardiac events and preterm deliveries should be anticipated in pregnant women with CHD, especially in those with mWHO classes III or IV. Therefore, these pregnancies should be under close surveillance and managed in specialized, multidisciplinary tertiary referral centers. Preconception counseling including individualized risk assessment is strongly recommended in women with CHD.

Here we generate up-to-date reference values of transthoracic echocardiographic aortic root dimensions matched by sex, age, and body surface area (BSA) derived from the population-based Hamburg City Health Study (HCHS) cohort. In 1687 healthy subjects (mean age 57.1 ± 7.7, 681 male and 1006 female), derived from the first prospectively-recruited 10,000 HCHS participants, dimensions of the aortic root were measured in systole and diastole using state-of-the-art 2-dimensional transthoracic echocardiography. Diameters were assessed at four levels: aortic annulus, Sinus of Valsalva, sinotubular junction, and ascending aorta. Female sex was associated with significantly smaller absolute aortic root dimensions, while indexing for BSA resulted in a reverse effect at all levels. There was a strong age dependency of all aortic root diameters as well as aortic annulus/sinotubular junction ratio for both sexes. Multivariate analysis revealed age, sex, weight, height, and BSA to be significant determinants of aortic root size. Finally, formulas were generated for the calculation of individual aortic root reference values considering age, sex, weight, and height. We provide population-based reference values of aortic root diameters based on a standardized transthoracic echocardiographic protocol of the population-based HCHS which may support the diagnosis, monitoring, and treatment of aortic root disease. Supplementary Information The online version contains supplementary material available at 10.1007/s10554-021-02354-5.

Chronic obstructive pulmonary disease (COPD) is a leading cause of death with a considerable part of the population dying from cardiovascular diseases. High-sensitivity troponin I (hs-TnI) might help to better identify COPD patients at high risk of mortality. We aimed to study the predictive value of hs-TnI for all-cause mortality beyond established COPD assessments, and after consideration of relevant cardiovascular risk factors and prevalent cardiovascular diseases, in a broad population with stable COPD.Circulating hs-TnI concentrations together with a wide range of respiratory and cardiovascular markers were evaluated in 2085 patients with stable COPD across all severity stages enrolled in the multicentre COSYCONET cohort study. The primary outcome was all-cause mortality over 3 years of follow-up.Hs-TnI was detectable in 2020 (96.9%) patients. The median hs-TnI concentration was 3.8 ng·L−1 (interquartile range 2.5–6.6 ng·L−1), with levels above the 99th percentile reference limit of 27 ng·L−1 observed in 1.8% of patients. In Cox regression analyses including adjustments for airflow limitation, dyspnoea grade, exercise capacity and history of severe exacerbations, as well as traditional cardiovascular risk factors, estimated glomerular filtration rate, ankle–brachial index, N-terminal pro-brain natriuretic peptides and prevalent cardiovascular diseases, hs-TnI was a significant predictor for all-cause mortality, both as a continuous variable (hazard ratio (HR) for log hs-TnI 1.28, 95% CI 1.01–1.62) and categorised according to the cut-off of 6 ng·L−1 (HR 1.63, 95% CI 1.10–2.42).In patients with stable COPD, hs-TnI is a strong predictor of all-cause mortality beyond established COPD mortality predictors, and independent of a broad range of cardiovascular risk factors and prevalent cardiovascular diseases. Hs-TnI concentrations well below the upper reference limit provide further prognostic value for all patients with COPD when added to established risk assessments.

Sarcoidosis is the most frequent immunologically related granulomatous disease and can serve as a model for understanding diseases within this category. The evidence on the diagnostics and treatment is so far limited. It is therefore all the more important that two new and significant guidelines on diagnosis and treatment of sarcoidosis were published during the last 2 years. Additionally, there were more new publications, which were considered for this review article. In this context, this review article provides a current update and overview of sarcoidosis. Pathophysiologically, there is an increasing understanding of the complex processes and interactions involved in the inflammatory processes and granuloma formation. The probability of a diagnosis of sarcoidosis is determined by compatible histology, the exclusion of differential diagnoses and if possible evidence of a multiorgan manifestation. The clinical course is variable and ranges from an asymptomatic manifestation to severe life-threatening organ failure. The most frequently affected organ are the lungs. Pulmonary fibrosis is the most severe form and is also decisive for mortality. An increasing focus is on the extrapulmonary organ manifestations, in particular, cardiac, hepatosplenic, gastrointestinal, renal, ocular and neurological involvement. Treatment, which consists primarily of immunosuppression, should be initiated in cases of organ-threatening or quality of life-impairing activity of the disease. Additional organ-specific management must also be evaluated. In cases of organ failure transplantation should be considered. Due to the limited evidence especially for the treatment of multiorgan sarcoidosis, when possible, patients with this disease should be included in clinical trials.Die Sarkoidose ist die häufigste immunologisch bedingte Granulomatose und kann beispielhaft für das Verständnis von Erkrankungen aus diesem Formenkreis herangezogen werden. Die Evidenz zur Diagnostik und Therapie ist bisher limitiert. Umso bedeutender ist es, dass in den letzten 2 Jahren gleich 2 neue Leitlinien zur Diagnose und Therapie veröffentlicht wurden. Darüber hinaus gab es weitere neue Publikationen, die in diesem Beitrag berücksichtigt werden. In diesem Kontext soll die vorliegende Übersichtsarbeit einen aktuellen Überblick über die Sarkoidose liefern. Pathophysiologisch zeigt sich ein zunehmendes Verständnis der komplexen Prozesse und Interaktionen, die an der Inflammation und Granulombildung beteiligt sind. Die Wahrscheinlichkeit einer Sarkoidosediagnose ist durch die Gewinnung einer passenden Histologie, den Ausschluss von Differenzialdiagnosen und möglichst den Nachweis einer Multiorganmanifestation bestimmt. Der klinische Verlauf ist variabel und reicht von einer asymptomatischen Manifestation bis zum schweren lebensbedrohlichen Organversagen. Das am häufigsten betroffene Organ ist die Lunge. Die schwerste Form ist dabei die Lungenfibrose, die auch mortalitätsbestimmend ist. Ein zunehmender Fokus liegt auf den extrapulmonalen Organmanifestationen, insbesondere der kardialen, hepatosplenischen, gastrointestinalen, renalen, okulären und neurologischen Beteiligung. Die Therapie sollte bei organbedrohender oder lebensqualitätseinschränkender Aktivität eingeleitet werden und ist geprägt durch eine Immunsuppression. Weitere organspezifische Therapien sind ebenfalls zu evaluieren. Bei einem Organversagen kann eine Transplantation erwogen werden. Aufgrund der eingeschränkten Datenlage, insbesondere in der Therapie der Multiorgansarkoidose, sollten Patienten mit dieser Erkrankung möglichst in Studien eingeschlossen werden.

The number of adults with congenital heart disease (ACHD) has increased over the last decades due to advancements in medical care, including interventional and surgical therapies. We are therefore more frequently challenged by the long-term consequences of palliative or corrective surgery carried out during childhood. Although patients with ACHD may develop conditions related to general cardiovascular risk factors, such as coronary artery disease, the most common complications leading to morbidity and mortality are arrhythmias, heart failure and thromboembolic events. For the management of arrhythmias, current recommendations regarding ablation and device therapy must be considered, whilst also taking into account the anatomical limitations of their congenital heart defect or surgical pathways. Heart failure treatment in acute and chronic settings must also consider the particular anatomy present, including the nature of the systemic ventricle. Treatments strategies for ACHD are typically extrapolated from the respective guidelines in non-ACHD patients, despite a lack of evidence to support this strategy. Right heart failure can be especially challenging to manage in conditions where either a systemic right ventricle or shunt lesions resulting in volume and/or pressure loading of the right ventricle are present. All physicians and cardiologists in particular should be acquainted with the most common diseases in ACHD, their complications and management regime, especially with regards to heart failure as this is a common reason for acute presentation in the emergency department.

AIMS Evidence suggests that peripheral vascular function is related to cardiovascular disease (CVD) and mortality. We evaluated the associations of noninvasive measures of flow-mediated dilatation and peripheral arterial tonometry with incident CVD and mortality. METHODS AND RESULTS In a post-hoc analysis of the community-based Gutenberg Health Study, median age 55 years (25th/75th percentile 46/65) and 49.5% women, we measured brachial artery flow-mediated dilatation (N = 12,599) and fingertip peripheral arterial tonometry (N = 11,125). After a follow-up of up to 11.7 years, we observed 595 incident CVD events, 106 cardiac deaths, and 860 deaths in total. Survival curves showed decreased event-free survival with higher mean brachial artery diameter and baseline pulse amplitude and better survival with higher mean flow-mediated dilatation and peripheral arterial tonometry ratio (all Plog rank

Aims The subcutaneous cardioverter-defibrillator (S-ICD) may be a valuable option in patients after successful transvenous lead extraction (TLE) without indication for pacemaker therapy and persistent risk of sudden cardiac death. The aim of this study was to evaluate device performance, postoperative outcome, and safety in patients who received a S-ICD after TLE compared to patients who underwent de-novo S-ICD implantation. Methods A retrospective analysis of all patients included into our institution's S-ICD database between September 2010 and May 2019 was conducted.The patients were divided in two groups, depending on whether they had received their S-ICD after TLE (n = 31) or de-novo (n = 113). Results The TLE group was significantly older with a mean age of 54.3 ± 15.7 versus 46.7 ± 14.4 years; p = .007. Leading S-ICD indication in the TLE group was previous infection (50%), whereas in the de-novo group the S-ICD was primarily chosen due to young patient age (74.6%). Median duration of follow-up was 527.0 versus 472.5 days, respectively; p = .576. Most common complication during follow-up was inappropriate ICD therapy (12.9% vs. 13.3%); p = 1.000. Pocket erosion/infection occurred in 3.2% versus 3.5% with no reported cases of systemic (re-)infection in either group; p = 1.000. All-cause mortality was low (6.2% vs. 2.7%) and entirely unrelated to S-ICD implantation or the device itself; p = .293. Conclusion The S-ICD is a safe and effective alternative for patients after TLE with very similar results regarding device performance and postoperative outcome, when compared to patients who underwent de-novo S-ICD implantation.

Background and Objective: The number of adults with congenital heart disease (ACHD) is increasing worldwide. Almost all congenital cardiac lesions can be successfully treated due to the progress in neonatal surgery and pediatric cardiology with a high likelihood of surviving until adulthood. However, ACHD frequently develop sequelae related to the initial cardiac anomaly. Heart failure (HF) is one of the most common complications associated with a high morbidity and mortality.Methods: The authors did search the PubMed database regarding relevant content covering publications up to March 2022. Relevant manuscripts were classified according to the impact factor of the journal, being a guideline manuscript, a position paper by a society or a comprehensive review of the current literature. Key content and findings: Optimal HF treatment remains an unmet need in ACHD. In particular, advanced HF therapy with cardiac resynchronization therapy, ventricular assist devices or organ transplantation is still very different and more specific in ACHD compared to non-ACHD. This review aims to compile international views and evidence from the literatures on the treatment of advanced HF in ACHD. Current challenges, but also the success of different treatment strategies in ACHD are illustrated by clinical cases.Conclusions: The main finding of the review is that data is still scarce regarding ACHD with advanced HF and international efforts to collect data regarding these patients needed to improve the current standard of care.

In Germany, 70,000-100,000 persons per year suffer from out-of-hospital cardiac arrest (OHCA). Despite medical progress, survival rates with good neurological outcome remain low. For many important clinical issues, no or only insufficient evidence from randomised trials is available. Therefore, a systemic and standardised acquisition of the treatment course and of the outcome of OHCA patients is warranted.The German Cardiac Arrest Registry (G-CAR) is an observational, prospective, multicentre registry. It will determine the characteristics, initial treatment strategies, invasive procedures, revascularisation therapies and the use of mechanical circulatory support devices with a focus on extracorporeal cardiopulmonary resuscitation. A special feature is the prospective 12-month follow-up evaluating mortality, neurological outcomes and several patient-reported outcomes in the psychosocial domain (health-related quality of life, cognitive impairment, depression/anxiety, post-traumatic stress disorder and social reintegration). In a pilot phase of 24 months, 15 centres will include approximately 400 consecutive OHCA patients ≥ 18 years. Parallel to and after the pilot phase, scaling up of G-CAR to a national level is envisaged.G-CAR is the first national registry including a long-term follow-up for adult OHCA patients. Primary aim is a better understanding of the determinants of acute and long-term outcomes with the perspective of an optimised treatment.NCT05142124. German Cardiac Arrest Registry (G-CAR).

Die Katheterablation ist eine etablierte Therapieoption zur Behandlung von symptomatischen Herzrhythmusstorungen. Trotz technischer Fortschritte geht die Katheterablation nach wie vor mit einer Inzidenz schwerer Komplikationen von bis zu 5 % einher. Insbesondere periprozedurale Komplikationen erfordern ein sofortiges und zielorientiertes Handeln. Es ist daher fur interventionelle Elektrophysiologen essenziell, etwaige Komplikationen fruhzeitig zu erkennen und notwendige Masnahmen sicher umsetzen zu konnen. Der vorliegende Artikel fasst die Inzidenz und die Risikofaktoren fur periprozedurale Komplikationen wie die Perikardtamponade, eine aortale Fehlpunktion, periprozedurale atrioventrikulare (AV) Blockierungen, Gefaskomplikationen und die Luftembolie im Rahmen einer Katheterablation zusammen, gibt Praventionsstrategien vor und zeigt einen entsprechenden Behandlungspfad auf.

Purpose Venoarterial extracorporeal membrane oxygenation (VA-ECMO) is an increasingly used treatment option for patients in need of mechanical cardiopulmonary support, while available outcome data is limited. The aim of this study was to identify predictors for 30-day in-hospital mortality. Material and methods We analyzed baseline characteristics and outcomes of 8351 VA-ECMO procedures performed in Germany from 2007 to 2015. Using a multivariable model, we identified the ten most important variables to allow for prediction of 30-day in-hospital mortality. Based on these variables, we created a mortality prediction score (ECMO-ACCEPTS score) to enhance decision making in patients considered for or treated with VA-ECMO support. Results Of 8351 patients (71.7% male) 3567 had prior CPR. Mean age was 62 years in the present cohort. The overall 30-day in-hospital mortality was 61%. The ECMO-ACCEPTS score, derived among randomly selected 4175 patients, included ten independent predictors for in-hospital mortality. Internal validation in the remaining 4176 patients confirmed strong differentiation between low and high risk of 30-day in-hospital mortality (r = 0.97 for correlation of predicted with observed mortality, p Conclusions The ECMO-ACCEPTS score might help clinicians to improve risk prediction among VA-ECMO patients for refractory cardiogenic shock.

ZusammenfassungDer echokardiografische Befund der linksventrikulären Hypertrophie impliziert im klinischen Alltag mehrere wichtige Differenzialdiagnosen. Häufig wird die linksventrikuläre Hypertrophie als Befund in einer sogenannten Routine-Echokardiografie erhoben. Für den Kliniker ist jedoch die zugrunde liegende Ursache für die linksventrikuläre Wandverdickung von entscheidender Bedeutung, um die weitere Diagnostik oder Behandlung zu planen. Der folgende Übersichtsartikel legt den Fokus auf physiologische und pathophysiologische Ursachen der linksventrikulären Wandverdickung. Eine physiologische Hypertrophie wird vor allem bei Sportlern beobachtet und als Sportlerherz charakterisiert, eine pathologische linksventrikuläre Wandverdickung liegt z. B. bei der hypertrophen Kardiomyopathie und bei der Amyloidose oder einer Ödembildung vor.

ZusammenfassungDie interventionelle Echokardiografie (also Echokardiografie während der Herzkatheterintervention) hilft, Strukturen zu visualisieren, die in der Fluoroskopie nicht oder nur schlecht sichtbar sind und erlaubt die Überprüfung der Behandlungsergebnisse vor Abschluss der Prozedur am „schlagenden Herzen“. Am Beispiel der Edge-to-Edge-Rekonstruktion mittels MitraClip lässt sich gut zeigen, dass die Echokardiografie bereits bei der Erfassung der Morphologie bzw. Anatomie eine entscheidende Rolle für die Selektion von Patienten spielt. Während der Prozedur wird die Navigation der Schleusen und Katheter durch zwei- und dreidimensionale Darstellungen in der transösophagealen Echokardiografie erleichtert. Die modernen kathetergestützten Verfahren zur Behandlung struktureller Herzerkrankungen wären ohne die interventionelle Echokardiografie zur Steuerung der Prozedur und direkten Erfolgskontrolle nicht möglich.

Assessment of severity and need for intervention in clinical practice often is in need for a thorough echocardiography regarding function of the ventricles and additional valvular dysfunction. Despite the indications recommended in the current guidelines, there is still need for further research to identify patients with a severe valvular dysfunction but a potential reversible status regarding the function of the ventricles. Strain imaging is suggested in the current literature to be an additional tool to identify ventricular dysfunction in the setting of preserved left ventricular ejection fraction.