Impact of a structured patient education programme on early diagnosis of prosthetic pulmonary valve endocarditis.

BACKGROUND: Infective endocarditis is a major threat after prosthetic pulmonary valve replacement. Early diagnosis may improve outcomes. METHODS: A structured patient education programme for prevention and early diagnosis of infective endocarditis was developed at our institution since 2016. Time delay between onset of symptoms of prosthetic pulmonary valve endocarditis and its diagnosis (defined as initiation of appropriate high-dose intravenous antibiotic treatment) was compared for patients presenting before (cohort 1) and after (cohort 2) initiation of the patient education programme. RESULTS: Between 2008-2019, 26 patients (median age 24.9, range: 16.8-62.0 years, 73% male) were diagnosed with prosthetic pulmonary valve endocarditis, 13 patients (cohort 1) before (1.7 cases/year) and 13 patients (cohort 2) after June 2016 (3.7 cases/year). There were no differences in baseline characteristics or clinical presentation between the study cohorts. Overall, the median delay between onset of symptoms and diagnosis of infective endocarditis was 6 days (range: 0-133 days) with a significantly longer delay among patients in cohort 1, compared to cohort 2 (25 days, range: 5-133 days versus 3 days, range: 0-13 days, p < 0.0001). A delay of >7 days was documented in 11/13 patients (85%) in cohort 1 as compared to 1/13 (8%) in cohort 2 (p < 0.001). Need for urgent valve replacement or permanent deterioration of prosthetic valve function was higher in cohort 1, compared to cohort 2 (11/13, 85% versus 5/13, 39%; p = 0.041). CONCLUSIONS: Prosthetic pulmonary valve endocarditis is increasingly recognised. A structured patient education programme may improve early diagnosis and clinical outcomes.

Athletes and Hypertension.

PURPOSE OF REVIEW: We reviewed most current medical literature in order to describe the epidemiology, clinical manifestation, outcome, and management of hypertension in athletes. RECENT FINDINGS: An estimated quarter of the world's population is suffering from hypertension and this prevalence is also reflected in athletes and in individuals involved in leisure time sport activities. Several studies found an inverse relationship between physical activity and blood pressure. Therefore, physical exercise is recommended to prevent, manage, and treat hypertension. On the other hand, the prevalence of hypertension may vary by sport and in some cases may even be higher in athletes competing in certain disciplines than in the general population. Hypertension is the most common medical condition in athletes and may raise concerns about its management and the individual's eligibility for competitive sports. A thorough clinical evaluation should be performed to correctly diagnose or rule out hypertension in athletes, describe the individual's risk profile, rule out secondary causes, and detect possible hypertension-mediated organ damage caused by hypertension at an early stage. Based on most recent clinical research and international consensus documents, we propose a diagnostic algorithm as well the non-pharmacological and pharmacological management of hypertension in athletes. Although elevated blood pressure levels are less common in the active population, athletes are not protected from hypertension. A thorough diagnostic approach may help to identify individual at risk for adverse cardiovascular events and to address the optimal treatment as well as sport recommendations.

High blood pressure response to exercise predicts future development of hypertension in young athletes.

Aims: Due to superior exercise performance, athletes show higher blood pressure (BP) at peak exercise compared to untrained individuals. Thus, higher reference values for peak exercise systolic and diastolic BP were reported specifically for athletes. However, the prognostic significance of high blood pressure response (HBPR) to exercise has not yet been clarified in this population. Methods and results: One hundred and forty-one normotensive athletes with HBPR to exercise were compared to 141 normotensive athletes with normal blood pressure response (NBPR) to exercise, matched for gender, age, body size, and type of sport. All athletes were followed up for 6.5 ± 2.8 years. Over follow-up, no cardiac events occurred; 24 athletes were diagnosed essential hypertension (8.5%). Specifically, 19 (13.5%) belonged to the HBPR compared with 5 (3.5%) in the NBPR group (P = 0.003). Kaplan-Meier analysis confirmed that the incidence of hypertension during follow-up was higher in the HBPR group (log-rank χ2P-value = 0.009). Multivariable analysis by Cox proportional hazard survival model showed that resting BP and HBPR at baseline evaluation were the strongest predictors of incident hypertension (χ2 for the model 30.099; P < 0.001). Specifically, HBPR was associated with a hazard ratio of 3.6 (95% confidence interval 1.3-9.9) of developing hypertension. Over follow-up exercise capacity, as well as morphologic and functional cardiac parameters in athletes from both groups did not change significantly. Conclusion: The present study showed that an exaggerated BP response to exercise increased the risk for incident hypertension in highly trained and normotensive athletes over a middle-term period.

Sudden death in patients with Ebstein anomaly.

Aims: Ventricular dysfunction or structural alteration of either ventricle is a well-established risk factor for sudden death (SD). Ebstein anomaly (EA) can present with both right and left heart abnormalities; however, predictors of SD have not been described. We therefore sought to characterize the incidence and risk factors of SD among a large cohort of patients with EA. Methods and results: All EA patients who underwent evaluation at a high-volume institution over a 4-decade period were retrospectively reviewed. Clinical variables, cardiovascular surgical procedure(s), and cause of death were recorded. Sudden death incidence from birth and following tricuspid valve (TV) surgery were estimated using the Kaplan-Meier method. Cox regression analysis was used to identify clinical and surgical predictors of SD. The cohort comprised of 968 patients [mean age 25.3 years, 41.5% male; 79.8% severe EA, 18.6% accessory pathway, 0.74% implantable cardioverter-defibrillator (ICD) placement]. The 10-, 50-, and 70-year cumulative incidences of SD from birth were 0.8%, 8.3%, and 14.6%, respectively. Prior ventricular tachycardia [hazard ratio (HR) 6.37, P < 0.001)], heart failure (HR 5.64, P < 0.001), TV surgery (HR 5.94, P < 0.001), syncope (HR 2.03, P = 0.019), pulmonic stenosis (HR 3.42, P = 0.001), and haemoglobin > 15 g/dL (HR 2.05, P = 0.026) were multivariable predictors of SD. In a similar subgroup analysis of patients who underwent TV surgery, all of the above factors except syncope were significantly associated with post-operative SD on multivariable analysis. Conclusion: Patients with EA are at significant risk for SD. Key clinical SD predictors identified can aid in risk stratification and potentially guide primary prevention ICD implantation.

Mid-term Outcome of 100 Consecutive Ross Procedures: Excellent Survival, But Yet to Be a Cure.

The Ross procedure offers excellent short-term outcome but the long-term durability is under debate. Reinterventions and follow-up of 100 consecutive patients undergoing Ross Procedure at our centre (1993-2011) were analysed. Follow-up was available for 96 patients (97%) with a median duration of 5.3 (0.1-17.1) years. Median age of the patient cohort was 15.2 (0.04-58.4) years with 76 males. 93% had underlying congenital aortic stenosis. Root replacement technique was applied in all. The most common valved conduits used for reconstruction of the right ventricular outflow tract were homografts (66 patients) and bovine jugular vein (ContegraR) graft (31 patients). Additional procedures included Ross-Konno procedure (14%), resection of subaortic stenosis/myectomy (11%) and reduction plasty of the ascending aorta (25%). One patient died within the first 30 days (1%). Late deaths occurred in 4 patients (4%) 0.5-4.5 years postoperatively: causes included pulmonary hypertension due to endocardial fibroelastosis (2), subarachnoid haemorrhage (1) and sudden cardiac death (1). Five-year survival was 93.6 (95% CI 88.1-99.1)%. Moderate or severe aortic (autograft) regurgitation needing reoperation occurred in 8 patients with a 5-year freedom from autograft reoperation of 98.5 (95.6-100)%. Five-year freedom from reintervention (surgery or catheter based) on the right ventricular outflow tract conduit was 91.5 (85.5-96.5)%. Univariate predictors of this reinterventions were smaller graft size (p = 0.03) and use of a ContegraR graft (p = 0.04). Ross procedure can be performed with low mortality and good survival in the long term. Most of the reinterventions are related to the neo-right ventricular outflow tract and may be partly attributed to the lack of growth. While the Ross Procedure remains an invaluable option for aortic valve disease in children, new solutions for the neo-pulmonary valve as well as for the less often occurring problems on the autograft are needed.

Aortic Root Surgery in Marfan Syndrome: Medium-Term Outcome in a Single-Center Experience.

BACKGROUND: The study aim was to analyze the authors' experience with aortic root surgery in Marfan syndrome (MFS), and to expand the surgical outcome data of patients meeting the Ghent criteria (Marfan registry). METHODS: Analyses were performed of data acquired from MFS patients (who met the Ghent criteria), including an aortic root surgery and Kaplan-Meier survival. RESULTS: Between April 2004 and February 2012, a total of 59 MFS patients (mean age at surgery 36 ± 13 years) underwent 67 operations for aortic root aneurysm (n = 52), aortic valve (AV) regurgitation (n = 15), acute aortic dissection (n = 2), and/or mitral valve (MV) regurgitation resulting from MV prolapse (n = 7). Of 59 initial operations, 21 (36%) involved AV-replacing root surgery, 38 (64%) AV-sparing root surgery, seven (12%) aortic arch or hemi-arch repair, and five (8%) simultaneous MV surgery. There were no early mortalities. The mean follow up was 6.8 ± 1.2 years, with five deaths (8%) and a relatively low reoperation rate (10 reoperations in nine patients; 14%). Seven reoperations involved AV or aortic root surgery (including four for AV regurgitation following failed AV-sparing surgery), two MV repair/replacements, and one coronary artery bypass graft. Eight patients (21%) with AV-sparing surgery had moderate/severe AV regurgitation at the last follow up before re-intervention. The mean five-year freedom from postoperative death was 91.2 ± 8.8%, from cardiac reoperation 86.3 ± 4.5%, and more-than-moderate AV regurgitation 90.3 ± 4.8%. CONCLUSIONS: Prophylactic aortic surgery in MFS patients with AV-replacing root or AV-sparing root surgery carries a low risk of operative morbidity and death when performed at an experienced center. AV-sparing root surgery increases the risk of AV regurgitation and, possibly, of re-intervention. Regular clinical follow up is important after any aortic root surgery in MFS patients, with a delineation of risk factors for AV regurgitation after AV rootsparing surgery.

Safety and efficacy of the nMARQ catheter for paroxysmal and persistent atrial fibrillation.

AIMS: Circular irrigated radiofrequency ablation using the nMARQ catheter has recently been introduced for the treatment of atrial fibrillation (AF). The aim of this study is to report the safety and efficacy of catheter ablation using this technology in patients with paroxysmal and persistent AF. METHODS AND RESULTS: The data of a prospective registry describing the experience of a single operator using this technology on 327 consecutive patients were analysed. The mean procedure time was 69 ± 22 min for paroxysmal AF (n = 228) and 75 ± 23 min for persistent AF (n = 97). Follow-up was available for 206 (63%) patients for 6 ± 5 months (range 1-23, median 3.3). Single procedure success off antiarrhythmic drugs was 75% in paroxysmal AF and 52% in persistent AF. Including the 5% redo cases and those on antiarrhythmic medication, freedom from AF was documented in 90 and 83% of paroxysmal and persistent AF patients, respectively. There were no serious complications in the first 325 patients, but the last two consecutive patients (0.6%) developed atrio-oesophageal fistulas and had a fatal outcome. The catheter has been recalled from market. CONCLUSION: The nMARQ catheter is a highly effective tool for treatment of paroxysmal and persistent AF. Nevertheless, the occurrence of life-threatening oesophageal fistulas is of major concern and requires further investigation.

Dilatation and Dysfunction of the Neo-aortic Root and in 76 Patients After the Ross Procedure.

Pulmonary autograft replacement (Ross procedure) is used as an alternative to prosthetic aortic valve replacement patients with aortic valve disease. There are limited data on incidence and risk factors for dilatation and dysfunction of the neo-aortic after the Ross procedure. Ross procedure was performed in 100 patients at our institution between 1993 and 2011. In 76 patients, complete follow-up data were available. Their median age at surgery was 16 (0.4-58) years (76 % males; 95 % with congenital aortic valve disease). Median follow-up duration was 5.2 years (0.3-16.0 years). We analyzed their clinical and echocardiographic follow-up to identify possible risk factors for neo-aortic root dilatation and dysfunction. Ross procedure included reduction plasty of the native ascending aorta in 25 % of patients. During follow-up, 21 patients (28 %) developed neo-aortic root dilatation, 38 patients (50 %) dilatation oft the native ascending aorta and 7 patients (9 %) at least moderate neo-aortic regurgitation. Univariate risk factors for neo-aortic root dilatation were preoperative aortic regurgitation (p = 0.04), concomitant reduction plasty of the ascending aorta (p = 0.009) and a longer duration of follow-up (p = 0.005). Younger age at surgery was associated with dilatation of the ascending aorta (p = 0.03). Reoperation on the neo-aortic root because of severe dilatation was necessary in 6 patients (8 %), where 2 patients had at least moderate neo-aortic root regurgitation. Neo-aortic root and aortic dilatation are common after the Ross procedure. This is often combined with neo-aortic valve dysfunction. Close follow-up of these patients is mandatory.

Management and outcome of Ebstein's anomaly in children.

OBJECTIVES: To assess clinical presentation, treatment, and outcome of children with Ebstein's anomaly. BACKGROUND: Data on long-term outcome of children with Ebstein's anomaly are scarce. METHODS: Retrospective analysis of all children with Ebstein's anomaly treated between February, 1979 and January, 2009 in a single tertiary institution. Primary outcomes included patient survival and need for intervention, either cardiac surgery or catheter intervention. RESULTS: A total of 42 patients were diagnosed with Ebstein's anomaly at a median age of 5 days ranging from 1 day to 11.7 years. Symptoms included cyanosis, heart murmur, and/or dyspnoea. Associated cardiac anomalies occurred in 90% of the patients. Average follow-up was 9.5 plus or minus 7.0 years. The overall mortality rate was 14%. Of the six patients, three died postnatally before treatment. Cardiac surgery and/or catheter-guided interventions were required in 33 patients (79%). Cardiac surgery was performed in 21 (50%) patients at a median age of 9.1 years (range 0.1-16.5 years), including biventricular repair in 13 (62%), one-and-a-half chamber repair in seven (33%), and a staged single-ventricle repair in one. Peri-operative mortality was 4%. Catheter-guided interventions consisted of device closure of an atrial septal defect in three cases and radiofrequency ablation of accessory pathways in nine patients. The estimated 10-year survival was 85.3 plus or minus 5.6%. CONCLUSION: In children, Ebstein's anomaly is usually diagnosed in the first year of age. Even though children with Ebstein's anomaly often require an intervention, their peri-operative mortality is low and long-term survival is good. Symptomatic newborns requiring an intervention may have a worse outcome.

Impact of pulmonary valve replacement on ventricular function and cardiac events in patients with tetralogy of Fallot. A retrospective cohort study.

INTRODUCTION AND OBJECTIVES: Our aim was to assess the impact of prosthetic pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot (rTOF) on changes in biventricular volumes and function and on adverse cardiac events. METHODS: Adults with rTOF were identified from the SACHER-registry. Data from serial cardiac magnetic resonance imaging, echocardiography, exercise capacity and n-terminal pro b-type natriuretic peptide (NT-proBNP) were collected. The primary endpoint was right ventricular ejection fraction (RVEF) as measured by cardiac magnetic resonance. Secondary endpoints were biventricular volumes, left ventricular ejection fraction, exercise capacity and NT-proBNP levels, and time to adverse cardiac outcomes (atrial and ventricular arrhythmia, endocarditis). Associations between previous PVR and longitudinal changes in functional outcomes and time to adverse cardiac outcomes were analyzed using linear mixed-effects models and Cox proportional hazards models, respectively. RESULTS: A total of 308 patients (153 with and 155 without PVR) with 887 study visits were analyzed. Previous PVR was not significantly associated with changes in RVEF (CE, -1.33; 95%CI, -5.87 to 3.21; P=.566). Previous PVR was associated with lower right ventricular end-diastolic volume but had no significant effect on left ventricular ejection fraction, exercise capacity, or NT-proBNP-levels. Previous PVR was associated with an increased hazard of atrial arrhythmias (HR, 2.09; 95%CI, 1.17-3.72; P=.012) and infective endocarditis (HR, 12.72; 95%CI, 4.69-34.49; P<.0001) but not with an increased hazard of sustained ventricular arrhythmias (HR, 0.64; 95%CI, 0.18-2.27; P=.490). CONCLUSIONS: Previous PVR was not significantly associated with changes in RVEF but was associated with an increased risk of atrial arrhythmias and infective endocarditis.

Determinants of Advanced Liver Fibrosis in Adult Patients After Fontan Palliation: Usefulness of Ultrasound Transient Elastography.

BACKGROUND: Fontan-associated liver disease is an increasing concern. Our aim was to assess prevalence and predictors of advanced liver fibrosis with a specific focus on utility of liver stiffness measurement by ultrasound transient elastography. METHODS: A total of 97 adult Fontan patients (55% male, median age: 23.1 years, interquartile range [IQR]: 18.7-30.6); 92 (95%) were evaluated with transient elastography, and 50 (52%) underwent transjugular liver biopsy. Advanced liver fibrosis was defined as congestive hepatic fibrosis score 3 or 4. RESULTS: Only 4 patients (4%) had liver stiffness values < 10 kilopascal (kPa). Liver-stiffness measurements correlated weakly with peak oxygen uptake on exercise testing and Fontan pressure but not with Model for End-Stage Liver Disease excluding INR (MELD-XI) score or spleen size. Serial follow-up liver stiffness measurements in 73 clinically stable patients showed large variability among individual patients. Advanced liver fibrosis was present in 35 of 50 (70%) patients on liver biopsy and was associated to MELD-XI-Score ≥ 11 and splenomegaly but not to liver-stiffness measurements. Advanced liver fibrosis was not associated with patient age or time since Fontan operation but with younger age at completion of Fontan (3.7 years, IQR: 2.3-6.3 vs 6.8 years; IQR: 3.5-12.1; P = 0.037). CONCLUSIONS: In our cohort, advanced liver fibrosis was present in the majority of adult Fontan patients. Liver stiffness as measured by transient elastography was not associated with the degree of liver fibrosis. Because of its high variability on serial measurements, it seems not to be useful for clinical decision making. The unexpected finding that younger age at completion of Fontan was associated with advanced liver fibrosis merits further evaluation.

Left ventricular hypertrophy in athletes, a case-control analysis of interindividual variability.

BACKGROUND: A variability in cardiac remodeling is observed in athletes regardless of age, sex, body size and sport participated. We sought to investigate whether other individual characteristics could affect the extent of Left ventricular hypertrophy (LVH). METHODS: From 2120 consecutive Olympic athletes, those with LVH (defined as LV Wall thickness ≥ 13 mm) were matched 1:1 by age, gender, body surface area and type of sport with non-LVH Athletes. Clinical and Echocardiographic variables were compared. RESULTS: 48 athletes with LVH (2.3%) and 48 matched non-LVH athletes were identified. LVH Athletes had higher body weight (90 ± 18 vs 81 ± 11Kg; p = 0.006) body mass index (26 ± 2 vs 24 ± 2 Kg/m2; p < 0.001) and body fat percentage (15 ± 7% vs 12 ± 4%; p = 0.016) compared to non-LVH Athletes. They also had higher systolic (123 ± 1 vs 116 ± 11 mmHg; p = 0.002) and diastolic blood pressure (76 ± 8 vs 71 ± 9 mmHg; p = 0.002). On exercise testing, LVH Athletes reached a lower index workload (3.7 ± 0.9 vs 4.1 ± 0.8 W/Kg; p = 0.013) and a higher peak diastolic blood pressure (79 ± 10 vs 74 ± 11 mmHg; p = 0.012) than those without LVH. Binary logistic regression analysis showed that diastolic blood pressure (OR 1.052; 95% CI from 1.011 to 1.130; p = 0.020) and BMI (OR 1.220; 95% CI from 1.016 to 1.465; p = 0.033) had the strongest association with LVH as categorical variable. CONCLUSIONS: Our study showed that increased blood pressure at rest and during exercise, together with larger body weight, body mass and fat percentage are associated with a higher degree of LVH, which is not associated with a greater physical performance and therefore possibly disproportionate to the sport activity.

Barlow disease: effect of mitral valve repair on ventricular arrhythmias in 82 patients in a retrospective long-term study.

BACKGROUND: Patients (pt) with mitral valve prolapse (MVP) due to Barlow disase (BD) have an increased incidence of ventricular arrhythmias (VA; including ventricular tachycardias VT) and sudden cardiac death (SCD). Data on the effect of MV repair on VA are scarce. METHODS: Pre- and postoperative VA in severe mitral regurgitation (MR) with MVP due to BD undergoing surgical mitral valve repair were analyzed. Patients with degenerative mitral valve disease not fulfilling BD criteria were excluded. Information was from charts, ECG/Holter ECG and/or pacemaker/ implantable cardioverter defibrillator (ICD) data. SCD, sustained VT>30 sec and/or ventricular fibrillation necessitating an ICD-shock were considered major events. Event probability was calculated using the Kaplan-Meier estimator throughout the follow-up period of 20.7 years. RESULTS: There were 82 pts (61% males), mean age at surgery 62±14 years. Bileaflet MVP was present in 54%, mitral annular dysjunction (MAD) in 37% and left ventricular ejection fraction (LVEF) <50% in 12%. MV repair included ring annuloplasty in all and artificial chords in 48%. Mean follow-up was 3.1 years (0.2 to 14.2 years). Postoperative rhythm surveillance by Holter ECG and/or pacemaker was available in 67%. A VA load of ≥10% and/or any VT was noted in 26% before and 32% after surgery (P=0.44). Postoperative VA load was not predicted by MAD, artificial chords, LVEF of <50%, age at surgery >50 years and/or residual ≥moderate MR (all P<0.05), it correlated only with bileaflet MVP (P=0.009). Major events occurred in 3 pts: SCD in 2 pts and ICD for sustained polymorphic VT in 1 pt (incidence 1.2%/year). The event probability of receiving a SCD or an ICD-shock was 4.9%. CONCLUSIONS: VA burden does not seem to change after MV repair in MVP due to BD. The occurrence of major arrhythmic events can not be predicted reliably, thus, patients with MVP due to BD may need lifelong postoperative follow-up, especially in bileaflet MVP which was an independent risk factor for increased VA burden in this retrospective long-term study in a small but well selected patient group.

High Variability of Right Ventricular Volumes and Function in Adults with Severe Pulmonary Regurgitation Late After Tetralogy of Fallot Repair.

Our aim was to assess changes of right ventricular end-diastolic volumes (RVEDVi) and right ventricular ejection fraction (RVEF) in asymptomatic adults with repaired tetralogy of Fallot, with native right ventricular outflow tract and severe pulmonary regurgitation by serial cardiac magnetic resonance imaging (CMR). The study included 23 asymptomatic adults who underwent ≥3 CMR studies (total of 88 CMR studies). We compared changes in RVEDVi and RVEF between first and last study (median follow-up: 8.8 years, interquartile range: 6.3 to 13.1 years) and between all study pairs. Variability of measurements between study pairs (65 consecutive and 139 nonconsecutive CMR study pairs) were assessed using Bland-Altman analysis and intraclass correlation coefficients. On average, there were no significant changes of RVEDVi or RVEF over the study period (change in RVEDVi: +0.4 ± 17.8 ml/m2, change in RVEF: -1.0 ± 5.5%). Assessment of variability of measurements between study pairs demonstrated no systematic change in RVEDVi and RVEF between study pairs with limits of agreement within the range of previously published studies (RVEDVi -29.1 to +27.2 ml/m2; RVEF -11.5% to 10.2%). High intraclass correlation coefficients for RVEDVi (0.943, 95% CI 0.906 to 0.965, p <0.001) and RVEF (0.815, 95% CI 0.697 to 0.887, p <0.0001) indicate high reliability of reported measurements. In conclusion, in asymptomatic adults with repaired tetralogy of Fallot with native right ventricular outflow tracts and severe pulmonary regurgitation, CMR measurements of RV volumes and RVEF remain stable during follow-up with variability between CMR studies in individual patients, as expected for interobserver and interstudy variability. Measurements derived from a single CMR study or changes occurring between 2 CMR studies should be used with caution for clinical decision-making.

The very low risk of myocarditis and pericarditis after mRNA COVID-19 vaccination should not discourage vaccination.

The benefits of vaccination - regarding COVID-19 infection and transmission, as well as COVID-associated complications - clearly outweigh the potential risk of vaccine-associated inflammation of the heart and other adverse events. Given the current state of knowledge, the outcome of myocarditis and pericarditis following vaccination is generally good. This review aims to guide physicians in the early diagnosis and management of suspected myocarditis following mRNA COVID vaccination. The initial work-up should include detailed history, a 12-lead electrocardiogram and serological biomarkers (high-sensitivity cardiac troponin T/I, natriuretic peptides and markers of inflammation) in accordance with the assessments recommended in current clinical practice guidelines for patients presenting with acute chest pain. In patients with suspected myocarditis, further assessment with transthoracic echocardiography and cardiovascular magnetic resonance imaging should be undertaken to confirm peri-/myocarditis and to distinguish the findings from other diseases with similar presentation. Patients with mRNA vaccine-associated myocarditis should be followed-up at least once to exclude chronic myocardial inflammation and deterioration of left ventricular ejection fraction. Consultation with an expert such as an immunologist with experience in vaccination regarding further mRNA vaccinations is advised in all patients with mRNA vaccine-associated perimyocarditis. Reporting of mRNA vaccine-associated myocarditis to Swissmedic is mandatory. Cohort studies prospectively follow-up on young adult and paediatric populations following immunisation with an mRNA COVID vaccine to monitor cardiac and immune parameters would generate valuable knowledge to better understand pathogenesis and risk factors for vaccine-associated perimyocarditis.

Utility of a bedside acoustic cardiographic model to predict elevated left ventricular filling pressure.

BACKGROUND: The authors previously described an acoustic cardiographic model that predicted echocardiographic correlates of elevated left ventricular (LV) filling pressure. This study evaluated this bedside acoustic cardiographic model against invasive measurements of LV filling pressure. METHODS AND RESULTS: Data were prospectively obtained from 68 adults referred for right heart catheterisation. Acoustic cardiographic measurements were obtained during right heart catheterisation. Elevated LV filling pressure was defined as a pulmonary capillary wedge pressure (PCWP) > or =15 mm Hg. Parameters generated from a previous dataset used for the current analysis were measures of LV systolic time, maximum negative area of the P wave, QTc interval and third heart sound (S3) score. Logistic regression was used to calculate area under the curve (AUC). Of the 66 patients included, 39 had elevated PCWP. Estimating the probability of an elevated PCWP from the derived model resulted in an AUC of 0.72 (95% CI 0.60 to 0.85). When the regression model's parameters were held constant but the parameter estimates were allowed to vary, the AUC in the validated model was 0.76 (95% CI 0.64 to 0.88). At a specificity of 90% the positive likelihood ratio (LR+) was 5.0 (1.7 to 15.3) and the negative likelihood ratio was 0.49 (0.34 to 0.71). CONCLUSION: These data demonstrate that the four-variable model predicts elevated filling pressure at the bedside with high specificity and an intermediate LR+. With improvements in sensitivity and further prospective validation of this model in a cohort of emergency department patients with undifferentiated dyspnoea this may be a useful bedside diagnostic modality.

Cardiovascular surgery in Turner syndrome - early outcome and long-term follow-up.

BACKGROUND: Cardiovascular disease is the leading cause of death in patients with Turner syndrome (TS), and cardiovascular surgery is frequently required for management of these patients. TS is associated with medical comorbidities than can complicate the care of this patient population. AIM: To describe the cardiovascular surgical outcomes of patients with TS. METHODS: A retrospective case series was compiled of 51 consecutive TS patients who had at least one cardiovascular surgery at Mayo Clinic Rochester from 1977-2017. The baseline clinical data of these patients were reviewed including demographics, medical comorbidities, congenital heart disease history, and medications. Echocardiographic reports were analyzed in detail. Operative reports and surgical hospital courses were reviewed. Long-term mortality was determined using medical records and the Social Security Death Index. Survival analysis was performed with the Kaplan Meier method. RESULTS: The cohort comprised 51 TS patients, average age at the time of surgery at Mayo Clinic was 28 (8-41) years, and 23 (45%) patients were under the age of 18. At the time of first Mayo Clinic surgery, 18 (35%) patients had previously undergone cardiac surgery at another institution. The most common procedures were repair of aortic coarctation in 14 (28%) patients, aortic valve replacement in 6 (12%) patients, and composite aortic root/ascending aorta replacement in 7 (14%) patients, with 7 patients undergoing repair of more than one lesion. Aortic dissection required operative intervention in 5 patients. After initial Mayo Clinic surgery, subsequent operations were required in 6 (13%) patients. Average hospital length of stay was 6 ± 2 d. There were 4 (8%) early surgical deaths. Freedom from death was 97% and 89% at 10 and 20 years, and the freedom from reoperation was 93% and 81% at 10 and 20 years. CONCLUSION: Cardiovascular surgery is associated with 8% early mortality given the medical complexity of TS patients. Those who survive to dismissal have good survival. Later cardiovascular reoperations are not rare.

Marfan syndrome and related connective tissue disorders in the current era in Switzerland in 103 patients: medical and surgical management and impact of genetic testing.

INTRODUCTION: Marfan syndrome (MFS) and related connective tissue disorders (CTDs) are increasingly recognised. Genetic testing has greatly improved the diagnostic outcome/power over the last two decades. In this study we describe a multicentre cohort of adults with MFS and related CTDs, with a particular focus on results from genetic testing. METHODS: All patients with MFS and related CTDs were identified from the databases of five centres in the canton of Zurich. Echocardiographic and clinical findings including systemic Marfan score, use of medication and genetic results were retrospectively analysed. MFS was diagnosed using the revised Ghent criteria (including FBN1 genetic testing if available); other CTDs (Loeys-Dietz syndrome) were diagnosed by genetic testing only. RESULTS: A cohort of 103 patients were identified (62 index patients, 41 relatives of family members): 96 patients with MFS and 7 patients with other CTD, 54 males (52%), median age 23 years (range 1–75). The median systemic Marfan score was 5 (range 0–18). Only 40 patients (40/103, 39%) fulfilled criteria for systemic involvement (≥7 points). A history of aortic dissection was present in 14 out of 103 patients (14%). Echocardiographic data were available for all: aortic root enlargement (Z-score ≥2 in adults, Z-score ≥3 in children) was found in 49 patients (48%) and mitral valve prolapse in 64 (62%). Genetic testing had been performed in 80 patients (78%); FBN1 mutations were present in 69 patients (86%); other pathogenic mutations could be identified in seven patients (9%); no disease-causing mutation was found in four patients, three of them fulfilling the Ghent criteria of MFS. Of the mutation-positive patients, 33 had a systemic score of ≥7 and 43 had a systemic score of ≥5. Revised Ghent criteria were fulfilled in 70 patients: in 69 patients with FBN1 mutations and 1 patient with another CTD. Recommended treatment (beta-blocker, angiotensin receptor blocker) was taken by 63% of patients. CONCLUSIONS: In this cohort a high percentage of patients fulfilling the revised Ghent criteria for MFS underwent genetic testing, often leading to or confirming the diagnosis of MFS. Other CTDs could be discriminated best by genetic testing. With respect to the diagnosis of MFS and related CTDs, the usefulness of the systemic score is limited, showing the importance of genetic testing, which enabled definitive diagnosis in 95% of tested patients. Patient education on medical treatment still has to be improved. (Trial registration no: KEK-ZH-Nr. 2013-0241).

Value of brain natriuretic peptides in primary care patients with the clinical diagnosis of chronic heart failure.

INTRODUCTION: Brain natriuretic peptide (BNP) and N-Terminal pro natriuretic peptide (NT-proBNP) are widely accepted to diagnose congestive heart failure (CHF) in the emergency room. The aim of this study was to evaluate the value of BNP and NT-proBNP to diagnose CHF in primary care. METHODS: Clinical and Doppler-echocardiographic assessment of patients referred by their general practitioner (GP) with the diagnosis of CHF. Receiver operating curves were used to evaluate the accuracy of BNP and NT-proBNP for echocardiographically confirmed systolic and/or diastolic heart failure. RESULTS: Three hundred and eighty four patients (mean age of 65) were included. One hundred and ninety three (50%) patients had systolic heart failure and 31 (8%) had isolated diastolic heart failure. Using currently recommended cut-off values of BNP (less than 100 pg/ml) and NT-proBNP (less than 125 pg/ml) for exclusion of CHF, BNP was false negative in 25% and NT-proBNP in 10% of the patients. The area under the curve was better for NT-proBNP than for BNP (0.742 vs. 0.691). CONCLUSION: In this population with a high prevalence of CHF, BNP and NT-proBNP failed to adequately rule out CHF. GP's should be cautious when using BNP and NT-proBNP in primary care. An echocardiography remains compulsory in unexplained dyspnea.

Bedside prediction of increased filling pressure using acoustic electrocardiography.

BACKGROUND: Patients presenting with acute dyspnea are often a diagnostic dilemma. A bedside tool that accurately and rapidly identifies increased left ventricular (LV) filling pressure would be helpful. We evaluated acoustic electrocardiography for this purpose. METHODS: We pooled 3 cohorts of patients for this analysis. Inclusion criteria required acoustic electrocardiography and echocardiography within 4 hours of each other. Increased LV filling pressure was defined as a pseudonormal or restrictive filling pattern on echocardiography. Area under the receiver operating characteristic curve (AUC) assessed multivariable model accuracy. RESULTS: The median age of the 324 patients was 61 years (range, 19-90 years), 67% were male, and 82% had a history of heart failure. The final multivariable model included mean LV systolic time, S(3) score, maximum negative area of the P wave, and the QTc interval. The AUC was 0.83 (95% confidence interval, 0.78-0.88). Although B-type natriuretic peptide (BNP) was an independent predictor of estimated increased filling pressure when considered alone (odds ratio = 1.002, 95% confidence interval, 1.000-1.003, P = .002), when added to the acoustic model, it did not improve overall model accuracy. In the subset of patients with indeterminate BNP levels (100-500 pg/mL), the acoustic model was more accurate than BNP (AUC = 0.82 vs 0.71). CONCLUSIONS: Bedside acoustic electrocardiography predicted echocardiographic correlates of increased pressures with high accuracy. For patients with an indeterminate BNP level (100-500 pg/mL), the acoustic electrocardiography model was superior to BNP. Prospective model validation is warranted.