Extraction of a 6-year-old leadless pacemaker (MICRA transcatheter pacing system) using commercially available removal tools: A case report.

INTRODUCTION: Leadless pacemakers are associated with a low risk of infection, so indications for their removal are rare. One can expect that the dwell time of the device correlates with a more difficult removal, but it has not been proved so far. METHODS AND RESULTS: We present a case of a patient in whom MICRA transcatheter pacing system was successfully removed with nondedicated commercially available tools, 70 months after implantation. CONCLUSION: A successful removal of the MICRA leadless pacemaker is possible, and may be safe even many years after the device implantation, despite a lack of dedicated tools. Due to the potential risk of complications, the benefits and risks of the procedure should be weighted before making a final decision.

Heart Failure with Preserved Ejection Fraction-a Concise Review.

PURPOSE OF REVIEW: Heart failure with preserved ejection fraction (HFpEF) is a relatively new disease entity used in medical terminology; however, both the number of patients and its clinical significance are growing. HFpEF used to be seen as a mild condition; however, the symptoms and quality of life of the patients are comparable to those with reduced ejection fraction. The disease is much more complex than previously thought. In this article, information surrounding the etiology, diagnosis, prognosis, and possible therapeutic options of HFpEF are reviewed and summarized. RECENT FINDINGS: It has recently been proposed that heart failure (HF) is rather a heterogeneous syndrome with a spectrum of overlapping and distinct characteristics. HFpEF itself can be distilled into different phenotypes based on the underlying biology. The etiological factors of HFpEF are unclear; however, systemic low-grade inflammation and microvascular damage as a consequence of comorbidities associated with endothelial dysfunction, oxidative stress, myocardial remodeling, and fibrosis are considered to play a crucial role in the pathogenesis of a disease. The H2FPEF score and the HFpEF nomogram are recently validated highly sensitive tools employed for risk assessment of subclinical heart failure. Despite numerous studies, there is still no evidence-based pharmacotherapy for HFpEF and the mortality and morbidity associated with HFpEF remain high. A better understanding of the etiological factors, the impact of comorbidities, the phenotypes of the disease, and implementation of machine learning algorithms may play a key role in the development of future therapeutic strategies.

Clinical aspects of myocardial fibrosis in adults with Ebstein's anomaly.

Heart failure and arrhythmia are common complications in adults with Ebstein's anomaly. They may result not only from hemodynamic alterations, but also from myocardial fibrosis. Late gadolinium enhancement (LGE) by CMR enables the evaluation of myocardial fibrosis. The aim of the study was to asses the presence of LGE and its relation to clinical outcome. We studied a group of 37 unoperated adults aged 43.0 ± 14.4 years with Ebstein's anomaly from the congenital heart disease outpatient clinic. Study protocol included: cardiopulmonary test, assessment of supraventricular arrhythmia (SVA), and CMR with evaluation of cardiac chambers' morphology and function, and presence of LGE. Variables following normal distribution were shown as mean ± SD if otherwise median (range) was applied. Fibrosis was found in 18 patients (48.6%) and was distributed as follows: 12 patients (32.4%) in the right atrium, 12 (32.4%) in the atrialized right ventricle, and 2 (5.4%) in the functional right ventricle. In patients with fibrosis, the tricuspid regurgitation fraction was bigger (48.3 ± 19.7 vs. 36.1 ± 22.6%, p = 0.048) and SVA was more frequent [12 (66.7%) vs. 6 (31.6%), p = 0.046] when compared to patients without fibrosis. However, exercise capacity did not differ between patients with and without LGE (peak VO2 24.0 ± 4.7 vs. 23.7 ± 4.4, p = 0.87). In adults with Ebstein's anomaly fibrosis estimated by LGE-CMR was localized in the right atrium and the right ventricle only. Volume overload resulting from tricuspid regurgitation might be a factor conducive to fibrosis. Myocardial fibrosis did not influence exercise capacity. Association between myocardial fibrosis and supraventricular arrhythmia was confirmed.

Lower air ozone concentration is associated with clinical remission in type 1 diabetes diagnosed in adulthood: a prospective, observational study in Wielkopolska region, Poland.

INTRODUCTION: Clinical remission in type 1 diabetes (T1D) results from metabolic compensation after insulin implementation and is caused by various factors. OBJECTIVES: Our aim was to investigate an association between air pollution defined based on ozone concentration in the month of T1D diagnosis and the early course of the disease, that is, glucose metabolism and the occurrence of remission. PATIENTS AND METHODS: This prospective, observational analysis included 96 adult patients with newly diagnosed T1D. The study group was divided according to the occurrence of remission at 12 months after the diagnosis. The levels of ambient ozone measured within the month of T1D diagnosis were calculated using the official data of Poland's Chief Inspectorate of Environmental Protection. Remission was defined according to the following formula: actual glycated hemoglobin (HbA1c)(%) level + [4 × insulin dose (units/kg per 24 h)] - value defining partial remission ≤9. RESULTS: The remission rate after 12 months was higher in the group where ozone concentration was below or equal to the median for the study population (P <0.001). Moreover, the patients in the group where ozone levels were above the median, presented lower C­peptide levels (P = 0.01), higher HbA1c concentration (P = 0.005), and higher daily insulin requirements (P = 0.02) after 12 months from the diagnosis. Also, in the group of participants achieving remission, the ambient ozone level was lower (P <0.001). In a multivariable logistic regression analysis, the increased ozone concentration in the month of diagnosis was the variable that influenced the lack of remission after 12 months, independently of sex and smoking (P <0.001). CONCLUSIONS: Increased ozone level may exacerbate metabolic outcomes and reduce remission in T1D.

A Twenty-Year Follow-Up of Adults with Ebstein Anomaly with Special Focus on Supraventricular Arrhythmias, Supraventricular Arrhythmias and Effectiveness of Catheter Ablation in 20-Year Follow-Up of Adults with Ebstein Anomaly.

Background: Ebstein anomaly (EA) is a rare congenital heart disease characterized by the apical displacement of the tricuspid leaflets, creating an enlarged functional right atrium. Supraventricular arrhythmias (SVA) are common, and catheter ablation remains challenging. SVA is considered a risk factor for sudden cardiac death in this population. Still, there are very few real-life data on the impact of SVA treated invasively or conservatively on a patient's prognosis. We aimed to analyze the incidence of SVA in adults with EA, evaluate the effectiveness of catheter ablation, and analyze the impact of SVA and catheter ablation on survival in this population. Methods and results: 71 pts (median age 53 years; range 24-84 years) with EA were evaluated retrospectively from 1988 to 2020. Forty patients (56.3%) had SVA, and eighteen of them (45.0%) required at least one catheter ablation (35 procedures in total). Indications for ablation were mostly intra-atrial reentrant tachycardia (IART) and atrioventricular reentrant tachycardia (AVRT) (14 pts [77.8% and 9 pts [50.0%], respectively. IART and AVRT coexisted in nine pts. One patient suffered from persistent atrial fibrillation. Procedural effectiveness was reported in 28 (80%) cases; over a longer follow-up (mean 12.6 ± 5.4 years), only eight (44.4%) patients were completely free from SVA after the first ablation. In total, 10 patients (14%) died due to cardiovascular events. There was no difference in survival between patients with or without SVA (p = 0.9) and between ablated and non-ablated EA individuals (p = 0.89). Conclusions: Supraventricular arrhythmia is frequent in adults with Ebstein anomaly. Patients often require more than one catheter ablation but eventually become free from arrhythmias. The imaging parameters assessed by echocardiography or cardiac magnetic resonance do not seem to be associated with ablation outcomes. The impact of supraventricular arrhythmia itself or treatment with radiofrequency ablation is questionable and should be thoroughly investigated in this population.

Microvolt T wave alternans in adults with congenital heart diseases characterized by right ventricle pathology or single ventricle physiology: a case control study.

BACKGROUND: Among adults with congenital heart diseases (CHD) evaluation of sudden cardiac death (SCD) risk remains a great challenge. Although microvolt T-wave alternans has been incorporated into SCD risk stratification algorithm, its role in adults with CHD still requires investigation. We sought to determine the incidence of MTWA in this specific group and its coincidence with ventricular arrhythmia (VA) and other clinical findings presumably associated with SCD. METHODS: A case-control study was performed in which 102 patients with CHD characterized by right ventricle pathology or single ventricle physiology (TGA, UVH, Ebstein's anomaly, ccTGA, Eisenmenger syndrome, DORV, CAT, unoperated ToF) were compared to 45 age- and sex-matched controls. All subjects underwent spectral MTWA test, ambulatory ecg monitoring, cardiopulmonary test, BNP assessment. After excluding technically inadequate traces, the remaining MTWA results were classified as positive(+), negative(-) and indeterminate(ind). Due to similar prognostic significance MTWA(+) and (ind) were combined into a common group labeled 'abnormal'. RESULTS: Abnormal MTWA was present more often in the study group, compared to controls (39.2% vs 2.3%, p = 0.00001). Sustained ventricular tachycardia (sVT) was observed more often among subjects with abnormal MTWA compared to MTWA(-): 19.4% vs 3.6%, p = 0.026. The patients with abnormal MTWA had a lower blood saturation (p = 0.047), more often were males (p = 0.031), had higher NYHA class (p = 0.04), worse cardiopulmonary parameters: %PeakVO2 (p = 0.034), %HRmax (p = 0.003). Factors proven to increase probability of abnormal MTWA on multivariate linear regression analysis were: sVT (OR = 20.7, p = 0.037) and male gender (OR = 15.9, p = 0.001); on univariate analysis: male gender (OR = 2.7, p = 0.021), presence of VA (OR = 2.6, p = 0.049), NYHA > I (OR = 2.06, p = 0.033), %HRmax (OR = 0.94, p = 0.005), %PeakVO2 (OR = 0.97, p = 0.042), VE/VCO2slope (OR = 1.05, p = 0.037). CONCLUSIONS: Abnormal MTWA occurs significantly more often in adults with the chosen forms of CHD than among healthy subjects. The probability of abnormal MTWA increases in patients with malignant VA, in males and among subjects with heart failure and cyanosis. MTWA might be of potential role in risk stratification for SCD in adults with CHD.

Clinical factors affecting survival in patients with D-transposition of the great arteries after atrial switch repair: A meta-analysis.

BACKGROUND: Atrial switch repair (AtrSR) was the initial operation method in patients with D-transposition of the great arteries (D-TGA) constituting the right ventricle as a systemic one. Currently, it has been replaced with arterial switch operation (ASO), but the cohort of adults after AtrSR is still large and requires strict cardiological management of late complications. For this reason, we aimed to evaluate potential long-term mortality risk factors in patients with D-TGA after AtrSR (either Mustard or Senning procedures) Methods: We searched the MEDLINE database for suitable trials. We included 22 retrospective and prospective cohort studies of patients with D-TGA with at least 5 years mean/median follow-up time after Mustard or Senning procedures, with an endpoint of non-sudden cardiac death (n-SCD) and sudden cardiac death (SCD) after at least 30 days following surgery. RESULTS: A total of 2912 patients were enrolled, of whom 351 met the combined endpoint of n-SCD/SCD. The long-term mortality risk factors were New York Heart Association (NYHA) class ≥III/heart failure hospitalization (odds ratio [OR], 7.25; 95% confidence interval [CI], 2.67-19.7), tricuspid valve regurgitation (OR, 4.64; 95% CI, 1.95-11.05), Mustard procedure (OR, 2.15; 95% CI, 1.37-3.35), complex D-TGA (OR, 2.41; 95% CI, 1.31-4.43), and right ventricular dysfunction (OR, 1.94; 95% CI, 0.99-3.79). Supraventricular arrhythmia (SVT; OR, 2.07; 95% CI, 0.88-4.85) and pacemaker implantation (OR, 2.37; 95% CI, 0.48-11.69) did not affect long-term survival in this group of patients. In an additional analysis, SVT showed a statistically significant impact on SCD (OR, 2.74; 95% CI, 1.36-5.53) but not on n-SCD (OR, 1.5; 95% CI, 0.37-6.0). CONCLUSIONS: This meta-analysis demonstrated that at least moderate tricuspid valve regurgitation, NYHA class ≥III/heart failure hospitalization, right ventricular dysfunction, complex D-TGA, and Mustard procedure are risk factors for long-term mortality in patients after AtrSR.

Pregnancy Outcomes in Women after the Fontan Procedure.

Women with single ventricle physiology after the Fontan procedure, despite numerous possible complications, can reach adulthood and give birth. Pregnancy poses a hemodynamic burden for distorted physiology of Fontan circulation, but according to the literature, it is usually well tolerated unless the patient is a "failing" Fontan. Our study aimed to assess maternal and fetal outcomes in patients after the Fontan procedure followed up in two tertiary Polish medical centers. We retrospectively evaluated all pregnancies in women after the Fontan procedure who were followed up between 1995-2022. During the study period, 15 women after the Fontan procedure had 26 pregnancies. Among 26 pregnancies, eleven ended with miscarriages, and 15 pregnancies resulted in 16 live births. Fetal complications were observed in 9 (56.3%) live births, with prematurity being the most common complication (n = 7, 43.8%). We recorded 3 (18.8%) neonatal deaths. Obstetrical complications were present in 6 (40%) out of 15 completed pregnancies-two (13.3%) cases of abruptio placentae, two (13.3%) pregnancies with premature rupture of membranes, and two (13.3%) patients with antepartum hemorrhage. There was neither maternal death nor heart failure decompensation during pregnancy. In two (13.3%) women, atrial arrhythmia developed. One (6.7%) patient in the second trimester developed ventricular arrhythmia. None of the patients suffered from systemic thromboembolism during pregnancy. Pregnancy in women after the Fontan procedure is well tolerated. However, it is burdened by a high risk of miscarriage and multiple obstetrical complications. These women require specialized care provided by both experienced cardiologists and obstetricians.

Out-of-hospital cardiac arrest: Do we have to perform coronary angiography?

Out-of-hospital cardiac arrest (OHCA) remains a leading cause of global mortality, while survivors are burdened with long-term neurological and cardiovascular complications. OHCA management at the hospital level remains challenging, due to heterogeneity of OHCA presentation, the critical status of OHCA patients reaching the return of spontaneous circulation (ROSC), and the demands of post ROSC treatment. The validity and optimal timing for coronary angiography is one important, yet not fully defined, component of OHCA management. Guidelines state clear recommendations for coronary angiography in OHCA patients with shockable rhythms, cardiogenic shock, or in patients with ST-segment elevation observed in electrocardiography after ROSC. However, there is no established consensus on the angiographic management in other clinical settings. While coronary angiography may accelerate the diagnostic and therapeutic process (provided OHCA was a consequence of coronary artery disease), it might come at the cost of impaired post-resuscitation care quality due to postponing of intensive care management. The aim of the current statement paper is to discuss clinical strategies for the management of OHCA including the stratification to invasive procedures and the rationale behind the risk-benefit ratio of coronary angiography, especially with patients in critical condition.

Preeclampsia and Fetal Growth Restriction as Risk Factors of Future Maternal Cardiovascular Disease-A Review.

Cardiovascular diseases (CVDs) remain the leading cause of death in women worldwide. Although traditional risk factors increase later-life CVD, pregnancy-associated complications additionally influence future CVD risk in women. Adverse pregnancy outcomes, including preeclampsia and fetal growth restriction (FGR), are interrelated disorders caused by placental dysfunction, maternal cardiovascular maladaptation to pregnancy, and maternal abnormalities such as endothelial dysfunction, inflammation, hypercoagulability, and vasospasm. The pathophysiologic pathways of some pregnancy complications and CVDs might be linked. This review aimed to highlight the associations between specific adverse pregnancy outcomes and future CVD and emphasize the importance of considering pregnancy history in assessing a woman's CVD risk. Moreover, we wanted to underline the role of maternal cardiovascular maladaptation in the development of specific pregnancy complications such as FGR.

Left, but not right, ventricular status determines heart failure in adults with Ebstein anomaly - A case-control study based on magnetic resonance.

BACKGROUND: Ebstein anomaly (EA) is a congenital heart defect affecting the right heart. Heart failure (HF) is a significant complication in adults with EA. It may result not only from the right ventricle (RV), but also from the left ventricle (LV) abnormalities. We evaluate the size and function of both ventricles in patients with EA in cardiac magnetic resonance (CMR); to assess their association with the clinical markers of HF. METHODS: Study group: 37 unoperated adults with EA (mean age 43.0 ± 14.4y, 21[56.8%] males). CONTROLS: 25 volunteers (mean age 39.9 ± 10.9y, 15[60%] males). Study protocol included: CMR [ejection fraction (EF), end-diastolic (EDVind) and stroke volumes (SVind) indexed by body surface area]; cardiopulmonary test (peak VO2, %peak VO2, VE/VCO2 slope). RESULTS: Size and systolic function of LV were reduced comparing to the controls [LVEDVind (ml/m2): 63.7(range 38.7-94.2) vs. 79.3(48.7-105.1), p < 0.001; LV SVind (ml/m2): 35.8(22.9-55.1) vs. 49.2(37.8-71.7), p < 0.0001; LVEF(%): 58.3(34-70.5) vs. 62.0(52.0-77.0), p = 0.009]. RV was enlarged comparing to the controls [RVEDVind (ml/m2): 124.3(52.8-378.9) vs. 83.0(64.0-102.0), p < 0.0001) with impaired systolic function (RV SVind (ml/m2): 22.7(11.1-74.1) vs. 48.0(37.8-71.7), p < 0.0001; RVEF(%): 38.0(21.0-66.1) vs. 59.0(49.0-69.0), p < 0.0001). A significant correlation was found between LVEDVind vs. peakVO2 (r = 0.52, p = 0.001); LV SVind vs. peakVO2 (r = 0.47,p = 0.005). There was no correlation between the right ventricular status and exercise capacity. CONCLUSIONS: In adults with Ebstein anomaly the size of left ventricle is reduced, right ventricle is enlarged; the function of both is impaired. Abnormal exercise capacity is associated with left ventricular status. Ventricular interdependence probably plays a role in heart failure pathomechanism.

Nuclear cardiology: an overview of radioisotope techniques used in the diagnostic workup of cardiovascular disorders.

Cardiovascular diseases are the most common cause of death in patients over 60 years old. Pivotal imaging modalities in cardiac diagnostic workup are echocardiography, magnetic resonance, multi­row detector computed tomography, coronary angiography, and radioisotope tests. In this study, we summarize the techniques of nuclear medicine (positron emission tomography, single­photon emission computed tomography, radionuclide ventriculography) that could be implemented in the cardiovascular diagnostic algorithms. Despite being acknowledged in a few cardiology guidelines, these imaging methods are still underestimated by practitioners. Nevertheless, noninvasive diagnostic tools are of increasing potential and should be implemented whenever possible. We discuss the usefulness of particular techniques in the management of patients with obstructive and nonobstructive coronary artery disease, including assessment of myocardial perfusion, contractility, viability, and detection of unstable atherosclerotic plaques. Radioisotope imaging can also be valuable in the diagnostic workup of infective endocarditis, as well as cardiac sarcoidosis and amyloidosis. Apart from theoretical principles of nuclear cardiology, we also provide 3 case reports illustrating a practical implementation of these imaging modalities.

Severity Scores for Ebstein Anomaly: Credibility and Usefulness of Echocardiographic vs Magnetic Resonance Assessments of the Celermajer Index.

BACKGROUND: The severity score of Ebstein anomaly (EA) that corresponds to clinical status is still under research, with the Celermajer index (Cel-ind) being one of those. The agreement between echocardiographic and cardiac magnetic resonance (CMR) assessment of Cel-ind is not known. We determined the agreement between echocardiography- and CMR-derived Cel-ind and its relationship with heart failure markers. METHODS: A total of 37 unoperated adults with EA (mean age, 43.0 ± 14.4 years) underwent echocardiography, CMR, and cardiopulmonary tests. For the Cel-ind, end-diastolic areas in echocardiography or end-diastolic volumes in CMR were used according to the following formula: Cel-ind = (right atrium + atrialized right ventricle)/(functional right ventricle + left atrium + left ventricle). On the basis of this assumption, patients were classified as follows: grade 1 = Cel-ind < 0.5, grade 2 = 0.5 to 0.99, grade 3 = 1.0 to 1.49, grade 4 > 1.5. The agreement between echocardiographic and CMR was determined with the intraclass correlation coefficient or Cohen's kappa (<0.2 poor agreement; 0.2-0.4 fair agreement; 0.4-0.6 moderate agreement; 0.6-0.8 good agreement; 0.8-1.0 very good agreement). RESULTS: The median echoCel-ind was 0.9 (range, 0.4-2.3), and the median cmrCel-ind was 0.7 (range, 0.3-5.3). Grade 1 or 2 was found in 19 patients (51.3%) by echocardiography and in 27 patients (72.9%) by CMR. The agreement between imaging methods was only fair (kappa = 0.39, P = 0.002) for the 4-grade classification and moderate (intra-class correlation coefficient = 0.43; 95% confidence interval, 0.13-0.66) for Cel-ind calculation. Significant correlations between Cel-ind in CMR and cardiopulmonary parameters were found (for peak oxygen uptake: R = -0.35, P = 0.034; for the ventilation/carbon dioxide slope: R = 0.46, P = 0.005). Neither of them correlated with echocardiographic severity score. CONCLUSIONS: The agreement between echocardiographic and CMR assessment of the Cel-ind is at most moderate; echocardiography usually overestimates, but rarely underestimates, EA severity. Cel-ind by CMR seems to be more valuable, because it is associated with heart failure markers.

Safety, feasibility and effectiveness of first in-human administration of muscle-derived stem/progenitor cells modified with connexin-43 gene for treatment of advanced chronic heart failure.

AIMS: To assess the safety and efficacy of transendocardial delivery of muscle-derived stem/progenitor cells with connexin-43 overexpression (Cx-43-MDS/PC) in advanced heart failure (HF). METHODS AND RESULTS: Thirteen subjects with advanced HF, New York Heart Association (NYHA) class II-III were enrolled and treated with targeted injection of Cx-43-MDS/PCs and then monitored for at least 6 months. Overexpression of Cx43 (Cx43+) was significantly higher in all but one subject (Cx43-). Injection of MDS/PCs was associated with significant improvement of exercise capacity: NYHA (3 ± 0 vs. 1.8 ± 0.7, P = 0.003), exercise duration (388.69 ± 141.83 s vs. 462.08 ± 176.69 s, P = 0.025), peak oxygen consumption (14.38 ± 3.97 vs. 15.83 ± 3.74 ml/kg.min, P = 0.022) and oxygen pulse (10.58 ± 2.89 vs. 18.88 ± 22.63 mLO2 /heart rate, P = 0.012). Levels of BNP, left ventricular (LV) ejection fraction and LV end-diastolic volumes tended to improve. There was a significant improvement of the mean unipolar voltage amplitudes measured for the injected segments and the entire left ventricle (9.62 ± 2.64 vs. 11.62 ± 3.50 mV, P = 0.014 and 8.83 ± 2.80 vs. 10.22 ± 3.41 mV, P = 0.041, respectively). No deaths were documented, Cx43+ (n = 12) subjects presented no significant ventricular arrhythmia; one Cx43- subject suffered from ventricular tachycardia (successfully treated with amiodarone). CONCLUSIONS: Injection of Cx-43-MDS/PCs in patients with severe HF led to significant improvement in exercise capacity and myocardial viability of the injected segments while inducing no significant ventricular arrhythmia. This may arise from improved electrical coupling of the injected cells and injured myocardium and thus better in-situ mechanical cooperation of both cell types. Therefore, further clinical studies with Cx43+ MDS/PCs are warranted.