Transcatheter Ablation of Atrial Fibrillation in Patients With Hypertrophic Cardiomyopathy: A Multicenter Propensity Score-Based Analysis.

Background: The prognostic impact of catheter ablation (CA) of atrial fibrillation (AF) in hypertrophic cardiomyopathy (HCM) patients has not yet been satisfactorily elucidated. Objectives: The aim of the study was to assess the impact of CA of AF on clinical outcomes in a large cohort of HCM patients. Methods: In this retrospective multicenter study, 555 HCM patients with AF were enrolled, 140 undergoing CA and 415 receiving medical therapy. 1:1 propensity score matching led to the inclusion of 226 patients (113 medical group, 113 intervention group) in the final analysis. The primary outcome was a composite of all-cause mortality, heart transplant and acute heart failure exacerbations. Secondary outcomes included AF recurrence and transition to permanent AF. Additionally, an inverse probability weighted (IPW) model was examined. Results: At propensity score matching analysis, after a median follow-up of 58.1 months, the primary endpoint occurred in 29 (25.7%) patients in intervention group vs 42 (37.2%) in medical group (P = 0.9). Thromboembolic strokes and major arrhythmic events in intervention vs medical group were 9.7% vs 7.1% (P = 0.144) and 4.4 vs 8.0% (P = 0.779), respectively. Fewer patients in intervention vs medical group experienced AF recurrences (63.7% vs 84.1%, P = 0.001) and transition to permanent AF pattern (20.4% vs 33.6%, P = 0.026). IPW analysis showed consistent results. Severe complications related to CA were uncommon (0.7%). Conclusions: After 5 years of follow-up, CA did not improve major adverse cardiac outcomes in a large cohort of patients with HCM and AF. Nevertheless, CA seems to facilitate the maintenance of sinus rhythm and slow the progression to permanent AF, without significant safety concerns.

Intramyocardial calcification in apical hypertrophic cardiomyopathy assessed using multimodality imaging: a case series.

Apical hypertrophic cardiomyopathy (ApHCM) is an HCM variant, affecting frequently males in midlife. It is characterized by apical obliteration and persistent diastolic contraction, often resulting in microvascular ischaemia. We report five cases of ApHCM, with evidence of intramyocardial calcification on echocardiogram. On cardiac magnetic imaging (MRI), a hypointense component at early gadolinium enhancement (EGE) sequences, compatible with calcium, and a deep layer, with hyperintensity at late gadolinium enhancement (LGE) sequences, referable to fibrosis, suggest an endomyocardial fibrosis (EMF) diagnosis. EMF pathologic hallmark is endocardium and myocardium scarring, evolving to dystrophic calcification. It is found only in few ApHCM patients. Our series is the largest one described until now. Analysing patients' history, coexistent inflammatory triggers were evident in all of them, so their co-morbidities could represent a further cause of small vessel disease, in the context of ischaemic microvascular stress due to hypertrophy, leading to fibrosis and dystrophic calcification. This series could demonstrate the relation between apical fibrosis/calcification and microvascular ischaemia due to hypertrophy and inflammatory triggers.

Real-world candidacy to mavacamten in a contemporary hypertrophic obstructive cardiomyopathy population.

AIMS: In the EXPLORER-HCM trial, mavacamten reduced left ventricular outflow tract obstruction (LVOTO) and improved functional capacity of symptomatic hypertrophic obstructive cardiomyopathy (HOCM) patients. We sought to define the potential use of mavacamten by comparing real-world HOCM patients with those enrolled in EXPLORER-HCM and assessing their eligibility to treatment. METHODS AND RESULTS: We collected information on HOCM patients followed up at 25 Italian HCM outpatient clinics and with significant LVOTO (i.e. gradient ≥30 mmHg at rest or ≥50 mmHg after Valsalva manoeuvre or exercise) despite pharmacological or non-pharmacological therapy. Pharmacological or non-pharmacological therapy resolved LVOTO in 1044 (61.2%) of the 1706 HOCM patients under active follow-up, whereas 662 patients (38.8%) had persistent LVOTO. Compared to the EXPLORER-HCM trial population, these real-world HOCM patients were older (62.1 ± 14.3 vs. 58.5 ± 12.2 years, p = 0.02), had a lower body mass index (26.8 ± 5.3 vs. 29.7 ± 4.9 kg/m2 , p < 0.0001) and a more frequent history of atrial fibrillation (21.5% vs. 9.8%, p = 0.027). At echocardiography, they had lower left ventricular ejection fraction (LVEF, 66 ± 7% vs. 74 ± 6%, p < 0.0001), higher left ventricular outflow tract gradients at rest (60 ± 27 vs. 52 ± 29 mmHg, p = 0.003), and larger left atrial volume index (49 ± 16 vs. 40 ± 12 ml/m2 , p < 0.0001). Overall, 324 (48.9%) would have been eligible for enrolment in the EXPLORER-HCM trial and 339 (51.2%) for treatment with mavacamten according to European guidelines. CONCLUSIONS: Real-world HOCM patients differ from the EXPLORER-HCM population for their older age, lower LVEF and larger atrial volume, potentially reflecting a more advanced stage of the disease. About half of real-world HOCM patients were found eligible to mavacamten.

Combination of percutaneous left appendage epicardial ligation and endo-epicardial atrial fibrillation ablation.

Introduction: Atrial fibrillation (AF) is the main cause of cardioembolic stroke. In high-bleeding-risk patients, long-life anticoagulation therapy is not permitted, and left atrial appendage (LAA) closure may be considered. LAA is also a critical substrate for AF. Epicardial LAA occlusion has several advantages: LAA ligation results in a favorable electrical and structural atrial remodeling, which decreases AF recurrence. Endocardial ablation alone is not efficient for all patients, and new evidence shows better outcomes in patients affected by persistent AF after a combined hybrid endo-epicardial ablation. Considering the synergic potential of these techniques, in this case series, they were both combined in a single procedure. Methods and results: We describe the treatment of 5 patients referred for refractory AF ablation and LAA closure. All patients had high thrombotic and previous major hemorrhage, with relative contraindication to life-long therapy with anticoagulation. A combined procedure of LAA ligation and endo-epicardial ablation was scheduled with short-term anticoagulation. LAA closure was performed with an epicardial approach using the LARIAT system. Then, LA mapping and ablation were performed, endocardially and then epicardially.All procedures were concluded without complications.At follow-up, in all patients, transesophageal echocardiography showed the complete occlusion of the LAA; therefore, anticoagulation therapy was interrupted. All patients were asymptomatic, and in the sinus rhythm, no hemorrhage or ischemic events occurred. Conclusion: The combination of percutaneous LAA ligation and endo-epicardial ablation was revealed to be feasible and safe and might represent a new approach for the treatment of refractory AF in patients with indication of LAA occlusion.

Left Atrial Anomalous Muscular Band: Case Report, Literature Review, and Role of Three-Dimensional Echocardiography.

Anomalous fibromuscular bands in the left atrium were already described in the 19th century. Recently, the greater attention to the anatomy of the left atrium and the technological improvement have made their finding more frequent. Here, we present six cases, out of approximately 30,000 unselected echocardiograms, in which the use of the three-dimensional echo allowed a better definition of their anatomy, course, and motility.

[The importance of intensive lipid-lowering therapy after acute coronary syndrome: changing the paradigm to improve the achievement of targets]. / L'importanza di una terapia ipolipemizzante intensiva dopo sindrome coronarica acuta: cambiare il paradigma per migliorare il raggiungimento dei target.

BACKGROUND: Despite the availability of effective lipid-lowering drugs, only few high-risk patients attain their LDL cholesterol (LDL-C) guideline-recommended risk-based goal because of underprescription of combination therapy. We present an 18-month experience with variation of prescription protocols after publication of the 2019 ESC/EAS guidelines for the management of dyslipidemias. METHODS: Overall, 621 consecutive patients hospitalized for acute coronary syndrome at Mauriziano Hospital in Turin, Italy, between January 2020 and June 2021 were enrolled. Lipid-lowering therapy recommended at discharge was registered to evaluate how many patients received statin monotherapy, statin plus ezetimibe combination or triple therapy with high-intensity statin plus ezetimibe and proprotein convertase subtilisin/kexin type 9 inhibitor (PCSK9i). At 6-month follow-up, the reduction in LDL-C, adverse events, compliance and cardiovascular recurrences was analyzed. RESULTS: Of 621 patients enrolled, 7 died during hospitalization. During the entire study period, 33% of patients received statin monotherapy, 50% were discharged on statin-ezetimibe combination, and PCSK9i (evolocumab) was prescribed to 17% of patients. Between April 2020 and June 2021, when new recommendations were introduced into clinical practice, 20% of patients received evolocumab, 56% combination therapy and only 24% were discharged on statin monotherapy. At the beginning of observation, evolocumab was prescribed to 3% of patients hospitalized for acute coronary syndrome, while at the end of the study period 27% of patients were discharged on PCSK9i, with an increase of the prescription rate by 759%; in the same period, prescription of statin monotherapy decreased by 75%. At 6-month follow-up, LDL-C reduction was 77% in patients treated with PCSK9i vs 48% in patients taking statin-ezetimibe combination therapy (p<0.001). All patients on evolocumab reached the guideline-directed goals and a low rate of adverse events was reported, mainly represented by local injection site reactions. Six patients experienced acute coronary syndrome recurrence; only one of them was treated with evolocumab. CONCLUSION: Prescription of intensive lipid-lowering therapy after acute coronary syndrome, eventually with introduction of PCSK9i during hospitalization or at discharge, leads to attainment of guideline-recommended goals for all patients, with a low incidence of adverse events and optimal compliance.

Conduction disorders as the first hallmark of isolated cardiac sarcoidosis in a highly active individual: a case report.

BACKGROUND: Cardiac sarcoidosis (CS) is an inflammatory disease with various clinical presentations depending on the extension of cardiac involvement. The disease is often clinically silent, therefore diagnosis is challenging. CASE SUMMARY: We discuss the case of a middle-aged highly active individual presenting with an occasional finding of low heart rate during self-monitoring. The electrocardiogram shows a Mobitz 2 heart block; thanks to multimodality imaging CS was diagnosed and corticosteroid therapy improved cardiac conduction. DISCUSSION: To our knowledge, this is one of the first documented cases of occasional, early findings of CS in a middle-aged highly active individual who presented with cardiac conduction involvement. Despite the very early diagnosis, multimodality imaging suggested an advanced disease with no oedema detection at the cardiac magnetic resonance. Nevertheless, prompt corticosteroid therapy was able to improve clinical conduction. Although non-sustained ventricular arrhythmias were detected, electrophysiological study allowed to discharge the patient safely without implantable cardioverter-defibrillator implantation. Light-to-moderate physical activity was allowed at mid-term follow-up. A multidisciplinary evaluation should be considered to resume a high-intensity training.

Late gadolinium enhancement on cardiac magnetic resonance and phenotypic expression in hypertrophic cardiomyopathy.

BACKGROUND: Cardiac magnetic resonance (CMR) imaging with late gadolinium enhancement (LGE) can identify areas of myocardial fibrosis in vivo in patients with hypertrophic cardiomyopathy (HCM). The aim of this study was to examine the association between clinical-morphological variables, risk factor for sudden death, and LGE findings in a consecutive, unselected population of HCM patients. METHODS: From January 2005 to August 2009, 124 HCM patients (53 ± 17 years, 86 men) were prospectively evaluated with CMR examination, assessing left ventricular (LV) hypertrophy, function, and LGE. RESULTS: In univariate analysis, patients were divided into tertiles according to the number of segments positive for LGE (first tertile, 0.3 ± 0.4; second tertile, 2.2 ± 0.4; third tertile, 5.2 ± 1.9 segments). Male gender (P = .05), maximum LV wall thickness (P = .002), nonsustained ventricular tachycardia (P = .001), ejection fraction <50% (P = .02), LV mass (P = .02), left atrium dilation (P = .04), perfusion defects (P ≤ .001), and telesystolic volume (P = .04) were all positively related with the number of segments of LGE. In multivariable analysis, male gender (P = .007), maximum LV wall thickness (P = .006), LV mass (P = .031), and perfusion alterations (P = .017) were independent predictors of LGE extent. CONCLUSIONS: Our study shows an independent association, even at multivariate analysis, between the entity of LGE and maximum LV wall thickness, mass, and perfusion defects in patients with HCM. Whether the presence and the extent of LGE translates into clinical events later on awaits further long-term follow-up studies.