Rita Amador , Joana Pereira , Pedro Freitas , Rita Lima , Mariana Paiva , Daniel Gomes , Carlos Eduardo Rochite , Edmundo Arteaga , Pedro Carmo , Diogo Cavaco , Pedro Adragão , António Ferreira
{"title":"Revisiting risk stratification in hypertrophic cardiomyopathy after the recent guidelines – Late gadolinium enhancement remains tough to beat","authors":"Rita Amador , Joana Pereira , Pedro Freitas , Rita Lima , Mariana Paiva , Daniel Gomes , Carlos Eduardo Rochite , Edmundo Arteaga , Pedro Carmo , Diogo Cavaco , Pedro Adragão , António Ferreira","doi":"10.1016/j.repc.2025.12.007","DOIUrl":"10.1016/j.repc.2025.12.007","url":null,"abstract":"<div><h3>Introduction and objectives</h3><div>Recommendations for implantable cardioverter-defibrillator (ICD) implantation to prevent sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM) have evolved over time, with late gadolinium enhancement (LGE) emerging as an important additional risk marker. Our study aimed to evaluate changes in the accuracy and discriminative performance of international guidelines for ICD recommendations and to determine whether LGE can improve this risk stratification.</div></div><div><h3>Methods</h3><div>We conducted a multicenter retrospective observational study of HCM patients who underwent cardiac magnetic resonance (CMR) imaging for diagnosis or risk assessment. ICD eligibility was determined based on the ESC guidelines (2014, 2022, 2023) and the American College of Cardiology (ACC) guidelines (2011, 2020, 2024). Our primary endpoint was a composite of sudden cardiac death (SCD), appropriate ICD discharge, or sustained ventricular tachycardia (VT).</div></div><div><h3>Results</h3><div>A total of 531 patients (median age 49 years; 57% male) were included. During a median follow-up of 49 months, twenty-eight events occurred (15 SCDs, six appropriate ICD discharges, seven sustained VTs). Over time, both European and American guidelines demonstrated improved sensitivity, positive predictive value, and discriminatory ability, while maintaining a high negative predictive value. The agreement between the recent ESC and ACC guidelines was moderate (κ≈0.6, p<0.001). Late gadolinium enhancement (LGE) was present in 80% of patients and LGE% independently predicted arrhythmic events (adjusted hazard ratio 1.09 per 1% increase; p<0.001). A burden ≥8% was the best predictor of events, while the absence of LGE identified patients without arrhythmic outcomes.</div></div><div><h3>Conclusions</h3><div>The extent of LGE burden improves risk stratification for SCD, with the complete absence of LGE indicating a very low-risk subgroup. LGE thresholds should be individualized by integrating imaging results, clinical factors and the patient's context.</div></div>","PeriodicalId":48985,"journal":{"name":"Revista Portuguesa De Cardiologia","volume":"45 4","pages":"Pages 171-178"},"PeriodicalIF":1.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147318899","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Agradecimento aos Revisores","authors":"","doi":"10.1016/S0870-2551(26)00159-9","DOIUrl":"10.1016/S0870-2551(26)00159-9","url":null,"abstract":"","PeriodicalId":48985,"journal":{"name":"Revista Portuguesa De Cardiologia","volume":"45 ","pages":"Pages VII-IX"},"PeriodicalIF":1.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148020624","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Índice por temas","authors":"","doi":"10.1016/S0870-2551(26)00167-8","DOIUrl":"10.1016/S0870-2551(26)00167-8","url":null,"abstract":"","PeriodicalId":48985,"journal":{"name":"Revista Portuguesa De Cardiologia","volume":"45 ","pages":"Pages S297-S319"},"PeriodicalIF":1.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148020847","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Margarida G. Figueiredo , José Miguel Viegas , Isabel Cardoso , Pedro Brás , Inês Almeida , Sofia Jacinto , Hélder Santos , Guilherme Portugal , Ana Lousinha , Pedro Silva Cunha , Silvia Aguiar Rosa , Mário Oliveira , Rui Cruz Ferreira
{"title":"Incremental value of implantable loop recorders in arrhythmia detection and management in cardiomyopathies: Prospective study","authors":"Margarida G. Figueiredo , José Miguel Viegas , Isabel Cardoso , Pedro Brás , Inês Almeida , Sofia Jacinto , Hélder Santos , Guilherme Portugal , Ana Lousinha , Pedro Silva Cunha , Silvia Aguiar Rosa , Mário Oliveira , Rui Cruz Ferreira","doi":"10.1016/j.repc.2025.12.008","DOIUrl":"10.1016/j.repc.2025.12.008","url":null,"abstract":"<div><h3>Introduction and objectives</h3><div>Implantable loop recorders (ILRs) are increasingly being used for the detection of infrequent arrhythmias in patients with cardiomyopathies, especially in the presence of risk markers. The role of these devices in improving the detection of significant arrhythmias requiring a change in clinical management remains to be determined. Our purpose was to evaluate the diagnostic yield, types of arrhythmia, and subsequent management in cardiomyopathy patients receiving an ILR.</div></div><div><h3>Methods</h3><div>Prospective single-centre study in cardiomyopathy patients considered at borderline risk for ventricular arrhythmias, who received an ILR. The primary endpoint was a meaningful arrhythmic event detection leading to a change in clinical management.</div></div><div><h3>Results</h3><div>A total of 45 patients were included, 51% were male, median age was 62 (48–71) years. The underlying disease was hypertrophic cardiomyopathy (HCM) in 31 patients (69%), dilated and non-dilated left ventricle cardiomyopathy (DCM/NDLVC) in 12 patients (26%) and transthyretin amyloid cardiomyopathy (ATTR-CM) in 2 patients (4%). The most frequent risk markers were brief run of nonsustained ventricular tachycardia in 42%, unexplained syncope/pre-syncope in 36%, family history of premature sudden cardiac death (SCD) in a first-degree relative in 36%, and palpitations suspicious of arrhythmic origin in 18% of patients. In the HCM cohort, median HCM Risk-SCD score was 3.07 (2.68–3.76)%, with 19% of patients having an estimated 5-year risk of SCD ≥4%. Mean maximum wall thickness was 20±4 mm, mean left atrial diameter was 43±7 mm, 23% of patients had obstructive HCM, late gadolinium enhancement (LGE) was present in 74% – with 52% of patients presenting extensive LGE – and left ventricle apical aneurysm in 3%. A sarcomeric pathogenic variant was identified in 26%. Among the DCM/NDLVC patients, 58% had left ventricular ejection <50%, 25% carried pathogenic/likely pathogenic variants on genetic testing and 25% exhibited an extensive ring-like scar pattern on cardiac magnetic resonance (CMR). During a mean follow-up of 19±13 months, 44% of patients had, at least, one ILR-guided diagnosis. De novo atrial fibrillation was diagnosed in 24% of patients and was the main detected event. Due to ILR-guided diagnosis, 20% (9 patients) received an implantable cardioverter-defibrillator (ICD), one of which with subsequent appropriate ICD-therapies.</div></div><div><h3>Conclusion</h3><div>This study provides insight into the possible role of ILR in this population, not only for the diagnosis of ventricular arrhythmias, but also for detection of atrial fibrillation, which can lead to a different clinical management.</div></div>","PeriodicalId":48985,"journal":{"name":"Revista Portuguesa De Cardiologia","volume":"45 4","pages":"Pages 193-202"},"PeriodicalIF":1.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147391024","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Seeing beneath the surface: Are current risk scores enough in hypertrophic cardiomyopathy?","authors":"Pedro Silva Cunha , Silvia Aguiar Rosa","doi":"10.1016/j.repc.2026.03.002","DOIUrl":"10.1016/j.repc.2026.03.002","url":null,"abstract":"","PeriodicalId":48985,"journal":{"name":"Revista Portuguesa De Cardiologia","volume":"45 4","pages":"Pages 179-181"},"PeriodicalIF":1.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147460609","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Índice por palavras-chave","authors":"","doi":"10.1016/S0870-2551(26)00166-6","DOIUrl":"10.1016/S0870-2551(26)00166-6","url":null,"abstract":"","PeriodicalId":48985,"journal":{"name":"Revista Portuguesa De Cardiologia","volume":"45 ","pages":"Pages S294-S296"},"PeriodicalIF":1.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148020622","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}