{"doi":"10.1161/circulationaha.124.069378","title":"Long-Term Outcomes After Septal Reduction Therapies in Obstructive Hypertrophic Cardiomyopathy: Insights From the SHARE Registry","abstract":"BACKGROUND: Septal reduction therapy (SRT) provides substantial symptomatic improvement in patients with obstructive hypertrophic cardiomyopathy (HCM). However, long-term disease course after SRT and predictors of adverse outcomes have not been systematically examined. METHODS: Data from 13 high clinical volume HCM centers from the international SHARE (Sarcomeric Human Cardiomyopathy Registry) were analyzed. Patients were followed from the time of SRT until last follow-up or occurrence of heart failure (HF) composite outcome (cardiac transplantation, implantation of a left ventricular assist device, left ventricular ejection fraction <35%, development of New York Heart Association class III or IV symptoms), ventricular arrhythmias composite outcome (sudden cardiac death, resuscitated cardiac arrest, or appropriate implantable cardioverter defibrillator therapy), or HCM-related death. Cox proportional hazards models were used to identify predictors of outcome. RESULTS: Of the 10 225 patients in SHARE, 1832 (18%; 968 [53%] male) underwent SRT, including 455 (25%) with alcohol septal ablation and 1377 (75%) with septal myectomy. The periprocedural 30-day mortality rate was 0.4% (8 of 1832) and 1499 of 1565 (92%) had a maximal left ventricular outflow tract gradient <50 mm Hg at 1 year. After 6.8 years (range, 3.4–9.8 years; 12 565 person-years) from SRT, 77 (4%) experienced HCM-related death (0.6% per year), 236 (13%) a composite HF outcome (1.9% per year), and 87 (5%) a composite ventricular arrhythmia outcome (0.7% per year). Among adults, older age at SRT was associated with a higher incidence of HCM death (hazard ratio, 1.22 [95 CI, 1.1–1.3]; P <0.01) and the HF composite (hazard ratio, 1.14 [95 CI, 1.1–1.2] per 5-year increase; P <0.01) in a multivariable model. Female patients also had a higher risk of the HF composite after SRT (hazard ratio, 1.4 [95 CI, 1.1–1.8]; P <0.01). De novo atrial fibrillation occurred after SRT in 387 patients (21%). Among pediatric patients followed for a median of 13 years after SRT, 26 of 343 (16%) developed the HF composite outcome, despite 96% being free of recurrent left ventricular outflow tract obstruction. CONCLUSIONS: Successful short- and long-term relief of outflow tract obstruction was observed in experienced multidisciplinary HCM centers. A subset of patients progressed to develop HF, but event-free survival at 10 years was 83% and ventricular arrhythmias were rare. Older age, female sex, and SRT during childhood were associated with a greater risk of developing HF.","journal":"Circulation","year":2024,"id":418736,"datarank":0.9610143332957173,"base_score":3.8712010109078907,"endowment":3.8712010109078907,"self_citation_contribution":0.5806801516361837,"citation_network_contribution":0.38033418165953364,"self_endowment_contribution":0.5806801516361837,"citer_contribution":0.38033418165953364,"corpus_percentile":78.95876846909569,"corpus_rank":2721,"citation_count":47,"citer_count":40,"citers_with_citation_signal":17,"citers_with_endowment":17,"datacite_reuse_total":0,"is_dataset":true,"is_dataset_confidence":0.7373,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2024-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":345231,"name":"Panagiotis Antiochos","orcid":"0000-0001-8466-7360","position":1,"is_corresponding":false},{"id":465189,"name":"Anjali Owens","orcid":"0000-0002-9669-8495","position":2,"is_corresponding":false},{"id":1207925,"name":"Neal Lakdwala","orcid":null,"position":3,"is_corresponding":false},{"id":244332,"name":"Sara Saberi","orcid":"0000-0003-4473-6725","position":4,"is_corresponding":false},{"id":396007,"name":"Mark W. Russell","orcid":"0000-0003-4855-9260","position":5,"is_corresponding":false},{"id":1207241,"name":"Carlo Fumagalli","orcid":"0000-0001-7963-5049","position":6,"is_corresponding":false},{"id":1207242,"name":"Ioannis Skalidis","orcid":"0000-0002-4374-0389","position":7,"is_corresponding":false},{"id":630501,"name":"Kimberly Y. Lin","orcid":"0000-0003-1582-9797","position":8,"is_corresponding":false},{"id":787073,"name":"Ashwin S. Nathan","orcid":"0000-0002-7227-6423","position":9,"is_corresponding":false},{"id":1207926,"name":"Alejandro De Feria Alsina","orcid":null,"position":10,"is_corresponding":false},{"id":225626,"name":"Nosheen Reza","orcid":"0000-0002-2942-3731","position":11,"is_corresponding":false},{"id":402329,"name":"John C. Stendahl","orcid":"0000-0002-1568-9280","position":12,"is_corresponding":false},{"id":344968,"name":"Dominic J. Abrams","orcid":"0000-0001-5266-2805","position":13,"is_corresponding":false},{"id":1207927,"name":"Christopher Scemsarian","orcid":null,"position":14,"is_corresponding":false},{"id":1207928,"name":"Brian Clagget","orcid":null,"position":15,"is_corresponding":false},{"id":323292,"name":"Rachel Lampert","orcid":"0000-0003-3313-0939","position":16,"is_corresponding":false},{"id":11028,"name":"Matthew T. Wheeler","orcid":"0000-0001-8721-3022","position":17,"is_corresponding":false},{"id":30821,"name":"Victoria N. Parikh","orcid":"0000-0002-5138-5559","position":18,"is_corresponding":false},{"id":3451,"name":"Euan A. Ashley","orcid":"0000-0001-9418-9577","position":19,"is_corresponding":false},{"id":232952,"name":"Michelle Michels","orcid":"0000-0001-6432-0431","position":20,"is_corresponding":false},{"id":361421,"name":"Joseph W. Rossano","orcid":"0000-0002-8284-0673","position":21,"is_corresponding":false},{"id":516257,"name":"Thomas D. Ryan","orcid":"0000-0003-4237-576X","position":22,"is_corresponding":false},{"id":244335,"name":"Jodie Ingles","orcid":"0000-0002-4846-7676","position":23,"is_corresponding":false},{"id":230115,"name":"James S. Ware","orcid":"0000-0002-6110-5880","position":24,"is_corresponding":false},{"id":1207929,"name":"Carolyn Ho","orcid":null,"position":25,"is_corresponding":false},{"id":244331,"name":"Adam Helms","orcid":"0000-0002-0234-6430","position":26,"is_corresponding":false},{"id":5580,"name":"Sharlene M. 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