PMID- 31412875 OWN - NLM STAT- MEDLINE DCOM- 20200504 LR - 20240117 IS - 1532-429X (Electronic) IS - 1097-6647 (Print) IS - 1097-6647 (Linking) VI - 21 IP - 1 DP - 2019 Aug 15 TI - The amount of late gadolinium enhancement outperforms current guideline-recommended criteria in the identification of patients with hypertrophic cardiomyopathy at risk of sudden cardiac death. PG - 50 LID - 10.1186/s12968-019-0561-4 [doi] LID - 50 AB - BACKGROUND: Identifying the patients with hypertrophic cardiomyopathy (HCM) in whom the risk of sudden cardiac death (SCD) justifies the implantation of a cardioverter-defibrillator (ICD) in primary prevention remains challenging. Different risk stratification and criteria are used by the European and American guidelines in this setting. We sought to evaluate the role of cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) in improving these risk stratification strategies. METHODS: We conducted a multicentric retrospective analysis of HCM patients who underwent CMR for diagnostic confirmation and/or risk stratification. Eligibility for ICD was assessed according to the HCM Risk-SCD score and the American College of Cardiology Foundation/American Heart Association (ACCF/AHA) algorithm. The amount of LGE was quantified (LGE%) and categorized as 0%, 0.1-10%, 10.1-19.9% and >/= 20%. The primary endpoint was a composite of SCD, aborted SCD, sustained ventricular tachycardia (VT), or appropriate ICD discharge. RESULTS: A total of 493 patients were available for analysis (58% male, median age 46 years). LGE was present in 79% of patients, with a median LGE% of 2.9% (IQR 0.4-8.4%). The concordance between risk assessment by the HCM Risk-SCD, ACCF/AHA and LGE was relatively weak. During a median follow-up of 3.4 years (IQR 1.5-6.8 years), 23 patients experienced an event (12 SCDs, 6 appropriate ICD discharges and 5 sustained VTs). The amount of LGE was the only independent predictor of outcome (adjusted HR: 1.08; 95% CI: 1.04-1.12; p < 0.001) after adjustment for the HCM Risk-SCD and ACCF/AHA criteria. The amount of LGE showed greater discriminative power (C-statistic 0.84; 95% CI: 0.76-0.91) than the ACCF/AHA (C-statistic 0.61; 95% CI: 0.49-0.72; p for comparison < 0.001) and the HCM Risk-SCD (C-statistic 0.68; 95% CI: 0.59-0.78; p for comparison = 0.006). LGE was able to increase the discriminative power of the ACCF/AHA and HCM Risk-SCD criteria, with net reclassification improvements of 0.36 (p = 0.021) and 0.43 (p = 0.011), respectively. CONCLUSIONS: The amount of LGE seems to outperform the HCM Risk-SCD score and the ACCF/AHA algorithm in the identification of HCM patients at increased risk of SCD and reclassifies a relevant proportion of patients. FAU - Freitas, Pedro AU - Freitas P AUID- ORCID: 0000-0001-9968-477X AD - Cardiovascular Magnetic Resonance and Computed Tomography Sector, Heart Institute (InCor), University of Sao Paulo Medical School, Avenida Dr. Eneas de Carvalho Aguiar, 44, Andar AB, Cerqueira Cesar, Sao Paulo, SP, 05403-000, Brazil. AD - Cardiology Department, Hospital de Santa Cruz, Centro Hospitalar de Lisboa Ocidental, EPE, Lisbon, Portugal. FAU - Ferreira, Antonio Miguel AU - Ferreira AM AD - Cardiology Department, Hospital de Santa Cruz, Centro Hospitalar de Lisboa Ocidental, EPE, Lisbon, Portugal. AD - Advanced Cardiovascular Imaging Department, Hospital da Luz, Lisbon, Portugal. FAU - Arteaga-Fernandez, Edmundo AU - Arteaga-Fernandez E AD - Laboratory of Genetics and Molecular Cardiology and Cardiomyopathies Unit, Heart Institute (InCor), University of Sao Paulo Medical School, Sao Paulo, Brazil. FAU - de Oliveira Antunes, Murrilo AU - de Oliveira Antunes M AD - Laboratory of Genetics and Molecular Cardiology and Cardiomyopathies Unit, Heart Institute (InCor), University of Sao Paulo Medical School, Sao Paulo, Brazil. FAU - Mesquita, Joao AU - Mesquita J AD - Cardiology Department, Hospital de Santa Cruz, Centro Hospitalar de Lisboa Ocidental, EPE, Lisbon, Portugal. FAU - Abecasis, Joao AU - Abecasis J AD - Cardiology Department, Hospital de Santa Cruz, Centro Hospitalar de Lisboa Ocidental, EPE, Lisbon, Portugal. AD - Cardiovascular Department, Hospital dos Lusiadas, Lisbon, Portugal. FAU - Marques, Hugo AU - Marques H AD - Advanced Cardiovascular Imaging Department, Hospital da Luz, Lisbon, Portugal. FAU - Saraiva, Carla AU - Saraiva C AD - Radiology Department, Centro Hospitalar de Lisboa Ocidental, EPE, Lisbon, Portugal. FAU - Matos, Daniel Nascimento AU - Matos DN AD - Cardiology Department, Hospital de Santa Cruz, Centro Hospitalar de Lisboa Ocidental, EPE, Lisbon, Portugal. FAU - Rodrigues, Rita AU - Rodrigues R AD - Advanced Cardiovascular Imaging Department, Hospital da Luz, Lisbon, Portugal. FAU - Cardim, Nuno AU - Cardim N AD - Advanced Cardiovascular Imaging Department, Hospital da Luz, Lisbon, Portugal. FAU - Mady, Charles AU - Mady C AD - Laboratory of Genetics and Molecular Cardiology and Cardiomyopathies Unit, Heart Institute (InCor), University of Sao Paulo Medical School, Sao Paulo, Brazil. FAU - Rochitte, Carlos Eduardo AU - Rochitte CE AD - Cardiovascular Magnetic Resonance and Computed Tomography Sector, Heart Institute (InCor), University of Sao Paulo Medical School, Avenida Dr. Eneas de Carvalho Aguiar, 44, Andar AB, Cerqueira Cesar, Sao Paulo, SP, 05403-000, Brazil. rochitte@incor.usp.br. LA - eng PT - Journal Article PT - Multicenter Study DEP - 20190815 PL - England TA - J Cardiovasc Magn Reson JT - Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance JID - 9815616 RN - 0 (Contrast Media) SB - IM MH - Adult MH - Brazil MH - Cardiomyopathy, Hypertrophic/complications/*diagnostic imaging/therapy MH - Clinical Decision-Making MH - Contrast Media/*administration & dosage MH - Death, Sudden, Cardiac/*etiology/prevention & control MH - Defibrillators, Implantable MH - Electric Countershock/instrumentation MH - Female MH - Humans MH - Magnetic Resonance Imaging/*standards MH - Male MH - Middle Aged MH - Patient Selection MH - Portugal MH - Practice Guidelines as Topic/*standards MH - Predictive Value of Tests MH - Primary Prevention MH - Prognosis MH - Retrospective Studies MH - Risk Assessment MH - Risk Factors PMC - PMC6694533 OTO - NOTNLM OT - Hypertrophic cardiomyopathy OT - Risk stratification COIS- The authors declare that they have no competing interests. EDAT- 2019/08/16 06:00 MHDA- 2020/05/06 06:00 PMCR- 2019/08/15 CRDT- 2019/08/16 06:00 PHST- 2019/01/16 00:00 [received] PHST- 2019/07/01 00:00 [accepted] PHST- 2019/08/16 06:00 [entrez] PHST- 2019/08/16 06:00 [pubmed] PHST- 2020/05/06 06:00 [medline] PHST- 2019/08/15 00:00 [pmc-release] AID - S1097-6647(23)00223-5 [pii] AID - 561 [pii] AID - 10.1186/s12968-019-0561-4 [doi] PST - epublish SO - J Cardiovasc Magn Reson. 2019 Aug 15;21(1):50. doi: 10.1186/s12968-019-0561-4.