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Diagnostic value of late gadolinium enhancement at cardiovascular magnetic resonance to distinguish arrhythmogenic right ventricular cardiomyopathy from differentials.
Rekker, Lian Y; Muller, Steven A; Gasperetti, Alessio; Bourfiss, Mimount; Oerlemans, Marish I F J; Cramer, Maarten J; Zimmerman, Stefan L; Dooijes, Dennis; Schalkx, Hanke; van der Harst, Pim; James, Cynthia A; van Tintelen, J Peter; Guglielmo, Marco; Velthuis, Birgitta K; Te Riele, Anneline S J M.
Afiliación
  • Rekker LY; Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands.
  • Muller SA; Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands; Netherlands Heart Institute, Utrecht, the Netherlands.
  • Gasperetti A; Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands; Division of Medicine, Department of Cardiology, Johns Hopkins University, Baltimore, Maryland, USA; Department of Clinical Electrophysiology & Cardiac Pacing, Centro Cardiologico Monzino, IRCCS, Milano, Italy.
  • Bourfiss M; Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands.
  • Oerlemans MIFJ; Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands.
  • Cramer MJ; Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands.
  • Zimmerman SL; Department of Radiology, Johns Hopkins University, Baltimore, Maryland, USA.
  • Dooijes D; Department of Genetics, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands.
  • Schalkx H; Department of Radiology, University Medical Center Utrecht, Utrecht, the Netherlands.
  • van der Harst P; Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands.
  • James CA; Division of Medicine, Department of Cardiology, Johns Hopkins University, Baltimore, Maryland, USA.
  • van Tintelen JP; Netherlands Heart Institute, Utrecht, the Netherlands; Department of Genetics, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands.
  • Guglielmo M; Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands.
  • Velthuis BK; Department of Radiology, University Medical Center Utrecht, Utrecht, the Netherlands.
  • Te Riele ASJM; Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands; Netherlands Heart Institute, Utrecht, the Netherlands. Electronic address: ariele@umcutrecht.nl.
J Cardiovasc Magn Reson ; 26(2): 101059, 2024 Jul 08.
Article en En | MEDLINE | ID: mdl-38986843
ABSTRACT

BACKGROUND:

While late gadolinium enhancement (LGE) is proposed as a diagnostic criterion for arrhythmogenic right ventricular cardiomyopathy (ARVC), the potential of LGE to distinguish ARVC from differentials remains unknown. We aimed to assess the diagnostic value of LGE for ARVC diagnosis.

METHODS:

We included 132 subjects (60% male, 47 ± 11 years) who had undergone cardiac magnetic resonance imaging with LGE assessment for ARVC or ARVC differentials. ARVC was diagnosed as per 2010 Task Force Criteria (n = 55). ARVC differentials consisted of familial/genetic dilated cardiomyopathy (n = 25), myocarditis (n = 13), sarcoidosis (n = 20), and amyloidosis (n = 19). The diagnosis of all differentials was based on the most current standard of reference. The presence of LGE was evaluated using a 7-segment right ventricle (RV) and 17-segment left ventricle (LV) model. Subsequently, we assessed LGE patterns for every patient individually for fulfilling LV- and/or RV-LGE per Padua criteria, independent of their clinical diagnosis (i.e. phenotype). Diagnostic values were analyzed using sensitivity and specificity for any RV-LGE, any LV-LGE, RV-LGE per Padua criteria, and prevalence graphs for LV-LGE per Padua criteria. The optimal integration of LGE for ARVC diagnosis was determined using classification and regression tree analysis.

RESULTS:

One-third (38%) of ARVC patients had RV-LGE, while half (51%) had LV-LGE. RV-LGE was less frequently observed in ARVC vs non-ARVC patients (38% vs 58%, p = 0.034) leading to a poor discriminatory potential (any RV-LGE sensitivity 38%, specificity 42%; RV-LGE per Padua criteria sensitivity 36%, specificity 44%). Compared to ARVC patients, non-ARVC patients more often had LV-LGE (91% vs 51%, p < 0.001) which was also more globally distributed (median 9 [interquartile range (IQR) 3-13] vs 0 [IQR 0-3] segments, p < 0.001). The absence of anteroseptal and absence of extensive (≥5 segments) mid-myocardial LV-LGE, and absence of moderate (≥2 segments) mid-myocardial LV-LGE predicted ARVC with good diagnostic performance (sensitivity 93%, specificity 78%).

CONCLUSION:

LGE is often present in ARVC differentials and may lead to false positive diagnoses when used without knowledge of LGE patterns. Moderate RV-LGE without anteroseptal and mid-myocardial LV-LGE is typically observed in ARVC.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: J Cardiovasc Magn Reson Asunto de la revista: ANGIOLOGIA / CARDIOLOGIA / DIAGNOSTICO POR IMAGEM Año: 2024 Tipo del documento: Article País de afiliación: Países Bajos Pais de publicación: Reino Unido

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: J Cardiovasc Magn Reson Asunto de la revista: ANGIOLOGIA / CARDIOLOGIA / DIAGNOSTICO POR IMAGEM Año: 2024 Tipo del documento: Article País de afiliación: Países Bajos Pais de publicación: Reino Unido