title: Cardiovascular magnetic resonance patterns of biopsy proven cardiac involvement in systemic sclerosis.
abstract: BACKGROUND
  To determine morphological and functional cardiovascular magnetic resonance (CMR) patterns in histopathologically confirmed myocardial involvement in patients with systemic sclerosis (SSc).
  
  
  METHODS
  Twenty patients (6 females; mean age 41 ± 11 years) with histopathologically proven cardiac involvement in SSc in the years 2008-2016 were retrospectively evaluated. Morphological, functional and late gadolinium enhancement (LGE) images were acquired in standard angulations at 1.5 T CMR. Pathologies were categorized: 1) Pericardial effusion; 2) pathologic left (LV) or right ventricular (RV) contractility (hypokinesia, dyssynchrony, and diastolic restriction); 3) reduced left (LV-EF) and right ventricular ejection fraction (RV-EF); 4) fibrosis and/or inflammation (positive LGE); 5) RV dilatation. 95 % confidence intervals (CI) were calculated for appearance of pathologic EF and RV dilatation.
  
  
  RESULTS
  Seven patients (35 %) had positive CMR findings in three categories, 9 patients (45 %) in four categories and 4 patients (20 %) in five categories. The distribution of pathologic findings was: minimal pericardial effusion in 7 patients (35 %), moderate pericardial effusion >5 mm in nine patients (45 %); abnormal LV or RV contractility in 19 patients (95 %), reduced LV or RV function in 14 patients (70 %; 95 % CI: 51-88 %), pathologic LGE in all patients, RV dilatation in 6 patients (30 %; 95 % CI: 15-54 %).
  
  
  CONCLUSIONS
  CMR diagnosis of myocardial involvement in SSc requires increased attention to subtle findings. Pathologic findings in at least three of five categories indicate myocardial involvement in SSc.
authors.affiliation: Department of Radiology, Diagnostic and Interventional Radiology, University of Tübingen, Hoppe-Seyler-Str. 3, Tübingen, 72076, Germany. patrick.krumm@uni-tuebingen.de.
authors.forename: Patrick
authors.identifier: http://orcid.org/0000-0003-1705-8439
authors.initials: P
authors.lastname: Krumm
authors.affiliation: Department of Internal Medicine III, Cardiology and Cardiovascular Medicine, University of Tübingen, Tübingen, Germany.
authors.forename: Karin A L
authors.initials: KA
authors.lastname: Mueller
authors.affiliation: Department of Pathology and Neuropathology, Molecular Pathology, University of Tübingen, Tübingen, Germany.
authors.forename: Karin
authors.initials: K
authors.lastname: Klingel
authors.affiliation: Department of Radiology, Diagnostic and Interventional Radiology, University of Tübingen, Hoppe-Seyler-Str. 3, Tübingen, 72076, Germany.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Kramer
authors.affiliation: Department of Radiology, Diagnostic and Interventional Radiology, University of Tübingen, Hoppe-Seyler-Str. 3, Tübingen, 72076, Germany.
authors.forename: Marius S
authors.initials: MS
authors.lastname: Horger
authors.affiliation: Department of Radiology, Diagnostic and Interventional Radiology, University of Tübingen, Hoppe-Seyler-Str. 3, Tübingen, 72076, Germany.
authors.forename: Tanja
authors.initials: T
authors.lastname: Zitzelsberger
authors.affiliation: Department of Pathology and Neuropathology, Molecular Pathology, University of Tübingen, Tübingen, Germany.
authors.forename: Reinhard
authors.initials: R
authors.lastname: Kandolf
authors.affiliation: Department of Internal Medicine III, Cardiology and Cardiovascular Medicine, University of Tübingen, Tübingen, Germany.
authors.forename: Meinrad
authors.initials: M
authors.lastname: Gawaz
authors.affiliation: Department of Radiology, Diagnostic and Interventional Radiology, University of Tübingen, Hoppe-Seyler-Str. 3, Tübingen, 72076, Germany.
authors.forename: Konstantin
authors.initials: K
authors.lastname: Nikolaou
authors.affiliation: Department of Radiology, Diagnostic and Interventional Radiology, University of Tübingen, Hoppe-Seyler-Str. 3, Tübingen, 72076, Germany.
authors.forename: Bernhard D
authors.initials: BD
authors.lastname: Klumpp
authors.affiliation: Department of Internal Medicine II, Rheumatology, University of Tübingen, Tübingen, Germany.
authors.forename: Joerg C
authors.initials: JC
authors.lastname: Henes
mesh_terms: D000328:Adult; D001706:Biopsy; D009202:Cardiomyopathies / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D003287:Contrast Media / Q000008:administration & dosage; D005260:Female; D006801:Humans; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D008875:Middle Aged; D009200:Myocardial Contraction; D009206:Myocardium / Q000473:pathology*; D009942:Organometallic Compounds / Q000008:administration & dosage; D010490:Pericardial Effusion / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications* / Q000175:diagnosis; D013318:Stroke Volume; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D018497:Ventricular Dysfunction, Right / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D016277:Ventricular Function, Left; D016278:Ventricular Function, Right
chemical_list: D003287:Contrast Media; D009942:Organometallic Compounds; C090600:gadobutrol
keywords: Cardiomyopathy; Cardiovascular magnetic resonance; Rheumatic heart disease; Systemic sclerosis