{
  "abstract": "Background Cardiac involvement is a major cause of morbidity and mortality in Fabry disease, progressing from myocardial injury to fibrosis, arrhythmia, and heart failure. While cardiovascular magnetic resonance imaging (CMR) detects oedema and fibrosis, a more sensitive marker of active inflammation is needed.Objectives To evaluate somatostatin receptor 2 (SST2) positron emission tomography (PET)/CMR using 68Ga-DOTATATE for imaging myocardial inflammation in Fabry disease.Methods Twelve patients with Fabry disease and cardiac involvement underwent prospective 68Ga-DOTATATE PET/CMR. PET uptake was assessed visually and quantified using standardised uptake values (SUV) in all 17 myocardial segments. Associations with clinical, biochemical, and CMR markers were assessed using non-parametric tests and mixed-effects modelling (figure 1).Results Patients were aged 62 (IQR 57–70) years; 58% male; 50% classical phenotype. All received disease-modifying therapy. Focal 68Ga-DOTATATE uptake was present in all patients with heterogeneous regional distribution. Segmental PET uptake was higher in segments with LGE (p<0.001) and T2 oedema (p=0.001). At the segmental level, quantitative T1 mapping independently predicted PET uptake (p<0.001), whilst quantitative T2 mapping showed a non-significant trend (p=0.105). Global PET signal correlated with NT-proBNP (ρ=0.66, p=0.020), hsCRP (ρ=0.71, p=0.022), and age (ρ=0.64, p=0.027). Discordance between PET and CMR was observed in 11/204 (5%) PET+/LGE-, 33/204 (16%) PET+/T2-, and 22/192 (11%) PET+/T1- normal segments, suggesting PET detects inflammation beyond structural MRI abnormality (figure 2–5).Conclusions In this first study of SST2 PET/CMR in Fabry cardiomyopathy, focal myocardial 68Ga-DOTATATE uptake correlated with CMR and clinical markers of disease severity and identified segments with active inflammation not detected by MRI. In this proof-of-principle study of 68Ga-DOTATATE PET/CMR in Fabry cardiomyopathy, which of the following best describes the relationship between PET uptake and myocardial T1 mapping at the segment level?A) Segmental PET uptake was higher in segments with low native T1, consistent with glycosphingolipid storageB) Segmental PET uptake correlated positively with native T1 values, independent of LGE status (β=0.001, p<0.001) (CORRECT)C) Native T1 mapping did not correlate with PET uptake at the segment levelD) Segmental PET uptake correlated with native T1 only in segments with co-existing late gadolinium enhancementAbstract 6 Figure 5Abstract 6 Figure 4Abstract 6 Figure 3Abstract 6 Figure 2Abstract 6 Figure 1E) Native T2 mapping was a stronger predictor of segmental PET uptake than native T1 mapping",
  "authors": [
    {
      "affiliations": [
        "Division of Cardiorespiratory Medicine, Victor Phillip Dahdaleh Heart & Lung Research Institute, University of Cambridge, Cambridge, UK"
      ],
      "name": "George Collins"
    },
    {
      "affiliations": [
        "Division of Cardiorespiratory Medicine, Victor Phillip Dahdaleh Heart & Lung Research Institute, University of Cambridge, Cambridge, UK"
      ],
      "name": "Andrej Ćorović"
    },
    {
      "affiliations": [
        "Lysosmal Disorders Unit, Department of Medicine, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK"
      ],
      "name": "Carmen Minea"
    },
    {
      "affiliations": [
        "Division of Cardiorespiratory Medicine, Victor Phillip Dahdaleh Heart & Lung Research Institute, University of Cambridge, Cambridge, UK"
      ],
      "name": "Christopher Wall"
    },
    {
      "affiliations": [
        "Department of Radiology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK"
      ],
      "name": "Sumit Karia"
    },
    {
      "affiliations": [
        "Department of Radiology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK"
      ],
      "name": "Marusa Kotnik"
    },
    {
      "affiliations": [
        "Division of Cardiorespiratory Medicine, Victor Phillip Dahdaleh Heart & Lung Research Institute, University of Cambridge, Cambridge, UK"
      ],
      "name": "Marta Peverelli"
    },
    {
      "affiliations": [
        "Department of Cardiology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK"
      ],
      "name": "Rosemary A Rusk"
    },
    {
      "affiliations": [
        "Department of Radiology, Imperial College Healthcare NHS Foundation Trust, Cambridge, UK"
      ],
      "name": "Deepa Gopalan"
    },
    {
      "affiliations": [
        "Lysosmal Disorders Unit, Department of Medicine, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK"
      ],
      "name": "Patrick B Deegan"
    },
    {
      "affiliations": [
        "Division of Cardiorespiratory Medicine, Victor Phillip Dahdaleh Heart & Lung Research Institute, University of Cambridge, Cambridge, UK"
      ],
      "name": "Jason M Tarkin"
    }
  ],
  "title": "6 Myocardial inflammation imaging in Fabry disease of the heart using somatostatin receptor PET/MRI",
  "uid": "2ccbeaf6-ef8f-5530-b51f-4f619690dc9a"
}