Figure 1
Ring-like LGE in patients with ACM and the hypokinetic nondilated LV phenotype. A: CMR showing normal LV volume and systolic function, together with linear ring-like LGE in the mid-anteroseptal, anterior, anterolateral and inferolateral segments (arrowheads), in a 62-year-old man with plakophilin 2-related ACM who presented with palpitations, a family history of ACM inverted T waves and epsilon waves in right precordial leads, and > 500 extrasystoles with LBBB morphology on 24-h Holter monitoring. B: CMR showing normal LV volume and systolic function, together with linear ring-like LGE in the basal and mid-anteroseptal, inferoseptal, and inferior segments (arrowheads), in a 46-year-old woman with LMNA-related ACM who had a family history of ACM and aborted sudden death and who presented with first degree atrioventricular block and LBBB.
Figure 2
Ring-like LGE in patients with ACM and the dilated LV phenotype. A: CMR showing severe LV dilatation and severe systolic dysfunction, together with linear circumferential ring-like LGE in the basal, middle, and apical LV (arrowheads), in a 28-year-old woman with desmoplakin-related ACM who presented with palpitations, heart failure, and a family history of premature sudden death, as well as low QRS voltages in limb leads, epsilon-like waves in inferior and left leads, and inverted T waves in left precordial leads. B: CMR showing LV dilatation and systolic dysfunction, together with linear circumferential ringlike LGE in the basal, middle, and apical LV (arrowheads), in a 42-year-old man with filamin-C–related ACM who presented with ventricular arrhythmias and family history of ACM.
Figure 3
Ring-like LGE in patients with familial DCM. A: CMR showing LV dilatation and systolic dysfunction, together with ring-like LGE in the basal and mid-anterior, anteroseptal, inferoseptal, and inferolateral segments (arrowheads), in a 47-year-old man with a troponin T2-related variant who presented with heart failure and a family history of premature sudden death, as well as atrial fibrillation, LBBB, and QRS fragmentation. B: CMR showing LV dilatation, systolic dysfunction, normal global native myocardial T1, and minimally increased myocardial extracellular volume, as well as ring-like LGE in the basal and mid-anterior, anteroseptal, inferoseptal, and inferior segments (arrowheads), in a 66-year-old man with a transthyretin-related variant who presented with heart failure, palpitations, and a family history of premature sudden death, together with negative 99mTc-pyrophosphate scintigraphy, first degree atrioventricular block, LBBB, and rare polymorphic ventricular contractions.
Figure 4
Ring-like LGE in patients with acute myocarditis. A,B: CMR showing LV dilatation, systolic dysfunction, and myocardial edema (A), with a prolonged T2 relaxation time (67 ms), as well as circumferential ring-like LGE in the middle and apical segments of the LV (arrowheads in B), in a 21-year-old woman with biopsy-proven acute lymphocytic myocarditis (presumably with a post-viral etiology) who presented with flu-like prodromes and fulminant myocarditis, with no family history of ACM or sudden premature death. C,D: CMR showing LV dilatation, systolic dysfunction, and myocardial edema with T2WI hyperintensity (C), together with ring-like LGE in the anterior, anteroseptal, inferoseptal, inferior basal, and inferior middle segments (arrowheads in D), in a 52-year-old woman with biopsy proven giant cell myocarditis who presented with fulminant myocarditis and no family history of ACM or sudden premature death.
Figure 5
CMR revealed pericardial effusion, normal LV volume and systolic function, myocardial edema (A) with prolonged myocardial T2 (58 ms), LGE in the RV (arrows in B), and ring-like LGE in the LV (arrowheads in B), in a 16-year-old woman who underwent heart transplantation because of idiopathic DCM and developed biopsy-proven acute cellular rejection two years after the surgery.
Figure 6
CMR, performed after the implantation of a pacemaker, showing severe LV dilatation, systolic dysfunction, and myocardial edema (A), with a prolonged T2 relaxation time (72 ms), together with circumferential ring-like LGE in the basal and middle segments (arrowheads in B), in a 48-year-old man with a biopsy-proven cardiac involvement in sarcoidosis who presented with total atrioventricular block. Axial magnetic resonance imaging scan of the chest, showing peribronchovascular interface irregularity (arrowheads in C), mediastinal lymph node enlargement (arrows in C), and pleural effusion.
Figure 7
CMR showing non-obstructive septal hypertrophy (A), as well as extensive, ill-defined, fibrosis centered in the anterior and septal segments (arrowheads in B), in a 54-year-old female with hyper-trophic cardiomyopathy who presented with syncope and progressive dyspnea, together with a family history of sudden death and myocardial hypertrophy. Follow-up CMR, performed eight years later, showing myocardial thinning, ventricular dilatation (C), and extensive confluent semi-circumferential myocardial fibrosis (arrowheads in D).
Figure 8
CMR showing non-obstructive, severe, symmetrical LV myocardial hypertrophy, and mid-wall multifocal ill-defined LGE (arrowheads in A), with sparing of the middle and basal septal segments, in a 27-year-old man with a previous diagnosis of Danon disease who had a family history of premature sudden death and myocardial hypertrophy. Laboratory tests showed elevated levels of transaminases, creatine kinase, and troponin. ECG revealed PR segment shortening, ventricular extrasystoles, and LV overload. Follow-up magnetic resonance imaging scan, acquired three years later, showing a reduction in myocardial thickness and LV dilation, together with confluent semi-circumferential mid-wall or subepicardial LGE involving in the anterior, anterolateral, infer-olateral, inferior middle, and inferior apical segments (arrowheads in B).
Figure 9
CMR showing severe LV systolic dysfunction and akinesia, as well as extensive semi-circumferential subepicardial LGE in the anterolateral, inferolateral, and inferior segments (arrowheads in A), with some transmural extension into the inferolateral segments, in a 22-year-old man with Duchenne muscular dystrophy who presented with progressive muscle weakness, significant physical limitation, and wheelchair dependence. B: Magnetic resonance imaging scan of the chest, showing fatty replacement of the muscles in the chest wall, particularly the periscapular muscles (arrows) and pectoralis muscles (arrowheads).
Figure 10
CMR showing LV dilatation, systolic dysfunction, apical aneurysm (arrowhead in A), and extensive confluent circumferential LGE (B), with a predominant subepicardial component (arrowheads), together with minor subendocardial foci (arrow) and minor transmural foci (double arrowheads), in a 53-year-old man with Chagas disease who presented with heart failure, right bundle block, and left anterosuperior fascicular block.
Figure 11
CMR showing LV dilatation and systolic dysfunction, together with extensive, multifocal, irregular, confluent semi-circumferential mid-wall LGE (arrowheads in A and B), in a 46-year-old man with Keshan disease who had previously undergone gastroplasty and presented with a severe malabsorption syndrome and subsequent heart failure. Laboratory tests revealed severe selenium deficiency.
Ring-like late gadolinium enhancement: differential diagnosis and mimics
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