Main demographic, clinical, and cardiac MRI characteristics of the 23 patients with ECD included in the study
| Pt. no. . | Age (y), sex . | TTD (mo) . | Extracardiac involvement . | Myocardial lesions on MRI . | Pericardial lesions on MRI . | MRI EF % . | Thoracic vessel involvement on MRI . | BRAF status . |
|---|---|---|---|---|---|---|---|---|
| 1 | 46, M | 55 | B, RP, HPG, O | Right atrial pseudomass | Pericardial thickening | 68 | AA, AArch, BT, LSA, LCCA | Wild type |
| Right AV sulcus infiltration and right pericoronary infiltration | Massive pericardial effusion | |||||||
| 2 | 47, M | 117 | B, RP, HPG, S | Right atrial pseudomass; both AV sulcus infiltration and right pericoronary infiltration | Massive pericardial effusion with tamponade | 68 | AA, LSA | V600E |
| 3 | 42, F | 190 | B, RP, HPG, L, O, S | Right atrial pseudomass; right AV sulcus infiltration and right pericoronary infiltration | Pericardial thickening | 61 | — | V600E |
| Mild pericardial effusion | ||||||||
| 4 | 49, F | 96 | O, S | — | — | 69 | — | Wild type |
| 5 | 71, M | 1 | RP, O | — | — | 51 | — | NA |
| 6 | 60, M | 5 | B, RP, HPG, CNS, O, S | Right atrial pseudomass; right AV sulcus infiltration and right pericoronary infiltration | Pericardial thickening | 63 | — | V600E |
| Mild pericardial effusion | ||||||||
| 7 | 43, M | 78 | B, CNS, S | — | — | 58 | — | NA |
| 8 | 41, M | 3 | RP | Right atrial pseudomass; right AV sulcus infiltration | Mild pericardial effusion | 65 | — | Wild type |
| 9 | 25, M | 15 | RP, O | Right atrial pseudomass; right AV sulcus infiltration and right pericoronary infiltration | Massive pericardial effusion with tamponade | 60 | AA, AArch, SVC, IVC, PA, PV | V600E |
| 10 | 22, M | 39 | B, HPG, CNS, S | — | — | 70 | — | V600E |
| 11 | 72, M | 1 | B, RP | — | — | 60 | — | V600E |
| 12 | 32, M | 12 | B, HPG, CNS, L, S | Right atrial pseudomass; right AV sulcus and auricolar infiltration and right pericoronary infiltration | Pericardial thickening | 66 | SVC | V600E |
| Mild pericardial effusion | ||||||||
| 13 | 59, M | 58 | B, CNS | — | — | 64 | — | NA |
| 14 | 70, F | 30 | B, RP, HPG, L, O | — | Pericardial thickening | 54 | AArch, BT, LCCA, LSA | V600E |
| Mild pericardial effusion | ||||||||
| 15 | 48, M | 216 | B (lytic and sclerotic), RP, HPG, L, S | Focal right atrial thickening | — | 55 | AA | Wild type |
| 16 | 50, M | 11 | B, RP | — | — | 71 | DA | Wild type |
| 17 | 44, M | 68 | B, RP, HPG, L | — | — | 55 | — | NA |
| 18 | 45, M | 107 | B, HPG | — | — | 53 | — | V600E |
| 19 | 55, M | 4 | B, RP, HPG | — | — | 58 | — | NA |
| 20 | 69, M | 36 | B, RP, HPG, S | — | — | 55 | — | V600E |
| 21 | 56, M | 30 | RP | — | — | 53 | — | V600E |
| 22 | 47, M | 36 | B, RP, HPG, O, S | Right atrial pseudomass; right AV sulcus infiltration and right pericoronary infiltration | Pericardial thickening | 58 | — | V600E |
| Mild pericardial effusion | ||||||||
| 23 | 48, M | 180 | RP, S | — | — | 69 | — | Wild type |
| Pt. no. . | Age (y), sex . | TTD (mo) . | Extracardiac involvement . | Myocardial lesions on MRI . | Pericardial lesions on MRI . | MRI EF % . | Thoracic vessel involvement on MRI . | BRAF status . |
|---|---|---|---|---|---|---|---|---|
| 1 | 46, M | 55 | B, RP, HPG, O | Right atrial pseudomass | Pericardial thickening | 68 | AA, AArch, BT, LSA, LCCA | Wild type |
| Right AV sulcus infiltration and right pericoronary infiltration | Massive pericardial effusion | |||||||
| 2 | 47, M | 117 | B, RP, HPG, S | Right atrial pseudomass; both AV sulcus infiltration and right pericoronary infiltration | Massive pericardial effusion with tamponade | 68 | AA, LSA | V600E |
| 3 | 42, F | 190 | B, RP, HPG, L, O, S | Right atrial pseudomass; right AV sulcus infiltration and right pericoronary infiltration | Pericardial thickening | 61 | — | V600E |
| Mild pericardial effusion | ||||||||
| 4 | 49, F | 96 | O, S | — | — | 69 | — | Wild type |
| 5 | 71, M | 1 | RP, O | — | — | 51 | — | NA |
| 6 | 60, M | 5 | B, RP, HPG, CNS, O, S | Right atrial pseudomass; right AV sulcus infiltration and right pericoronary infiltration | Pericardial thickening | 63 | — | V600E |
| Mild pericardial effusion | ||||||||
| 7 | 43, M | 78 | B, CNS, S | — | — | 58 | — | NA |
| 8 | 41, M | 3 | RP | Right atrial pseudomass; right AV sulcus infiltration | Mild pericardial effusion | 65 | — | Wild type |
| 9 | 25, M | 15 | RP, O | Right atrial pseudomass; right AV sulcus infiltration and right pericoronary infiltration | Massive pericardial effusion with tamponade | 60 | AA, AArch, SVC, IVC, PA, PV | V600E |
| 10 | 22, M | 39 | B, HPG, CNS, S | — | — | 70 | — | V600E |
| 11 | 72, M | 1 | B, RP | — | — | 60 | — | V600E |
| 12 | 32, M | 12 | B, HPG, CNS, L, S | Right atrial pseudomass; right AV sulcus and auricolar infiltration and right pericoronary infiltration | Pericardial thickening | 66 | SVC | V600E |
| Mild pericardial effusion | ||||||||
| 13 | 59, M | 58 | B, CNS | — | — | 64 | — | NA |
| 14 | 70, F | 30 | B, RP, HPG, L, O | — | Pericardial thickening | 54 | AArch, BT, LCCA, LSA | V600E |
| Mild pericardial effusion | ||||||||
| 15 | 48, M | 216 | B (lytic and sclerotic), RP, HPG, L, S | Focal right atrial thickening | — | 55 | AA | Wild type |
| 16 | 50, M | 11 | B, RP | — | — | 71 | DA | Wild type |
| 17 | 44, M | 68 | B, RP, HPG, L | — | — | 55 | — | NA |
| 18 | 45, M | 107 | B, HPG | — | — | 53 | — | V600E |
| 19 | 55, M | 4 | B, RP, HPG | — | — | 58 | — | NA |
| 20 | 69, M | 36 | B, RP, HPG, S | — | — | 55 | — | V600E |
| 21 | 56, M | 30 | RP | — | — | 53 | — | V600E |
| 22 | 47, M | 36 | B, RP, HPG, O, S | Right atrial pseudomass; right AV sulcus infiltration and right pericoronary infiltration | Pericardial thickening | 58 | — | V600E |
| Mild pericardial effusion | ||||||||
| 23 | 48, M | 180 | RP, S | — | — | 69 | — | Wild type |
—, absent; AA, ascending aorta; AArch, aortic arch; AV, atrioventricular; B, bone; BT, brachiocephalic trunk; DA, descending aorta; EF, ejection fraction; F, female; HPG, hypothalamic-pituitary gland axis; IVC, inferior vena cava; L, lung; LCCA, left common carotid artery; LSA, left subclavian artery; M, male; NA, not available; O, orbits; PA, pulmonary artery; Pt, patient; PV, pulmonary vein; RP, retroperitoneum; S, skin; SVC, superior vena cava; TTD, time from symptom onset to diagnosis.