Table E1.
Clinical, pathologic, and CT findings in all patients
| Clinical findings |
Pathologic findings |
|||||||
|---|---|---|---|---|---|---|---|---|
| Case no. | Age at surgery (y) and sex | Presentation/symptoms | Fibrointimal hyperplasia | Mural smooth muscle disarray and hyperplasia | Mucoid extracellular matrix accumulation | Mural fibrosis | Fiber disorganization | Fiber fragmentation |
| 1 | 9 M | Syncope on several occasions | Absent | Moderate | Minimal | Minimal | Moderate | Minimal |
| 2 | 15 M | Chest pain and syncopal attacks | Absent | Moderate | Minimal | Moderate | Moderate | Minimal |
| 3 | 17 M | Ventricular dysrhythmias | Absent | Moderate | Minimal | Minimal | Moderate | Moderate |
| 4 | 15 F | Syncope | Minimal | Moderate | Marked | Minimal | Moderate | Marked |
| 5 | 9 M | Chest pain | Absent | Marked | Minimal | Moderate | Marked | Marked |
| 6 | 11 M | Presyncopal episodes | Minimal | Minimal | Minimal | Minimal | Moderate | Moderate |
| 7 | 9 F | Asymptomatic, incidentally diagnosed on echo workup following adoption | Minimal | Moderate | Minimal | Minimal | Moderate | Marked |
| 8 | 7 M | Chest pain during a soccer game | Absent | Minimal | Minimal | Moderate | Minimal | Moderate |
| 9 | 18 M | Chest pain on exertion | Absent | Moderate | Moderate | Marked | Moderate | Minimal |
| 10 | 13 M | Chest pain on exertion | Absent | Minimal | Moderate | Marked | Marked | Moderate |
| 11 | 8 M | Asymptomatic; patient is known to have this condition since infancy | Absent | Absent | Absent | Minimal | Minimal | Minimal |
| 12 | 12 F | Chest pain on exertion | Minimal | Moderate | Marked | Minimal | Moderate | Moderate |
| 13 | 12 M | Presyncopal episodes | Absent | Moderate | Marked | Marked | Marked | Moderate |
| 14 | 9 M | Dizziness and “intestinal angina” | Minimal | Moderate | Marked | Moderate | Moderate | Moderate |
| 15 | 10 M | Chest pain | Minimal | Minimal | Moderate | Moderate | Marked | Moderate |
| 16 | 15 F | Incidentally discovered at the time of echo for murmur secondary to a large ASD | Absent | Moderate | Minimal | Marked | Marked | Moderate |
| 17 | 13 M | Incidentally diagnosed when evaluated for pectus excavatum and scoliosis | Minimal | Minimal | Moderate | Minimal | Minimal | Minimal |
| 18 | 17 M | Incidentally discovered at the time of radiofrequency ablation of his accessory pathway | Minimal | Minimal | Moderate | Moderate | Minimal | Minimal |
| 19 | 7 F | Chest pain, recurrent | Minimal | Minimal | Minimal | Absent | Minimal | Absent |
| 20 | 14 F | Chest pain after a 5-kilometer run | Minimal | Moderate | Minimal | Moderate | Moderate | Moderate |
| Case no. | Intraoperative findings |
CT measurements |
||||||
|---|---|---|---|---|---|---|---|---|
| Coronary artery with anomalous origin by direct inspection | Coronary ostium morphology | Intramural course | CT length of presumed intramural course, mm | Coronary artery with anomalous origin | Sinus of origin | Relationship between right and left ostia (same sinus) | Take-off angle, degrees | |
| 1 | LCA | Slit-like | N | Not available | Not available | Not available | Not available | Not available |
| 2 | RCA | Narrowed | Y, unspecified length | 11.1 | RCA | Left | Two contiguous orifices adjacent to each other | 9.9 |
| 3 | RCA | Slit-like | Y, 10-11.5 mm | 15.2 | RCA | Left-sided sinus∗ | Two separate noncontiguous | 11.8 |
| 4 | RCA | Slit-like | Y, unspecified length | 11.8 | RCA | Left | Two contiguous orifices adjacent to each other | 16.1 |
| 5 | RCA | Slit-like | Y, unspecified length | 12.2 | RCA | Above right sinus | Not applicable | 12.6 |
| 6 | LCA | Slit-like | Y, 8 mm | 10.3 | LCA | Right | Two separate noncontiguous | 9.1 |
| 7 | RCA | Not mentioned | Y, 3 mm | 4.7 | RCA | Above left sinus | Two separate noncontiguous | 18.7 |
| 8 | RCA | Slit-like | Y, unspecified length | 2.6 | RCA | Left | Two contiguous orifices adjacent to each other | 13.2 |
| 9 | RCA | Slit-like | Y, unspecified length | Not available | Not available | Not available | Not available | Not available |
| 10 | RCA | Slit-like | Y, unspecified length | 8.6 | RCA | Above left sinus | Two separate noncontiguous | 12.1 |
| 11 | RCA | Slit-like | Y, 8 mm | 5.3 | RCA | Left | Two contiguous orifices adjacent to each other | 13.9 |
| 12 | RCA | Slit-like | Y, 10 mm | 9.7 | RCA | Above intercoronary commissure | Not applicable | 15.1 |
| 13 | RCA | Slit-like | Y, 8 mm | 6.4 | RCA | Left | Two contiguous orifices adjacent to each other | 18,7 |
| 14 | LCA | Slit-like | Y, 6-7 mm | 7.7 | LCA | Top of left noncoronary commissure with minimal extension to left sinus | Not applicable | 18.4 |
| 15 | RCA | Slit-like | Y, 3.5 mm | 4.6 | RCA | Left | Two contiguous orifices adjacent to each other | 17.3 |
| 16 | RCA | Slit-like | Y, 2 mm | 3.6 | RCA | Left | Two separate noncontiguous | 15 |
| 17 | LCA | Slit-like | Y, unspecified length short | 3 | LCA | Right | Two contiguous orifices adjacent to each other | 20.4 |
| 18 | RCA | Very narrowed | Y, unspecified length | 11.9 | RCA | Left | Two contiguous orifices adjacent to each other | 15.8 |
| 19 | RCA | Slit-like | Y, 7 mm | 7.3 | RCA | Left | Two contiguous orifices adjacent to each other | 16.2 |
| 20 | RCA | Slit-like | Y, 9-11 mm | 13.2 | RCA | Above right sinus | Not applicable | 8.5 |
| CT measurements | |||||
|---|---|---|---|---|---|
| Case no. | CT proximal coronary minor dimension, mm | CT proximal coronary eccentricity index | Coronary artery course | Distance from commissure, mm | Crosses an ICC? |
| 1 | Not available | Not available | Not available | Not available | Not available |
| 2 | 0.8 | 0.27 | Interarterial | 0 | Crosses ICC |
| 3 | 1.9 | 0.90 | Unable to evaluate | 3 | Does not cross ICC |
| 4 | 0.7 | 0.41 | Interarterial | 3 | Crosses ICC |
| 5 | 0.6 | 0.30 | Interarterial | 4 | Does not cross ICC |
| 6 | 1.4 | 0.35 | Interarterial | −2† | Crosses ICC |
| 7 | 0.7 | 0.33 | Interarterial | 4 | Origin above ICC |
| 8 | 1 | 0.43 | Unable to evaluate | 3 | Crosses ICC |
| 9 | Not available | Not available | Not available | Not available | Not available |
| 10 | 0.8 | 0.38 | Interarterial | 3 | Crosses ICC |
| 11 | 0.9 | 0.47 | Interarterial | 4 | Crosses ICC |
| 12 | 1.3 | 0.41 | Interarterial | 4 | Does not cross ICC |
| 13 | 1.5 | 0.43 | Interarterial | 0 | Crosses ICC |
| 14 | 1.1 | 0.58 | Retroaortic (posterior to aortic root) | 2 | Does not cross ICC |
| 15 | 0.9 | 0.47 | Interarterial | 0 | Crosses ICC |
| 16 | 1.8 | 0.40 | Interarterial | 0 | Crosses ICC |
| 17 | 2.6 | 0.63 | Interarterial | 2 | Crosses ICC |
| 18 | 1.2 | 0.29 | Interarterial | 2 | Crosses ICC |
| 19 | 1.2 | 0.60 | Interarterial | 3 | Crosses ICC |
| 20 | 1.3 | 0.20 | Unable to evaluate | 14 | Does not cross ICC |
CT, Computed tomography; M, male; F, female; ASD, atrial septal defect; LCA, left coronary artery; N, no; RCA, right coronary artery; Y, yes; ICC, intercoronary commissure.
∗
Bicommissural aortic valve with anterior–posterior directed-commissures and left- and right-sided sinuses. Both coronary orifices were in or above the left-sided sinus.
†
Negative value = below the sinotubular junction.