Table 5.
Comparison of risk stratification with Kid-SMS and diagnosis according to Ghent-2 at follow-up visit. (Ghent-2 Pos, revised Ghent Criteria positive; Ghent-2 Neg, revised Ghent Criteria negative; FBN1 Pos, FBN1 mutation positive; FBN1 Neg, FBN1 mutation negative; SV, dilatation of sinus of valsalvae; PA, dilatation of pulmonary artery; MVP, mitral valve prolapse; TVP, tricuspid valve prolapse; 3Skel, at least 3 skeletal features of the systemic score of the revised Ghent Criteria; EL, ectopia lentis; FH, family history).
| Patients Ghent-2 Pos (Ghent-2), n = 27 | Patients Ghent-2 Neg, n = 79 | ||
|---|---|---|---|
| Patients FBN1 Pos, n = 5 | Patients FBN1 Neg, n = 74 | ||
| Very high risk SV + EL | 2 | 0 | 0 |
| High risk SV + 3Skel | 8 | 0 | 2 |
| High risk SV + MVP + TVP | 7 | 0 | 0 |
| High risk SV + PA | 3 | 0 | 0 |
| High risk EL + MVP + TVP | 0 | 0 | 0 |
| High risk EL + PA | 2 | 0 | 0 |
| Moderate risk (FH) | 2 | 2 | 7 |
| Moderate risk (SV) | 3 | 0 | 5 |
| Negative | 0 | 3 | 60 |