Patients ID | Age at diagnosis/referred to our center (years) | Sex | Diagnostic criteria | ||||||||
---|---|---|---|---|---|---|---|---|---|---|---|
BAV morphology | MAS: diameter (mm)/site | BAV hemodynamics | MVP | Eye EL | Systemic features | Systemic features score | Family history | FBN1 mutations | |||
P1 | 15/24 | M | RL | 50/AoR | AR moderate | - | - | - | 0 | TAA | Arg529Gln |
P2 | 19/25 | M | RL | 57/AoR | AR moderate | + | - | MVP, My | 2 | - | Arg636Gly |
Arg2726Trp | |||||||||||
P3 | 35/40 | M | RL | 45/AoR | - | - | - | My, PE, Sc, Th+ | 4 | - | - |
P4 | 27/40 | F | RL | 41/AoR | AR mild | + | - | MVP, PP, CS | 3 | TAA/AAA | - |
P5 | 17/24 | M | RL | 47/AscA | - | - | - | Sc, PP, CS | 3 | - | - |
P6 | 40/40 | M | RL | 44/AoR | - | - | - | PE, CS, facies | 3 | - | - |
P7 | 42/42 | M | RL | Prothesic tube | - | - | - | CS | 1 | - | na |
P8 | 31/31 | M | RL | 42/AscA | - | + | - | MVP, PE, PC, PP, CS | 6 | BAV | na |
P9 | 24/24 | M | RL | 40/AoR | - | - | - | PE, Ky, CS, My | 4 | TAA | - |
P10 | 31/31 | F | RL | 48/AoR | AR mild | + | - | MVP, CS, DE, PP, Sc | 5 | - | - |