Table 2.
Study (Author/Y) | No. of Relatives Screened | Patients Affecteda (Aortic Aneurysm+Aortic Dissection) | Sudden Death (Unexplained) | Aortic Aneurysma | Aortic Dissectiona | ||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
No. | % | Male | % | No. | % | No. | % | No. | % | Age at Dissection, y | Range (Age, y) | ||
Barbier et al 201422 | 13 | 9 | 23 | 3 | 33 | n/a | ··· | 8 | 89 | 1 | 11 | 58 | n/a |
Bee et al 201223 | 32 | 21 | 39 | 16 | 76 | n/a | ··· | 21 | 100 | 0 | 0 | ··· | ··· |
Chamney et al 201524 | 6 | 6 | 43 | 4 | 67 | 0 | 0 | 3 | 50 | 3 | 50 | 49±10.4 | 37–55 |
Disabella et al 201125 | 29 | 15 | 41 | 8 | 53 | 1 | 3 | 6 | 40 | 9 | 60 | 49.3±16.3 | 29–73 |
Disertori et al 199126 | 14 | 4 | 13 | 4 | 100 | n/a | ··· | 2 | 50 | 2 | 50 | 46±2.8 | 44–48 |
Dong et al 201427 | 39 | 9 | 14 | 7 | 78 | 1 | 2 | 6 | 67 | 3 | 33 | 39±6.9 | 35–47 |
Francke et al 199528 | 23 | 10 | 38 | 6 | 60 | n/a | ··· | 8 | 80 | 2 | 20 | 55±14.1 | 45–65 |
Gago‐Diaz et al 201429 | 12 | 7 | 23 | 5 | 71 | n/a | ··· | 5 | 71 | 2 | 29 | 37.5±4.9 | 34–41 |
Gago‐Diaz et al 201630 | 14 | 11 | 37 | 6 | 55 | 1 | 3 | 5 | 45 | 6 | 55 | 34.2±12.9 | 15–48 |
Guo et al 200131 | 121 | 73 | 33 | 47 | 64 | n/a | ··· | n/a | ··· | n/a | ··· | ··· | ··· |
Guo et al 200732 | 130 | 53 | 25 | 33 | 62 | n/a | ··· | 8 | 15 | 45 | 85 | 37.3±13.9 | 13–67 |
Guo et al 200933 | 163 | 66 | 25 | 39 | 59 | n/a | ··· | n/a | ··· | n/a | ··· | ··· | ··· |
Guo et al 201134 | 18 | 9 | 32 | 9 | 100 | n/a | ··· | 8 | 89 | 1 | 11 | 32 | n/a |
Guo et al 201335 | 39 | 37 | 42 | 16 | 43 | n/a | ··· | 15 | 41 | 22 | 59 | 31.1±10.3 | 17–51 |
Guo et al 201536 | 34 | 8 | 17 | 5 | 63 | 1 | 2 | 8 | 100 | 0 | 0 | ··· | ··· |
Guo et al 201637 | 21 | 21 | 32 | 17 | 81 | 2 | 3 | 17 | 81 | 4 | 19 | 44.8±15.1 | 25–60 |
Hannuksela et al 201538 | 106 | 44 | 17 | 32 | 73 | 0 | 0 | 27 | 61 | 17 | 39 | 48b | 15–75 |
Hannuksela et al 201639 | 19 | 6 | 13 | 4 | 67 | 0 | 0 | 0 | 0 | 6 | 100 | 53.2±21.1 | 23–75 |
Harakalova et al 201340 | 40 | 15 | 20 | 10 | 67 | 3 | 4 | 4 | 37 | 11 | 73 | 46.6±19.5 | 18–70 |
Hasham et al 200341 | 52 | 17 | 25 | 14 | 82 | n/a | ··· | 9 | 53 | 8 | 47 | 45.4±21.5 | 14–72 |
Kakko et al 200342 | 115 | 39 | 18 | 25 | 64 | n/a | ··· | 26 | 67 | 13 | 33 | 53.2±15.5 | 26–80 |
Kent et al 201343 | 93 | 48 | 37 | 37 | 77 | n/a | ··· | n/a | ··· | n/a | ··· | ··· | ··· |
Keramati et al 201044 | 15 | 13 | 57 | 6 | 46 | n/a | ··· | 10 | 77 | 3 | 23 | n/a | n/a |
Khau Van Kien et al 200445 | 49 | 8 | 12 | 6 | 75 | 3 | 4 | 4 | 50 | 4 | 50 | n/a | n/a |
Khau Van Kien et al 200546 | 78 | 8 | 9 | 6 | 75 | 2 | 2 | 4 | 50 | 4 | 50 | n/a | n/a |
Kuang et al 201647 | 16 | 11 | 28 | 11 | 100 | n/a | ··· | 0 | 0 | 11 | 100 | 44.3±22.6 | 9–88 |
Loscalzo et al 200748 | 138 | 57 | 29 | 42 | 74 | n/a | ··· | n/a | ··· | n/a | ··· | ··· | ··· |
Marwick et al 198749 | 4 | 2 | 12 | 1 | 50 | 0 | 0 | 0 | 0 | 2 | 100 | 26.5±3.5 | 24–29 |
McManus et al 198750 | 8 | 6 | 32 | 5 | 83 | n/a | ··· | 0 | 0 | 6 | 100 | 33.5±14.9 | 22–62 |
Milewicz et al 199851 | n/a | 30 | 24 | 18 | 60 | 9 | 7 | 12 | 40 | 18 | 60 | 42.9±11.3 | 22–62 |
Morisaki et al 200952 | 9 | 14 | 30 | 10 | 71 | 5 | 11 | 3 | 21 | 11 | 79 | 36.8±10.1 | 25–52 |
Pannu et al 200553 | 72 | 58 | 25 | 39 | 66 | n/a | ··· | 27 | 46 | 32 | 54 | 46.1±16.3 | 14–73 |
Pannu et al 200754 | 23 | 6 | 22 | 4 | 67 | n/a | ··· | 1 | 17 | 5 | 83 | 45±8.8 | 37–56 |
Regalado et al 201155 | 12 | 52 | 23 | 35 | 67 | 7 | 3 | 9c | 17 | 43b | 83 | 50.8±13.7 | 25–76 |
Regalado et al 201156 | 36 | 23 | 22 | 14 | 61 | 1 | 1 | 9 | 39 | 14 | 61 | 42d | 25–54 |
Regalado et al 201157 | 11 | 15 | 52 | 8 | 53 | n/a | ··· | 7 | 47 | 8 | 53 | 32.3±9.9 | 18–50 |
Renard et al 201358 | 29 | 29 | 30 | 16 | 55 | 3 | 3 | 14 | 48 | 15 | 52 | 48.0±21.2 | 33–63 |
Robertson et al 201659 | 581 | 486 | 38 | 266 | 72 | n/a | ··· | 370 | 76 | 116 | 24 | 50±13 | n/a |
Sherrah et al 201660 | 119e | n/a | n/a | 68 | 76 | n/a | ··· | n/a | ··· | n/a | ··· | n/a | ··· |
Takeda et al 201561 | 9 | 5 | 29 | 4 | 80 | 0 | 0 | 1 | 20 | 4 | 80 | 47.8±16.6 | 32–70 |
Teixidó‐Turà et al 201462 | 10 | 3 | 8 | 2 | 67 | 1 | 3 | 1 | 33 | 2 | 67 | 46.5±12 | 38–55 |
Tortora et al 201763 | 77 | 25 | 26 | 61 | 79 | n/a | ··· | 25 | 100 | 0 | 0 | ··· | ··· |
Tran‐Fadulo et al 200664 | 9 | 21 | 14 | 7 | 33 | 0 | 0 | 4 | 19 | 17 | 81 | 32.0±12.3 | 16–55 |
Tran‐Fadulo et al 200965 | 49 | 29 | 37 | 17 | 59 | 0 | 0 | 15 | 52 | 14 | 48 | n/a | 14–62 |
Vaughan et al 200166 | 63 | 30 | 45 | 8 | 27 | n/a | ··· | n/a | ··· | n/a | ··· | ··· | ··· |
Wang et al 201067 | 21 | 10 | 21 | 5 | 50 | 2 | 4 | 0 | 0 | 10 | 100 | 54.3±20.8 | 16–78 |
Wang et al 201368 | 8 | 2 | 20 | 2 | 100 | 0 | 0 | 1 | 50 | 1 | 50 | n/a | n/a |
Ware et al 201469 | 7 | 2 | 20 | 2 | 100 | 0 | 0 | 0 | 0 | 2 | 100 | 17 | ··· |
Warnes et al 198570 | 2 | 2 | 33 | 2 | 100 | 0 | 0 | 0 | 0 | 2 | 100 | 35.0±18.4 | 22–48 |
Weigang et al 200771 | 23 | 9 | 35 | 5 | 56 | 0 | 0 | 3 | 33 | 6 | 67 | 32c | 18–47 |
Yoo et al 201072 | 6 | 5 | 25 | 1 | 20 | 0 | 0 | 0 | 0 | 5 | 100 | 32.5±12.9 | 20–46 |
Zhu et al 200673 | 49 | 8 | 16 | 7 | 88 | n/a | ··· | 5 | 63 | 3 | 38 | n/a | n/a |
Ziganshin et al 201574, f | 15 | 4 | 15 | 2 | 50 | n/a | ··· | 1 | 25 | 3 | 75 | n/a | n/a |
Ziganshin et al 201574, f | 15 | 7 | 41 | 4 | 57 | n/a | ··· | 4 | 57 | 3 | 43 | n/a | n/a |
n/a indicates not available.
Percentage calculated in the family pedigree (as per protocol).
Median available only.
Data available from 4 families only (TAA288, TAA062, TAA549, TAA395).
Mean available only.
Comprehensive of patients affected by bicuspid aortic valve.
Data of 2 different screened families obtained from the same study.