Table 2.

Details of Newly Diagnosed Diseases of the Thoracic Aorta in the Screened Relatives

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.

^aPercentage calculated in the family pedigree (as per protocol).

^bMedian available only.

^cData available from 4 families only (TAA288, TAA062, TAA549, TAA395).

^dMean available only.

^eComprehensive of patients affected by bicuspid aortic valve.

^fData of 2 different screened families obtained from the same study.