Table 3.

Clinical data of hypertrophic cardiomyopathy patients with advanced heart failure

Patient no.	Gender	Gene mutation	Age at Dx, years	Age at symptom onset, years	CAD	LA entry, mm	LA FU, mm	AF entry	AF FU	LVOT peak gradient entry, mmHg	LVOT peak gradient FU, mm Hg	EF entry, %	EF FU, %	Max- LV, mm	LVED entry, mm	LVED FU, mm	PW LV filling pattern entry	PW LV filling pattern FU	MR entry	MR FU	NYHA class entry	NYHA class FU	Surgery	SE	Outcome (age)
End-stage
1	M	0	5	26	0	30	49	0	parox	0	0	62	38	25	45	60	E/A > 1	E/A > 1	0	1	I	III	0	0	Alive
2	F	0	11	40	—	51	50	0	0	15	0	58	40	32	43	62	E/A > 1	E/A > 1	0	1	I	III	0	0	List for TP
3	F	0	17	47	0	46	54	0	perm	0	0	61	46	20	48	57	E/A > 1	RES	0	1	II	III	0	0	Alive, PM
4	M	0	38	38	—	65	68	0	perm	0	0	44	45	21	56	56	RES	RES	1	0	II	IV	0	0	TP (50)
5	M	TNNT2	39	56	+	60	70	perm	perm	0	0	26	30	13	58	62	RES	RES	1	1	II	III	0	0	TP (59)
		EX 9
		Arg94Leu
6	M	—	39	59	—	48	56	0	perm	0	0	60	46	22	48	57	E/A > 1	RES	0	1	II	III	0	0	Alive, ablation
7	M	—	48	74	—	53	56	0	parox	0	0	74	46	25	51	47	E/A > 1	E/A > 1	1	—	I	III	0	0	Alive, PM
8	F	—	54	54	0	44	45	0	parox	0	0	47	46	25	45	31	RES	RES	2	2	III	III	0	+	HF death (55)
9	F	—	55	55	0	58	59	pers	perm	0	0	25	47	19	60	61	—	—	1	2	III	III	0	0	Alive, PM, ICDa
10	F	0	59	66	—	50	48	perm	perm	0	0	32	36	18	40	38	—	RES	1	1	II	IV	0	0	HF death (68)
11	F	MYBPC3	59	63	0	43	51	perm	perm	0	0	34	31	16	55	58	RES	RES	2	2	II	III	0	0	TP (63)
		EX 13
		Gln366X
12	M	—	64	65	0	42	60	0	0	0	0	40	32	18	35	68	E/A > 1	RES	2	3	I	III	0	0	HF death (77)
13	F	—	65	65	—	50	52	0	0	64	0	70	31	25	52	64	E/A < 1	—	2	0	III	III	0	0	Alive
14	M	—	65	80	+	52	58	0	perm	0	0	71	43	23	53	60	E/A > 1	—	1	0	I	III	CABG	0	Alive
15	F	—	66	66	—	38	55	0	0	0	0	54	41	17	60	60	E/A > 1	E/A > 1	0	0	IV	III	0	0	Alive
LV outflow obstruction
16	M	MYH7	16	16	—	45	51	0	0	80	50	83	58	35	50	40	E/A > 1	E/A > 1	2	1	III	III	0	0	Alive
		EX 22
		Met877Ile
17	M	—	41	41	—	41	45	0	parox	40	190b	75	66	21	41	40	E/A > 1	E/A < 1	1	1	III	III	0	0	Alive
18	M	—	45	64	0	48	64	0	perm	55	40	75	50	25	52	55	E/A > 1	—	1	0	II	III	0	0	Alive
19	F	—	47	47	—	60	65	0	0	50	80	68	57	25	50	42	E/A > 1	E/A > 1	1	2	IV	III	0	0	Alive
20	M	—	51	61	—	35	38	0	0	60	60	50	50	18	53	47	E/A > 1	—	0	1	III	III	0	0	Alive, PM
21	F	—	51	54	—	46	55	perm	perm	50	60	62	66	28	38	40	—	—	2	2	III	III	0	0	Alive
22	M	0	52	58	0	38	58	0	perm	45	75 (0)	68	63	27	42	42	E/A > 1	—	2	2	III	III (I)	Myectomy + MVR	0	Alive
23	F	—	54	62	—	36	56	0	parox	40	40 (0)	77	67	29	40	41	E/A < 1	—	1	2	IV	III (I)	Myectomy + MVR	0	Alive
24	F	—	60	60	—	50	48	0	0	110	200	71	59	23	41	46	E/A > 1	—	2	2	III	IV	0	0	Alive, PM
25	F	—	60	60	—	40	54	0	perm	65	95	62	53	25	46	38	E/A > 1	—	1	1	II	IV	0	0	HF death (67)
26	F	—	72	72	—	45	—	0	0	45	—	68	—	19	51	—	E/A > 1	—	2	—	IV	—	0	0	Lost
Non-obstructive with preserved systolic function + atrial fibrillation
27	F	—	14	34	—	57	60	0	parox	0	0	50	50	18	46	45	E/A > 1c	RES	3	3	II	IV	0	+	HF death (39)
28	M	—	15	18	—	70	70	0	perm	0	0	72	65	14	40	40	RES	—	1	0	III	IV	0	0	TP (20)
29	M	—	20	20	—	56	58	0	parox	0	0	70	64	18	57	38	RES	—	0	3 (0)	III	III (II)	MVR	0	Alive
30	M	—	22	25	—	38	40	parox	parox	0	0	46	56	25	60	60	E/A > 1	—	1	1	II	III	0	+	Alive, ICDa
31	F	—	30	31	—	70	70	pers	perm	0	0	63	60	20	25	36	RES	RES	0	0	III	IV	0	0	HF death (34)
32	M	—	32	47	0	40	43	0	pers	0	0	63	70	23	45	46	E/A < 1	E/A < 1	1	1	I	III	0	0	Alive
33	F	0	32	32	+	47	48	0	perm	0	0	68	67	25	40	38	E/A < 1	—	0	1	II	IV	0	+	PM, HF death (53)
34	F	—	35	43	0	38	61	0	perm	12	0	64	50	26	35	45	E/A < 1	—	1	2	II	III	0	0	Alive, PM, list for TP
35	M	0	36	36	—	44	127	0	perm	20	—	75	55	22	50	48	E/A > 1	—	2	3 (0)	II	III (II)	MVR	0	Alive,
36	M	—	42	44	—	70	—	perm	perm	0	—	58	—	15	40	—	—	—	2	—	III	—	0	0	Lost
37	M	0	48	55	—	47	50	perm	perm	0	0	57	65	32	40	42	—	—	1	1	III	III	0	0	Alive, PM
38	F	MYH7, EX 36 Thr1760Met	48	58	—	60	56	0	pers	0	0	76	51	18	38	44	E/A > 1	E/A < 1	1	1	II	III	0	0	Alive
39	M	MYH7, EX 27 Ile1207Met	49	58	—	62	90	perm	perm	20	0	57	57	18	52	55	—	RES	1	2	III	IV	0	0	TP (60)
40	F	—	57	57	—	40	44	0	perm	0	0	67	65	16	50	48	E/A > 1	RES	0	0	I	III	0	0	Alive, PM
41	F	—	63	65	0	55	—	0	perm	0	0	62	—	15	47	—	RES	—	2	—	II	III	0	0	Alive, ablation + PM
Non-obstructive with preserved systolic function + normal sinus rhythm
42	F	MYBPC3	1	1	0	30	33	0	0	15	10	84	72	30	32	36	E/A < 1	E/A > 1c	1	0	IV	IV	0	0	TP (12)
		EX 12
		Ala364Thr
43	M	0	4	4	—	60	61	0	0	0	0	52	53	15	30	29	RES	RES	0	2	III	III	0	0	TP (9)
44	F	—	5	5	0	25	25	0	0	0	0	68	72	20	32	32	E/A > 1c	E/A > 1c	0	0	III	III	0	0	Alive
45	F	TNNI3	28	28	—	53	—	0	0	0	0	59	58	17	38	—	RES	RES	0	0	III	—	0	0	TP (28)
		EX 8
		Lys207Thr
46	F	—	37	41	—	60	—	0	—	0	0	55	—	18	55	—	RES	—	0	0	IV	—	0	0	HF death (41)
47	M	—	45	56	—	39	46	0	0	0	0	60	68	24	49	48	E/A < 1	RES	1	1	II	IV	0	0	ICDd, HF death (56)
48	F	0	45	45	—	70	55	0	0	0	0	56	51	16	49	48	RES	RES	1	1	II	IV	0	0	PM, HF death (66)
49	F	—	65	70	—	46	75	0	0	0	0	64	57	15	40	53	RES	RES	0	0	I	IV	0	0	Alive
50	M	—	76	76	—	35	—	0	0	0	0	53	—	25	37	—	E/A > 1c	—	1	—	II	IV	0	0	Non-HCM-related death (76)

AF, atrial fibrillation classified as paroxysmal (parox), persistent (pers) or permanent (perm); CABG, coronary artery bypass graft; CAD, coronary artery disease; Dx, diagnosis; EF, ejection fraction; FU, follow-up; HCM, hypertrophic cardiomyopathy; HF, heart failure; ICD, implantable cardioverter-defibrillator; LA, left atrium; LVED, left ventricle end-diastolic diameter; LV, left ventricle; LVOT, left ventricular outflow tract; MR, mitral regurgitation; MVR, mitral valve replacement; NYHA, New York Heart Association; PM, pacemaker; RES, restrictive (defined in setting of early LV diastolic filling deceleration time <150 ms and E/A ratio >2 at sinus rhythm); SE, systemic embolism; TP, transplant; —, data non available; +, present; 0, absent; () data after surgery.

^aImplanted for secondary prevention.

^bZero gradient at rest, and 190 mmHg with exercise.

^cPseudo-normal LV filling pattern [defined in the setting of E/A ratio = 0.9–1.5, early LV diastolic filling deceleration time = 160–240 ms, Valsalva positive (= E/A ratio decrease by ≥0.5 and increase in A velocity during Valsalva manoeuvre)].⁸

^dImplanted for primary prevention.