NYHA class | ||||||||||||||||||
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Patient | Sex | NSVT | Age at Dx (years) | Age at death (years) | Follow up (years) | NSVT to death (years) | Family history | Initial | Most recent | LVWT max (mm) | LA (mm) | LVED (mm) | FS (%) | Basal LVOTG (mm Hg) | PAP (mm Hg) S/D | AF | End stage HCM* | Cause of death |
1 | M | None | 40 | 53 | 12.7 | – | HCM+ SD | II | IV | 30 | 52 | 41 | 29 | 32 | 55/25 | C | + | CHF |
2 | F | None | 59 | 72 | 13.1 | – | HCM+ SD | IV | III | 30 | 40 | 43 | 25 | 100 | 42/14 | C | + | CHF/stroke |
3 | M | None | 60 | 73 | 12.8 | – | 0 | II | III | 28 | 53 | 48 | 41 | 0 | – | 0 | 0 | CHF |
4 | F | None | 66 | 75 | 8.8 | – | HCM | IV | IV | 30 | 70 | 26 | 61 | 0 | 75/40 | C | 0 | CHF |
5 | F | Iso | 25 | 48 | 23.2 | 15.2 | 0 | II | II | 26 | 53 | 40 | 24 | 100 | 52/12 | P | 0 | SD |
6 | M | MR | 30 | 31 | 1.0 | 1.0 | 0 | III | III | 24 | 49 | 41 | 39 | 60 | 60/34 | 0 | 0 | CHF |
7 | M | MR | 20 | 48 | 28.0 | 26.5 | HCM+ SD | I | IV | 23 | 43 | 45 | 38 | 0 | – | C | + | CHF |
8 | M | MR | 59 | 62 | 3.8 | 3.8 | 0 | II | III | 26 | 50 | 44 | 32 | 0 | – | C | + | CHF/stroke |
9 | F | MR | 70 | 73 | 2.8 | 2.8 | 0 | II | III | 28 | 47 | 27 | 22 | 100 | – | 0 | 0 | CHF |
–, data not available; +, present; 0, absent; *, defined as severe progressive heart failure associated with left ventricular wall thinning (with or without asynergy), relative cavity enlargement, decreased ejection fraction or any combination of these.
AF, atrial fibrillation; C, chronic; CHF, congestive heart failure; Dx, diagnosis; Hx, history; Iso, Isolated; LA, left atrium; LVWT, left ventricular wall thickness; LVED, left ventricular end diastolic dimension; LVOTG, left ventricular outflow tract pressure gradient estimated with continuous wave Doppler echocardiography33; MR, multiple repetitive; P, paroxysmal; PAP (S/D), pulmonary arterial pressure (systolic/diastolic); SD, sudden death; FS, fractional shortening.