Table 1 |.
The onset and severity of cardiomyopathy are variable in TV-Dhet patients
| Patient | Sex | Diagnosis | Age of onset (yr)a | Left ventricular ejection fraction (%) | Left ventricular D/S diameter (cm) | Therapy/intervention |
|---|---|---|---|---|---|---|
| A | M | DCM | 14 | 25 | 8.6/7.6 | Left ventricular assist device/ orthotopic heart transplant |
| B | M | DCM | 35 | 40 | 6.9/5.4 | Automatic implantable cardioverter-defibrillator |
| C | F | Sudden cardiac death, mild hypertrophy consistent with early DCM | 15 | 61 | 4.46/3.0 | N/A |
| D | F | DCM | 60 | 45 | 5.9/3.5 | Medical therapy |
| E | M | DCM | 50 | 46 | 6.1/4.5 | Medical therapy |
| F | M | DCM | 0.5 | 29 | 6.4/5.5 | Mitral valve annuloplasty |
The clinical characteristics of affected individuals marked in Fig. 1 with a red patient identifier (A–F) are described. The mean age of onset ± s.d. was calculated. Cardiac function is represented as left ventricular ejection fraction. The left ventricular diameter was measured during the diastole (D) and systole (S) phases. M, male; F, female; N/A, not applicable.
a
Mean = 29.1±23.1.