Table 1. Anatomical measures in hearts of CLN2-affected dogs that received ICV TPP1 gene therapy.
Measurement | Dog 7a 10 months | Dog 6a 10 months | Dog 4 18 months | Dog 1 18 months | Dog 2 21 months | Reference range |
---|---|---|---|---|---|---|
H/BW %b | 0.78% | 0.74% | 1.13% ↑ | 0.93 | 0.94 ↑ | 0.58–0.94% |
LV+S/RVc | 2.86 | 3.18 | 2.36 ↓ | 2.4 ↓ | 2.42 ↓ | 2.76–3.88 |
RV/BW, %d | 0.18 | 0.16 | 0.27 ↑ | 0.23 ↑ | 0.24 ↑ | 0.1–0.18 |
LV+S/BW, %e | 0.54 | 0.49 | 0.65 ↑ | 0.56 ↑ | 0.59 ↑ | 0.35–0.55 |
RV/TC, %f | 23.8 ↑ | 21.0 ↑ | 24% ↑ | 24.9 ↑ | 25.7 ↑ | 15.3–20.9% |
LV+S/TC, %g | 68.2 ↑ | 66.8 ↑ | 56 | 59.7 | 62.3 | 52.5–66.5% |
Dogs 6 and 7 were untreated affected dogs; dogs 1, 2 and 4 correspond to the dogs listed in Figures 1, 2, 3.
Total heart weight/body weight.
Left ventricle and septum weight/right ventricle weight.
Right ventricle weight/body weight.
Left ventricle and septum weight/body weight.
Right ventricle weight/total cardiac weight.
Left ventricle and septum weight/total cardiac weight.