Table.
RV samples were obtained from 12 non-failing controls and 26 patients with HLHS
| Subject ID |
Age (y) |
Gender | FGP | β -AR |
AC 5/6 |
β-AR binding |
α-MHC WB |
PLB WB |
PLB-Thr17 WB |
PLB- Ser16 WB |
cAMP | CaMK activity |
Inotrope | Digoxin | ACEI | BB | Diuretic | Palliation history/indication for transplant for D-HLHS group |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| NF-RV | ||||||||||||||||||
| NF1 | 9 | M | X | X | X | X | X | X | X | X | + | − | − | + | − | |||
| NF2 | 7 | M | X | X | X | X | X | X | X | + | − | − | − | − | ||||
| NF3 | 13 | M | X | X | X | X | X | X | X | X | X | X | + | − | − | + | − | |
| NF4 | 3 | F | X | X | X | X | X | X | X | X | X | + | − | − | − | − | ||
| NF5 | 8 | F | X | X | X | X | X | X | X | X | X | X | − | − | − | − | − | |
| NF6 | 1.3 | F | X | X | X | X | X | X | X | X | X | X | + | − | − | − | − | |
| NF7 | 7 | M | X | X | X | X | X | X | X | X | X | X | − | − | − | − | − | |
| NF8 | 1.4 | M | X | X | X | X | X | X | X | X | X | − | − | − | − | − | ||
| NF9 | 12 | M | X | X | X | − | − | − | − | − | ||||||||
| NF10 | 14 | M | X | X | X | X | X | NA | NA | NA | NA | NA | ||||||
| NF11 | 8 | F | X | + | − | − | − | − | ||||||||||
| NF12 | 13 | F | X | X | X | X | NA | NA | NA | NA | NA | |||||||
| 11 | 10 | 8 | 6 | 7 | 9 | 8 | 9 | 8 | 9 | 6 | 0 | 0 | 2 | 0 | ||||
| C-HLHS | ||||||||||||||||||
| CH1 | 0.5 | M | X | X | X | X | X | − | − | − | − | − | PDA stent and PAB | |||||
| CH2 | 0.14 | F | X | X | X | X | X | X | X | − | − | + | − | − | PDA stent | |||
| CH3 | 0.22 | M | X | X | X | X | X | X | X | X | − | − | + | − | + | PDA stent | ||
| CH4 | 0.6 | F | X | X | X | X | X | X | X | − | − | + | − | + | Atrial septal, PDA stent and PAB | |||
| CH5 | 0.05 | F | X | X | X | X | X | X | X | X | X | − | − | − | − | + | On PGE | |
| CH6 | 0.17 | M | X | X | X | X | X | X | X | X | X | − | − | − | − | + | PDA stent | |
| CH7 | 0.07 | M | X | X | X | X | X | X | X | X | X | − | − | − | − | + | Atrial septal stent and on PGE | |
| CH8 | 0.17 | M | X | X | X | X | X | X | X | X | − | − | − | − | + | PDA stent | ||
| CH9 | 0.02 | F | X | X | − | − | − | − | − | On PGE | ||||||||
| CH10 | 0.1 | M | X | X | − | − | − | − | − | PDA stent | ||||||||
| CH11 | 0.1 | M | X | X | X | X | − | + | − | − | − | PDA stent | ||||||
| CH12 | 0.1 | F | X | X | X | − | − | + | − | + | On PGE | |||||||
| CH13 | 0.09 | M | X | X | X | X | − | − | + | − | + | On PGE | ||||||
| CH14 | 0.3 | F | X | X | X | X | − | − | − | − | + | PDA stent and PAB | ||||||
| 8 | 10 | 8 | 4 | 7 | 9 | 10 | 9 | 7 | 10 | 0 | 1 | 5 | 0 | 9 | ||||
| D-HLHS | ||||||||||||||||||
| DH1 | 7.9 | M | X | X | X | X | X | − | + | + | − | + | Fontan (h/o BT shunt)/PLE | |||||
| DH2 | 3.8 | M | X | X | + | + | − | − | + | Fontan (h/o BT shunt)/RV failure | ||||||||
| DH3 | 3.2 | M | X | X | X | X | X | + | + | + | − | + | Fontan/RV failure | |||||
| DH4 | 1.0 | F | X | X | X | X | X | X | X | X | X | X | − | + | + | − | + | Norwood with Sano shunt/RV failure |
| DH5 | 6.0 | F | X | X | X | X | X | X | X | X | X | X | + | − | − | − | + | Fontan (h/o Sano shunt)/PLE and RV failure |
| DH6 | 2.8 | F | X | X | X | X | X | X | X | X | X | X | + | + | + | − | + | Glenn (h/o Sano shunt)/RV failure |
| DH7 | 9.9 | M | X | X | X | X | X | X | X | X | X | + | − | + | − | + | Fontan/PLE and RV failure | |
| DH8 | 0.4 | M | X | X | X | X | X | X | X | X | X | + | − | + | − | + | Norwood with BT shunt/RV failure | |
| DH9 | 4.8 | M | X | X | X | X | + | − | + | − | + | Fontan (h/o Sano shunt)/PB and RV failure | ||||||
| DH10 | 0.7 | F | X | X | X | X | X | X | X | − | + | − | − | + | PDA stent and PAB/RV failure | |||
| DH11 | 1.3 | M | X | X | X | X | X | X | X | X | − | − | + | − | + | PAB/RV failure | ||
| DH12 | 7.5 | M | X | X | X | NA | NA | NA | NA | NA | Glenn (h/o BT shunt)/RV failure | |||||||
| 11 | 12 | 8 | 7 | 7 | 9 | 6 | 7 | 8 | 7 | 7 | 6 | 8 | 0 | 11 |
RV samples were obtained from 12 non-failing controls and 26 patients with HLHS. Of the HLHS cohort, 14 are defined as compensated (C-HLHS) and 12 are defined as decompensated (D-HLHS). The age, gender, specific studies performed on each patient sample, medications, palliation history and indications for transplant (for the D-HLHS group) are noted. Inotropes include dopamine (3mcg/kg/min in one patient) or milrinone (0.5–0.75mcg/kg/min in 7 patients) for the D-HLHS group and dopamine, vasopressin and/or norepinephrine for the NF-RV group. Duration of inotropic therapy in the D-HLHS group was a median of 98 days (range 30–385 days).
ACEI, angiotensin converting enzyme inhibitor; BT shunt, Blalock-Taussig shunt; BB, beta-blocker; F, female; h/o, history of; M, male; NA, information not available; PAB, surgically placed bilateral pulmonary artery bands; PDA, patent ductus arteriosus; PGE, prostaglandin E1 infusion; PLE, protein losing enteropathy; WB, western blot.