A 4-y follow-up of a patient with severe pulmonary hypertension and type-Ia glycogen-torage disease
| Year | ||||
| 1996 | 1997 | 1998 | 1999 | |
| Right atrial pressure kPa (mmHg) | ND | 0.93 (7) | 1.86 (14) | 1.73 (13) |
| Systolic pulmonary artery pressure kPa (mmHg) | 80¶ | 13.3 (100) | 15.69 (118) | 11.97 (90) |
| Mean pulmonary artery pressure kPa (mmHg) | ND | 8.65 (65) | 10.50 (79) | 8.38 (63) |
| Mean pulmonary capillary wedge pressure kPa (mmHg) | ND | 1.20 (9) | 1.20 (9) | 0.93 (7) |
| Cardiac index L·min−1·m−2 | ND | 3.30 | 3.77 | 3.32 |
| Pulmonary vascular resistance kPa (mmHg·L·min·m−2) | ND | 2.06 (15.5) | 2.77 (20.8) | 2.53 (19.0) |
| Mixed venous oxygen saturation % | ND | 59 | 58 | 59 |
| Acute vasodilator response# | ND | Negative | Negative | ND |
| 6-min walk test m | ND | 450 | 510 | 450 |
| NYHA functional class | II | II | II | II |
| Pulmonary hypertension therapy | None | Diuretics+warfarin | Diuretics+warfarin | Diuretics+warfarin |
ND: not determined
NYHA: New York Heart Association
#: response to nitric oxide 10 ppm
¶: systolic pressure measured echography with doppler