Table 1. Clinical characteristics of index patients at diagnosis.
Characteristic | Index patient Family 1 | Index patient Family 2 |
---|---|---|
Gender | male | male |
Age at diagnosis, years | 33 | 13 |
Heart rate, min-1 | 89 | 96 |
Oxygen saturation, % | 90 | 98 |
Mean pulmonary artery pressure, mmHg | 70 | 46 |
Pulmonary vascular resistance, dyn*s*cm-5 | 2000 | 1055 |
Cardiac index, l/min/m2 | 1.9 | 3.6 |