Mutation carriers# | Non-carriers# | p-value | |
Patients | 12 | 28 | N/A |
Mean±sd follow-up years | 5.5±3.6 | 5±2.3 | NS |
NYHA I–II | 11 (91%) | 19 (68%) | NS |
NYHA III–IV | 1 (9%) | 9 (32%) | |
Oral combination + prostanoid i.v./s.c. (triple therapy) | 6 (50%) | 8 (30%) | NS |
Potts anastomosis | 2 (15%) | 5 (17.5%) | NS |
Transplantation | 1 (8%) | 0 | NS |
Death | 0 | 4 (14%)¶ | NS |
NYHA: New York Heart Association (classes I-IV); N/A not applicable; NS: nonsignificant. #: pulmonary veno-occlusive disease excluded; ¶: two triple therapy; one combined therapy; and one calcium channel blocker.