Table 2 Demographic details for patients with APAH
Total APAHEisenmengerPostoperativeLungConnective tissueMiscellaneous†Complex CHD
Number of cases1564947299148
Age at presentation (years) mean (range)7.89 (5.9)*12.07 (1.6–17.45)6.86 (0.2–19.5)3.8 (0.1–17.7)8.79 (1.16–15.9)4.18 (0.1–15.7)6.36 (0.5–17.3)
sex male:female76:8018:3125:2215:146:38:64:4
WHO (range)2.95 (0.61)*3 (2–4)3 (2–4)2.6 (2–4)3.4 (3–4)2.42 (2–3)3.25 (3–4)
Catheter study, n (%)83 (53)25 (51)32 (68)12 (41)4 (44)5 (36)5 (62)
PAP (mm Hg) (range)48.8 (16.03)*58.4 (53–83)45.0 (23–88)39.8 (26–66)47.25 (43–53)55 (28–80)42.6 (34–51)
PVR (U/m2) (range)15.6 (11.3)*22.7 (6–57)13.8 (3.4–49.8)7.9 (5–13.7)13.81 (8.25–19.1)13.14 (5.5–23.9)12.7 (3.6–18.1)
Treatment, n (%)
None35 (22)11 (22)7 (15)7 (24)2 (22)6 (43)2 (25)
Nifedipine10 (6)1 (2)4 (8)3 (10)1 (11)01 (12.5)
Bosentan/epoprostenol/sildenafil111 (72)37 (76)36 (77)19 (66)6 (67)8 (57)5 (62.5)
Deaths, n (%)
With treatment18 (11)2 (4)9 (19)4 (14)3 (33)00
Without treatment5 (3)02 (4)1 (3)02 (14)0
  • *SD.

  • †Miscellaneous, disorders including HIV, bone marrow transplantation and metabolic disorders; Complex CHD, complex unoperated or palliated congenital heart disease.

  • n, number of cases; PAP, pulmonary artery pressure; PVR, pulmonary vascular resistance; WHO, World Health Organization functional class.