First author (Ref. no.) | Age at diagnosis of PH yrs | Sex | Shunt operation | Intracardiac shunt | Hepatic tumors | SPAP (mPAP) (kPa) | Evolution | Pathology (small pulmonary arteries) |
Pizzo 5 | 16 | F | Yes Porto-caval shunt at 12 yrs | No | Yes Focal nodular hyperplasia | ND | CPA Death | Intimal fibrosis Medial hypertrophy Plexiform lesions |
Furokawa 6 | 12 | F | No | No | No | ND | RHF Death (1 month after PH diagnosis) | Fibrous occlusion Plexiform lesions |
Hamaoka 7 | | | | | | | | |
Hamaoka 7 | 16 | M | Yes Intestinal vein/inferior vena cava at 10 yrs | No | No | 120 | RHF Death (2 months after PH diagnosis) | ND |
Bolz 8 | 4 | F | No | Yes Atrial septal defect of the second type | No | 72 (6.92) | RHF Death (4 years ofter PH diagnosis) | Intimal fibrosis |
Ohura 9 | 21 | F | No | No | No | 90 | RHF Death (1 week after PH diagnosis) | ND |
Kishnani 10 | 24 | F | No | No | No | 115 | RHF Death (8 months after PH diagnosis) | ND |
Humbert 2# | 25 | M | Yes Porto-caval shunt at 8 yrs | No | Yes Hepatic adenomas | 100 (8.65) | Alive NYHA II (4 years after PH diagnosis) | ND |