PULMONARY ARTERY HYPERTENSION IN CHILDREN WITH LIVER DISEASE: DIAGNOSIS. OUTCOME AND POTENTIAL REVERSAL AFTER LIVER TRANSPLANTATION.:

175 Pulmonary artery hypertension (PAH) occurs in 1-2% of adults with cirrhosis and results in death on average 15 months after diagnosis; its reversal after liver transplantation is still debated. There have not been many reports on pulmonary hypertension in children with liver disease and the outcome after liver transplantation is not known. We report on 8 children whose liver disease was complicated by PAH between 1984 and 1995. Liver disease was portal vein obstruction (2pts), congenital hepatic fibrosis (2 pts), cirrhosis (3 pts) and congenital portocaval fistula (1 pt). Three pts had undergone surgical portosystemic shunt 1 to 8 yrs before the first signs of PAH. Presenting symptoms were noted at ages 3-19 yrs and consisted in 7 pts of dyspnoea on exertion, with fainting on exertion in 3 pts and chest pain in 1, and in 1 pt of abnormal findings on a routine echocardiogram. Hemodynamic exploration showed raised mean pulmonary artery pressures (PAP) ranging from 30 to 52 mmHg (median: 41), raised pulmonary resistances (PR) ranging from 5 to 12 IU.m2 (median: 7), cardiac indices between 2.9 and 7 l/mn/m2 (median: 5) and normal capillary pressures. Before the era of transplantation. 3 pts died 1 mo, 6 mo and 6 yrs after diagnosis and 1 was lost to follow-up after 4 yrs. Partial surgical closure of the portocaval fistula was associated with a decrease in mean PAP and PR from 30 to 22 mmHg and from 7 to 5 IU.m2 respectively. Two children underwent livert transplantation: mean PAP decreased from 46 and 36 to 22 and 14 mmHg respectively, and PR from 12 and 5.4 to 4.8 and 3.6 IU.m2 respectively. Liver transplantation was attempted in 3rd patient but, in the 11 mos waiting time, mean PAP rose from 45 to 65 mmHg; ventricular tachycardia occurred at the onset of surgery and the child died 15 days later. These results indicate that 1) PAH may complicate all kinds of liver disease in children even without cirrhosis, 2) the spontaneous prognosis is ominous. 3) liver transplantation may allow regression of PAH provided the lesions are not too advanced. 4) systematic screening with echocardiography may thus be warranted in all children at risk.