Liver and systemic hemodynamics in children with cirrhosis: Impact on the surgical management in pediatric living donor liver transplantation

Abstract

Cirrhosis in adults is associated with modifications of systemic and liver hemodynamics, whereas little is known about the pediatric population. The aim of this work was to investigate whether alterations of hepatic and systemic hemodynamics were correlated with cirrhosis severity in children. The impact of hemodynamic findings on surgical management in pediatric living donor liver transplantation (LT) was evaluated. Liver and systemic hemodynamics were studied prospectively in 52 children (median age, 1 year; 33 with biliary atresia [BA]). The hemodynamics of native liver were studied preoperatively by Doppler ultrasound and intraoperatively using invasive flowmetry. Portosystemic gradient was invasively measured. Systemic hemodynamics were studied preoperatively by Doppler transthoracic echocardiography and intraoperatively by using transpulmonary thermodilution. Hemodynamic parameters were correlated with Pediatric End-Stage Liver Disease (PELD) score and the histological degree of fibrosis (collagen proportionate area [CPA]). Cirrhosis was associated with a 60% reduction of pretransplant total liver flow (n = 46; median, 36 mL/minute/100 g of liver) compared with noncirrhotic livers (n = 6; median, 86 mL/minute/100 g; P = 0.002). Total blood flow into the native liver was negatively correlated with PELD (P < 0.001) and liver CPA (P = 0.005). Median portosystemic gradient was 14.5 mm Hg in children with cirrhosis and positively correlated with PELD (P < 0.001). Portal vein (PV) hypoplasia was observed mainly in children with BA (P = 0.02). Systemic hemodynamics were not altered in our children with cirrhosis. Twenty-one children met the intraoperative criteria for PV reconstruction using a portoplasty technique during the LT procedure and had a smaller PV diameter at pretransplant Doppler ultrasound (median = 3.4 mm; P < 0.001). Cirrhosis in children appears also as a hemodynamic disease of the liver, correlated with cirrhosis severity. Surgical technique for PV reconstruction during LT was adapted accordingly. Liver Transplantation 23 1440-1450 2017 AASLD.

Response to Han et al

“…Diagnosis of hyperdynamic circulation is difficult because accurate measurements of cardiac output and systemic vascular resistance are mandatory; thus, the choice of hemodynamic measurement is crucial. Although studies using transthoracic echocardiography have revealed a high incidence of hyperdynamic circulation among children with cirrhosis, our results indicate a low incidence of hyperdynamic circulation, a result compatible with that of a recent study by de Magnée et al Both studies used the transpulmonary thermodilution method 5 . In addition, portal hypertension was common in the patient cohort in the study by de Magnée et al, regardless of the presence or absence of hyperdynamic circulation.…”

Severity of Ascites Is Associated with Increased Mortality in Patients with Cirrhosis Secondary to Biliary Atresia

“…Although recent reports have demonstrated high mortality among infants with BA on liver waiting lists [6], few studies have reported the impact of decompensated cirrhosis in children and establishing prognosis for these patients remains a challenge [7,8]. In adult cirrhotic patients, ascites is often the first sign of CLD decompensation and is usually considered a landmark sign of cirrhosis aggravation [9][10][11][12].…”

Novel technique for pediatric living donor liver transplantation in patients with portal vein obstruction: The “pullout technique”

“…Liver cirrhosis can be associated with PV hypoplasia and severe alterations of the liver hemodynamics. PV hypoplasia may lead to technical difficulties in PV reconstruction during LT due to size mismatch with the donor PV, elasticity, or the intimal thickening of the recipient PV, which increases the risk of posttransplant PV complications, and thus increases the risk of graft loss, especially in pediatric patients …”