Role of Beta Blockers in EHPVO in Children

Pathophysiology and Hemodynamic Effects

Hemodynamic data from animal models with pre-hepatic portal hypertension and patients with non-cirrhotic portal hypertension demonstrate beneficial effects of non-selective beta adrenergic blockade on splanchnic hemodynamics 1, 2
NSBBs cause vasoconstriction of the splanchnic circulation through β2-receptor inhibition and decrease cardiac output via β1-receptor blockade, leading to decreased portal venous inflow and lower portal pressure 3
The theoretical concerns about deleterious effects of NSBBs on patients with extended thrombosis have never been proven in clinical practice 1

According to multivariate analysis, beta adrenergic blockade decreases the risk of bleeding in patients with large varices and improves survival in patients with chronic portomesenteric venous obstruction 1, 2
Endoscopic variceal band ligation is superior to sclerotherapy according to short-term randomized controlled trials in children 1
In children, a combination of ligation and sclerotherapy may provide marginal advantage over either technique alone 1
When comparing propranolol to band ligation in adults with non-cirrhotic portal hypertension, no difference in rebleeding rates was observed at two years follow-up 1

The Cystic Fibrosis Foundation cannot recommend for or against the use of NSBBs for primary prophylaxis of variceal bleeding in children due to insufficient evidence 4, 5
The desired reduction of 20% in portal pressure gradient is achieved in about 50-75% of patients with propranolol or carvedilol, respectively 3
Beta blockers may have potential adverse effects on pulmonary function, though no studies specifically assessing worsening of lung disease in children with EHPVO have been conducted 4, 5
Hypotension is a contraindication to beta blocker use 3
Mesenterico-Rex shunt has shown high feasibility and long-term patency in children with patent vessels 1, 2