Clinical characteristics and therapeutic outcomes of pulmonary arterial hypertension secondary to congenital portosystemic shunts

Conclusion: CPSS may be a more likely cause of unexplained PAH in pediatric patients than previously thought. Shunt closure or liver transplantation may prevent the progression of PAH, or even improve it for the majority of CPSS patients.What is Known:•Congenital portosystemic shunts (CPSS) are increasingly recognized as a cause of pulmonary arterial hypertension (PAH) in children, but the prevalence of CPSS in pediatric PAH patients is largely unknown, and the therapeutic outcomes of these conditions are not well described.What is New:•This is the largest cohort of CPSS-associated PAH ever published. CPSS may be a not uncommon cause of unexplained PAH in pediatric patients.• Shunt closure or liver transplantation may prevent the progression of PAH, or even improve it for the majority of CPSS patients.• Shunt closure might cause unique pulmonary hemodynamic changes in CPSS patients associated with hepatopulmonary syndrome (HPS), even leading to the transition from HPS to PAH.
Source: European Journal of Pediatrics - Category: Pediatrics Source Type: research