Characteristics of pediatric pulmonary artery hypertension due to
congenital porto-systemic shunt
Abstract
Background: Congenital porto-systemic shunt (CPSS) is a rare condition
that can cause pulmonary artery hypertension (PAH). Methods: We reviewed
six patients who received treatment in our institution between 2011 and
2019. Results: Median age at diagnosis of CPSS was 2.5 months (0 to 9
years) and that of PAH was 5.6 years (0 to 7.1 years). All patients
underwent intervention for CPSS. The median duration between diagnosis
of CPSS and intervention for CPSS was 2.5 months (2 months to 8 years)
and between diagnosis of PAH and intervention for CPSS was 2.5 months (1
month to 6 years). All patients received combination PAH-specific
therapy, three drugs in three cases and two drugs in three cases. Only
one infant who underwent intervention for CPSS at 3 months old
discontinued pulmonary vasodilator after intervention, but all other
patients continued taking it; three patients needed to boost the
therapy. Conclusion: When treatment for CPSS is delayed, pulmonary
arterial pressure does not decrease only by ligation of the shunt
vessel, and PAH-specific therapy cannot be discontinued.