Preoperative pulmonary hemodynamics and clinical decision making to determine operability and risk of long-term pulmonary hypertension in infants with open shunt under 1 year.

INTRODUCTION: Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) can potentially be reversed by early shunt closure, but its efficacy decreases over time. This study provides valuable descriptive data on: which factors were associated with clinical decision-making of operability and the outcomes of those decisions in infants under 1 year with PAH-CHD and an open shunt.

METHODS: We retrospectively analysed 100 infants with PAH-CHD who underwent right heart catheterization for operability assessment. Acute vasodilator testing (AVT) was performed in 64 patients.

RESULTS: Eleven patients (11%) were deemed non-operable and 10/11 had developmental lung disease (DLD). Positive AVT was more common among infants deemed operable, whereas developmental lung disease was more common among those deemed non-operable. In the patients who had shunt closure (89, 89%), preoperative pulmonary haemodynamic did not relate to prevalence of long-term PH. At long-term follow-up, 5 patients had PH (2 persistent PH with DLD, 1 with PAH-CHD, 2 recurrent PH after shunt closure with Down syndrome), with 4 requiring targeted pulmonary arterial hypertension treatments.

CONCLUSION: In this cohort, decisions regarding operability were more closely associated with clinical features such as DLD than with PVRi. However, due to the small number of non-operable cases, these associations should be considered exploratory.