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.