Pulmonary artery banding in infants and young children with end-stage left ventricular dilated cardiomyopathy-Cohort Study.

Abstract

Dilated cardiomyopathy (DCM) is the most common cardiomyopathy and 40-50% patients may die or need heart transplant in 5 years after diagnosis. Although heart transplantation being the most effective life-saving option of end-stage DCM, scarcity of donors and series of complications prevent many patients from receiving timely treatment. Pulmonary artery banding (PAB) is recently described as an alternative strategy for end-stage DCM, with low left ventricular function (LVEF) but preserved right ventricular function, may potentially restore heart function and delay the need for heart transplantation, but current clinical evidence is still insufficient. On the other hand, the medication treatment of DCM in pediatric patients is mostly based on the experience of adults. It remains unclear whether PAB combing medication treatment could benefit infants and young children patients. The aim of this study was to assess the short-term efficacy of PAB combined with medication therapy in infants and young children with end stage DCM, comparing with medication therapy alone. This is a retrospective analysis of 18 consecutive pediatric patients aged ranging from 1 month to 44 months old who diagnosed with end-stage DCM (LVEF30%) with preserved right ventricular function between 2019 and 2023 in our hospital. All patients had been treated with conventional medications for two months. Then they were divided in two groups: PAB surgery group (6/18), and non-surgery group (12/18). Regardless of whether surgery was performed, both groups continued to receive medication treatment. Recovery of ventricular function was primary endpoints. Secondary endpoints included 180-day mortality and severe heart failure (LVEF≤30%). We found there were no differences in age, weight, height, BMI, renal function, liver function, pulmonary hypertension, tricuspid valve regurgitation, mitral valve regurgitation and genetic abnormalities between those with and without PAB surgery. Comparing with non-surgery group, 5 patients in surgery group regain the normal cardiac ejection fraction (LVEF≥50%) (5/6, 83.3% vs. 4/12, 33.3%, P=0.131). A total of 3 patients had sudden death in non-surgery group, and there was no death in surgery group (P=0.180). 5 patients (5/12, 41.7%) still remain the low heart failure (LVEF≤40%) after 6 months of enrollment only given medical therapy, and none of patients present with LVEF≤40% in PAB surgery group (0/6, 0% vs. 8/12, 67.7%, P=0.034). Pulmonary artery banding is safe and effective in infants and young children with end-stage DCM with preserved right ventricular function. Combined with conventional heart failure medication therapy, it may provide short-term benefits post-operatively, decrease the cardiogenic shock, and act as a bridge to recovery and potentially reduce the need for heart transplantation. Long-term effects remain further observation, and larger randomized controlled trials would be more persuasive in validating its efficacy.