Abstract

Background: Percutaneous balloon valvuloplasty (PBPV) is recommended as a first-choice treatment for critical pulmonary stenosis (CPS). A concept of perinatal integrative management has been developed. Unfortunately, the evidence on the advantage of integrative management for CPS during the perinatal period is absent.Methods: Single-center, observational, preliminary research has been developed, and three groups have been enrolled. There were 42 children with CPS enrolled for this study between January 2014 and December 2017 in our center, and their follow-up duration is at least 1 year. Three groups were set up: the integrative perinatal management group (group I), who received prenatal diagnosis with perinatal management to maintain circulation and an optimized PBPV procedure; the prenatal diagnosis group (group PR), who received a diagnosis of pulmonary stenosis before birth without any monitoring and perinatal management; and the postnatal diagnosis group (group PO), who received the CPS diagnosis after birth.Result: There were 13 patients enrolled in group I, 11 babies enrolled in group PR, and 18 cases included in group PO. Integrative management helped to put the timing of PBPV in advance. The age for PBPV in group I was 9.38 ± 5.58 days, and groups PR and PO were 24.54 ± 4.87 and 49.11 ± 9.50 days, respectively. The average peak transvalvular gradient (PGs) of the perinatal management group (group I) and prenatal diagnosis group (group PR) remained at a stable level. However, the average PGs of group PO were progressively elevated during follow-up. Moreover, the follow-up data from group I revealed an advantage in RV development and functional restoration. There was no difference among the three groups in the ratio of reintervention and postoperative moderate pulmonary regurgitation during 1-year follow-up (p >0.05).Conclusion: Prenatal diagnosis helps to improve the outcomes of PBPV. Moreover, perinatal integrative medical management enhances the advantage of prenatal diagnosis. However, this research is still a small-size cohort study, and the limited population number and follow-up duration were the major limitations to expand the conclusions.

Highlights

  • Critical pulmonary stenosis (CPS) is a type of ductal-dependent disease, along with pulmonary atresia with intact ventricular septum (PAIVS), which account for 3% of all congenital heart diseases (CHD) [1]

  • Preliminary, and observational study, from January 2014 to December 2017, we recruited a cohort with CPS that presented with significant pressure differences across the affected area (>90 mmHg) based on echocardiography measurement or any evidence on right ventricular (RV) and/or pulmonary artery (PA) hypoplasia, and fetal echocardiography demonstrated a bidirectional shunt between the right and left atria

  • The peak transvalvular gradient (PG) increased to 84.54 ± 17.81 mmHg around the perinatal time and reached 96.23 ± 15.67 mmHg just before the Percutaneous balloon pulmonary valvuloplasty (PBPV) procedure

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Summary

Introduction

Critical pulmonary stenosis (CPS) is a type of ductal-dependent disease, along with pulmonary atresia with intact ventricular septum (PAIVS), which account for 3% of all congenital heart diseases (CHD) [1]. Percutaneous balloon pulmonary valvuloplasty (PBPV) is considered the most common treatment choice for CPS, which is predicted with biventricular heart management, for some single-ventricle predicted cases, surgical treatment, includes the Fontan, Glenn procedure [4, 5]. Ronai et al reported that fetal cardiac intervention is likely to provide better outcomes in some cases because it would be difficult to achieve acceptable ventricular function with extremely poor right heart hypoplasia [7]. The earlier pulmonary valvuloplasty is conducted in CPS patients, the better the benefits from shortening the duration of hypoxemia exposure and more capabilities for RV function restoration to be achieved [11]. Percutaneous balloon valvuloplasty (PBPV) is recommended as a first-choice treatment for critical pulmonary stenosis (CPS). The evidence on the advantage of integrative management for CPS during the perinatal period is absent

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