Immediate effects and outcome of in‐utero pulmonary valvuloplasty in fetuses with pulmonary atresia with intact ventricular septum or critical pulmonary stenosis

Abstract

ObjectiveTo assess the immediate effects of fetal pulmonary valvuloplasty on right ventricular (RV) size and function as well as in‐utero RV growth and postnatal outcome.MethodsPatients with pulmonary atresia with intact ventricular septum (PAIVS) or critical pulmonary stenosis (CPS) who underwent fetal pulmonary valvuloplasty at our center between October 2000 and July 2017 were included. Echocardiographic data obtained before and after the procedure were analyzed retrospectively (median interval after intervention, 1 (range, 1–3) days) for ventricular and valvular dimensions and ratios, RV filling time (duration of tricuspid valve (TV) inflow/cardiac cycle length), TV velocity time integral (TV‐VTI) × heart rate (HR) and tricuspid regurgitation (TR) velocity. Longitudinal data were collected from only those fetuses followed up in our center. Outcome was assessed using the scoring system as described by Roman et al. for non‐biventricular outcome.ResultsThirty‐five pulmonary valvuloplasties were performed in our institution on 23 fetuses with PAIVS (n = 15) or CPS (n = 8). Median gestational age at intervention was 28 + 4 (range, 23 + 6 to 32 + 1) weeks. No fetal death occurred. Immediately after successful intervention, RV/left ventricular length (RV/LV) ratio (P ≤ 0.0001), TV/mitral valve annular diameter (TV/MV) ratio (P ≤ 0.001), RV filling time (P ≤ 0.00001) and TV‐VTI × HR (P ≤ 0.001) increased significantly and TR velocity (P ≤ 0.001) decreased significantly. In fetuses followed longitudinally to delivery (n = 5), RV/LV and TV/MV ratios improved further or remained constant until birth. Fetuses with unsuccessful intervention (n = 2) became univentricular, all others had either a biventricular (n = 15), one‐and‐a‐half ventricular (n = 3) or still undetermined (n = 3) outcome. Five of nine fetuses with a predicted non‐biventricular outcome, in which the procedure was successful, became biventricular, while two of nine had an undetermined circulation.ConclusionIn selected fetuses with PAIVS or CPS, in‐utero pulmonary valvuloplasty led immediately to larger RV caused by reduced afterload and increased filling, thus improving the likelihood of biventricular outcome even in fetuses with a predicted non‐biventricular circulation. © 2018 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of the International Society of Ultrasound in Obstetrics and Gynecology.

Severe Pulmonary Stenosis or Atresia with Intact Ventricular Septum in the Fetus: The Natural History

“…This dilemma has encouraged selected centers to promote intrauterine valvuloplasty of the pulmonary valve (PV) in cases of PSAIVS in order to prevent the intrauterine evolution to hypoplastic right heart, and consequently the sequela of Fontan palliation [24,31,[33][34][35][36][37][38]. Fetal pulmonary valvuloplasty may relief RV outflow tract obstruction, promote RV growth and function, and increase the likelihood of a BV circulation postnatally.…”

Fetal cardiac intervention for right sided heart disease: Pulmonary atresia with intact ventricular septum

“…The group at the Children's Hospital Heart Center at Linz has published a large cohort of patients at a single center (Tulzer et al, ). They reported 35 FCI on 25 maternal fetal pairs for either PA/IVS ( n = 15) or critical pulmonary valve stenosis ( n = 8).…”

“…In the technically successful FCI group, of the live born infants, 5/7 (70%) had a BV outcome, 2/7 died prior to discharge. The group at the Children's Hospital Heart Center at Linz has published a large cohort of patients at a single center (Tulzer et al, 2018). They reported 35 FCI on 25 maternal fetal pairs for either PA/IVS (n = 15) or critical pulmonary valve stenosis (n = 8).…”

“…The bulk of the literature to date has examined feasibility and short and medium term outcomes on left sided heart disease. However, there is an increasing amount of data examining the feasibility and short term outcome of balloon dilation of the pulmonary valve in right sided heart disease (Edwards et al, 2015;Gardiner et al, 2008;Gomez Montes, Herraiz, Mendoza, & Galindo, 2012;Huhta, Quintero, Suh, & Bader, 2004;Kohl, 2002;Moon-Grady et al, 2015;Pedra et al, 2014;Tulzer et al, 2018;Tulzer et al, 2002;Tulzer & Gardiner, 2006;Tworetzky et al, 2009). The rationale for FCI is to alter the natural history of right sided heart disease or to prevent hemodynamic compromise in the setting of hydrops fetalis.…”

“…The high mortality is attributable to a variety of reasons occurring before any post-natal intervention, at the time of surgery/catheter intervention and also late after surgery (Moon-Grady et al, 2015;Tulzer et al, 2002). A wide range of survival rates has been published with a 1-year survival from 65% to 92% and a 10-year survival from 43% to 76% (Edwards et al, 2015;Tulzer et al, 2018). The ultimate goal is to achieve a biventricular (BV) repair but this is only feasible in 32% to 63% of reported series of patients with postnatal cardiac intervention (either surgery or catheter based intervention; Edwards et al, 2015;Gomez Montes et al, 2012).…”