Prenatal Features of Ductus Arteriosus Constriction and Restrictive Foramen Ovale in d-Transposition of the Great Arteries

Abstract

Background-Although most neonates with d-transposition of the great arteries (TGA) have an uncomplicated preoperative course, some with a restrictive foramen ovale (FO), ductus arteriosus (DA) constriction, or pulmonary hypertension may be severely hypoxemic and even die shortly after birth. Our goal was to determine whether prenatal echocardiography can identify these high-risk fetuses with TGA. Methods and Results-We reviewed the prenatal and postnatal echocardiograms and outcomes of 16 fetuses with TGA/intact ventricular septum or small ventricular septal defect. Of the 16 fetuses, 6 prenatally had an abnormal FO (fixed position, flat, and/or redundant septum primum). Five of the 6 had restrictive FO at birth. Five fetuses had DA narrowing at the pulmonary artery end in utero, and 6 had a small DA (diameter z score of ϽϪ2.0). Of 4 fetuses with the most diminutive DA, 2 also had an abnormal appearance of the FO, and both died immediately after birth. One other fetus had persistent pulmonary hypertension. Eight fetuses had abnormal Doppler flow pattern in the DA (continuous high-velocity flow, nϭ1; retrograde diastolic flow, nϭ7). Conclusions-Abnormal

Sensitivity and Specificity of Prenatal Features of Physiological Shunts to Predict Neonatal Clinical Status in Transposition of the Great Arteries

“…Indeed, there is now a significant number of reported cases of patients with TGA who presented either at autopsy without prior diagnosis or of children who were moribund on admission and who died within a few hours of birth. [5][6][7][8][9]15,16 We showed that prenatal diagnosis reduced the mortality rate of patients with TGA. 3 However, Soongswang et al 5 and Maeno et al 9 reported 3 cases of prenatally diagnosed TGA in patients who died immediately after birth.…”

“…Prenatal features of the foramen ovale and of the ductus arteriosus were reviewed retrospectively for the period November 1997 and November 1999 and described prospectively during the second period of the study. According to Maeno et al 9 and Wilson et al, 14 the foramen ovale was considered at risk of postnatal early restriction if the aneurysmal septum primum bulged Ͼ50% of the way across to the left atrial free wall; if the angle between the septum primum and the rest of the atrial septum was Ͻ30°; or if the septum did not have the typical swinging motion during the cardiac cycle. For the ductus arteriosus, the diameter was measured at the narrowest portion, typically at the pulmonary end.…”

“…3,[5][6][7][8] Indeed, antenatal restriction of the foramen ovale and/or the ductus arteriosus were found predictive of significant neonatal morbidity and mortality. 9 Because the surgical mortality for the arterial switch operation is as low as 2% in many institutions, 10 -13 the preoperative mortality is a major issue in the management and outcome of infants with TGA. Here, we reviewed the prenatal features of the foramen ovale and of the ductus arteriosus in all our cases of prenatally diagnosed TGA to address the relation between prenatal echo features and need for urgent intervention.…”

Early discontinuation of intravenous prostaglandin E1 after balloon atrial septostomy is associated with an increased risk of rebound hypoxemia

“…14,15 Premature closure of the foramen ovale and/or ductus arteriosus also has been shown to be associated with severe postnatal hypoxemia [16][17][18] and pulmonary hypertension, thought to occur as a result of pulmonary vascular remodeling. 18 The rationale to discontinue the PGE 1 infusion is based on a theoretical risk that if the left ventricle (LV) is exposed to a lower PVR preoperatively, it is less likely to adapt to the sudden increase in LV afterload that occurs following the arterial switch procedure. We did not identify an obvious increased risk of cardiorespiratory stability as judged by the duration of assisted ventilation, need for cardiotropic support or frequency of cardiac dysrhythmias.…”

Late-onset pulmonary arterial hypertension after successful early arterial switch surgery for simple transposition of the great arteries

“…Antenatal ductal constriction is known to be associated with persistent pulmonary hypertension in neonates. (12,24) In infants with transposition of the great arteries and intact ventricular septum as young as one week considerable intimal proliferation with luminal occlusion has been reported. (12,25) With persistence of extensive bronchial-pulmonary shunts resulting in increased blood flow, and inherent structural defects of the muscular pulmonary arteries secondary to chromosomal abnormalities, arteriolar spasm and a gradual increase in pulmonary vascular resistance follows.…”