Morphologic and Functional Predictors of Eventual Circulation in the Fetus With Pulmonary Atresia or Critical Pulmonary Stenosis With Intact Septum

Gardiner, Helena M.; Belmar, Cristián; Tulzer, Gerald; Barlow, Anna; Pasquini, Lucia; Carvalho, J. S.; Daubeney, Piers E.F.; Rigby, Michael L.; Gordon, Fabiana; Kulinskaya, Elena; Franklin, Rodney C. G.

doi:10.1016/j.jacc.2007.08.073

Cited by 115 publications

(100 citation statements)

References 28 publications

(37 reference statements)

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“…Furthermore, obstructive lesions can develop or progress as pregnancy advances and may not be recognisable around 20 weeks of gestation. 32,33 We believe that another factor that adds to the persistent low detection rates is the fact that training programmes do not sufficiently teach how to diagnose those anomalies in fourchamber and sagittal views. The difficulty in the diagnosis of anomalies of the aortic arch has been widely acknowledged, 34 yet this study showed a significant increase in the detection rate of coarctation of the aorta.…”

Section: Discussionmentioning

confidence: 99%

Prenatal detection of congenital heart disease—results of a national screening programme

Velzen

Clur

Rijlaarsdam

et al. 2015

BJOG

188

226

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Objective Congenital heart disease (CHD) is the most common congenital malformation and causes major morbidity and mortality. Prenatal detection improves the neonatal condition before surgery, resulting in less morbidity and mortality. In the Netherlands a national prenatal screening programme was introduced in 2007. This study evaluates the effects of this screening programme. Design Geographical cohort study. Setting Large referral region of three tertiary care centres. Population Fetuses and infants diagnosed with severe CHD born between 1 January 2002 and 1 January 2012. Methods Cases were divided into two groups: before and after the introduction of screening. Main outcome measures Detection rates were calculated. Results The prenatal detection rate (n = 1912) increased with 23.9% (95% confidence interval [95% CI] 19.5–28.3) from 35.8 to 59.7% after the introduction of screening and of isolated CHD with 21.4% (95% CI 16.0–26.8) from 22.8 to 44.2%. The highest detection rates were found in the hypoplastic left heart syndrome, other univentricular defects and complex defects with atrial isomerism (>93%). Since the introduction of screening, the ‘late’ referrals (after 24 weeks of gestation) decreased by 24.3% (95% CI 19.3–29.3). Conclusions This is the largest cohort study to investigate the prenatal detection rate of severe CHD in an unselected population. A nationally organised screening has resulted in a remarkably high detection rate of CHD (59.7%) compared with earlier literature.

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Section: Discussionmentioning

confidence: 99%

Prenatal detection of congenital heart disease—results of a national screening programme

Velzen

Clur

Rijlaarsdam

et al. 2015

BJOG

188

226

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“…Several reports have been published regarding the prediction of a non‐biventricular/univentricular outcome in fetuses with PAIVS/CPS5, 11, 15, 21. Using the criteria published by Roman et al 15,.…”

Section: Discussionmentioning

confidence: 99%

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

Tulzer

Arzt

Gitter

et al. 2018

Ultrasound in Obstet & Gyne

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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.

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“…We did not have longitudinal data for analysis during these pregnancies; TV annulus growth may be impaired through gestation (crossing Z-score lines) as is true in all forms of left and right outflow obstruction [4] . RV length as well may progressively shorten in critical outflow lesions with little ventricular inflow [6][7][8] . Unfortunately, due to the high pregnancy termination rates in our study and in others, there are not sufficient available data to prognosticate well-based earlier observations and subsequent growth velocities, therefore extrapola- …”

Section: Discussionmentioning

confidence: 99%

327: Prenatal tricuspid valve size as a predictor of postnatal outcome in patients with severe pulmonary stenosis or pulmonary atresia with intact ventricular septum

Moon‐Grady

Lemley

Kipps

et al. 2011

American Journal of Obstetrics and Gynecology

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Morphologic and Functional Predictors of Eventual Circulation in the Fetus With Pulmonary Atresia or Critical Pulmonary Stenosis With Intact Septum

Abstract: The best predictive scores for post-natal outcome in fetal PAIVS/CPS are a combination of morphologic and physiological variables, which predict a BV circulation with a sensitivity of 92% and specificity of 100% before 26 weeks.

Cited by 115 publications

References 28 publications

Prenatal detection of congenital heart disease—results of a national screening programme

Prenatal detection of congenital heart disease—results of a national screening programme

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

327: Prenatal tricuspid valve size as a predictor of postnatal outcome in patients with severe pulmonary stenosis or pulmonary atresia with intact ventricular septum

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