H. M. Gardiner

Fetal pulmonary valvuloplasty for critical pulmonary stenosis or atresia with intact septum

Progressive stenosis of the semilunar valves in utero can be life threatening. We treated two fetuses with complete or almost complete pulmonary atresia and imminent hydrops (increased cardiothoracic ratio, pericardial effusion, holosystolic tricuspid regurgitation extending into diastole, and abnormal venous Dopplers). We dilated the pulmonary valve of two fetuses in utero at 28 and 30 weeks gestation, through the mothers abdomens. After the procedure, the fetuses had decreased signs of circulatory failure and gestation continued until near term. In the neonatal period, we did a repeat valvuloplasty with systemic-to-pulmonary arterial shunt. Both children (now aged 18 months and 12 months) now have biventricular circulation. Surgery on selected fetuses with semilunar valve stenosis or atresia, or both, can extend pregnancy and favourably change the postnatal surgical options.

Z-scores of the fetal aortic isthmus and duct: An aid to assessing arch hypoplasia

Prenatal diagnosis of isolated coarctation of the aorta suffers from high false positive and false negative rates. The aim of our study was to develop Z‐scores for the aortic isthmus in normal fetuses as a reference for fetuses with suspected coarctation.

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

OBJECTIVESnThe purpose of this study was to determine the morphologic and physiological predictors of post-natal surgical pathway in a longitudinal series of fetuses with pulmonary atresia with intact ventricular septum (PAIVS) and/or critical pulmonary stenosis with reversal of ductal flow (CPS) using statistical modeling.nnnBACKGROUNDnPulmonary atresia with intact ventricular septum is rarely associated with chromosomal or extra cardiac malformations, so decisions about continuing a pregnancy are strongly influenced by the prediction of univentricular (UV) or biventricular (BV) circulation.nnnMETHODSnPredictive scores were derived, using a combination of z-scores of fetal cardiac measurements (for femoral length) and tricuspid/mitral valve (TV/MV) ratios, to facilitate early prediction of UV or BV circulation in 21 fetuses with PAIVS (18 fetuses) or CPS (3 fetuses) between 1998 and 2004. We also assessed the predictive value of coronary fistulae and right atrial pressure (RAP) score (comprising the tricuspid valve, foramen ovale, and ductus venosus Doppler).nnnRESULTSnOne-half of the cohort was first assessed before 23 gestational weeks (range 15.7 to 33.7 weeks). The TV z-score was a good predictor at all gestations, but the best predictive scores for specific gestations were pulmonary valve (PV) z-score (<23 weeks), median TV z-score (<26 weeks), the combination of median PV z-score and the median TV/MV ratio (26 to 31 weeks), and the combination of median TV z-score and median TV/MV ratio (>31 weeks). The RAP score and coronary fistulae were good independent predictors: RAP score >3 predicted BV with area under the curve of 0.833, and detection of fistulae usually predicted a UV route.nnnCONCLUSIONSnThe 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.

Total anomalous pulmonary venous connection: impact of prenatal diagnosis: Fetal TAPVC

To investigate whether prenatal screening is effective in the detection of total anomalous pulmonary venous connection (TAPVC) and to identify common prenatal features.

Erratum: Z-scores of the fetal aortic isthmus and duct: An aid to assessing arch hypoplasia (Ultrasound in Obstetrics and Gynecology (2007) 29, (628-633))

The original article to which this Erratum refers was published in Ultrasound in Obstetrics and Gynecology 29 2007, 628–633.

OC15.06: Fetal PR interval: a comparison of electrical and mechanical methods

and post-mortem data from the regional neonatal and paediatric pathology centre for the same time. We defined as major cardiac anomalies those cases that needed intervention by either surgery or catheterisation within the first month of life. Prior to 1998 most ultrasonographers would be trained to examine the 4 chamber view of the heart. In 1998 we introduced a course that included hand’s on training in examination of the outflow tracts and demonstrating videos of common abnormalities. We have retrospectively examined detection rates and correlated these with the introduction of the regional training scheme. Results: During the study period there were 951 cases with CHD. The incidence of CHD was 0.33% for the study population. The overall prenatal detection rate for CHD was 40.3%. For anomalies usually seen at the 4 chamber view the detection rate improved from 47.2% to 65.4% after the training courses (p < 0.005). This improvement was also evident for anomalies that require the cardiac outflow tracts to be visualised (from 20.2% to 32% p < 0.005). The biggest gain was in cases with isolated CHD where detection rates improved from 33%–54% and from 8%–19% respectively (p < 0.005). Conclusions: Improved training of the ultrasonographers, particularly enabling them to identify the outflow tracts with confidence, can help to increase prenatal detection of major cardiac defects.

P05.29: Evaluation of PR interval in fetuses of Anti Ro positive pregnancies

Objective: To establish the normal limits of FHR and reliability of fetal heart anatomy evaluation during obstetric scan at 12 to 16 + 6 weeks of gestation. Material and methods: Analysis of transabdominal ultrasound scans performed in 343 pregnant women at 12 to 16 + 6 weeks of gestation. 2D, M-mode and Doppler techniques were used. Gestational week was established on the basis of CRL, BPD, HC, AC and FL measurements. Demographic factors, obstetrical history and follow up of pregnancies were collected. All data were statistically reviewed (Chi test, Student T test, Mc Person correlation). Results: Maternal age varied between 18 and 44 years (mean 32.7 ± 6) and paternal age: 22–62 years (mean 35.4 ± 7). There were 56% primigravidas, 18% women had a history of two or more deliveries and 14% had one or more abortions. FHR varied between 127 and 185 bpm (mean 151 ± 9). The statistically significant difference was found between FHR at 12 weeks (mean 160 ± 8) and following gestational weeks (149–155 bpm). The r – correlation ratio between FHR and gestational week was −0.41 (ns), maternal age – r = 0.40 (ns), paternal age r = 0.28 (ns) and nuchal translucency r = −0.11 (ns). The ultrasound visualization of four chamber view was possible from 12 weeks (72%) to 14 weeks – full evaluation of fetal heart structures could be done in about half of patients then (54%). The ratio between fetal heart area and fetal chest area varied from 0.22 to 0.29. There was no significant correlation between that ratio and gestational week. Conclusions: Fetal heart rate at 12 to 16 weeks was irrespective of the duration of gestation. Parents’ age and nuchal translucency did not have influence on the fetal heart rate. It is possible to evaluate fetal heart anatomy as early as at 12 to 16 weeks. The fetal heart size is then smaller than later in pregnancy.

OC30.07: Selection of cases for fetal pulmonary valvuloplasty

Background: Critical pulmonary stenosis or atresia (PAIVS) diagnosed in the second trimester progresses resulting in right ventricular (RV) growth failure. Fetal pulmonary valvuloplasty may improve outcome. We explore the determinants of right-sided growth in the fetus with PAIVS. Methods: Serial fetal echocardiography in 10 of 14 consecutive fetuses diagnosed with PAIVS in a fetal medicine centre (1999–2003). Assessment of morphology, right and left sided valvular and ventricular growth, Doppler across valves and in arterial and venous ducts (DV). DV waveforms were classified as ‘‘abnormal’’ if absent or reversed end diastolic flow (AREDF), signifying high right atrial pressure (RAP). Postnatal echocardiography confirmed morphology, Doppler & oval foramen restriction. Fetal pulmonary valvuloplasty (× 3) was performed percutaneously under ultrasound guidance at 21–30 weeks. Results: Three pregnancies with PAIVS were terminated. Six of the remaining 11 survived infancy, four with biventricular circulation. Fetal pulmonary valvuloplasty was offered in selected cases. Morphology was unsuitable in three. Two further monochorionic diamniotic twin pregnancies with good anatomy were excluded leaving three suitable for valvuloplasty. Valvuloplasty was successful in 2 cases but placental bleeding and bradycardia precipitated emergency delivery before valvuloplasty in the third. Eleven had high RAP (tricuspid regurgitation) with AREDF and hydrops in 1 case. The three with low RAP had the worst TV and PV growth velocities. At birth, median (95% CI) TV annulus Z score was −4.29 (−6.57 to −1.71). TV growth was significantly better in those with high RAP (−4.29 vs −6.00, p = 0.02) and oval foramen restriction. Conclusions: Fetuses with high RAP show the best RV growth, but may develop hydrops. AREDF may paradoxically be associated with better outcome, as fetal valvuloplasty may be most effective in those with high RAP.

P01.06: Significance of left superior caval vein in the fetus

Objective: To analyze the consequences of termination we studied the abortion rate, the distribution of CHD’s (inand excluding the abortions) and the year of termination, and compared the data to a contemporaneous postnatal population. Methods: We reviewed data from all isolated structural CHD’s either born or terminated between January 1 1991 and July 1 2003 at the Yale New Haven Hospital. We subdivided CHD diagnoses into those altering and not altering the four-chamber view. We developed a severity score to code for all CHD’s. Code 1 was given to a CHD amenable to non-complex biventricular repair, code 2 to complex biventricular repair, code 3 to non-complex univentricular repair and code 4 to complex univentricular repair. Results: Of the 236 prenatally diagnosed patients, 110 opted for an abortion (47%) and the remaining 126 decided to carry out their pregnancy (53%). Within the group of 206 CHD’s with abnormal four-chamber views termination of pregnancy was chosen significantly more often than in the group (n = 30) that did not have an altered four-chamber view. The abortion rate is the highest in group 3 (75%), going down to group 4 (51%), to group 2 (30%), to group 1 (7%), with a p-value 0.0001 in all steps. The distribution of severity scores comparing preand postnatally (n = 127) diagnoses showed a different pattern with the inor exclusion of abortions. The likelihood of abortion did not change in any severity score group over the 12.5 years this study covers. Conclusions: The spectrum of neonatal CHD’s is influenced by the option to terminate. Termination was chosen more often when the CHD alters the four-chamber view or when the CHD is not amenable to biventricular repair. The abortions changed the pattern of severity scores between the prenatal and postnatal diagnosis group. We have not seen a trend in the rate of termination within any of the severity score groups in the 12.5 year study period.