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Objective: Fetal aortic stenosis may progress to hypoplastic left heart syndrome. Fetal valvuloplasty (FV) has been proposed to improve left heart hemodynamics and maintain biventricular (BV) circulation. The aim of this study was to assess FV efficacy by comparing survival and postnatal circulation between fetuses that underwent FV and those that did not. Methods: This was a retrospective multicenter study of fetuses with aortic stenosis that underwent FV between 2005 and 2012, compared with contemporaneously enrolled natural history (NH) cases sharing similar characteristics at presentation but not undergoing FV. Main outcome measures were overall survival, BV-circulation survival and survival after birth. Secondary outcomes were hemodynamic change and left heart growth. A propensity score model was created including 54/67 FV and 60/147 NH fetuses. Analyses were performed using logistic, Cox or linear regression models with inverse probability of treatment weighting (IPTW) restricted to fetuses with a propensity score of 0.14–0.9, to create a final cohort for analysis of 42 FV and 29 NH cases. Results: FV was technically successful in 59/67 fetuses at a median age of 26 (21–34) weeks. There were 7/72 (10%) procedure-related losses, and 22/53 (42%) FV babies were delivered at < 37 weeks. IPTW demonstrated improved survival of liveborn infants following FV (hazard ratio, 0.38; 95% CI, 0.23–0.64; P = 0.0001), after adjusting for circulation and postnatal surgical center. Similar proportions had BV circulation (36% for the FV cohort and 38% for the NH cohort) and survival was similar between final circulations. Successful FV cases showed improved hemodynamic response and less deterioration of left heart growth compared with NH cases (P ≤ 0.01). Conclusions: We report improvements in fetal hemodynamics and preservation of left heart growth following successful FV compared with NH. While the proportion of those achieving a BV circulation outcome was similar in both cohorts, FV survivors showed improved survival independent of final circulation to 10 years' follow-up. However, FV is associated with a 10% procedure-related loss and increased prematurity compared with the NH cohort, and therefore the risk-to-benefit ratio remains uncertain. We recommend a carefully designed trial incorporating appropriate and integrated fetal and postnatal management strategies to account for center-specific practices, so that the benefits achieved by fetal therapy vs surgical strategy can be demonstrated clearly.

Objective: Fetal aortic stenosis may progress to hypoplastic left heart syndrome. Fetal valvuloplasty (FV) has been proposed to improve left heart hemodynamics and maintain biventricular (BV) circulation. The aim of this study was to assess FV efficacy by comparing survival and postnatal circulation between fetuses that underwent FV and those that did not. Methods: This was a retrospective multicenter study of fetuses with aortic stenosis that underwent FV between 2005 and 2012, compared with contemporaneously enrolled natural history (NH) cases sharing similar characteristics at presentation but not undergoing FV. Main outcome measures were overall survival, BV-circulation survival and survival after birth. Secondary outcomes were hemodynamic change and left heart growth. A propensity score model was created including 54/67 FV and 60/147 NH fetuses. Analyses were performed using logistic, Cox or linear regression models with inverse probability of treatment weighting (IPTW) restricted to fetuses with a propensity score of 0.14–0.9, to create a final cohort for analysis of 42 FV and 29 NH cases. Results: FV was technically successful in 59/67 fetuses at a median age of 26 (21–34) weeks. There were 7/72 (10%) procedure-related losses, and 22/53 (42%) FV babies were delivered at < 37 weeks. IPTW demonstrated improved survival of liveborn infants following FV (hazard ratio, 0.38; 95% CI, 0.23–0.64; P = 0.0001), after adjusting for circulation and postnatal surgical center. Similar proportions had BV circulation (36% for the FV cohort and 38% for the NH cohort) and survival was similar between final circulations. Successful FV cases showed improved hemodynamic response and less deterioration of left heart growth compared with NH cases (P ≤ 0.01). Conclusions: We report improvements in fetal hemodynamics and preservation of left heart growth following successful FV compared with NH. While the proportion of those achieving a BV circulation outcome was similar in both cohorts, FV survivors showed improved survival independent of final circulation to 10 years' follow-up. However, FV is associated with a 10% procedure-related loss and increased prematurity compared with the NH cohort, and therefore the risk-to-benefit ratio remains uncertain. We recommend a carefully designed trial incorporating appropriate and integrated fetal and postnatal management strategies to account for center-specific practices, so that the benefits achieved by fetal therapy vs surgical strategy can be demonstrated clearly. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Objectives\ud \ud Fetal aortic valvuloplasty (FV) aims to prevent fetal aortic valve stenosis progressing into hypoplastic left heart syndrome (HLHS), which results in postnatal univentricular (UV) circulation. Despite increasing numbers of FVs performed worldwide, the natural history of the disease in fetal life remains poorly defined. The primary aim of this study was to describe the natural history of fetal aortic stenosis, and a secondary aim was to test previously published criteria designed to identify cases of emerging HLHS with the potential for a biventricular (BV) outcome after FV.\ud Methods\ud \ud From a European multicenter retrospective study of 214 fetuses with aortic stenosis (2005–2012), 107 fetuses in ongoing pregnancies that did not undergo FV were included in this study and their natural history was reported. We examined longitudinal changes in Z-scores of aortic and mitral valve and left ventricular dimensions and documented direction of flow across the foramen ovale and aortic arch, and mitral valve inflow pattern and any gestational changes. Data were used to identify fetuses satisfying the Boston criteria for emerging HLHS and estimate the proportion of these that would have been ideal FV candidates. We applied the threshold score whereby a score of 1 was assigned to fetuses for each Z-score meeting the following criteria: left ventricular length and width > 0; mitral valve diameter > −2; aortic valve diameter > −3.5; and pressure gradient across either the mitral or aortic valve > 20 mmHg. We compared the predicted circulation with known survival and final postnatal circulation (BV, UV or conversion from BV to UV).\ud Results\ud \ud Among the 107 ongoing pregnancies there were eight spontaneous fetal deaths and 99 livebirths. Five were lost to follow-up, five had comfort care and four had mild aortic stenosis not requiring intervention. There was intention-to-treat in these 85 newborns but five died prior to surgery, before circulation could be determined, and thus 80 underwent postnatal procedures with 44 BV, 29 UV and seven BV-to-UV circulatory outcomes. Of newborns with intention-to-treat, 69/85 (81%) survived ≥ 30 days. Survival at median 6 years was superior in cases with BV circulation (P = 0.041). Those with a postnatal UV circulation showed a trend towards smaller aortic valve diameters at first scan than did the BV cohort (P = 0.076), but aortic valve growth velocities were similar in both cohorts to term. In contrast, the mitral valve diameter was significantly smaller at first scan in those with postnatal UV outcomes (P = 0.004) and its growth velocity (P = 0.008), in common with the left ventricular inlet length (P = 0.004) and width (P = 0.002), were reduced significantly by term in fetuses with UV compared with BV outcome. Fetal data, recorded before 30 completed gestational weeks, from 70 treated neonates were evaluated to identify emerging HLHS. Forty-four had moderate or severe left ventricular depression and 38 of these had retrograde flow in the aortic arch and two had left-to-right flow at atrial level and reversed a-waves in the pulmonary veins. Thus 40 neonates met the criteria for emerging HLHS and BV circulation was documented in 13 (33%). Of these 40 cases, 12 (30%) had a threshold score of 4 or 5, of which five (42%) had BV circulation without fetal intervention.\ud Conclusions\ud \ud The natural history in our cohort of fetuses with aortic stenosis and known outcomes shows that a substantial proportion of fetuses meeting the criteria for emerging HLHS, with or without favorable selection criteria for FV, had a sustained BV circulation without fetal intervention. This indicates that further work is needed to refine the selection criteria to offer appropriate therapy to fetuses with aortic stenosis.

A 21 week fetus was diagnosed with complete heart block. From the first diagnosis until 38 weeks gestation, umbilical venous pulsations occurred during ventricular systole. The ventricular frequency was 49 bpm. The venous pulsations corresponded with the ventricular frequency. At 38 weeks venous pulsations also occurred during ventricular diastole. Umbilical pulsations during diastole were associated with a decrease of ventricular frequency to 28 bpm and fetal hydrops. The venous pulsations during ventricular systole seem to be characteristic of complete heart block. The pulsations during ventricular diastole reflect the fetal heart failure. Doppler ultrasound aids in the diagnosis and surveillance of the fetus with complete heart block.

Symptom Polyhydramnios: Analysis of 56 Cases: 56 cases of polyhydramnios, which were diagnosed sonographically over a period of three years, were analysed retrospectively in this study. In 9334 births, the prevalence of this symptom was 0.6%. Malformations were the most common cause (26.7%) of polyhydramnios (p

The purpose of this study was to assess the effects of smoking one cigarette (nicotine mean 0.63 +/- 0.17 mg) on uterine- and foetal cardiovascular Doppler parameters in healthy pregnant smokers. All pregnancies (n = 16; mean gestational age: 36 +/- 4 weeks) had been uneventful and all foetuses were appropriate for gestational age with normal baseline Doppler parameters and normal foetal outcome (birthweight: 3254 +/- 340 grams). Measurements, performed immediately before and after smoking, included pulsatility index (PI) of umbilical artery (UA), middle cerebral artery (MCA), foetal descending aorta and uterine artery as well as maternal and foetal heart rate. The ratio of UA/MCA PI was used to assess centralisation. Changes in foetal cardiac output were determined by: time-velocity integral times heart rate, at aortic and pulmonary valve level. Foetal heart rate (p < 0.0005, paired t-test) and maternal heart rate (p < 0.05) increased significantly. All other parameters did not change significantly. However, in one additional woman with labile hypertension and increased baseline uterine artery PI (1.9), smoking of one cigarette caused a substantial rise in uterine artery PI to 3.25 ten minutes after smoking. Middle cerebral artery PI decreased from 2.2 to 1.18 with an unchanged cardiac output and umbilical artery PI raising the UA/MCA PI ratio from 0.51 to 0.81, suggesting a brain sparing effect. Smoking of one cigarette raised maternal and foetal heart rate. There was no evidence of other cardiovascular effects or centralisation in healthy foetuses of normal pregnancies, but this might not be true in foetus of pathologic pregnancies.

Tetralogy of Fallot with Absent Pulmonary Valve - Prenatal Diagnosis and Management in the Second Trimester: Tetralogy of Fallot with absent pulmonary valve is a rare congenital cardiac malformation detected in 3% to 6% of tetralogy of Fallot patients. The prognosis depends on respiratory complications. In the present case the diagnosis was made at 18 week's gestation with two-dimensional and Doppler echocardiography. A ventricular septal defect and overriding aorta, absent valve echo, pulmonary regurgitation, and cystic pulsatile dilatation of the left pulmonary artery were the main diagnostic criteria. Severe respiratory complications were assumed in combination with a hyperechogenic left lung and termination of pregnancy was performed at 20 weeks' of gestation.