Evidence for uteroplacental malperfusion in fetuses with major congenital heart defects.

AIMS: Fetuses affected by congenital heart defects (CHD) are considered to be at increased risk of fetal growth restriction and intrauterine demise. Whether these risks are a direct consequence of fetal CHD or a result of associated uteroplacental dysfunction is not evident from the data of recent studies. The aim of this study was to investigate the prevalence of uteroplacental dysfunction reflected by abnormal uterine artery Doppler indices and reduced fetal growth in CHD pregnancies. METHODS: This is a retrospective case-control study including singleton pregnancies referred for detailed fetal cardiac assessment subsequently diagnosed with or without CHD. Mid-trimester uterine artery Doppler assessment at 20-24 weeks as well as third trimester fetal biometry and arterial Doppler pulsatility indices (PI) were performed. All fetal biometry were converted into centiles and Doppler values to multiples of median (MoM) to adjust for physiological changes with gestation. RESULTS: The study included 811 pregnancies including 153 cases where the fetus was diagnosed with CHD. Mid-pregnancy uterine artery PI was significantly higher in women with fetal CHD compared to controls (0.90MoM vs 0.83MoM; p = 0.006). In the third trimester, median centiles for fetal head circumference (45.4 vs 57.07; p<0.001), abdominal circumference (51.17 vs 55.71; p = 0.014), estimated fetal weight (33.6 vs 56.7; p<0.001) and cerebroplacental ratio (CPR: 0.84MoM vs 0.95MoM; p<0.001) were significantly lower in fetuses with CHD compared to controls. The percentage of small for gestational age births <10th centile (24.0% vs 10.7%; <0.001) and low CPR <0.6MoM (11.7% vs 2.5%; p<0.001) were significantly higher in the fetal CHD cohort. CONCLUSIONS: Mid-pregnancy uterine artery resistance is increased and subsequent fetal biometry reduced in pregnancies with CHD fetuses. These findings suggest that fetal CHD are associated with uteroplacental dysfunction, secondary to impaired maternal uteroplacental perfusion resulting in relative fetal hypoxaemia and reduced fetal growth

Fetal aorto-pulmonary window: case series and review of the literature

Aorto-pulmonary window is a rare congenital cardiac anomaly characterized by a communication between the aorta and the pulmonary artery above the semilunar valves. Prenatal diagnosis is rare. We report four fetuses with aorto-pulmonary window and review the relevant literature. Approximately half of the reported cases had additional cardiac defects; none had chromosomal abnormalities. In cases with normal cardiac connections, the diagnosis can be made prenatally on the standard three–vessel view as seen in two of our cases. In one fetus with complete transposition, the diagnosis was made retrospectively on sagittal views. In the remaining case the window was seen post-natally but could not be identified retrospectively due to the abnormal supero-inferior relationship of the ventricles and vessels

Perinatal changes in fetal ventricular geometry, myocardial performance and cardiac function in normal term pregnancies.

Background: The fetal heart at term is exposed to an increase in hemodynamic work as a consequence of fetal growth, increased circulating volume and alteration in loading patterns due to maturational changes in fetoplacental circulation. The extent to which these cardiovascular changes influence the human fetal and neonatal cardiac adaptation has not been fully elucidated. The aim of this study was to evaluate perinatal cardiovascular changes in ventricular geometry and myocardial performance in normal term fetuses. Methods: Prospective study of 108 uncomplicated pregnancies delivering at term. M-mode, twodimensional (2D) or B-mode, pulsed wave (PW) Doppler, PW tissue Doppler and 2D speckle tracking imaging were performed a few days before, and within 24 hours of birth. Results: Analysis of paired fetal and neonatal echoes demonstrated significant perinatal changes (p<0.0001 for all) in right ventricular (RV) and left ventricular (LV) geometry (RV/LV enddiastolic dimension ratio: 1.2 vs. 0.8, RV sphericity index: 0.53 vs. 0.40, LV sphericity index: 0.46 vs. 0.49). There were corresponding significant (p<0.001 for all) perinatal changes in global myocardial performance: LV myocardial performance index (MPI’): 0.60 vs. 0.47, RV MPI’: 0.61 vs. 0.42; systolic function: LV longitudinal systolic strain rate: -1.4 /s vs. -1.0 /s, RV longitudinal systolic strain rate: -1.5 /s vs. -1.0 /s; RV systolic annular peak velocity (S’): 5.3 cm/s vs. 6.5 cm/s; and diastolic function: LV diastolic annular peak velocity ratio (E’/A’): 0.8 vs.1.1. Conclusion: The findings support the concept that the perinatal period is associated with major changes in fetal ventricular geometry and cardiac function in response to significant alterations in loading conditions. Improved knowledge of perinatal cardiac changes in normal fetuses could facilitate better understanding of cardiac adaptation in normal and pathological pregnancies

Fetuses with right aortic arch Multicentre cohort study and meta-analysis.

OBJECTIVES: Recent antenatal screening guidelines for cardiac abnormalities has increased fetal diagnosis of right aortic arch (RAA). We aimed to establish outcome of fetal RAA without intra-cardiac abnormalities (ICA) to guide postnatal management. METHODOLOGY: Retrospective cohort study. Outcome measures were rates of chromosomal abnormalities, 22q11.2 deletion, fetal extra-cardiac abnormalities (ECA), postnatal ICA and ECA, symptoms and surgery for vascular ring. A systematic review and meta-analysis (reference: CRD42015016097) was performed; results are reported as proportions. Kaplan Meier analysis of vascular ring cases with surgery as endpoint was performed. RESULTS: Our cohort included 86 cases; 41 had a vascular ring. Rates of chromosomal abnormalities, 22q11.2 deletion, and fetal ECA were 14.1%, 6.4% and 17.4% respectively. Sixteen studies including our cohort (312 fetuses) were included in the systematic review. Overall chromosomal abnormalities and 22q11.2 deletion rates were 9.0% (95% CI 6.0-12.5) and 6.1% (95% CI 3.6-9.3) whilst rates for cases with no ECA were 4.6% (95% CI 2.3-7.8) and 5.1% (95% CI 2.4-8.6). ECA were seen in 14.6% (95% CI 10.6-19.0) prenatally and 4.0% (95%CI 1.5-7.6) after birth. Postnatal ICA were identified in 5.0% (95% CI 2.7-7.9). Rate of symptoms (follow up ≥24 months) was 25.2% (95% CI 16.6-35.0) while 17.1% (95% CI 9.9-25.7) had surgery. Two-year freedom from surgery was 83.0% (95% CI 74.3-90.1) CONCLUSIONS: Fetal RAA without ICA is more frequently associated with ECA than chromosomal abnormalities. Most cases however, are isolated. Vascular ring symptoms occur in about 25% of cases. Postnatal surveillance is required mainly in the first 2 years of life

Stressors in anaesthesiology: development and validation of a new questionnaire: A cross-sectional study of Portuguese anaesthesiologists

BACKGROUND: Stress in anaesthesiologists is a common and multifactorial problem related to patients, colleagues and organisations. The consequences of stress include depression, work-home conflicts and burnout. Reduction in stress can be achieved by reducing the number and magnitude of stressors or by increasing resilience strategies. OBJECTIVES: We have created the self-reporting 'Stress Questionnaire in Anaesthesiologists' (SQA), to qualify the sources of stress in anaesthesiologists' professional lives, and measure the level of associated stress. Our study aimed to develop and validate the SQA using exploratory and confirmatory factor analyses. Construct validity was assessed through correlations between SQA and negative psychological outcomes as well as by comparing perception of stress among different known groups. DESIGN: A questionnaire-based cross-sectional, correlational, observational study. SETTINGS: The study was conducted between January 2014 and December 2014, throughout different anaesthesia departments in Portuguese hospitals. Data collection was from a representative subset at one specific time point. PARTICIPANTS: A sample of 710 anaesthesia specialists and residents from Portugal. MAIN OUTCOME MEASURES: The primary outcome measure was to identify specific stressors in anaesthesiologists. Secondary outcome was the association between stressors and burnout, depression symptoms, anxiety, stress, rumination, satisfaction with life and functional impairment. RESULTS: The exploratory analysis showed the SQA is a tri-dimensional instrument and confirmatory analysis showed the tri-dimensional structure presented good model fit. The three dimensions of SQA correlated positively with other stress measures and burnout, but negatively with satisfaction with life. CONCLUSION: SQA is a well adjusted measure for assessing stressors in anaesthesia physicians and includes clinical, organisational and team stress factors. Results showed that the SQA is a robust and reliable instrument.info:eu-repo/semantics/publishedVersio

Natural history of fetal pyelocaliectasia

INTRODUCTION: It is estimated that genitourinary anomalies comprise 20% of all antenatally detected fetal anomalies, and pyelocaliectasia is the most common one. Detection of antenatal dilatation of the urinary tract does not always indicate postnatal urinary tract obstruction or even a significant genitourinary anomaly. Most cases will improve spontaneously, representing a temporary physiologic impedence and do not require surgery. MATERIALS AND METHODS: In a two-year period we studied 197 newborns, with prenatal pyelocaliectasia, without concomitant anomaly, delivered at Maternidade Dr. Daniel de Matos. In the postnatal follow-up period, the infants were followed at Pediatrics Department, at our Center. RESULTS: A male predilection was found. Ultrasonic follow-up of the 197 infants showed that pyelocaliectasia resolved in 97%, while 3% still presented it in the postnatal ultrasound. Complementary renal evaluation, with voiding cystourethrography and radionuclide imaging, was realised in 29 (15%) infants to further characterize the abnormality detected in postnatal ultrasound. Surgery was performed in four (2%) children. CONCLUSION: Prenatally diagnosed pyelocaliectasia may be safely observed, and surgical correction should be performed only if renal compromise occurs

Total anomalous pulmonary venous connection to an unroofed coronary sinus diagnosed in a fetus with associated spinal muscular atrophy type I

Total anomalous pulmonary venous connection (TAPVC) to unroofed coronary sinus is a rare cardiac condition. We report the first case of antenatal diagnosis which had a rapid and fatal neonatal course due to spinal muscular atrophy (SMA) type I (Werdnig-Hoffmann disease). The diagnosis of TAPVC with unroofing of the coronary sinus was made at 26 weeks’ gestation, although a dilated inferior caval vein had been recognised at 23 weeks’ gestation. Due to profound hypotonia after birth, genetic review followed by muscle biopsy was performed due to presumptive diagnosis of SMA. The infant deteriorated rapidly, became ventilator dependent and died at the age of 6 weeks. The worst types of SMA are associated with only one copy of SMN2 (survival motor neuron 2) protein, which was also the case of our infant. Although a powerful association with congenital heart defects was described, it has not been reported with TAPVC to unroofed coronary sinus. Moreover, this cardiac condition has not been previously recognised antenatally