Isolated noncompaction of left ventricular myocardium with fetal sustained bradycardia due to sick sinus syndrome

Noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. This disease is characterized by numerous and prominent trabeculations and deep intertrabecular recesses. It may be isolated or associated with other congenital heart diseases. The disorder is clinically accompanied by depressed ventricular function, systemic embolization and arrhythmias. Sustained bradycardia is infrequent in prenatal life and associated with maternal collagen vascular diseases, structural heart diseases or long QT syndrome. Herein we report a case of isolated noncompaction of left ventricular myocardium diagnosed in the first day of life and followed previously by serial fetal echocardiograms for the sustained sinus bradycardia. On postnatal electrocardiography, there was junctional escape rhythm due to profound sinus bradycardia, leading us to consider sick sinus syndrome. To our knowledge, this is the first case in the literature of isolated noncompaction of left ventricular myocardium with sustained bradycardia due to sick sinus syndrome

Pentalogy of Cantrell: two patients and a review to determine prognostic factors for optimal approach

Two patients with incomplete pentalogy of Cantrell are described. The first was a girl with a large omphalocele with evisceration of the heart, liver and intestines with an intact sternum. Echocardiography showed profound intracardiac defects. The girl died 33 h after birth. The second patient was a female fetus with ectopia cordis (EC) without intracardiac anomalies; a large omphalocele with evisceration of the heart, stomach, spleen and liver; a hypoplastic sternum and rib cage; and a scoliosis. The pregnancy was terminated. A review of patients described in the literature is presented with the intention of finding prognostic factors for an optimal approach to patients with the pentalogy of Cantrell. In conclusion the prognosis seems to be poorer in patients with the complete form of pentalogy of Cantrell, EC, and patients with associated anomalies. Intracardial defects do not seem to be a prognostic factor

Serum screening with Down's syndrome markers to predict pre-eclampsia and small for gestational age: Systematic review and meta-analysis

<p>Abstract</p> <p>Background</p> <p>Reliable antenatal identification of pre-eclampsia and small for gestational age is crucial to judicious allocation of monitoring resources and use of preventative treatment with the prospect of improving maternal/perinatal outcome. The purpose of this systematic review was to determine the accuracy of five serum analytes used in Down's serum screening for prediction of pre-eclampsia and/or small for gestational age.</p> <p>Methods</p> <p>The data sources included Medline, Embase, Cochrane library, Medion (inception to February 2007), hand searching of relevant journals, reference list checking of included articles, contact with experts. Two reviewers independently selected the articles in which the accuracy of an analyte used in Downs's serum screening before the 25^thgestational week was associated with the occurrence of pre-eclampsia and/or small for gestational age without language restrictions. Two authors independently extracted data on study characteristics, quality and results.</p> <p>Results</p> <p>Five serum screening markers were evaluated. 44 studies, testing 169,637 pregnant women (4376 pre-eclampsia cases) and 86 studies, testing 382,005 women (20,339 fetal growth restriction cases) met the selection criteria. The results showed low predictive accuracy overall. For pre-eclampsia the best predictor was inhibin A>2.79MoM positive likelihood ratio 19.52 (8.33,45.79) and negative likelihood ratio 0.30 (0.13,0.68) (single study). For small for gestational age it was AFP>2.0MoM to predict birth weight < 10^thcentile with birth < 37 weeks positive likelihood ratio 27.96 (8.02,97.48) and negative likelihood ratio 0.78 (0.55,1.11) (single study). A potential clinical application using aspirin as a treatment is given as an example.</p> <p>There were methodological and reporting limitations in the included studies thus studies were heterogeneous giving pooled results with wide confidence intervals.</p> <p>Conclusion</p> <p>Down's serum screening analytes have low predictive accuracy for pre-eclampsia and small for gestational age. They may be a useful means of risk assessment or of use in prediction when combined with other tests.</p

The value of middle cerebral artery systolic velocity for initial and subsequent management in fetal anemia

Objective: To evaluate the fetal middle cerebral artery Doppler waveform for the prediciton of anemia in the RhD-alloimmunized fetus. Study Design: Doppler velocimetry of the fetal middle cerebral artery peak systolic velocity was measured in 52 non-hydropic, RhD-alloimmunized fetuses who underwent 103 cordocenteses. Normal values were obtained from 70 normal cases. The peak systolic velocity values were expressed as multiples of the median (MoM) for gestation. Hemoglobin threshold for developing hydrops was developed front 22 RhD-alloimmunized hydropic fetuses and severe anemia was defined as Hb less than or equal to 0.60 MoM. The most efficient threshold values fur the prediction of severe anemia in groups with no prior transfusion and those with prior transfusion were obtained by constructing ROC curves. Results: The mean gestational age (+/-S.D.) at cordocentesis was 28.0 +/- 4.6 weeks. Severe anemia was noted in 53 (51.5%) occasions. At a threshold middle cerebral artery peak systolic velocity value of greater than or equal to1.35 MoM, the sensitivity for severe anemia detection was 100% with a false-positive rate of 18%. In sub-analysis, in patients with one or more prior transfusion a sensitivity or 97% and a false-positive rate of 14% obtained at a threshold of greater than or equal to1.45 MoM. In patients with no previous transfusion the sensitivity for severe anemia was 100%, with a false-positive rate of 9.1% at a threshold value of greater than or equal to1.35 MoM. Conclusion: The middle cerebral artery peak systolic velocity can be used to time both the initial diagnostic procedure and retransfusion. (C) 2002 Elsevier Science Ireland Ltd. All rights reserved

Case of an Abductor Pollicis Longus Muscle: Variation or Differentiation?

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