Isolated Coronary Artery Fistula in Fetal Heart: Case Reports and Literature Review.

We report two cases of prenatally diagnosed isolated coronary artery fistula. The first fetus had a fistulous communication between the left circumflex coronary artery and the right atrium. The second fetus had right coronary artery to left ventricle fistula. In addition, we have also reviewed previously published reports on prenatally diagnosed coronary artery fistulae, focusing on their clinical profiles, outcome and postnatal treatment.

[Echocardiographic diagnosis for ventricular non-compaction cardiomyopathy in foetus and the pathologically comparative study].

OBJECTIVE: To evaluate the echocardiographic diagnosis for ventricular non-compaction cardiomyopathy (NCCM) in foetus and to analyze the pathologic features of NCCM.
 METHODS: A total of 9 patients with fetal NCCM were examined by prenatal echocardiography from 2004 to 2013, which was compared with postnatal echocardiography or autopsy to analyze the fetal characteristic of myocardial ultrastructure.
 RESULTS: The results of echocardiography displayed an excessive muscle trabecular meshwork and muscle trabecular crypt, and the ventricular myocardium and non-compaction/compaction ratio was ≥2.0. Among the 9 fetuses of NCCM, 6 fetuses were involved in left ventricle, 2 in both left and right ventricles and 1 in right ventricle. Two fetuses were confirmed by postnatal echocardiography, the remaining 7 patients were chosen to terminate their pregnancies, which were confirmed by autopsy later. Muscle biopsies revealed the abnormal myocardial mitochondria, sarcomeres and myocardial fibrosis.
 CONCLUSION: It is feasible to accurately diagnose NCCM by prenatal echocardiography. Fetal NCCM most often involves the left ventricle, but it can involve the right ventricle or both, too. The myocardial ultrastructure of fetal NCCM possesses certain unique characteristics, such as the low maturation of the mitochondria, sarcomeres and myocardial fibers.

Noninvasive assessment of myocardial mechanics of the left ventricle in rabbits using velocity vector imaging.

BACKGROUND: Our study aimed to investigate the feasibility of velocity vector imaging (VVI) to analyze left ventricular (LV) myocardial mechanics in rabbits at basal state. MATERIAL AND METHODS: The animals used in this study were 30 New Zealand white rabbits. All rabbits underwent routine echocardiography under VVI-mode at basal state. The 2-dimensional (2-D) echocardiography images acquired included parasternal left long-axis views and short-axis views at the level of LV mitral valve, papillary muscles, and apex. Images were analyzed by VVI software. RESULTS: At basal state, longitudinal LV velocity decreased from the basal to the apical segment (P<0.05). In the short axis direction, the highest peak myocardial velocity was found between the anterior septum and anterior wall for each segment at the same level; the peak strains and strain rates (SR) were the highest in the anterior and lateral wall compared to other segments (all P<0.05). During systole, LV base rotated in a clockwise direction and LV apex rotated in a counter-clockwise direction, while during diastole, both LV base and apex rotated in the direction opposite to systole. The rotation angle, rotation velocity and unwinding velocity in the apical segment were greater than the basal segment (P<0.05). CONCLUSIONS: VVI is a reliable tool for evaluating LV myocardial mechanics in rabbits at basal state, and the LV long-axis short-axis and torsional motions reflect the normal regular patterns. Our study lays the foundation for future experimental approaches in rabbit models and for other applications related to the study of human myocardial mechanics.

Ventricular non-compaction cardiomyopathy: prenatal diagnosis and pathology.

OBJECTIVE: The objective of this study is to evaluate the prenatal diagnosis of ventricular non-compaction cardiomyopathy (NCCM) and to report the pathologic features and the characteristic myocardial ultrastructure of fetal NCCM. METHOD: We examined nine cases of fetal NCCM by prenatal echocardiography from 2004 to 2013. These cases were evaluated with respect to prenatal diagnosis, outcomes, pathology, and characteristics of the myocardial ultrastructure. RESULTS: Trabeculated myocardium and non-compaction/compaction ratio (N/C ratio) ≥2.0 is the echocardiographic feature that allowed diagnosis. Among the nine fetuses with a prenatal diagnosis of NCCM, six were diagnosed with left ventricular non-compaction (LVNC), two with non-compaction of both ventricles (biventricular NCCM), and one with right ventricular non-compaction. Among the nine fetuses, the LVNC diagnosis was confirmed by postnatal echocardiography in 2; the other patients chose to terminate their pregnancies, and those cases were confirmed at autopsy. Muscle biopsies were performed on three of the aborted fetuses, and abnormal mitochondria, sarcomeres, and myocardial fibers were observed. CONCLUSION: Non-compaction can be identified in the fetus. Fetal non-compaction most often involves the left ventricle, but can involve the right ventricle or both ventricles. The myocardial ultrastructure of fetal ventricular non-compaction has certain unique characteristics regarding the maturation of the mitochondria, sarcomeres, and myocardial fibers. © 2014 John Wiley & Sons, Ltd.

Echocardiographic follow-up and pregnancy outcome of fetuses with cardiac asymmetry at 18-22 weeks of gestation.

OBJECTIVE: The goal of our study was to evaluate the evolution of cardiac findings and assess the pregnancy outcome of fetuses with cardiac asymmetry at midgestation. METHODS: We reviewed all fetuses with cardiac asymmetry at echocardiograms performed at 18-22 weeks of gestation from 2006 to 2013. The data collected included the gestational age at diagnosis, serial echocardiographic findings, karyotype testing, and pregnancy outcome. Excluded were cases of classical hypoplastic left heart syndrome (HLHS) at initial echocardiogram (i.e., aortic (AO) and/or mitral atresia or dysplasia with normal intracardiac connections), heterotaxy syndrome, twin pregnancy, fetal growth restriction, and coarctation of the aorta. RESULTS: Our study included 278 fetuses with cardiac asymmetry (defined as Z-scores of left ventricle length or diameter of less than -2). A total of 202 (72%) fetuses had normal variations in cardiac growth by prenatal and neonatal echocardiography, 69 (25%) fetuses were confirmed to have HLHS variant by autopsy or neonatal echocardiography (with an overall survival of 4.3%), and seven (3%) fetuses were terminated before 24 weeks of gestation because of abnormal karyotypes. None of the cardiac measurements at the initial echocardiogram was significantly different between HLHS variant and normal cases. In the 69 fetuses ultimately diagnosed with HLHS variant, but not in the 202 normals, the Z-scores of all measured left heart structures decreased progressively between the first and the last prenatal echocardiograms (P < 0.01). CONCLUSIONS: The majority of the fetuses with cardiac asymmetry at 18-22 weeks have a good outcome. A minority of them progress to develop HLHS variant with advancing gestation.

[Four-dimensional reconstruction by inversion mode of spatio-temporal image in prenatal diagnosis of congenital heart diseases].

OBJECTIVE: To investigate the value of 4-dimensional reconstruction by inversion mode of spatio-temporal image correlation (STIC) in prenatal diagnosis of congenital heart diseases. METHODS: Cardiac volume images of 30 normal fetuses and 22 fetuses with congenital heart diseases were obtained by STIC gray-scale 2-dimensional imaging technique. Under the guidance of cross-sectional display, the images were collected by inversion mode. RESULTS: A total of 53 and 38 qualified volume images were obtained in the 30 normal fetuses and 22 fetuses with congenital heart diseases respectively. All volume images were inverted successfully and showed the spacial position of the heart and blood vessel directly. CONCLUSION: Four-dimensional reconstruction by inversion mode of STIC is feasible. This new method can provide more morphologic information, and may be used for prenatal diagnosis of congenital heart diseases.

Four-dimensional echocardiography with B-flow imaging and spatiotemporal image correlation in the assessment of congenital heart defects.

OBJECTIVE: The objective of the present study was to evaluate the role of four-dimensional ultrasound with B-flow imaging and spatiotemporal image correlation (STIC) in the detection of congenital heart defects. METHODS: Automated longitudinal and transverse sweeping of the fetal chest was utilized to obtain the volume datasets of the heart. Among the fetuses recruited in the study, 20 were normal and 13 were fetuses with congenital heart anomalies. Reconstructed images of abnormal hearts were compared with those of normal fetuses. RESULTS: Of the 20 normal fetuses, all extracardiac vessels such as aorta, pulmonary artery, ductus artery, inferior vena cava, and ductus venosus could be detected on reconstructed images of 16 fetuses. In five normal cases, a four-dimensional image was recorded to allow simultaneous visualization of all four pulmonary veins. In the 13 fetuses with cardiac anomalies, four-dimensional sonography with B-flow imaging and STIC detected the 'digital casts' of the outflow tracts, great arteries, and veins draining into the heart. These results demonstrate spatial relationship among these structures which provide important anatomical information. CONCLUSIONS: The use of four-dimensional ultrasound examination with B-flow imaging and STIC is a very useful technique in identifying anatomical features of different congenital cardiac anomalies.

Evaluation of regional left ventricular longitudinal function in 151 normal fetuses using velocity vector imaging.

OBJECTIVE: The purpose of this study was to investigate the clinical value of velocity vector imaging (VVI) in the assessment of normal fetal regional myocardial performance and to establish a normative data set for normal Chinese fetuses. METHODS: One hundred and fifty-one healthy Chinese fetuses were divided into five groups according to their gestational age. Digital dynamic four-chamber views were collected and analyzed offline. The regional tissue velocity, strain, and strain rate of the interventricular septum and left lateral wall were measured in systole and diastole. RESULTS: Normal systolic and diastolic values for tissue velocity, strain, and strain rate were established. Tissue velocity decreased gradually from the basal segment to the apical segment (P < 0.01), whereas the strain and strain rate were stable among all segments in every group (P > 0.05). Tissue velocity was dependent on gestational age (P < 0.05), whereas strain and strain rate were stable throughout gestation (P > 0.05). CONCLUSION: VVI is a novel noninvasive tool for quantitative assessment of regional systolic and diastolic function of the fetal heart. Fetal myocardial velocity, strain, and strain rate measurements can be measured easily and reproducibly. This technique is useful for evaluating cardiac function.

Prenatal diagnosis of type 1 tracheal agenesis without tracheoesophageal fistula.

Tracheal agenesis is a rare and uniformly lethal congenital malformation. We recently had the opportunity to diagnose prenatally a case of type I tracheal agenesis without fistula in a 22-week-old fetus, in which the hyperechogenicity of the enlarged lungs was associated with ascites, and tracheal dilatation in the lower half. We report the ultrasonographic and autopsy findings and review the literature on this rare condition to contribute to the understanding of type 1 tracheal agenesis without tracheoesophageal fistula.

Assessment of regional myocardial function in patients with dilated cardiomyopathy by velocity vector imaging.

OBJECTIVE: To assess the left ventricular (LV) longitudinal systolic and diastolic function in patients with dilated cardiomyopathy (DCM) by syngo Velocity Vector Imaging (VVI). METHODS: Digital dynamic images of 30 DCM patients and 30 healthy subjects were collected; then the longitudinal velocity, strain, and strain rate were measured in systolic early and late diastolic periods, and the time to peak systolic velocity, strain, and strain rate were measured and recorded. The parameters of the two groups were compared. RESULTS: All of the parameters of the DCM were significantly lower than those of the normal group (P < 0.05-0.01), except that the parameter of late diastolic strain was not different between the two groups (P > 0.05). CONCLUSIONS: VVI is a novel noninvasive tool to assess quantitatively and objectively LV regional systolic and diastolic function in patients with DCM; it provides another useful modality for evaluating cardiac function.