Genetic dilated cardiomyopathy with inflammation in an infant that responded to immunosuppressive therapy evaluated using cardiovascular magnetic resonance.

Cardiovascular magnetic resonance T1 and T2 mapping reflects inflammation, fibrosis, and myocardial oedema. However, its application in infants remains uncertain. Herein, we report a three-month-old boy with dilated cardiomyopathy successfully treated with steroids. Cardiovascular magnetic resonance was useful for diagnosis based on the elevated native T1, T2, and extracellular volume and evaluation of response to immunosuppressive therapy in infantile inflammatory dilated cardiomyopathy.

Fusion imaging of single-photon emission computed tomography and magnetic resonance lymphangiography for post-Fontan chylothorax.

A preschool male patient with an extensive cardiac surgical history developed refractory chylothorax after a total cavopulmonary connection. Neither lymphoscintigraphy nor single-photon emission computed tomography (SPECT)/computed tomography could identify the lymphatic system leakage sites. Non-contrast heavy T2-weighted magnetic resonance lymphangiography (MRL) was performed to visualize the lymphatic system. Nevertheless, distinguishing lymphatic ducts from other watery structures of the patient remained difficult. Therefore, non-contrast MRL and SPECT images were fused. This hybrid diagnostic tool elucidated the pathophysiology of the prolonged chylothorax; pulmonary lymphatic perfusion syndrome and illustrated the anatomical connection of the thoracic duct and an abnormally dilated lymphatic network in the neck and left hilar regions. Subsequent intranodal lymphangiography with ethiodized oil confirmed these findings. SPECT/MRL may become an alternative modality for revealing the mechanism of prolonged chylothorax by visualizing the lymphatic system when dynamic contrast-enhanced magnetic resonance lymphangiography is unavailable.

Thrombus calcification after removing peripherally inserted central catheters in extremely preterm infants.

Pericatheter thrombus calcification is a complication that arises due to central venous catheter insertion and is particularly rare in peripherally inserted central catheters (PICCs). In this case report, we reviewed the clinical course of two neonates experiencing thrombus calcification. The first case involved a male neonate weighing 445 g. His PICC dwelt in the superior vena cava for over 49 days. Although a radiograph after removal did not show any silhouette, subsequent radiographs and CT depicted a catheter-like outline. Percutaneous intravascular retrieval was performed to salvage the object. Pathological examination revealed it to be a calcified cast. The calcified thrombosis was successfully dissolved with 6 months of warfarin therapy. The second case involved a male neonate weighing 534 g. After PICC removal, a catheter-like structure was shown on ultrasonograms. It was determined that invasive procedures were unnecessary for diagnosing the calcified thrombosis based on experience with the first case.

A rare association of left pulmonary artery sling with right pulmonary hypoplasia and total anomalous pulmonary venous connection.

We present the second reported case of left pulmonary artery sling with dextrocardia, right pulmonary hypoplasia, and total pulmonary venous connection in a fetus. This case highlights the importance of the determination of pulmonary artery arrangement by fetal echocardiography if right pulmonary hypoplasia and/or congenital heart disease is suspected.

Prenatal Diagnosis of Criss-Cross Heart Using 4-Dimensional Color Doppler Rendering.

Criss-cross heart is a rare congenital cardiac anomaly characterized by the crossing of two ventricular inflow streams. We have demonstrated the utility of 4-dimensional color Doppler rendering in diagnosing the criss-cross heart in a fetus. Four-dimensional color Doppler rendering can demonstrate the relative direction of intracardiac blood flows and facilitate recognition of the crossover of inflow streams in the same plane, confirming the criss-cross heart diagnosis in the fetus.

Serial echocardiography for immune-mediated heart disease in the fetus: results of a risk-based prospective surveillance strategy.

OBJECTIVE: Mothers carrying anti-Ro antibodies are frequently referred for weekly echocardiograms to early detect and treat antibody-mediated fetal heart disease. We tested a surveillance strategy based on anti-Ro antibody titers. METHODS: From 2009 to 2014, 232 pregnancies were referred for maternal anti-Ro antibodies. At the baseline echocardiogram, anti-Ro titers were measured by enzyme-linked immunosorbent essay and results categorized as negative (<8 U/mL; n = 43; excluded), low-moderate positive (8-49 U/mL; n = 62; group 1) or high positive (50 - >100 U/mL; n = 127; group 2). Serial echocardiograms to ≥24 weeks were only recommended for group 2 mothers. RESULTS: Group 1 patients underwent significantly less fetal echocardiograms when compared with group 2 mothers (median 2 vs. 4; p < 0.001). Isolated endocardial fibroelastosis (n = 1) and incomplete (n = 4) or complete (n = 4) heart block were diagnosed in 9 (8%) pregnancies with anti-Ro titers >100 U/mL but none with lower titers (odds ratio 17.78; p = 0.004). Incomplete block and endocardial fibroelastosis regressed with transplacental corticosteroid and immune globulin therapy. CONCLUSIONS: Limiting serial fetal echocardiograms to women with high anti-Ro antibody levels is safe and more cost effective. While numbers of echocardiograms were significantly reduced in referrals with anti-Ro titers <50 U/mL, reversible abnormalities with prenatal treatment were detected by serial echocardiography in group 2 patients. © 2017 John Wiley & Sons, Ltd.

Impact and issues of detecting fetal congenital heart defects in Kyushu, Japan.

AIM: To determine the current status of fetal CHD screening in our region and to establish a CHD screening system in Japan. MATERIAL AND METHODS: Subjects were 168 fetuses prenatally-diagnosed with CHD at four referral centers in Japan from 2003 to 2007. Subjects were divided into two groups: group A (n = 84) included cases without extracardiac sonographic abnormalities and known risk factors for CHD and group B (n = 84) included those with extracardiac sonographic abnormalities or risk factors. The diagnostics and outcomes between the groups were analyzed. RESULTS: There were more cases of single ventricle and restrictive ductus arteriosus and fewer cases of ventricular septal defect and double outlet right ventricle in group A than in group B (P < 0.05). In group A, the most frequent referral reason was an abnormal four-chamber view. In group B, 37 cases had chromosomal anomalies. The mortality rates in group B were higher than those in group A (P < 0.05). There were no differences in mortality rates between fetuses without chromosomal anomalies in group B and group A. CONCLUSION: Prenatally-diagnosed CHD were mostly limited to those cases with obvious abnormalities in the four-chamber view or those with chromosomal anomalies. Prenatal detection of CHD is useful for the prediction of outcomes.