Idiopathic constriction of the fetal ductus arteriosus: three cases and review of the literature.

Premature constriction or closure of the ductus arteriosus can occur during fetal life. It is a rare phenomenon and has been described secondary to medication or structural lesions or as idiopathic constriction. Premature closure of the ductus arteriosus can lead to progressive right heart dysfunction with tricuspid regurgitation, congestive heart failure, fetal hydrops, and intrauterine death. This series describes diagnosis of fetal ductus arteriosus constriction of unknown etiology in 3 cases, prenatal management, and outcomes. Constriction of the ductus arteriosus can be diagnosed prenatally with careful interrogation of the ductal arch using pulsed Doppler sonography and complete fetal echocardiography. Close monitoring is mandatory to rule out development of right heart failure and to determine the intervention time.

Fetal and neonatal diagnosis of interrupted aortic arch: associations and outcomes.

OBJECTIVE: Interrupted aortic arch (IAA) is a rare but serious anomaly. Prenatal diagnosis is challenging and published data are limited. The aim of the study was to review the data of fetuses and neonates diagnosed with IAA during a 16-year period at Children's Hospital Giessen. METHODS: Retrospective ascertainment of 8 fetuses and 20 neonates with a confirmed diagnosis of IAA from 1994 to 2010 by reviewing the hospital database of the cardiovascular program of the prenatal and pediatric cardiology clinics at the University Hospital Giessen. RESULTS: Eighteen cases with IAA type B and 10 cases with IAA type A were found. After 2005, prenatal diagnosis was achieved in 8 cases and postnatal imaging confirmed IAA in all 8 neonates. Twenty-nine percent of individuals had a chromosomal anomaly, with microdeletion 22q11.2 being the most common abnormality (n = 6, 21%). In 46% (13/28) other complex cardiac anomalies were present. Mortality after surgery was 18%. Long-term morbidity and mortality was due to neurological impairment in the presence of microdeletion 22q11.2 and the need of surgical or catheter re-intervention. CONCLUSION: Despite the difficulties and challenges in diagnosis, the prenatal detection rate of IAA is increasing. Associated complex cardiac and chromosomal abnormalities influence the outcome of patients with IAA and are important issues of parental counseling.

Increased endothelial thrombomodulin (TM) expression in pregnancies complicated by IUGR.

Thrombomodulin (TM) is a cell surface receptor playing an important role in endothelial cell anticoagulant activity. TM is also known as a factor of angiogenesis; low TM activity correlates with impaired angiogenesis. Since vascular lesions with disorders of the placental coagulation and inadequate angiogenesis can be associated with IUGR, we hypothesized that thrombomodulin expression in the villous vasculature and syncytiotrophoblast of placentae complicated by IUGR might differ from those of normal pregnancies. Representative tissue samples of normal, IUGR as well as 1st and 2nd trimester (n = 12) placentae were collected. Immunohistochemistry (APAAP) of paraffin tissue sections was performed using monoclonal antibodies specific for TM and PECAM. The percentage of immunopositive vessels and the intensity of immunoreactivity was assessed. Vascular endothelium and syncytiotrophoblast stained positive for TM. Immunoreactivity for TM in villous vasculature rose significantly with gestational age. Villous vessels of IUGR placentae, showed a higher expression of TM, compared to placentae of appropriately grown fetus (p < 0.05). The number of terminal villi and the number of blood vessels per intermediate villi was significantly reduced in IUGR placentae (p < 0.05). These differences reflect inadequate vascularisation and impaired angiogenesis in IUGR.