Arterial venosus malformation

After thickening of the cardiac chamber walls during embryogenesis, oxygen and nutrients can no longer be adequately supplied to cardiac cells via passive diffusion; therefore, a primitive vascular network develops to supply these vital structures. This plexus further matures into coronary arteries and veins, which ensures continued development of the heart. Various models have been proposed to account for the growth of the coronary arteries. However, lineage-tracing studies in the last decade have identified 3 major sources, namely, the proepicardium, the sinus venosus, and endocardium...

A link between congenital vascular malformation (CVM) of the liver and trisomy 21 has been suggested. We reviewed all children with trisomy 21 referred for investigation to a specialist pediatric hepatobiliary unit (1985-2015). Forty-five children with trisomy 21 were identified; 7 (15%) had a defined CVMs (4 girls). All such infants were also diagnosed with a range of cardiac defects. CVMs were divided according to the nature of the vascular connection. Group (i) (n = 3): Abnormal venovenous anomaly. This included portocaval shunt and patent ductus venosus (n = 2)...

Introduction Congenital umbilical arteriovenous malformations (AVMs) are extremely rare. We present the first case of congenital umbilical AVM with feeding arteries originating not only from abdominal but also from the mammary arteries. Case Report A 34-week gestational age newborn was transferred to our hospital with a supraumbilical murmur. Abdominal Doppler ultrasound (US) showed a large vascular AVM, with multiple feeding arteries and several venous drainage structures to the umbilical vein and also a persistent ductus venosus...

OBJECTIVES: The purpose of this study was to describe the echocardiographic features and perinatal outcomes of congenital absence of the aortic valve diagnosed by first-trimester echocardiography. METHODS: This retrospective study assessed the feasibility of first-trimester echocardiography in detecting absence of the aortic valve. All cases of absence of the aortic valve diagnosed by first-trimester fetal echocardiography from January 2010 to December 2014 were identified at a single referral center using an established perinatal database...

OBJECTIVE: We analyzed the association between renal and cardiovascular parameters in fetuses with isolated severe urinary tract malformations. METHODS: 39 fetuses at a mean gestational age of 23.6 weeks with nephropathies or urinary tract malformations and markedly impaired or absent renal function were prospectively examined. Fetal echocardiography was performed, and thicknesses of the interventricular septum, and left and right ventricular wall were measured...

The use of the patent ductus venosus via a transjugular approach to access the portal system for endovascular treatment of hepatic vascular anomalies in three infants is reported. Two patients had an arterioportal fistula, and one had a rapidly involuting congenital hemangioma. All patients underwent arteriography followed by embolization of the vascular anomalies without complications. This alternative route is technically simpler and likely safer than transarterial and transhepatic approaches.

As operations for lung cancer become more common, more anomalies of various pulmonary arteries and veins are being encountered. Persistent left superior vena cava (PLSVC) is caused by abnormal development of the sinus venosus in early fetal life. In cases of PLSVC with left lung cancer, particular care must be exercised during the excision of the pulmonary vein, focusing on the region into which the PLSVC flows. In such anomalous cases, proper excision of the pulmonary artery and vein and lymph node dissection require a firm grasp of anatomic detail using preoperative computed tomographic scanning...

INTRODUCTION: Triploidy in the second trimester is a sporadic, rare lethal chromosomal abnormality characterized by an extra haploid chromosome set (3n = 69). Doppler blood flow study in fetal triploidy syndrome is rarely reported in the literature. CASE PRESENTATION: A 19-year-old woman at 18 weeks of gestation was referred to our fetal medicine unit. Examination revealed a digynic triploid fetus presenting with asymmetric intrauterine growth restriction, oligohydramnios, relative macrocephaly together with a small thin trunk, low-set ears, micrognathia, bilateral talipes, bilateral syndactyly on the third, fourth and fifth fingers and toes, a large ventricular septal defect, bradycardia, bilateral hyperechogenic kidneys and small placenta...

Fetal arterial and venous Doppler is a useful tool for the monitoring of growth restricted fetuses. Our aim in this study was to compare outcomes when fetuses were grouped according to the combinations of the Doppler results and also according to each vessel Doppler. Deliveries during the period 2002-2008 were reviewed retrospectively and cases with a birth weight less than the 10th percentile were selected for the study. Cases with congenital malformations or chromosomal abnormalities were excluded. Cases were then grouped according to umbilical artery (UA), middle cerebral artery (MCA) and ductus venosus (DV) Doppler results...

OBJECTIVE: Many published studies have shown that application of three-dimensional (3D) and real-time 3D (4D) ultrasound modalities can improve certain aspects of fetal echocardiography, but have left open the question of whether these modalities improved the accuracy of prenatal detection of anatomical fetal cardiovascular malformations. We aimed to determine whether 3D/4D ultrasound improved diagnostic ability in cases of congenital heart disease (CHD). METHODS: Women who attended for early- or midtrimester targeted organ scans had complete fetal echocardiography according to our five-planes protocol, as well as examination of the ductus venosus and longitudinal aortic arch planes, performed with 2D ultrasound combined with 2D color Doppler, spatiotemporal image correlation (STIC), STIC with color Doppler, and STIC with B-flow...

BACKGROUND: Monochorionic biamniotic (MC/BA) twin pregnancies are at higher risk for twin-to-twin transfusion syndrome (TTTS), selective intrauterine growth restriction (sIUGR) and structural defects. MATERIAL AND METHOD: During an 18 months period all MC/BA twins referred to our unit for clinical surveillance were evaluated. Chorionicity was assessed based on the characteristics of the intertwin amniotic membrane. MC complicated cases were labeled either as, TTTS or sIUGR and classified according to severity...

Tetralogy of Fallot (TOF) with concomitant absent pulmonary valve syndrome (APVS) constitutes a rare prenatal condition characterized by rudimentary cusps of the pulmonary valve, pulmonary regurgitation, and a variable degree of dilatation of the main and branch pulmonary arteries. Although early prenatal diagnosis of this complex malformation is feasible, the antenatal course of affected fetuses clearly depends on the presence of associated structural (absence of the ductus venosus) and chromosomal anomalies (microdeletion 22q11, DiGeorge syndrome)...

OBJECTIVE: Maternal diabetes during pregnancy is associated with congenital cardiac malformations and hypertrophic cardiomyopathy. Blood flow in the ductus venosus (DV) has been postulated to reflect cardiac function. The aim of our study was to investigate if diabetic pregnancies exhibit abnormal DV hemodynamics, hence indicating changes in fetal cardiac function. METHODS: The pulsatility index of the DV (DV-PI) was analyzed retrospectively in 142 diabetic patients and compared to previously published DV-PI reference values from a non-diabetic low-risk population...

OBJECTIVE: To evaluate the intrauterine course and outcome of tricuspid atresia detected in the fetus. METHODS: This was a retrospective review of all confirmed cases of tricuspid atresia detected prenatally between 1998 and 2006 in three tertiary referral centers in Germany. RESULTS: Fifty-four cases of tricuspid atresia were detected prenatally during the study period and confirmed postnatally: 28 (51.9%) cases had a concordant ventriculoarterial connection of which 14 also had pulmonary outflow obstruction; 25 (46...

Stella Van Praagh, MD (1927-2006) of Children's Hospital Boston was one of the greatest pediatric cardiologists and pediatric cardiac pathologists of the 20th and early 21st centuries. Née Stella Zacharioudaki from Crete, Greece, in addition to her stellar professional attainments, she was also an outstanding cuisinière, hostess, linguist, philosopher, and philanthropist. In 1962, she married Richard Van Praagh, MD, beginning a life-long collaboration that was in every sense an affaire de coeur. They had three children and seven grandchildren...

We report a rare association of vein of Galen malformation in a young infant with associated sinus venosus atrial septal defect and aortic arch hypoplasia. This unique association of lesions provides insights into the embryological basis as well as offers potential echocardiographic clues for the diagnosis of the cerebral arterio-venous malformation.

OBJECTIVE: To evaluate the relationship between ductus venosus Doppler findings on the day of delivery and postnatal outcomes in pregnancies with absent or reversed end-diastolic (ARED) flow in the umbilical arteries. STUDY DESIGN: Postnatal outcomes of 103 newborns of pregnancies with a diagnosis of ARED flow on Doppler velocimetry of the umbilical arteries were analyzed retrospectively between January 1997 and December 2004. Single pregnancies and fetuses without malformations were included...

An abnormal course of the umbilical vein is a rare anomaly. Its association with the congenital absence of the ductus venosus is common. We found 3 cases of an abnormal course of the umbilical vein and an absent ductus venosus. In 2 of these cases, the umbilical vein turned down and continued in the internal iliac vein, and no ductus venosus was found. One of these pregnancies was terminated. From the continued pregnancy a growth-retarded baby was born. At follow-up examinations, mild microcephaly, mildly elevated levels of ammonia, delayed speech and mild muscular hypotonia were found...

Absence of the ductus venosus is a rare vascular anomaly, but clinicians should be aware that it can be diagnosed antenatally and the prognosis is dependent on the type of associated malformation of the fetal vascular system. Antenatal detection of a single umbilical artery and unexplained cardiomegaly should prompt detailed examination of the umbilical and portal veins. Absent ductus venosus is associated with three main patterns of abnormal venous circulation, the worst prognosis being seen when the umbilical vein bypasses the liver and connects to the right atrium...

OBJECTIVE: To investigate arterial and venous blood flow in fetuses with absent or reversed end-diastolic flow in the umbilical arteries and to correlate the Doppler results with umbilical artery blood pH at birth to predict the probability of acidosis at birth. METHODS: Ninety-one fetuses from singleton pregnancies without fetal malformations with a diagnosis of absent or reversed end-diastolic flow in the umbilical arteries were prospectively studied. On the day of delivery, Doppler velocimetry of the umbilical arteries, middle cerebral artery, and ductus venosus was performed and the results were correlated with umbilical artery pH at birth at the following cutoff levels: pH < 7...