Search

Right ventricular (RV) failure is a major concern in grown-up congenital heart diseases. Cell-based myocardial repair may be an innovative approach to restore overloaded RV function. A repaired Tetralogy of Fallot pig model was used. After 5 months, cell therapy was surgically administrated using human embryonic stem cell-derived Nkx2.5+ cardiac progenitor cell-seeded collagen patch. A large patch containing 107 cells was engineered to cover the whole RV free wall and applied on the epicardium of 4 pigs (treated group). A control group (n = 4) received a cell-free patch (3) or no patch (1). Myocardial function was assessed by echocardiography using standard and strain parameters before and 2 months after cell graft. The fate of progenitors was tracked using human-and cardiac specific antibodies. Indexed RV diameter and telediastolic RV area significantly decreased in treated group whereas increased in control. The free wall thickness significantly increased in treated group compared to control. In the treated group, RV free wall strain significantly improved by contrast to control (respectively from −13.4 to −15.9% vs. −18.9 to −12.9%, P < 0.001). The global function was maintained in the treated group while it decreased in the control. Differentiating human cardiac progenitors were found in the patch and migrated within the myocardium (Fig. 1). Although still immature, some cells featured TnT+ actinin+ sarcomeric units. Proliferating small Ki-67+ TnT+ labelled pig myocytes were observed. Expression of collagen genes col1a3, col3a1 monitored in real-Time QPCR reflecting myocardial fibrosis status significantly decreased in the treated group. Human cardiac progenitors migrated and differentiated, contributing together with triggered endogenous mechanism of repair to maintain the RV adaptation to overload and to improve free wall contractility. They also induced a proliferation of host cells supporting potential paracrine effects. [ABSTRACT FROM AUTHOR]

Abstract: Objective: The objective of this study is to describe the evolution of 8 cases of congenital malformations of the umbilical-portal-hepatic venous system diagnosed before the first month of life. Materials and methods: All cases of congenital malformation of the portal and hepatic venous system diagnosed prenatally or during the first month of life in our institution were systematically reviewed since November 2000. Clinical features, imaging, and anatomical findings were reviewed, focusing primarily on clinical and radiologic evolution. Results: Eight cases of congenital malformation of the umbilical-portal-hepatic venous system were studied. Fifty percent of these malformations were diagnosed prenatally. We report 4 portosystemic shunts. Three involuted spontaneously, and the fourth one required surgical treatment. We report a variation of the usual anatomy of portal and hepatic veins that remained asymptomatic, an aneurysmal dilatation of a vitelline vein causing portal vein thrombosis that needed prompt surgical treatment with good result, a complex portal and hepatic venous malformation treated operatively, and a persistent right umbilical vein that remained asymptomatic. Conclusion: Prenatal diagnosis of malformations of the umbilical-portal-hepatic venous network is uncommon. Little is known about the postnatal prognosis. Clinical, biologic, and radiologic follow-up by ultrasonography is essential to distinguish pathologic situations from normal anatomical variants. [Copyright &y& Elsevier]

Presents a letter to the editor about medical liability.

Abstract: Umbilical vein varix is a well-described prenatal anomaly in which the prognosis remains unclear. We describe a very rare venous malformation that mimicked an umbilical vein varix consisting of a persistent vitelline vein. From 2003 to 2010, three patients were referred starting at 20weeks gestation to our prenatal centers for an umbilical vein varix diagnosis. Fetal follow up was unremarkable, with the exception of the dilated vein size (mean: 35mm at 33weeks gestation). After birth, the three children presented with thrombosis from the aneurysmal sac to the portal trunk. All the children underwent surgical thrombectomy and resection of the aneurysmal sac after birth. Operative findings showed no umbilical vein but an abnormal dilated and thrombosed vein coming from the umbilicus to the portal vein following the right vitelline vein trajectory. One child was treated with systemic heparin. Median follow up is 5.6years. Currently, one patient has a normal portal flow. The other two have persistent portal vein thrombosis with portal cavernoma and portal hypertension. This malformation is rare and should be considered in cases of early diagnosed umbilical vein varix whose diameter is greater than 20mm. We advocate an early surgical thrombectomy with heparinization to prevent portal vein thrombosis. [Copyright &y& Elsevier]

Abstract: “Wandering liver” describes an excessive mobility of the liver caused by abnormalities of hepatic fixation that could lead to hepatic pedicle torsion or bowel obstruction. It is considered a rare entity, but because of the evolution in medical imaging techniques, this unusual condition is being identified more often. We report 2 cases presenting with chronic vague abdominal pain, diagnosed by abdominal ultrasonography and the use of cine–magnetic resonance imaging sequences with dynamic maneuvers. We tried to correlate our ultrasound and magnetic resonance imaging findings to peroperative findings and insist on the usefulness of dynamic maneuvers when confronted with atypical symptoms and a normal abdominal ultrasound scan finding. [Copyright &y& Elsevier]

Objective: To describe the diagnostic criteria and outcome of fetal megacystis according to the gestational age at diagnosis. Methods: A 7-year retrospective study was carried out from 2004 to 2011, including cases of megacystis referred to 2 prenatal fetal medicine units. The following data were collected and analyzed: maternal age, term at diagnosis (gestational weeks), ultrasonographic and magnetic resonance imaging data, karyotype, decision of the multidisciplinary prenatal team, fetopathology in cases of termination of pregnancy or fetal death, final diagnosis at birth after ultrasonography and voiding cystourethrography, and medical and surgical follow-up. Results: Of the 69 fetuses included in this study, 82.6% were males; 26 were diagnosed during the first trimester, 21 during the second, and 22 during the third. During the first trimester, the main etiologies were urethral occlusions and prune-belly syndrome with poor fetal prognosis. Nineteen pregnancies (69%) were terminated for medical reasons including the association with other malformations, poor evolution, or miscarriage. Only 4 children were born alive. The main etiologies of megacystis discovered during the second and third trimesters were vesicoureteral reflux and urethral occlusion. Twenty of 22 fetuses (91%) were born alive when the fetal megacystis was discovered after 27 weeks of gestation. Conclusion: Antenatal discovery of megacystis is a complex and challenging prognostic situation. The prognosis depends on the gestational age at discovery. Megacystis is not always associated with obstruction. In a newborn with megacystis, bladder outlet obstruction has to be excluded. Optimal counseling of the involved parents requires a multidisciplinary approach. [ABSTRACT FROM AUTHOR]