M. G. Russo

Characteristics, associations and outcome of absent pulmonary valve syndrome in the fetus

To assess in a population of 21 fetuses diagnosed with absent pulmonary valve syndrome (APVS) the accuracy of prenatal diagnosis, the incidence of extracardiac and chromosomal anomalies and the perinatal outcome.

Similar cardiac remodelling after transcatheter atrial septal defect closure in children and young adults

Objective: To study the cardiac geometric changes after transcatheter closure of large atrial septal defects (ASDs) according to patient age at the time of the procedure. Design: Prospective echocardiographic follow-up study. Setting: Tertiary referral centre. Patients and intervention: 25 asymptomatic patients younger than 16 years (median 8 years; group 1) and 21 asymptomatic adults (median 38 years; group 2) underwent percutaneous closure of large ASD with the Amplatzer septal occluder device (mean 25 (SD 7) mm). Main outcome measures: Cardiac remodelling was assessed by M mode and two dimensional echocardiography one and six months after ASD closure. Results: By six months, right atrial volume decreased from 31 (15) to 19 (5) ml/m2 (p < 0.001) and right ventricular (RV) transverse diameter decreased from 29.8 (8.6) to 23.2 (5.6) mm/m2 (p < 0.001). Conversely, left atrial volume did not change significantly (from 18 (6) to 20 (6) ml/m2, NS) and left ventricular (LV) transverse diameter increased from 27.8 (6.4) to 31.8 (7.3) mm/m2 (p < 0.05). Ventricular remodelling resulted in an RV:LV diameter ratio decrease from 1.1 (0.2) to 0.7 (0.1) (p < 0.001). The magnitude and time course of cardiac remodelling did not differ significantly between the age groups. Indeed, right atrial volume decreased by 33 (26)% versus 37 (23)%, RV diameter decreased by 26 (10)% versus 20 (13)%, LV diameter increased by 17 (15)% versus 15 (10)%, and RV:LV diameter ratio decreased by 36 (8)% versus 27 (15)% in groups 1 and 2, respectively. Conclusions: Cardiac remodelling after percutaneous ASD closure seems to be independent of the patient’s age at the time of the procedure up to early adulthood. Thus, postponing ASD closure for a few years may be a reasonable option for potentially suitable asymptomatic children.

Diagnosis, characterization and outcome of congenitally corrected transposition of the great arteries in the fetus: A multicenter series of 30 cases

To describe the anatomy, associated anomalies and outcome of 30 cases of congenitally corrected transposition of the great arteries (ccTGA) detected prenatally.

Transposition of the great arteries in the fetus: assessment of the spatial relationships of the arterial trunks by four‐dimensional echocardiography

Coronary arterial abnormalities can be one of the few negative prognostic indicators in transposition of the great arteries (TGA), and their occurrence is related to the type of spatial relationship of the great arteries. The main objective of this study was to assess whether the use of the reconstructed en‐face view with color Doppler imaging of the four cardiac valves can demonstrate the different types of spatial relationship of the arterial trunks in fetuses with TGA, in order to derive the risk of coronary abnormalities. A secondary end‐point was the evaluation of the type of coronary arterial branching pattern.

Neonatal Patent Ductus Arteriosus Recanalization and Stenting in Critical Ebstein’s Anomaly

A critically ill 3-day-old neonate with severe tricuspid valve Ebstein’s anomaly, functional pulmonary atresia, and closed ductus arteriosus, unresponsive to prostaglandin infusion, underwent percutaneous ductal recanalization and stenting as an alternative to a surgical shunt. After local prostaglandin infusion through an end-hole catheter, the ductus was passed using a hydrophilic, high-support coronary guidewire. It was then stabilized by coronary stent implantation, after which the arterial oxygen saturation showed a sudden rise. In conclusion, ductus arteriosus recanalization and stenting can be successfully achieved within a few days after spontaneous closure as a cost-effective alternative to a surgical shunt for critical neonatal, duct-dependent Ebstein’s anomaly.

Prenatal echocardiography in a case of Uhl's anomaly

Uhl’s anomaly is a rare form of congenital heart disease (CHD) characterized by a partial or complete absence of the right ventricular myocardium1. This disorder confers a parchment-like appearance to the heart wall, as described by Uhl in 1952. To our knowledge, only two cases of the prenatal diagnosis have been reported previously2,3. We describe a further case of Uhl’s anomaly diagnosed during fetal life and followed up to 1 year postnatally. A 23-year-old woman was referred to our tertiary center at 31 weeks’ gestation because of suspicion of fetal CHD on routine fetal ultrasonographic examination. Echocardiographic evaluation showed an enlarged right ventricular cavity (Figure 1), without apical trabeculation, and a hypokinetic thin ventricular wall which prompted the diagnosis of Uhl’s anomaly. Color Doppler examination showed moderate tricuspid regurgitation without inferior displacement of the leaflets. Pericardial effusion and polyhydramnios were also observed. There was no evidence of aortic coarctation, and the pulmonary valve and pulmonary and systemic venous return were normal. At 37 weeks’ gestation a 2750-g male was delivered by Cesarean section because of hydrops and the mother’s history of previous Cesarean section. On physical examination the baby had tachypnea, enlargement of the liver and a gallop heart rhythm. Arterial gas blood analysis initially showed metabolic acidosis (pH, 7.15; pCO2, 56 mmHg). The baby underwent mechanical ventilatory support for 15 days. The work-up also revealed a proto-meso-systolic murmur on the right sternal border (3/6 on Levine’s scale). An electrocardiogram showed sinus rhythm with a heart rate of 150 beats per minute, QRS-axis: −30◦ on the frontal plane, right atrial enlargement and low QRS amplitude in the right precordial leads. An X-ray

The Role of von Willebrand Factor in Vascular Inflammation: From Pathogenesis to Targeted Therapy

Beyond its role in hemostasis, von Willebrand factor (VWF) is an emerging mediator of vascular inflammation. Recent studies highlight the involvement of VWF and its regulator, ADAMTS13, in mechanisms that underlie vascular inflammation and immunothrombosis, like leukocyte rolling, adhesion, and extravasation; vascular permeability; ischemia/reperfusion injury; complements activation; and NETosis. The VWF/ADAMTS13 axis is implicated in the pathogenesis of atherosclerosis, promoting plaque formation and inflammation through macrophage and neutrophil recruitment in inflamed lesions. Moreover, VWF and ADAMTS13 have been recently proposed as prognostic biomarkers in cardiovascular, metabolic, and inflammatory diseases, such as diabetes, stroke, myocardial infarction, and sepsis. All these features make VWF an attractive therapeutic target in thromboinflammation. Several lines of research have recently investigated “tailor-made” inhibitors of VWF. Results from animal models and clinical studies support the potent anti-inflammatory and antithrombotic effect of VWF antagonism, providing reassuring data on its safety profile. This review describes the role of VWF in vascular inflammation “from bench to bedside” and provides an updated overview of the drugs that can directly interfere with the VWF/ADAMTS13 axis.

Partial atrioventricular septal defect in the fetus: diagnostic features and associations in a multicenter series of 30 cases

To assess the anatomical features and the associations of partial atrioventricular septal defect (pAVSD) in the fetus.

Von Willebrand Factor as a Novel Player in Valvular Heart Disease: From Bench to Valve Replacement

von Willebrand Factor (vWF) is a well-known mediator of hemostasis and vascular inflammation. Its dynamic modulation in the bloodstream, according to hemodynamic conditions, makes it an appealing biomarker in patients with valvular heart disease (VHD). Recent studies highlight the close connection between vWF and VHD, with possible implications in the pathogenesis of VHD, promoting valve aging and calcification or favoring the development of infective endocarditis. Moreover, vWF has been recently proposed as a new diagnostic and prognostic tool in patients with valve stenosis or regurgitation, showing a strict correlation with severity of valve disease, outcome, and bleeding (Heyde syndrome). A novel role for vWF is also emerging in patients undergoing percutaneous or surgical valve repair/replacement to select and stratify patients, evaluate periprocedural bleeding risk, and detect procedural complications. We also report our single-center experience, suggesting, for the first time, possible clinical implications for vWF in percutaneous mitral valve repair (MitraClip). This review summarizes recent advances in the role of vWF in VHD with an updated overview going from bench to operating room.

OC170: Prenatal diagnosis improves short term outcome in Transposition of the Great Arteries

Echogenic ovarian foci (EOF) are common ultrasound findings of unclear significance. Objectives: to follow up natural course of EOF and to determine potential for malignant transformation. Materials and Methods: Sixty five patients with EOF in one or both ovaries were followed with yearly pelvic ultrasound for 10 years. Results: EOF were bilateral in 53 patients, unilateral in 12 patients. EOF had clustered pattern in 83 ovaries and were singular in 35 ovaries. EOF were peripheral in 67 ovaries, had central location in 18 and had mixed distribution in 33 ovaries. During longitudinal follow up EOF stayed unchanged in 99 ovaries, increased in size in 12, decreased or became undetectable in 7 ovaries. Ovarian specimens were obtained in 6 patients (total 9 ovaries) who had gynecologic surgery for reasons unrelated to EOF. Epithelial inclusion cysts were detected in 5 ovaries, simple calcifications in 4 ovaries. None of the patients developed ovarian malignancy over 10-year observation period. Conclusion: EOF are benign findings and their appearance changes over time. In our series EOF did not signify ovarian pathology and required no follow-up.