Biomedical subjects

Sylvia Abadir

Publications and source records attributed to Sylvia Abadir.

6 recordsLinked to original sources

PubMed2006

Severe aortic valvar stenosis in familial Noonan syndrome with mutation of the PTPN11 gene.

Noonan's syndrome is an autosomal dominant genetic disease, in which mutation of the PTPN11 gene is found in from one-third to half of all cases. Pulmonary valvar stenosis and myocardiopathy are frequently associated cardiac malformations, whereas aortic valvar stenosis is rarely described. We report, as far as we know, the first case of familial Noonan syndrome with severe aortic valvar stenosis, demonstrating mutation of the PTPN11 gene in the father of the patient.

Aortic Valve Stenosis↗

PubMed2006

Expandable right ventricular-to-pulmonary artery conduit: an animal study.

This study was performed to assess a new vascular stent graft as an expandable valved conduit for right ventricular outflow tract (RVOT) reconstruction in sheep. Conduits were constructed by sewing an 18-mm valved conduit inside a stent. Crimped to 16 mm, they were implanted either under or without extracorporeal circulation in seven (group A) and in five (group B) sheep, respectively. Six weeks and 3 mo after their insertion, conduits were dilated intraluminally. A valved stent was implanted percutaneously into conduits before they were killed. Two animals from group A recovered normally, whereas five animals had a complicated postoperative course. In group B, one died acutely due to kinking of the conduit. Balloon dilatations were performed in all surviving animals. First dilatations had a slight impact on valvular function in all animals but one, whereas second dilatations led to significant PR in all. Transcatheter valve implantation was performed successfully. When animals were killed, no bleeding was found around the surgically implanted device. In conclusion, we designed a biologic valved conduit for RVOT reconstruction that can be dilated sequentially to follow animal growth. This new device can have tremendous applications in children with congenital heart diseases involving the RVOT.

Animals↗

PubMed2006

Transcatheter closure of perimembranous ventricular septal defects with Amplatzer occluder assessed by real-time three-dimensional echocardiography.

We report our initial experience in two patients using real-time three-dimensional echocardiography to assess perimembranous ventricular septal defect and device morphology and their relation with contiguous cardiac structure. Defect size and rims as well as device position and profile were displayed from the three-dimensional "en face" views. We think that real-time three-dimensional echocardiography could be a complementary approach to angiography and transesophageal echocardiography in performing transcatheter closure of perimembranous ventricular septal defect.

Cardiac Catheterization↗

PubMed2006

Ebstein's anomaly assessed by real-time 3-D echocardiography.

The outcome of patients with Ebstein's malformation depends mainly on the severity of the tricuspid valve malformation. Accurate description of the tricuspid anatomy by two-dimensional echocardiography remains difficult. We applied real-time three-dimensional echocardiography to 3 patients with Ebstein's anomaly. Preoperative and postoperative descriptions of the tricuspid valve were obtained from views taken inside the right ventricle. Surface of the leaflets as well as the commissures were obtained by three-dimensional echocardiography. Real time three-dimensional echocardiography is a promising tool, providing new views that will help to evaluate the ability and efficiency of surgical valve repair in patient with Ebstein's malformation.

Adolescent↗

PubMed2006

Off-label use of an adjustable gastric banding system for pulmonary artery banding.

BACKGROUND: Pulmonary artery banding is proposed as a first palliation in infants with complex congenital heart disease and high pulmonary blood flow. In addition, it may be used to retrain the left ventricle. Optimal tightening may be difficult to obtain, leading to reoperation. An implantable device for pulmonary artery banding with telemetric control was recently developed allowing for repeated adjustments, but it is presently limited to patients weighing less than 20 kg. In large animals, we tested an off-label adjustable gastric banding system for pulmonary artery banding. METHODS AND RESULTS: Fourteen ewes weighing 50 to 75 kg underwent implantation of the Lap-Band device (BioEnterics Corp, Santa Barbara, Calif) around the main pulmonary artery through a left thoracotomy. All had functional evaluation with progressive occlusion and opening of the device at implantation and every 2 weeks until sacrifice immediately after implantation (group 1, n = 8), at 1 month (group 2, n = 3), at 3 months (group 3, n = 3), or death. Invasive pressure measurements in the right ventricle and aorta were carried out each time. Devices were easily implanted in all animals. Progressive occlusion and reopening were possible in all animals during each time point. Two animals died of right heart failure related to excessive tightening of the band. Retrieval of the device without any major damage was possible in 12 of 14 animals. CONCLUSION: With this implantable device, we were able to adjust the pulmonary artery diameter in animals. Patients requiring left ventricle retraining and weighing more than 30 kg would benefit from the device's use in humans.

Animals↗

PubMed2005

Real-time three-dimensional fetal echocardiography using matrix probe.

OBJECTIVE: To investigate the feasibility of using real-time three-dimensional echocardiography (3DE) to evaluate the fetal heart. METHODS: Sixty fetuses were studied with gestational age between 22 and 34 weeks. The fetal heart was normal in 44 fetuses. In 16 fetuses, the fetal heart had morphologic abnormalities (hypoplastic left ventricle in 4, tetralogy of Fallot in 2, Ebstein anomaly in 2, rhabdomyomes in 2, hypoplastic right ventricle in 1) or myocardial dysfunction (in 5 fetuses). Real-time 3DE was performed with a cardiac matrix probe (2-4 MHz). Two modalities of 3D imaging were performed: Biplane and Live 3D imaging. RESULTS: Real-time 3DE was performed successfully in 93%. Biplane imaging allowed a multiplanar scanning of the fetal heart. Using rotation, lateral and vertical tilts, the normal cardiac structures were identified from a unique reference image plane: atria and ventricles, atrioventricular valves, aorta and pulmonary artery. Live 3D imaging allowed surface imaging of the fetal heart. 'En face' view of the foramen ovale from the right atrium was obtained, showing the shape of the orifice. By cropping the pyramidal imaging volume, ascending aorta, aortic arch and ductus arteriosus were depicted from a single dataset. In pathologic fetal hearts, 3DE was helpful for (1) localizing multiple cardiac tumors; (2) estimating size and function of the right and left ventricles; (3) evaluating mechanism of valvular regurgitation and pulmonary obstruction. CONCLUSION: Real-time 3DE is a feasible and non-time-consuming method, allowing a multiplanar scanning and new inside 3D views of the fetal heart.

Echocardiography, Three-Dimensional↗