Cardiogenesis and Congenital Cardiopathies
Course in Cardiogenesis and Congenital Cardiopathies :
From Developmental Models to Clinical Applications
June 7th-10th 2008
neonatal cardiology
AEPC TEACHING COURSE INTERVENTIONAL CATHETERISATION
The AEPC interventional working group is pleased to announce the second
TEACHING COURSE in pediatric and congenital INTERVENTIONAL CARDIAC
CATHETERISATION, which will take place on 4-5 april 2008, in Milan (Italy).
Aorto-ventricular tunnel
Aorto-ventricular tunnel is a congenital, extracardiac channel which connects the ascending aorta above the sinutubular junction to the cavity of the left, or (less commonly) right ventricle………………….>
Cantrell’s pentalogy
Pentalogy of Cantrell is a rare congenital malformation
characterized by midline defects, resulting from
defective development in the septum transversum. The
constellation of findings includes deficiency of the
diaphragmatic pericardium, lower sternum, anterior
diaphragm, supraumbilical abdominal wall often
allowing an omphalocele as well as an intrapericardial
diaphragmatic hernia, and a cardiac lesion, most often
a ventricular septal defect. In severe cases, the
heart herniates through the diaphragmatic defect,
causing thoracoabdominal ectopia cordis. Other
associated congenital cardiac lesions may include an
atrial septal defect, pulmonary valve stenosis, tetralogy
of Fallot, dextrocardia, anomalous pulmonary venous
connection, tricuspid atresia, and truncus arteriosus.
FETAL AND NEONATAL PRESENTATION OF NON-COMPACTED VENTRICULAR
Noncompaction of the ventricular myocardium (NCVM) is a rare cardiomyopathy characterized by
numerous, prominent trabeculations and deep intertrabecular recesses caused by arrest in myocardial embryogenesis.
This study reviews our experience with neonatal and fetal NCVM. In contrast to the past reports of fetal and
neonatal NCVM, all pts in this group improved, and survived infancy.
Diagnosis, characterization and outcome of ccTGA
Diagnosis, characterization and outcome of congenitally
corrected transposition of the great arteries in the fetus:
a multicenter series of 30 cases
D. PALADINI*, P. VOLPE†, M. MARASINI‡, M. G. RUSSO§, M. VASSALLO*, M. GENTILE¶,and R. CALABR ` O§
*Fetal Cardiology Unit, Department of Gynecology and Obstetrics, University Federico II of Naples and §Department of Pediatric Cardiology, 2nd University of Naples, Monaldi Hospital, Naples, †Department of Obstetrics and Gynecology, ‘‘Di Venere-Giovanni XXIII’’ Hospital and ¶Department of Medical Genetics, I.R.C.C.S. ‘‘Di Venere-Giovanni XXIII’’ Hospital, Bari and ‡Pediatric Cardiology,
I.R.C.C.S. Giannina Gaslini Hospital, Genoa, Italy
Congenitally corrected transposition of the great arteries
(ccTGA) is characterized by atrioventricular and ventriculoarterial discordance. It represents a rare cardiac
defect, accounting for 1.1% of cases of major congenital
heart disease (CHD), and has an incidence at birth of
0.02 per 1000 live births. Prenatal diagnosis of this
lesion is feasible but may be difficult to detect at routine
screening because the atrioventricular discordance may
be overlooked if other significant anomalies of the fourchamber
view are absent.
Familial Origin of Atrial Septal Aneurysm
Familial Origin of Atrial Septal Aneurysm
Ioannis A. Paraskevaidis, MDa,b,*, Dimitrios Tsiapras, MDa, Stamatis Kyrzopoulos, MDa,
and Dimitrios T. Kremastinos, MDb
The familial origin of atrial septal defects has been previously reported.
This is the first study describing 2 families with atrial septal aneurysm of familial origin.
Williams-Beuren syndrome (WBS)
Williams-Beuren syndrome (WBS) is a multisystem genetic disorder caused by a microdeletion on chromosome 7. The majority of patients demonstrate cardiovascular pathology, most commonly supravalvular aortic and/or other vascular stenoses……………
Workshop on Magnetic Resonance on CHD
2nd Workshop on :
Magnetic Resonance Imaging in Diagnosis and Management of Congenital Heart Disease
21-22 october 2005 – Massa
Deletion 22q11.2 syndrome (DiGeorge/Velo-Cardio-Facial syndrome).
Digilio MC,1 Marino B,2 Capolino R,1 Dallapiccola B.3 Clinical manifestations of Deletion 22q11.2 syndrome (DiGeorge/Velo-Cardio-Facial syndrome). Images Paediatr Cardiol 2005;23:23-34
1 Medical Genetics, Bambino Gesù Hospital, Rome, Italy
2 Pediatric Cardiology, Institute of Pediatrics, University “La Sapienza”, Rome, Italy
3 Experimental Medicine and Pathology, University “La Sapienza”, and CSS-Mendel Institute, Rome, Italy
Abstract
Deletion 22q11.2 syndrome (Del22) (DiGeorge/Velo-Cardio-Facial syndrome) is characterized by congenital heart defect (CHD), palatal anomalies, facial dysmorphisms, neonatal hypocalcemia, immune deficit, speech and learning disabilities. CHD is present in 75% of patients with Del22.