Hypoplastic left heart syndrome(HLHS) refers to the abnormal development of the left-sided
cardiac structures, resulting in obstruction to blood flow from the left ventricular outflow tract. In
addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as
well as mitral atresia or stenosis…………….>
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.
Cardiac hemangioma during fetal life
The prevalence of cardiac tumors has been reported to be 0.05% in an autopsy study of infants only. Cardiac hemangioma in infancy is extremely rare and resection of these tumors after prenatal diagnosis has been rarely reported
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.
Prenatal diagnosis of persistent left superior vena
cava and its associated congenital anomalies
C. BERG*, M. KN¨ UPPEL*, A. GEIPEL*, T. KOHL*, M. KRAPP†, G. KN ¨ OPFLE‡, U. GERMER§, M. HANSMANN* and U. GEMBRUCH*
*Department of Prenatal Medicine and Obstetrics, University of Bonn, and ‡Department of Pathology, Rheinische
Friedrich-Wilhelms-Universit ¨ at, Bonn, †Division of Prenatal Medicine, Department of Obstetrics and Gynecology, University Hospital
Schleswig-Holstein, Campus L¨ ubeck and §Department of Prenatal Medicine, University of Regensburg, Germany
Persistent left superior vena cava (PLSVC) represents the
most common form of anomalous systemic venous return
in adults. It has been observed in 0.3% of autopsies in
the general population and in 4–8% of patients with
congenital heart disease. The PLSVC usually drains into the right atrium via the coronary sinus and may lead to a dilatation of the latter in the prenatal period.
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
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) 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……………
