Cross sectional echocardiographic assessment of the extent of the atrial septum relative to the atrioventricular junction in atrioventricular septal defect.

OBJECTIVE: To study patients with atrioventricular septal defect to determine the pathognomonic morphological features of the lesion and the relation between the septal structures and the atrioventricular junction. Setting : Tertiary level paediatric cardiology centre. METHODS: Cross sectional echocardiograms from 60 patients were reviewed using qualitative and quantitative analysis. The unifying feature was the presence of a common atrioventricular junction. The overall dimensions of the septal defect were determined and related to the plane of the common junction; the extent of both the atrial and the ventricular septal components was then measured according to the site of closure of the bridging leaflets. RESULTS: In 48 cases, the common junction was guarded by a common valvar orifice, but in 12 cases there were separate right and left valvar orifices. Irrespective of the valvar morphology, no significant difference was found between the groups in terms of the dimensions of the atrial and ventricular septal components. In all patients, the hole permitting shunting at atrial level extended below the plane of the atrioventricular junction, with a variable position of the leading edge of the atrial septum itself. CONCLUSIONS: The atrioventricular junction is a common structure irrespective of valvar morphology. In spite of the presence of unequivocal shunting at atrial level, the atrial septum is usually a well formed structure, even extending in some below the level of the common atrioventricular junction.

Morphologic features of the hypoplastic left heart syndrome--a reappraisal.

The report describes the morphologic features of 102 specimens with hypoplasia of the chambers of the left heart, all with either a concordant atrioventricular connection (66 cases) or absence of the left atrioventricular connection, the right atrium being connected to a dominant right ventricle (36 cases). Either the aorta was connected to the left ventricle, having a patent valve in 23 cases and an imperforate valve in 5, or else the aortic trunk was atretic (74 cases). It was not possible to determine with certainty the ventricular origin of the aorta when its trunk was atretic, but in all hearts but 1 it seemed to be connected to the left ventricle. The atrial septum was intact in 11 hearts and grossly restrictive in a further 8. Deviation of the atrial septum relative to the infolded atrial roof (septum secundum) was seen in 32 specimens. The ventricular septum was intact in 88 hearts. The length of the left ventricle compared to the right was greatest in the 14 hearts with a ventricular septal defect. The left ventricular length was also significantly greater in hearts with a patent aortic valve. The state of the atrial septum, in contrast, bore no relationship to the dimensions of the left ventricle. Aortic coarctation was found in 44 of the hearts, and in all but 2 the shelf lesion was preductal in location. Coarctation was significantly more frequent in those hearts in which the ascending aorta was less than 3 mm in diameter. The anatomic findings are discussed in relation to recent advances in the surgical palliation of these malformations.

Solitary arterial trunk with pulmonary atresia and arteries with supply to the left lung from both an arterial duct and systemic-pulmonary collateral arteries.

We report a case of pulmonary atresia with ventricular septal defect and absent pulmonary trunk. The sources of pulmonary blood supply were the arterial duct and systemic pulmonary collateral arteries. The left lung received blood from both sources which is very rare. One of the systemic pulmonary collateral arteries arose from the right coronary artery in an unusual fashion.