Success of atrioventricular septal defect repair is defined by post-operative atrioventricular valve function and presence of residual intracardiac shunting. We evaluated differences in interpretation of atrioventricular valve function and residual defects between transesophageal and transthoracic echocardiography in a contemporary cohort of infants undergoing atrioventricular septal defect repair. Among 106 patients, we identified an increase in left and right atrioventricular valve regurgitation, right atrioventricular valve inflow gradient, and increased detection rate of residual intracardiac shunting on transthoracic compared to transesophageal echocardiograms, although residual shunts identified only on transthoracic echocardiogram were not haemodynamically significant. Findings may help inform expectation of post-operative transthoracic echocardiogram findings based on intraoperative assessment.

We report a case of a 25-year-old man diagnosed with an unusual case of cor triatriatum sinister with a mobile left atrial thrombus. He was hospitalised with aggravating dyspnoea. Transthoracic echocardiography revealed a membrane-like structure traversing the left atrial and a small orifice of about 7.1 mm. The mean pressure gradient was 12.94 mmHg across the orifice of the membrane-like structure and there was a mobile mass in the post-erosuperior chamber. The anomaly was rectified by a surgical resection. Timely diagnosis and surgical repair may prevent stroke in patients with unusual cor triatriatum sinister.

Objectives: This study sought to investigate the prevalence of atrial septal aneurysms in the paediatric population and to define coexisting abnormalities and their incidence. Background: Few papers refer to the prevalence of atrial septal aneurysms in childhood. Methods: We enrolled a total of 4522 children aged more than 12 months who underwent a transthoracic echocardiography. Atrial septal aneurysm was defined as a protrusion of the interatrial septum or part of it >15 mm beyond the plane of the atrial septum or phasic excursion of the interatrial septum during the cardiorespiratory cycle of at least 15 mm in total amplitude and a diameter of the base of the aneurysm of at least 15 mm. Results: Atrial septal aneurysms were found in 47 children (1.04%). They involved almost the entire septum in 14 patients (28.89%) and were limited to the fossa ovalis in 33 (71.11%). An atrial septal aneurysm was an isolated structural defect in 17 (35.56%). In 30 (64.44%) patients, it was associated with interatrial shunting – atrial septal defect and patent foramen ovale. At the echo follow-up after a year, no changes were recorded. Conclusions: Prevalence of atrial septal aneurysms is almost 1%. The most common abnormalities associated are interatrial shunts, that is, a patent foramen ovale and an atrial septal defect. From a medical point of view, it is suggested that no action is to be taken during childhood, as a child with an atrial septal aneurysm is not at increased risk compared with a child without one. Follow-up is scheduled on an individual basis.

There is a collection of rare congenital cardiac defects that can produce significant haemodynamic embarrassment. Owing to their rarity, these lesions may be overlooked or mistaken for other more common congenital cardiac defects. Using careful segmental echocardiographic techniques, such as multiple planes of imaging and inferential Doppler findings, it is possible to identify these lesions and thus plan surgical management. Some of the lesions of importance reviewed include the aortopulmonary window, the aorto-ventricular tunnels, the aorto-atrial tunnels, and aneurysm of the sinuses of Valsalva. Some conditions such as the aortopulmonary window and aneurysm of the sinuses of Valsalva often occur in the setting of other congenital cardiac lesions. At times, the presence of coexisting lesions may mask some of the characteristic features of these defects. With prompt identification, all of these rare conditions are amenable to complete surgical repair. Newer modalities such as three-dimensional echocardiography can further enhance surgical planning.

Aims: Our aims were to use transthoracic three-dimensional echocardiography to assess the morphology of atrial septal defects in children prior to closure, and to compare the three-dimensional echocardiographic data with transcatheter and surgical findings. Methods and results:We used transthoracic three-dimensional echocardiography in 62 consecutive patients, aged from 2 to 18 years, with atrial septal defects, measuring the maximal diameter and the extent of the rims. Subsequent to the study, we referred 42 patients for transcatheter closure, the rims being measured at greater than 4 mm. We found a good correlation between the maximal diameter of the defect as measured at transthoracic three-dimensional echocardiography and using a balloon (y = 3.45 − 0.73x; r = 0.78; p < 0.0001), the mean difference between the measurements being 2.4 ± 2.8 mm. Successful closure with the Amplatzer septal occluder, having a mean size of 22 ± 4 mm, was achieved in 95% of the patients. Of the original cohort, 20 patients were referred for surgical closure. In these patients, the inferior rim had been deemed insufficient in 5, the postero-superior rim in 6, and the postero-inferior rim in 9. Complete agreement was found when the deficiency of the rim as judged using transthoracic three-dimensional echocardiography was compared with intraoperative findings. The correlation between measurements of the deficiency of the rim achieved by transthoracic three-dimensional echocardiography and at surgery was excellent (y = 0.2 + 0.98x; r = 0.93; p < 0.0001), the mean difference between the measurements being no more than 0.6 ± 0.4 mm. Conclusions: Transthoracic three-dimensional echocardiography proved accurate in measuring the maximal diameter and rims of atrial septal defects within the oval fossa. This non-invasive method will be valuable in selecting children for transcatheter or surgical closure of such defects.

The aortopulmonary window is a communication between the ascending aorta and the pulmonary trunk in the presence of two separate arterial valves. This uncommon congenital anomaly is reported rarely in the literature. We present here our experience with 16 patients, emphasizing the importance of early closure of the defect by a transaortic approach.

We performed surgery on 16 patients over a period of 13 years using a transaortic approach under cardiopulmonary bypass. The median age of the patients at the time of operation was 6.5 months, with a range from 1 month to 11 years. Preoperative pulmonary arterial systolic pressure ranged from 30 to 100 mmHg. Associated cardiac anomalies were present in 7 of the patients, and were repaired at the same stage. The defect was between the ascending aorta and the proximal pulmonary trunk in 13 patients, and between the ascending aorta and the distal pulmonary trunk, with overriding of the orifice of the right pulmonary artery, in 3 patients. For closure, we used a patch of 0.4 mm Gore-Tex in 11, and gluteraldehyde-treated autologous pericardium in 5 of the patients.

One patient died during surgery. The mean follow-up period for the surviving 15 patients was 52.2 months, with a range from 12 to 130 months. All the patients were in good condition during the follow-up, and no residual defects have been detected.

Aortopulmonary window is a rare congenital cardiac anomaly, which can be repaired with very good operative results if surgery is performed before the development of irreversible pulmonary hypertension. We advise early correction of the defect with a transaortic patch, repairing all associated cardiac anomalies at the time of diagnosis.