In neonatal Ebstein’s anomaly of the tricuspid valve, prolonged ductal patency in patients without anatomic pulmonary valve atresia can be deleterious. Circular shunts may develop in patients with different degrees of pulmonary and tricuspid insufficiency. Closure of the arterial duct may result in haemodynamic improvement in particular scenarios. The transcatheter approach is a valuable closure alternative despite some technical difficulties in large-sized arterial ducts and low birth weight neonates. Herein, we report on two consecutive term newborns with Ebstein’s anomaly and large arterial ducts in whom mechanical stimulus of the arterial duct during failed attempts of transcatheter closure led after two days to definitive closure followed by good clinical outcomes.

We present a case of an 18-year-old male with large anterior muscular ventricular septal defect. Assessment by echocardiography showed that the defect has completely closed by the growth of muscle bundles that formed a pouch-like structure on the right ventricular side. This unusual mechanism of closure has been reported in one prior case report. In our report, we present images obtained by three-dimensional echocardiography.

Direct communication between the right pulmonary artery and the left atrium is rare. We report a case that was first detected in utero and confirmed by a two-dimensional echocardiographic study performed postnatally. The patient remained asymptomatic, and hence was managed conservatively. The fistula gradually decreased in size and closed spontaneously at 15 months of age. The rarity of the case is discussed.

The incidence of spontaneous closure of uncomplicated defects of the oval fossa was determined in cohort of 498 children. Surgical repair was accomplished in 428 at a median age of 4.5 years. Of the 70 who did not undergo surgery, 20 showed spontaneous closure or a decrease in size of the defect. The defect was unchanged in the other 50. The age at diagnosis, pulmonary-to-systemic flow ratio and size of the defect were not statistically significantly different in the changed and unchanged groups, and there was no difference in prevalence of symptoms, the triad of systolic and diastolic murmurs and abnormal splitting of the second sound, or of right ventricular enlargement by echocardiography. The rate of spontaneous diminution in size or actual closure of a defect in this series was 4%, and no clinical predictor of change in the size of a defect was identified. Thus, delay in repair of a large flow atrial septal defect in the hope of spontaneous closure is not justified, whether or not symptoms are present.

Muscular ventricular septal defects were diagnosed by echocardiography in 97 neonates within 7 days of birth. In 82 of the neonates (84.5%), the defect was solitary, while 15 had multiple defects. The solitary defects was located at mid-septal, apical, anterior and inlet locations in 42 (51.2%), 21 (25.6%), 14 (17.1%) and 5 (6.1%) neonates, respectively. Multiple defects occurred in the apical, anterior and mid-septal areas. The diameter of the solitary defects ranged from 1 to 6 mm (2.3 ± 0.8 mm), while the multiple lesions were 1 to 4 mm in diameter (2.1 2.3 ± 0.8 mm 0.8 mm) in 28 instances in which they could measured. It proved possible to follow 79 of the patients for period of 10 to 13 months. The defects closed spontaneously in 56 (84.8%) of 66 patients with a single defect, and in 7 (53.8%) of 13 of those with multiple defects (P<0.05). For the solitary defects, the position and size were factors determining the likelihood and speed of closure. Defects located at the apical septum, or defects larger than 4 mm in diameter, closed slowly and at a later stage. Echocardiography is an useful technique in establishing of natural history of muscular ventricular septal defects encountered in neonates.

We describe 3 siblings with muscular ventricular septal defects, two requiring surgical closure. One of their offspring had a rare congenital aneurysm of the muscular ventricular septum, also requiring surgery. Another had a small muscular ventricular septal defect which closed spontaneously. Their father had echocardiographic evidence suggestive of a closed muscular defect. Paternal cousins have had ventricular septal defect, hypertrophic cardiomyopathy, and tetralogy of Fallot. There was no evidence of 22q11 deletion.

Although ventricular septal defects are the most common congenital heart defect, such familial clustering is uncommon. The distribution of cases in this family suggests autosomal dominant inheritance. With echocardiography, and more precise diagnosis of defects which close, a larger genetic component may be revealed in other families.

Objective: To emphasize the formation of fibrous aneurysmal structures in the setting of muscular ventricular septal defects Methods: Among patients with muscular ventricular septal defects followed in our institute, we described six who were diagnosed with formation of fibrous aneurysms. Of the patients, one was female and others were male. The age at diagnosis ranged from eight days to 16 years. In one, the defect opened between the ventricular inlets, but in the others the defects occupied the middle part of the apical trabecular septum. Results: The sizes of defects at initial echocardiographic evaluation ranged from 2.9 to 8 millimetres. The period of follow up for four patients was from 5 months to 7 years, one patient could not be followed up, and the other had already been diagnosed when first seen in our Institution. In our second and third patients, no aneurysm was seen during the initial echocardiographic evaluation, and it was recognized during follow-up. In these two patients, sequential echocardiographic examinations showed that the formation of the aneurysm made the defect smaller, with it almost closing in one. Conclusion: Although seen most frequently in the setting of perimembranous defects, fibrous aneurysms can also form adjacent to, and reduce the size of, muscular ventricular septal defects.

Objectives: To estimate the incidence and timing of spontaneous closure of atrial septal defects within the oval fossa, to study the modalities for diagnosis, and predict the need for therapeutic intervention. Methods: We reviewed retrospectively the medical records of patients with isolated atrial septal defects within the oval fossa, so-called “secundum defects”, diagnosed between January 1990 and February 2003. Based on the initial echocardiographic evaluation, we divided defects into small ones measuring from 3 to 5 mm, medium ones from 5 to 8 mm, and large ones greater than 8 mm. Results: We identified 121 patients, 50 (41.3%) of whom had failed to thrive, and 14 (11.6%) had congestive heart failure. At a mean of 44.9 ± 22.1 months following diagnosis, with a range from 12 to 102 months, the defects had closed spontaneously in 31 patients (25.6%). Of 22 patients having small defects, spontaneous closure occurred in 18 (82%) at a mean age of 18.9 ± 10.2 months. Of 27 patients with defects of medium size, 12 (44%) either experienced spontaneous closure, or else the defect effectively became a patent oval foramen, at a mean age of 51.2 ± 32.2 months. Only 1 (1.4%) of the 72 patients with a defect larger than 8 mm in size underwent spontaneous closure. The defects increased in size in 8 patients (6.6%). Intervention was necessary in 76 patients (63%) at a mean age of 75.5 ± 15.2 months. Conclusion: The initial size of a defect within the oval fossa at diagnosis is the best predictor of its natural history. Some defects increase in size with growth, irrespective of their initial size.