Categorized Genetic Analysis in Childhood-Onset Cardiomyopathy.

Background: Childhood-onset cardiomyopathy is a heterogeneous group of conditions the cause of which is largely unknown. The influence of consanguinity on the genetics of cardiomyopathy has not been addressed at a large scale.

Methods: To unravel the genetic cause of childhood-onset cardiomyopathy in a consanguineous population, a categorized approach was adopted.

Cardiac rhabdomyoma presenting as infective endocarditis: a case report.

We report a case of cardiac rhabdomyomas in an infant who presented with right ventricular tachycardia, and a clinical picture of infective endocarditis. Typical features of tuberous sclerosis developed subsequently. To the best of our knowledge, cardiac rhabdomyoma has not been reported previously in association with infective endocarditis.

Spontaneous closure of atrial septal defects within the oval fossa.

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.

Successful surgical repair of Uhl's anomaly.

We report a case of Uhl's anomaly in a 5-month-old cyanotic infant who presented with thromboembolic stroke and acute hemiparesis. The patient underwent successfully an initial surgical repair, which included exclusion of the right ventricle by patch closure of the tricuspid valve, atrial septectomy and construction of a bidirectional Glenn shunt. This was followed by successful construction of a total cavopulmonary connection.