J. Lintermans

The natural history of cardiac rhabdomyoma with and without tuberous sclerosis

The aim of the present study is to contribute to the knowledge of the natural history of cardiac rhabdomyoma in children with and without tuberous sclerosis. In a retrospective study, 33 children with cardiac rhabdomyoma were collected from three pediatric cardiology centres. In 30/33 patients tuberous sclerosis was associated. High prevalence of cardiac rhabdomyoma was found in infancy, with 21/23 detected before the age of 1 year, and 11/33 before 1 month of age. Cardiac manifestations were present in 19 patients: cardiac rhythm disturbances were detected in 13; in 6/33 a Wolff‐Parkinson‐White syndrome was documented, of which 4 presented paroxysmal arrhythmias. Obstructive or regurgitative phenomena were present in 5; and in 2 patients surgical removal proved necessary. With the exception of one tumoural mass in the right atrium, all 77 tumours were located somewhere in the ventricles, including at atrioventricular valve level. Because of spontaneous regression of most of the tumoural masses, treatment should at first be symptomatic, while surgical removal is required only in life‐threatening conditions, as documented in 2 of our 33 patients.

Myxomatous degeneration of the mitral valve in a child with Turner syndrome and partial anomalous pulmonary venous return.

This a report of myxomatous mitral valve degeneration in a child with Turner syndrome. The diagnosis was first suspected at 7 months of age. At 5 years of age, ultrasonic and angio-cardiographic confirmation prompted a successful mitral valve replacement with a Starr prosthesis. The child also had partial anomalous pulmonary venous return and a dysplastic right kidney. The child has remained well for 4 years after surgery.

Doppler and 2D echocardiographic diagnosis of congenital coronary artery fistulae to the right cardiac chambers: Report of 3 cases

We present three cases of coronary artery fistulae to the right cardiac chambers. The first was a 2-day-old neonate in congestive heart failure: 2D and Doppler echocardiography revealed a dilated proximal left coronary artery and a fistulous connection to the right atrium. The other two patients, respectively 4 and 3 years old, were asymptomatic and presented with a continuous heart murmur: a left coronary artery fistula into the right ventricle was detected by ultrasound in one, and a dilated proximal right coronary artery in the other. The diagnosis was confirmed in all three patients. The first patient was operated upon at 18 months of age; the second patient is awaiting surgery, and in the third patient the fistula was ligated at the age of 3 years. The possibility of ultrasound diagnosis without invasive procedures is suggested.

Atrioventricular-conduction and Right Atrial Volume in Children With and Without Secundum Atrial Septal-defects

Atrioventricular conduction intervals and right atrial end-diastolic volumes were studied in 43 patients (26 with secundum atrial septal defect and 17 controls) to test the hypothesis that the increase in intra-atrial conduction in atrial septal defect is due to right atrial dilatation. There was no relation between any of the atrioventricular conduction intervals and right atrial dilatation. The PH, PR, and AH intervals were, however, related to both age and the RR interval.