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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 atrio-ventricular 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.

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.

The electrocardiographic features of Noonan's syndrome have been known for several years, but the discordance between these electrical findings and the underlying haemodynamic disorders remains unexplained. In an attempt to elucidate the genesis of electrical abnormalities, we present here a retrospective study of 14 children with Noonan's disease, aged from a few days to 16 years and evaluated by electrocardiography, vectography, one- or two-dimensional echocardiography, angiography and His bundle electrophysiology. The electrocardiographic abnormalities observed concerned ventricular depolarization and intracardiac electric conduction with, notably, a QRS axis directed towards the right upper part of the electric field and a first degree infra-hisian atrioventricular block (His bundle potentials). Vectography showed in some cases an image of inferior pseudo-necrosis due to the absence of initial inferior forces; this image is highly characteristic. In other cases the QRS loop showed an image of left segmental block which is unusual in this type or cardiac pathology (pulmonary stenosis with or without atrial septal defect of the ostium secundum type).

The process of diagnosis by echocardiography of transposition of the great vessels is based fundamentally on the recognition of the position and relative orientation of the two ventricles and of the two vessels of the base of the heart. Traditional two-dimensional echocardiography allows us to establish the position of the left ventricle because of the atrio-ventricular (mitral) valve and its direct continuity with a sigmoid valve. If this continuity is absent, the mitral valve may be recognised because it closes after the tricuspid valve. It the tricuspid valve is on the right of and/or in front of the mitral valve, the aorta will in principle be found on the right of the pulmonary artery ("loop rule"). The orientation of the great vessels may then be determined. Two-dimensional systems are particularly useful in diagnosis of vascular disorders. If these are not available, traditional echocardiography may lead us to suspect this abnormality: 1. Provided that the orientation of the transducer is known at all times during the recording; 2. Provided the recording demonstrates simultaneously the two great vessels with their respective valves. Two other criteria are important if Van Praagh's rule or the "loop rule" is unhelpful: the diameter of the two vascular trunks, and identification of the valve by a study of the ejection times.

His bundle electrograms were recorded in 42 patients with secundum atrial septal defects (ASD) and in 50 controls, aged 1 month to 16 years, in normal sinus rhythm and with normal PR intervals. Intervals PA (P less than 0.01) and PR (P less than 0.02) were found to be significantly prolonged in ASD patients. Analyzing the results according to age, a significant increase for intervals AH (P less than 0.05), HV (P less than 0.001) and PR (P less than 0.001) was noted in the oldest ASD children as compared to the younger ASD children, whereas in controls only the PR interval was found to be significantly prolonged (P less than 0.05) in the oldest subgroup. Under the age of three years, atrioventricular conduction intervals were not significantly different whether or not an ASD was present. However, in older children with ASD as compared to controls, a significant increase was noted for interval PA (P less than 0.03) in children four to eight years old and for intervals HV (P less than 0.01) and PR (P less than 0.001) in children 9 to 16 years old. ASD patients showed a more significant correlation with age for interval PR with a significantly steeper slope of the regression line. Results show the impact of ASD on AV conduction to be age-related, with involvement not only of the PA interval but also of the HV interval.

An unusual form of left ventricular aneurysm affecting young Negroes has recently been described at the aortic or mitral valve ring; constitutional factors have been postulated. A 4-year-old Negro boy was observed with a left ventricular submitral aneurysm, probably one of the largest and one of the most calcified to be reported. Tuberculous lymphadenitis involving some of the mediastinal lymph nodes was found on postmortem examination; tuberculosis was believed to be a secondary contributing factor. A review of the literature disclosed only 5 other children with calcified left ventricular aneurysms, of which 2 were of the subvalvular type.

Numerous neurological disorders are associated with myocardial disease or involve the cardiovascular system. Echocardiography thus proves quite helpful in the evaluation and management of patients with problems such as hydrocephalus, tuberous sclerosis, Friedreich ataxia, mitochondrial encephalo-myopathies, Werdnig-Hoffmann disease, convulsive disorders, syncope, central nervous system infections, etc.

A retrospective study allowed us to examine 23 cases with aneurysm of the left ventricle. The aneurysm was discovered incidentally in 6 patients, and as a result of cardiac symptoms in an other 14. The diagnosis made following a cerebrovascular accident and an episode of ventricular tachycardia respectively in two cases. Radiological and electrocardiographical investigation revealed the classical findings in 21 cases, while inflammatory changes were also present in 19. Tuberculosis was found to coexist in 4 patients, and was suspected in a further 4. The aetiology of these aneurysms has remained obscure until now. We feel that a debilitating conditions such as tuberculosis or late pregnancy, or perhaps a change in cardiac function, might lead to the development or enlargement of a sub-annular aneurysm in a predisposed subject. One of our patients presented with an apical aneurysm of the left ventricle in association with a complete situs inversus. A further patient had sustained a severe precordial ingiury 10 years previously, and 2 others had a past history of rheumatic joint disease many years previously. It appears to us that cases with calcification have a better prognosis.

A method for positioning the cardiac end of a ventriculoatrial shunt with the aid of echocardiography is described. This simple procedure has resulted in safe and accurate shunt placement in infants.

Iatrogenic acute spondylytis with secondary bacterial meningeal infection following lumbar punctures was observed in an 11 month old Negro boy. The infection healed but led to a spondylotic deformity of the lumbar spine.

Three cases of endocardial fibroelastosis with vectorcardiographic findings suggestive of an anterior myocardial infarct are presented. It had been previously reported that such vectorcardiographic features were diagnostic of anomalous origin of the left coronary artery. We conclude that the two diseases can not be differentiated on the basis of electrocardiography or vectorcardiography alone. Two of the cases presented were in identical twins; serial scalar electrocardiograms and vectorcardiograms were obtained during the development and progression of endocardial fibroelastosis.