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Seven patients with hemodynamic and angiocardiographic diagnosis of supravalvular aortic stenosis were studied by single plane echocardiography. 3 patients had the hypoplastic and 3 the "hourglass" type of the disease. 3 patients underwent surgical repair of the malformation, with postoperative echocardiographic control. There was no relationship between the lumen dimension variations as measured by echocardiography and the transtenotic pressure gradient. With respect to angiocardiography, echocardiography underestimates the severity of the disease. The method was always diagnostic, but the anatomic type of stenosis could not be determined. The severity of the disease can be only indirectly assessed by evaluating left ventricular hypertrophy induced by pressure overload.

Two-dimensional echocardiography in the right oblique subxiphoid view (ROSV) was used to visualize all right ventricular components in 351 patients younger than 2 years of age (100 normal and 251 with various types of congenital heart disease). The ROSV, which is anterior and parallel to the ventricular septum, displays all right-sided cardiac components, the left atrium and the aorta. This view provided the correct diagnosis in patients with pulmonary stenosis or atresia with intact ventricular septum, Ebstein's anomaly, tricuspid atresia, infundibular ventricular septal defect, tetralogy of Fallot and truncus arteriosus. In all patients studied with the ROSV, an excellent definition of morphologic characteristics, equivalent to that in the right oblique axial angiocardiographic view, was achieved. The ROSV should be used routinely to delineate the right ventricular anatomy in all infants with congenital heart disease.

Before Mustard's operation 27 children, from 2 days to 11 months old, with simple or complex transposition of the great vessels, underwent an electrocardiogram at rest. After operation, all the children underwent a standard electrocardiogram and 24 hours continuous Holter monitoring. Successive electrocardiograms at rest were recorded in all patients, and eleven children were successfully examined by dynamic electrocardiogram. The electrocardiogram at rest showed a sinusal rhythm in 66.7 of the patients; during the follow-up, in 33.3% non sinusal rhythm was recorded. The continuous 24 hours dynamic electrocardiogram, immediately after surgery, revealed 25.9% of the patients with non-sinusal rhythm. After 7 months, the Holter monitoring showed, in one case, a severe sinus node disfunction. Our data and those of the literature confirm the importance to follow these patients during a long period of time, because it was found that, even bedately, can develop serious arrhythmias.

The surgical approach to relief of mitral stenosis in children is still a controversial problem. We describe our experience with four severely symptomatic children in whom a valved conduit from the left atrium to the left ventricle was successfully used to bypass a hypoplastic systemic atrioventricular valve. A left atrial-left ventricular extracardiac conduit was implanted in these patients with a hypoplastic mitral anulus and an adequate left ventricular chamber. There were no early or late deaths. Postoperative cardiac catheterization performed in all patients 1 month after the operation showed reduced size of the left atrium, a reduction of the left atrial-left ventricular gradient from a mean of 14 mm Hg to a mean of 5 mm Hg, and an increase of the left atrial outlet from a mean diameter of 10.7 mm to 28.7 mm (including the diameter of the native mitral valve plus the internal diameter of the valved conduit). The application of this unconventional operation in children with congenital or acquired stenosis of the systemic atrioventricular valve should be considered when the mitral valve obstruction cannot be relieved by conventional valve repair or replacement. Furthermore, the left atrial-left ventricular conduit does not preclude future alternative surgical options.

Heart lesions induced in mice, rats, rabbits and dogs by Doxorubicin administered i.v. according to various schedules were studied by light and electron microscopy Vacuolization of myocardial cytoplasm due to distention of the sarcoplasmic reticulum, the T-tubule system and the Golgi vesicles was one of the most common findings. Myocytolysis, clumping and loss of fibrils, fragmentation of sarcomeres, swelling of mitochondria and an increase in lysosomes and residual bodies were also observed. The severity of the cardiomyopathy, quantitatively evaluated by a score system, proved to be dose-dependent. Cardiomyopathy was more severe when the treatment was given in a short period by administration of high doses than when the same cumulative dose was administered as low doses repeated for a long period. The left atrium was more severely affected than the ventricles when high doses were given, whereas it was less affected in animals given low doses. The cardiomyopathy was less severe in animals receiving the same dose in a high volume of solvent and during a long perfusion time. Threshold doses were needed both to induce the cardiomyopathy and to establish it as a progressive disease.

Subcostal oblique 2-dimensional echocardiography was performed in 64 infants younger than 2 years with complete transposition of the great arteries (TGA) (situs solitus, concordant atrioventricular and discordant ventriculoarterial connections). All patients examined before cardiac catheterization had a correct diagnosis by 2-dimensional echocardiography using the subcostal oblique views. Twelve patients had associated left ventricular (LV) outflow tract obstruction and 7 had right ventricular (RV) outflow obstruction. The standard parasternal views failed to diagnose obstruction in 1 patients with LV outflow obstruction and 5 with RV outflow obstruction; the subcostal left oblique cut and long axis of the left ventricle visualized all left-sided obstructions, and right-sided obstructions were correctly displayed in 5 of 7 cases using a combination of left oblique and right oblique cuts. Two-dimensional echocardiographic subcostal oblique views allow an excellent definition of the morphologic characteristics of RV and LV outflow tracts in patients with TGA and improve the diagnosis of the outflow obstruction in these malformations.

The possibility of using reflectance NIRS to assay 2-(benzoylphenyl)-propionic acid (Ketoprofen) in solid-state and gel preparations is investigated. The number of samples and amplitude of the percentage content distribution of the samples is examined in order to define the best possible calibration system.

From June, 1983, to February, 1987, 91 patients with pulmonary outflow tract obstruction underwent a systemic-to-pulmonary artery shunt without an invasive study. The surgical indication was based on two-dimensional (2-D) and Doppler echocardiography only. In 82 patients (90.1%), 2-D and Doppler echocardiography allowed correct assessment of the intracardiac anatomy and of the morphology of the pulmonary arteries, pulmonary veins, and aortic arch. The aortic arch anatomy was misdiagnosed in 6 patients (6.6%) who underwent a modified instead of a classic Blalock-Taussig shunt. In 3 patients (3.3%), the indication for the systemic-pulmonary artery shunt was inappropriate; in 1, operative death resulted from the incomplete diagnosis. After the preselection of patients based on knowledge of the anatomical patterns, a systemic-pulmonary artery shunt may be performed without cardiac catheterization if these rules for definitive patient selection are followed: (1) accurate clinical assessment to identify the patients with restricted pulmonary blood flow; (2) precise intracardiac diagnosis by 2-D and Doppler echocardiography; and (3) definitive 2-D echocardiographic visualization of the pulmonary venous connection and the confluence of the pulmonary arteries.

To identify the pattern of pulmonary anatomy and pulmonary blood supply, we analyzed the angiocardiograms of 40 patients with pulmonary atresia and complex congenital heart disease. All patients but two (95%) had confluent pulmonary arteries supplied by a patent ductus arteriosus. These two patients (5%) had nonconfluent pulmonary arteries, one with major systemic-pulmonary collateral arteries and absent patent ductus arteriosus and the other with a patent ductus arteriosus supplying the right lung and small collateral arteries supplying the left. The low incidence of pulmonary arterial abnormalities and the uniform pattern of pulmonary blood supply make the patients with pulmonary atresia and complex congenital heart disease potential candidates for palliative operation without cardiac catheterization in the neonatal period.

Two patients with Multiple Lentiginosis (ML) had hypertrophic cardiomyopathy with severe obstruction of the right and left ventricular outflow tracts. ML is a rare syndrome in which there is a typical skin pigmentation disorder together with various other abnormalities like slight mental retardation, sensorineural deafness, genital and skeletal anomalies and sometimes a characteristic facies. Obstruction of the right ventricular outflow tract is frequent in such patients. The association of ML and obstructive hypertrophic cardiomyopathy has been recently reported. A critical examination of the literature in addition to our clinical observations suggests that the biventricular obstructive hypertrophic cardiomyopathy may play a major role in the clinical feature of ML syndrome.

We describe the use of percutaneous balloon angioplasty of a stenotic truncal valve in a newborn with a common arterial trunk. A 12 mm balloon catheter was chosen, this representing 80% of the diameter of the ventriculo-truncal junction. The peak systolic gradient dropped from 45 to 15 mm Hg. The procedure can avoid, or certainly delay, surgical treatment of the stenotic truncal valve.

The authors describe a case of aneurysm of the non-coronary sinus of Valsalva and aortic insufficiency caused by Salmonella Tiphi in a 7 year old child. The exceptional ethiology is discussed as well as the problems connected with surgical correction.

The acute and chronic iv toxicity of 4'-epidoxorubicin, a new antitumor anthracycline antibiotic, was compared with doxorubicin. The LD50 of 4'-epidoxorubicin was 16.07 mg/kg in mice, 14.27 mg/kg in rats, and about 2 mg/kg in dogs; the LD50 of doxorubicin was 11.98 mg/kg in mice, 10.51 mg/kg in rats, and about 2.5 mg/kg in dogs. Rats and dogs were also dosed iv for 91 days (3 injections/week) with 4'-epidoxorubicin or doxorubicin at doses of 0.128, 0.32, and 0.8 mg/kg to rats and 0.064, 0.16, and 0.4 mg/kg to dogs. A comprehensive toxicological evaluation of the animals was carried out before, throughout, and at the end of the study. High-dose 4'-epidoxorubicin induced toxic clinical signs in dogs, and in both species caused loss of body weight, antiproliferative effects on blood-forming organs and testes, and degenerative lesions in kidneys and heart. The cardiac damage was moderate in rats and very mild in dogs; only three male rats died at this dose. The medium dose induced less pronounced changes and no heart lesions and the low dose was practically nontoxic. Doxorubicin showed similar antiproliferative activity, but more evident toxic effects, especially on the heart; many rats given the high dose died and some at the medium dose showed initial cardiac lesions. Thus 4'-epidoxorubicin appeared less toxic than doxorubicin; in particular cardiac damage was much less evident in animals chronically injected with the new drug.

Seventy-nine specimens with transposition of the great arteries were reviewed. Among them were 45 with ventricular septal defect, including 6 with so-called "posterior aorta transposition". The morphology of the mitral valve was carefully studied. Mitral valve anomalies were found in 24 cases (30%). The anomalies involving the mitral valve were classified into 4 groups according to the different components of the valve: A) specimens with anomalies of the leaflets--2 cases with mitral cleft; B) those with anomalies of the commissures--1 case with a mitral gap; C) those with abnormalities of the chordae tendinae--2 cases of malattachment of the chordae tendinae to the ventricular septum and 2 with bridging chordae between the papillary muscles; and D) those with abnormalities of the papillary muscles--5 cases with a parachute valve, 10 with a "form fruste" of the parachute mitral valve (hypoplasia of one papillary muscle and short chordae tendinae) and 2 with abnormally placed papillary muscles, which reached the pulmonary annulus. In 14 cases, abnormalities of the tricuspid valve were also encountered, including 8 cases with a tricuspid gap and 6 with a malattachment of tricuspid chordae on the border of a ventricular septal defect. It can be concluded that mitral valve anomalies are not uncommon in transposition of the great arteries. This pathology must be carefully evaluated at the time of surgery, particularly in those patients for whom an anatomic correction is considered, for in those cases the left ventricle will be supplying the systemic circulation and therefore, a completely normal mitral valve will be required.(ABSTRACT TRUNCATED AT 250 WORDS)

Bronchial compression by a dilated left pulmonary artery complicated the postoperative course in two infants: one after a Mustard operation for transposition of the great arteries, restrictive ventricular septal defect, and moderate pulmonary stenosis, the second after correction of tetralogy of Fallot with absent pulmonary valve. In both cases the bronchial compression was successfully treated by division of the left pulmonary artery and its prolongation by interposition of a conduit.

From March 1982 to December 1983, five patients with a mean age 7 years (4 months-16 years) underwent a palliative Mustard operation for complex cardiac anomalies. The diagnoses were: univentricular heart with pulmonary vascular obstructive disease (PVOD) (2 cases); criss-cross heart with intact atrial septum, ventricular septal defect (VSD), transposition of the great arteries (TGA) and moderate pulmonary stenosis; isolated atrioventricular discordance with VSD; TGA, diminutive right ventricle and multiple VSDs. Cardiac catheterization showed unfavourable direction of flow: the oxygen saturation in the aorta was lower than in pulmonary artery, with a mean difference of 13.5% (range 6% to 30%). In no case was anatomical or physiological repair considered advisable because of the unfavourable intracardiac anatomy or the presence of PVOD. In two patients the atrial rerouting was accompanied by pulmonary artery banding. There were no hospital or late deaths in a mean follow-up of 36 months (range 29 to 50 months). The mean post-operative arterial oxygen saturation was 95.4% (range 92% to 99%).

Rabbits were injected with adriamycin 3 days/week/7 weeks. Heart tissues of treated rabbits showed myocardial degeneration, but the tissues of controls were normal. The deficiency in the activity of the succinate dehydrogenase-coenzyme Q10 reductase in the heart tissues of the rabbits treated with adriamycin was higher (0.001 less than P less than 0.01) than that for control tissues. These data indicate a correlation of at least a part of the cardiotoxicity of adriamycin in cancer patients to inhibition of CoQ10-enzymes, since coenzyme Q10 is indispensable to bioenergetics in the myocardium of both humans and rabbit.