[Amiodarone therapy in childhood: efficacy and side effects]. / La terapia con amiodarone in età pediatrica: efficacia ed effetti collaterali.

In the adult, Amiodarone is a very effective drug in the treatment of ventricular and supraventricular arrhythmias. The presence of severe side effects such as some alterations in the thyroid function and/or pulmonary fibrosis have restricted the use in children. Nevertheless, research has shown that there is a low incidence of collateral side effects and this therapy in infancy can be very effective. For this reason we evaluated a group of 27 children with supraventricular (19 patients) and ventricular (8 patients) arrhythmias. The mean age of patients treated was 6 +/- 5 years (2 days-13 years). The follow-up period was of 13 +/- 10 months. Amiodarone has been used in 9 patients intravenously, with the loading dose of 5 mg/Kg followed by an infusion of 10 mg/Kg/day. In 18 patients we administered the drug orally with a loading dose of 10 mg/Kg/day for a period of 10 days, thereafter the maintenance was of 5-7 mg/Kg/day for 5 days every week. The patients were all checked for thyroid function and Holter monitoring quarterly; they were given an ophthalmologic examination (every 6 months) and a chest-x-ray and echocardiography annually. The efficacy of intravenous treatment was judged successful in 56% of patients, partially successful in 22% and ineffective in the remaining 22%. The oral treatment was completely effective in 77% of children, partially in 5% and ineffective in 18%. In one case we had to suspend the therapy because we found high values of T3 and T4. During the treatment, in 86% of cases, we had blood level fluctuations of T3 and T4, however these did not exceed the normal ranges. The most important side effect observed has been the photosensitivity found in 22% of children. Moreover we observed a reduction of sinusal automatism, which was more marked in patients less than year old. In 4 cases an A/V block of first degree appeared. In all patients we found changes of ventricular repolarization, while corneal deposits appeared in only one child after a year of therapy and did not cause an impairment of visual acuity. In conclusion we can assert that Amiodarone is a very effective drug in children, specially in small babies, where it can safety be used as a first choice drug.

Pulmonary valve agenesis and supravalvular pulmonary stenosis. Report of a case and review of literature.

Pulmonary valve agenesis is a rare malformation, and is usually associated with a ventricular septal defect. Occasionally it may be associated also with right ventricular outflow obstruction. Children with this syndrome usually die early in infancy either of intractable heart failure or of severe respiratory distress. Rare instances of association with peripheral pulmonary stenoses have also been reported. We report the case of a three-year-old girl, asymptomatic except for failure to thrive, with pulmonary valve agenesis and coarctation of the pulmonary artery trunk.

[Exercise test in 100 healthy children between 5 and 12 years of age (author's transl)]. / Valutazione ergometrica di 100 bambini normali tra i 5 e i 12 anni.

100 healthy children, between 5 and 12 years of age underwent maximal exercise test on a bicycle ergometer in sitting position. Particularly interesting seem to be the observations on maximal heart rate during exercise, maximum work load, energy cost in terms of heart rate, EGG during exercise. As concerning the maximal heart rate, it was observed a levelling of around 195 beats min. for all the subjects. A low increase of systolic blood pressure was observed during strenuous exercise. The maximum tolerated work load increases accordingly with the increase of body surface area and age; taking into account the maximum value of heart rate during exercise (195 for all subjects), it arises a different energetic cost in terms of beats per min. in favour of 12 years subjects compared to youngers. None rhythm or conduction disturbances was observed during exercise, neither variations of the ventricular repolarization appeared during exercise.

[beta-Blocking therapy in obstructive hypertrophic cardiomyopathy. Long term results (author's transl)]. / Risultati a distanza della terapia betabloccante nella cardiomiopatia ipertrofica ostruttiva.

34 patients have been controlled after beta-blocking therapy, for a mean period of 5 years. Symptoms and evolution: syncope disappeared, angoy passed from 47% to 23%, dyspnea from 65% to 47%, dizziness from 70% to 54%, weakness from 30% to 37%. A systolic murmur was present in 75% of the cases. Two patients died by heart failure. Phonocardiogram: the systolic murmur was unchanged, like the carotid pulse. Paradoxical splitting of the 2 degrees sound was more frequent, atrial sound unimodified, isometric contraction shortened (60%) and the Q-1 degree sound interval prolonged (90%). Electrocardiogram: 1 degree A/V block appeared in 24% of the cases, complete A/V block in 9%, atrial fibrillation in 3%. Left atrial enlargement was more frequent; left ventricular hypertrophy unchanged. Heart catheterization (10 cases, after a mean period of 5.5 years): left ventricular pressure gradient passed from 80% to 90%; a low cardiac index from 20% to 30%; telediastolic pressure of left ventricle was unmmodified in 10% of cases, more elevated in 50%, less elevated in 40%. Chest X ray: cardiac size was unchanged in 65% of cases, enlarged in 32%; smaller in 3%. In conclusion, symptoms improved in most of the patients; no case of sudden death was observed. Some data however show that the evolution of the myocardiopathy goes on to congestive heart failure and arise doubts on the real usefullness of beta-blocking drugs in the disease.

[Echo-angiocardiographic correlations in congenital abnormalities of the mitral valve (author's transl)]. / Correlazioni eco-angiocardiografiche nelle anomalie congenite della mitrale.

Echocardiographic findings satisfactorily compared with angiocardiographic data in six infants affected by congenital abnormalities of the mitral valve. Peculiar echocardiographic features were as follows: a) mitral stenosis: reduced E-F slope of the anterior mitral leaflet, paradoxical diastolic movement of the posterior leaflet, enlargement of the left atrium; b) "parachute mitral valve": small, dysmorphic, stiff mitral valve, with an extra echo projecting between those of the two leaflets; c) "cor triatriatum": enlarged left atrium whose cavity is divided in two portions by an extra echo; d) "hypoplastic left heart syndrome": the mitral leaflets are thin, short. The ventricular cavity and the diameter of the aorta are small; the right ventricle and the pulmonary artery are on the contrary markedly dilated.

[The incomplete atrio-ventricular canal. Operative and long term results in 100 cases (author's transl)]. / Il canale atrio-ventricolare incompleto. Risultati immediati e a distanza di 100 casi operati.

100 patients with endocardial cushion defect of the partial type underwent surgical correction between the years 1957-1975. The age of patients ranged from 3 to 57 years with a mean of 18 years. In 89 patients there was a mitral insufficiency. The repair of the mitral valve was performed in 78 patients; in 21 was repaired also the tricuspidal valve. The hospital mortality was of 8 patients. The mortality was correlated with the age of the patients, the pulmonary pressure and with the presence of malformations of the tricuspid valve. A major complication was represented by a complete atrioventricular block that occurred in 8 patients, in 5 of these the block disappeared before the dimission from the hospital. 86 patients have been followed for a period variable from 2 to 228 months and an average of 61 months. The late mortality was of 4 patients. Two patients have been reoperated of mitral valve replacement because of residual important mitral insufficiency. The phonocardiographic study, done in 50 patients, showed a presence of a holosystolic murmur in 20. The standard X-ray chest and electrocardiogram iid not show important changes between the pre and post operative examination.

[Cardiac fibroma: a successfully operated case and a review of the literature (author's transl)]. / I fibromi del cuore. Un caso operato con successo e revisione della letteratura

A case of cardiac fibroma in a three years old child successfully operated on is presented. The tumor was localized on the interventricular septum and was provoking an obstacle in the outflow of the right ventricle. The preoperative diagnosis was formulated by angiocardiography. A year after the operation, the hemodynamic control showed that he interventricular pressures were normal. The problems and the importance of an early diagnosis are emphasized in patients affected by cardiac fibroma. Surgical indication, technical aspects related to the operation, the immediate and more long-term results from surgical operation of this kind of pathology, with reference to the data taken from the literature, are discussed.

[Surgical treatment of cardiac abnormalities associated with corrected transposition of the great arteries (author's transl)]. / Il trattamento chirurgico delle anomalie cardiache associate alla trasposizione corretta dei grossi vasi

Twelve patients with corrected transposition of the great arteries, who underwent surgical repair, are reported. In four cases a ventricular septal defect was present; in six cases, a ventricular septal defect and pulmonary stenosis and in two an incompetence of the systemic atrio-ventricular valve. The anatomic types of corrected transposition and the pertinent diagnostic characteristics were reviewed and discussed. The problems related to the surgical correction and the different approaches to the intracardiac lesions are considered and the difficulties of treating the obstruction of the venous ventricle successfully are emphasized.