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Both immediate and long-term prognosis of Kawasaki's disease (K. D.) are due to cardiac involvement and, particularly, to coronary artery aneurysms formation. Of 19 cases that we studied, age ranging between 7 months and 8 years, 18 has been followed clinically and with echocardiographic procedure. In 1 case (10 years old), which underwent a triple bypass surgical operation due to the presence of multiple aneurysms, diagnosis was made retrospectively. In 2 of 19 cases (10.52%) coronary artery aneurysms were present. One case showed aneurysm's partial regression two years later. In 10 of 18 cases (55.5%) clinical evidence of cardiac compromise was present, whereas in no patient ECG alterations occurred at all. Our data prove the poor benefit of clinical approach and ECG interpretation in K. D., whereas we believe that echocardiographic study is the best method in early identification of aneurysms.

An apparently unique case of double right tracheal bronchus supplying the whole right upper lobe is described in a 12-month-old infant presenting with a right paratracheal opacity, persisting cough, and ventricular septal defect. The two tracheal bronchi, initially discovered on tomography, were confirmed by tracheobronchography, which demonstrated also the absence of other upper lobe branches. At surgery, the upper lobe was atelectatic, and its blood supply was abnormal.

Thrombosis in the right atrium or ventricle is a rare compli cation of permanent endocardial pacing in adults. To the best of our knowledge, this complication has not been previously reported at all in the pediatric age group. We report on a case of a 7-year-oId boy who had large left ventricular thrombi that occurred during permanent endocardial electrical stimulation. Subsequent pulmonary emboli complicated congestive heart failure in this patient. As a diagnostic approach, echocardiography and pulmonary perfusion scintigraphy were used. We comment on possible causes of this serious complication and suggest hemorrheological and platelet activation studies in patients with permanent endocardial pacing.

Four cases of "absent pulmonary valve" (APV) are described. This congenital heart disease consists of aplasia or extreme hypoplasia of pulmonary semilunar cusps and is always combined with aneurysmatic dilatation of pulmonary artery. In two of them the diagnosis was confirmed at autopsy. The remaining two are clinical reports surgically confirmed. One of them was studied by single cristal and two-dimensional echocardiography. In three cases APV was associated with tetralogy of Fallot, while in one case there was an intact ventricular septum. Review of literature allowed us to select 149 cases of APV anatomically confirmed, besides our ownes. It is stressed on that APV is usually associated with dextroposition of the aorta and ventricular septal defect by conoventricular malallignment. It is suggested that pathogenesis of this malformation is a consequence of an anomalous development of mesenchimal tissue of pulmonary cusps rather than an asymmetrical truncal sepimentation. We favour the hypothesis that aneurysmatic dilatation of pulmonary artery is caused by altered hemodynamics acting both in foetal and extrauterine life, even if differently expressed. Pathophysiologic and diagnostic value of cyanosis, dyspnea, and systo-diastolic murmur are discussed. Some outlines of the most important diagnostic procedures are reviewed and particularly echocardiography, which shows aortic overriding and dilatation of right ventricular outflow tract and pulmonary artery separated by a restricted pulmonary annulus. Prognosis and therapy are also mentioned.

Thirty-one hearts with aortic arch obstruction and patent ductus arteriosus were examined with special reference to associated cardiac anomalies. Six presented with complete interruption of the aortic arch, four with atretic isthmus, twelve with coarctation, and three with tubular hypoplasia. Associated cardiac anomalies were divided into two main groups: (1) septal defect with left-to-right shunt, and (2) left ventricular inflow and/or outflow obstruction. A high incidence (9/19=47.4%) of ventriculo-infundibular malalignment type of ventricular septal defect with subaortic stenosis was observed. Associated cardiac lesions that reduce blood flow in the aortic arch during fetal life may be responsible for poor development of this structure.

We have evaluated 46 patients with Turner syndrome by clinical examination, M-mode and two-dimensional echocardiography, dynamic exercise testing and 24 h Holter monitoring. Twelve patients (26.1%) had mitral valve prolapse and 7 patients (15.2%) had isolated non stenotic bicuspid aortic valve. Aortic root dilation was present in 2 patients (4.3%). Our data indicate that incidence of mitral valve prolapse is significantly higher in Turner syndrome than in the general population (P less than 0.025).

A child with microcephaly, facial dysmorphisms, seizures, and congenital car diopathy is presented. On the basis of skull x-rays, electroencephalogram, trans- illumination of the head, and computed tomography (CT) scan, the diagnosis of semilobar holoprosencephaly was made. The heterogeneous etiology of the disorder is discussed in order to evaluate the recurrence risk. The usefulness of CT scan for the classification in the different types of holoprosencephaly is stressed.

We tested some cardiovascular reflexes (Valsalva ratio, deep-breathing, postural hypotension) in 53 subjects suffering from diabetes type I in childhood with an average disease length of 13 +/- 6 years, in order to estimate possible damage to the autonomic nervous system without clinical symptomatology. A rather high percentage of signs of neurovegetative involvement combined with the progressive increase of further complications during the course of the disease, suggests early neurologic damage. We confirm the sensitivity of adopted tests and deep-breathing in particular.

The AA. describe the clinical, surgical and pathological features of a 13-days old infant with multiple cardiac rhabdomyomas diagnosed by two-dimensional echocardiography (2DE). The clinical picture simulated hypoplastic left heart syndrome (HLHS). 2DE showed a well developed left heart. The apical four chamber view disclosed a mass of echoes between the left atrium and ventricle, across the mitral valve. Another little mass of echoes was attached to the left surface of the ventricular septum. The other cardiac chambers and the great arteries were normal. These multiple intracardiac masses were interpreted as rhabdomyomas. The infant underwent an operation at the age of 21 days because of mitral valve obstruction and CHF. At surgery, a left atrial rhabdomyoma was found, that severely obstructed the mitral valve orifice. Although after resecting the tumour we felt that the obstruction was adequately relieved, the little patient died of pulmonary oedema one hour after surgery. At autopsy all the previously detected myomas were confirmed and a small additional one was discovered within the crista supraventricularis. Unexpectedly, the chordae tendineae of the mitral valve were extremely short. This anomaly, masked preoperatively by the rhabdomyoma, was probably the cause of the fatal postoperative mitral regurgitation.

Echocardiographic studies were performed in 15 patients, aged 8 months to 20 years, with congenital valvular aortic stenosis confirmed by hemodynamic findings. Seven patients showed an anomalous pattern of the valvular echo in diastole (multiple valve echoes), seven exhibited an eccentric closure, and only in two patients the area was significantly reduced in systole. Therefore aortic valve diastolic pattern was more important than systolic appearance in the echocardiografic diagnosis of congenital valvular aortic stenosis. In all cases a correlation was found between the left ventricular pressure obtained with this non invasive method and the pressure measured in the left ventricle at the cardiac catheterization (p less than 0.001).

Twenty-five patients with tricuspid atresia, ranging in age between 2 months and 23 years, previously morphologically studied, were assessed by M-mode echocardiography with regard to left ventricular function. Fifteen of them underwent palliative cardiac surgery (systemic-pulmonary anastomosis). Thirty normal subjects, same age and sex, were selected as controls. Patients with tricuspid atresia had a significant increase of left ventricular dimensions and mass and significant decrease of shortening fraction and ejection fraction. The left ventricular pre-ejection/ejection ratio (LPEP/LVET) was significantly higher in patients with tricuspid atresia compared to normals. In patients without surgical shunt there was no significant correlation between Qp/Qs determined by oximetry and the end-diastolic dimension and left ventricular function parameters. In patients with surgical shunt a significant correlation was found between ejection fraction, shortening fraction, systolic time intervals and Qp/Qs. Qp/Qs significantly correlated with end-diastolic dimension. Shortening fraction and ejection fraction were higher in patients with satisfactory systemic saturation, while left ventricular mass was increased in patients with longstanding surgical shunt. These data suggest that patients with tricuspid atresia, with or without surgical shunt, may present with a decreased left ventricular function, early detectable by echocardiography.

Our report concerns 18 cases of mitral valve prolapse, all documented by M-mode and D2-mode echocardiographic study. Of these patients three presented severe cardiac arrhythmias and therefore therapeutic treatment was necessary. One of them presented repeated episodes of paroxysmal supraventricular tachycardia and premature supraventricular and ventricular contractions. In another the arrhythmia consisted of numerous ventricular premature contractions. The third presented a sinus tachycardia which necessitated pharmacological treatment. In this study we have examined several forms of arrhythmias associated with mitral valve prolapse and discussed the antiarrhythmic therapy with quinidine, verapamil, amiodarone and propranolol. Since most people with mitral valve prolapse are young, arrhythmia suppression therapy might subject them to a course of treatment for possibly several decades. Therefore, the physician must weigh the risk of antiarrhythmic therapy against the risk of morbidity without therapy in each individual patient.

This paper describes 2 cases of tricuspid valvular dysplasia (TVD) associated with aortic stenosis and mitral incompetence in newborns. Mitral regurgitation was due to dysplasia, which resembles its tricuspid counterpart and should be termed 'mitral valve dysplasia'. This association of tricuspid and mitral valve dysplasia has been reported only once before. The concomitance of mitral and tricuspid incompetence is noteworthy, since mitral regurgitation can produce left heart failure and mask tricuspid valve disease.