[Primary cardiac tumours in infancy]. / Tumores cardíacos primarios en la infancia.

INTRODUCTION: Primary cardiac tumours are very rare in the paediatric age, their incidence varies from 0.0017 % to 0.28 %. More than 90 % are benign in nature. The most common variety is the rhabdomyoma, present in over 60 % of cases with tuberous sclerosis. MATERIAL AND METHODS: We performed a retrospective analysis of medical records with a diagnosis of primary cardiac tumor between March 1977 and March 2007, finding a total of 27 patients. RESULTS: The age of initial diagnosis is more prevalent in the neonatal period, beginning with the discovery of a heart murmur (11 cases). There was no difference in gender distribution. In 14 patients were found cardiomegaly on chest radiograph. According to the echocardiography characteristics there were diagnosed 20 rhabdomyomas, 2 fibromas, 2 pericardial teratomas and 3 non classifiable tumours. Most were located in the left ventricle. Echocardiography, cardiac catheterization was also performed in 3 cases and angioresonance in 5 cases. During their evolution, episodes of arrhythmias were observed in 11 patients, 5 patients required some sort of surgical procedure, which confirmed the histopathology diagnosis. In 3 patients the initial cause of death was cardiological. The 75 % of cases with rhabdomyomas presented or developed tuberous sclerosis. In most of the rhabdomyomas (13 cases), there was a spontaneous regression. CONCLUSIONS: Firstly, there is shown to be a low prevalence of this disorder in children. Rhabdomyoma is the most common primary cardiac tumour in our study and it was associated in 75 % of cases with tuberous sclerosis. The diagnosis is more common in the early neonatal period after auscultation of a cardiac murmur and echocardiography, the diagnostic technique of choice, other imaging techniques, such as angioMRI not being of much for diagnosis in children. The emergence of foetal echocardiography allows early detection. The course is benign in most tumours, rhabdomyomas tending to regress spontaneously. It must be monitored as the occurrence of arrhythmias during its evolution will require medical treatment. Surgery is needed in cases with severe symptoms, due to obstruction in the ventricular output tracts. One option is the surgical cardiac transplant in non-resectable life-threatening tumours.

[Anomalous origin of the right pulmonary artery from the ascending aorta. Its surgical correction in early infancy]. / Origen anómalo de la arteria pulmonar derecha de la aorta ascendente. Corrección quirúrgica en la primera infancia.

Anomalous origin of the right pulmonary artery from the ascending aorta is a rare congenital malformation associated with a high mortality during the first year of life. Up to 1990, 75 cases have been reported in the literature. We are reporting 2 cases diagnosed and operated before 3 months of age. Repair was facilitated by the use of deep hypothermic circulatory arrest. The duration of follow up is 5 and 6 years, respectively. Pre and postoperative cardiac catheterization pressure data and angiograms are shown. We review the literature, presenting the physiopathological, embryological and surgical features of this rare congenital anomaly.

Hypertrophic cardiomyopathy in preterm infants treated with dexamethasone.

UNLABELLED: Steroid therapy has been widely used in neonates for its beneficial effects. Adverse side-effects have been described, also affecting the cardiovascular system. We report two cases of hypertrophic cardiomyopathy in two preterm newborns secondary to dexamethasone treatment. Full recovery occurred after discontinuing steroids. Risk/benefit ratios must be carefully considered before using steroids in the neonatal period. CONCLUSION: Serial echocardiographic evaluations should be performed to evaluate prevalence and clinical relevance of hypertrophic cardiomyopathy in preterm infants treated with dexamethasone.