Conservative management of a left ventricle cardiac fibroma in an asymptomatic child patient.

Primary cardiac tumors in infancy and childhood are rare and usually benign. Fibroma is the second most common bening cardiac tumor after rhabdomyoma. Surgery is required when fibromas cause ventricular outflow tract obstruction, ventricular dysfunction and life-threatening arrhythmia. This case report describes a 9-year-old asymptomatic male presenting with a giant left ventricular cardiac fibroma who was followed up using conservative treatment.

Discrete supravalvular aortic stenosis in children: Is it necessary to reconstruct the whole aortic root?

OBJECTIVE: Discrete supravalvular aortic stenosis (SAS) is known to involve the whole aortic root. Some surgeons have therefore changed their approach from relief of obstruction using a single-patch to symmetric reconstruction of the whole aortic root - three-patch technique. The advantages are said to be preserved long-term aortic valve function and allowance for growth. This is unproven. We compare growth and aortic root geometry in patients who have undergone relief of discrete SAS using either single-or three-patch technique. METHODS: Twenty-five patients (14 male, 11 female, mean age of 11+/-4 years, range 4-18) underwent surgery for discrete SAS. No patients with diffuse SAS were included in this retrospective analysis. Twelve patients had features of Williams syndrome. Five patients had other concomitant procedures. A single-patch was inserted into the longitudinal incision, which passed across the stenosis into the non-coronary sinus in 14. A three-patch technique was used in 11 patients. Changes in aortic root following repair were documented in patients using both echocardiography and magnetic resonance imaging (MRI). RESULTS: There were no operative deaths. The mean preoperative gradient was 66+/-17 mmHg (range 50-100 mmHg), which decreased to 14+/-7 mmHg (range 4-18 mmHg) early postoperatively. The late mean gradient was 15+/-5 mmHg. There was no significant difference in the incidence of postoperative aortic regurgitation or gradient across the repair between two techniques according to the echocardiograms and MRI findings. CONCLUSION: According to our study, we cannot demonstrate any benefit in reconstructing the whole aortic root for discrete SAS. A single-patch technique is easy, safe and appears durable.

Clinical investigations over 13 years to establish the nature of the cardiac defects in patients having abnormalities of lateralization.

INTRODUCTION: The first step in diagnosing congenital cardiac malformations is to assess the arrangement of the atrial appendages. In patients with abnormal lateralization of the organs of the body, the arrangement of atrial appendages is neither normal, nor a mirror image of normal. There are 2 categories of abnormal arrangement based on the morphology of the atrial appendices, namely right isomerism and left isomerism, and in almost all instances these are found in the setting of so-called heterotaxy syndromes. OBJECTIVE: To evaluate the various congenital cardiac malformations those are associated with abnormalities of lateralization, and to discuss the diagnostic tools, therapeutic options, and outcome for these patients. PATIENTS AND METHODS: We studied 134 patients, who had been admitted to our department of paediatric cardiology with known abnormalities of lateralization and congenital cardiac defects between 1990 and 2003. The data relating to each patient was evaluated retrospectively. The arrangement of the atrial appendages was established echocardiographically, and/or angiographically, and/or on the basis of morphologic investigations during the operation. These studies showed that 43 (32.1%) of the patients had right isomerism, and 88 (65.7%) had left isomerism. In 2 (1.5%), there was mirror-imaged arrangement, while in the final patient (0.07%), we were unable to determine sidedness with certainty. The median age at diagnosis was 0.66 years, and the females outnumbered the males in a ratio of 3 to 1. Patients in the 2 isomeric groups were compared with regard to age, cardiac defects, diagnostic tools and outcomes. RESULTS: The difference in mean ages of the two groups of patients was statistically significant, those with right isomerism being 1.0 minus or plus 1.5 years, as opposed to those with left isomerism being 3.3 minus or plus 4.7 years (P is less than 0.005). Of the patients with right isomerism, 32 (74.4%) had left-sided, and 11 (25.6%) right-sided hearts, whereas in those with left isomerism, the hearts were left-sided in 65 (73.9%), and right-sided in 21 (23.9%), with 2 (2.2%) positioned in the midline. Extracardiac totally anomalous pulmonary venous connection was more common in those with right isomerism, being found in 13 patients (30.2%) as opposed to 5 patients (5.7%) with left isomerism. In only 8 of those with right isomerism did we find two perforate atrioventricular valves (18.6%), this arrangement being found in 34 (38.6%) of those with left isomerism. Pulmonary atresia and stenosis were present in 40 (93.0%) of those with right isomerism, but also in 41 (46.6%) of the patients with left isomerism. Angiographic and echocardiographic investigations were concordant in about three-quarters of patients with both right and left isomerism. All patients with extracardiac totally anomalous pulmonary venous connection died. Overall, 22 of the patients with right isomerism died (51.2%), as opposed to 20 (22.7%) of those with left isomerism. CONCLUSION: Our experience confirms that patients with right isomerism have more complex cardiac defects than those with left isomerism. Overall, the presence of isomerism carries a poor prognosis, the more so for right isomerism, with this related to the complex cardiac abnormalities. In our cohort, extracardiac totally anomalous pulmonary venous connection with pulmonary arterial obstruction was always a fatal combination. The mapping of cardiac and abdominal morphologies is still essential for proper diagnosis of these syndromes, especially in fetal life.

Sequential balloon dilatation for combined aortic valvular stenosis and coarctation of the aorta in a single catheterization procedure: a prognostic evaluation based on long-term follow up.

BACKGROUND: Combined aortic valvular stenosis (AVS) and coarctation of the aorta (CoA) is uncommon. There are only a few case reports that discuss the treatment choices and prognosis. We present the immediate and long-term results for a group of children with combined AVS and CoA who underwent sequential percutaneous balloon dilatation in a single catheterization procedure. PATIENTS AND METHODS: The cases of 13 children with combined AVS and CoA who underwent balloon dilatation in a single catheterization session between August 1995 and May 2002 were retrospectively evaluated. The group was comprised of 9 boys and 4 girls of mean age 14.9 +/- 24.2 months (range = 19 days to 7 years). RESULTS: The pressure gradients at the valvular level before and after the intervention were 51.5 +/- 22.3 mmHg (range = 8 to 85 mmHg) and 22.4 +/- 18.3 mmHg (range = 2 to 57 mmHg), respectively (p < 0.001). The corresponding findings for the coarctation segment were 22.3 +/- 13.5 mmHg (range = 0 to 45 mmHg), and 5.2 +/- 7.0 mmHg (range = 0 to 24 mmHg; p < 0.001). After the intervention, mild aortic regurgitation occurred in 5 children (38.5%). Mild aortic regurgitation became moderate in 1 patient (7.7%). Three patients developed peripheral arterial occlusion treated with heparin and streptokinase after intervention. There were no deaths during or early after the procedures. Four patients (30.8%) died, all in the first 6 months after the intervention, and the mean follow-up time for the 9 survivors was 57.6 +/- 38.9 months (range = 6 to 107 months). Recurrence of stenosis and coarctation occurred in 2 (15.4%) and 4 (30.8%) cases, respectively. Four patients (30.8%) underwent cardiac surgery. The event-free survival rates were 76.9% at 6 months, 61.5% at 12 months through 24 months, and 30.8% at 60 months. The overall survival rates were 76.9% at 3 months, 69.2% at 6 months, and it remained 69.2% for the rest of the follow-up period. NOTE: Outcomes for 13 patients with combined AVS and CoA who underwent single-session sequential balloon dilatation are described. The results were favorable; there were no severe complications related to the procedures, and no deaths occurred during or in the early period after the intervention.