Percutaneous occlusion of patent foramen ovale in patients with paradoxical embolism.

INTRODUCTION: A patent foramen ovale can be found in about one quarter of adults and in a small percentage it is a wide opening and may be associated with aneurysmal formation. The association between a wide patent foramen ovale and paradoxical embolism is well established. In such cases percutaneous closure is indicated, as an alternative to life-long anticoagulant therapy or surgery. Percutaneous closure is an attractive technique and is more advantageous than other methods. METHODOLOGY: We describe the first cases of percutaneous occlusion of patent foramen ovale performed in Portugal, using the Amplatzer PFO occluder, in three female patients with documented cerebrovascular accidents due to paradoxical embolism. We also analyze the rationale for using this technique in such patients and its preliminary results. RESULTS: All three patients submitted to percutaneous occlusion of patent foramen ovale had a similar history of ischemic cerebrovascular accident. Transesophageal echocardiography showed a wide-open foramen ovale ranging from 9 to 12 mm, with spontaneous right-to-left shunt in all patients, and one of them also had an aneurysmal formation. Total procedure time ranged from 30 to 55 minutes and fluoroscopic time from 9 to 12 minutes. There were no complications and during the short follow up all patients are asymptomatic and free of recurrent events. CONCLUSIONS: Percutaneous closure of patent foramen ovale is a safe and promising technique in the prevention of recurrent systemic thromboembolism in appropriately selected patients. Prospective studies comparing antithrombotic therapy or surgery with percutaneous closure should clarify its efficacy and therapeutic value.

Spontaneous closure of congestional coronary fistula.

Coronary fistula is a rare congenital anomaly. In the pediatric population they are usually asymptomatic and this entity is suspected from the presence of a continuous murmur. They usually have a favorable course, although rarely they can complicate or occlude spontaneously. Early treatment is generally advised. The authors describe a case of spontaneous closure of congenital right coronary fistula draining into the right ventricle in a twelve-year-old boy and they comment on the therapeutic management of this anomaly.

Late cardiac tamponade after transcatheter closure of atrial septal defect with Cardioseal device.

Cardiac tamponade occurring late after interventional closure of defects within the oval fossa is a very rare but life-threatening complication. We describe such an occurrence after use of a Cardioseal device to close an interatrial communication. Two arms of the device had perforated left atrial wall. The device was removed at surgery, and the defect closed uneventfully. All available means should be used to identify this complication.

Transcatheter occlusion of a residual left superior vena cava causing right-to-left shunt in a Fontan patient with a new occlusion device.

We describe the management of a residual left superior vena cava connected to the coronary sinus and causing right-to-left shunt at atrial level in an 8 year-old-child, with modified Fontan operation (total cava pulmonary connection) by transcatheter closure with a new duct occluder device.

Idiopathic dilated cardiomyopathy in children: clinical profile and prognostic determinants.

Idiopathic dilated cardiomyopathy is a severe disease with a high mortality rate in childhood. Its clinical evolution and prognosis are important for the selection of cardiac transplantation candidates. In order to characterize its evolution and identify prognostic factors, the clinical records of 41 children with the diagnosis of idiopathic dilated cardiomyopathy, admitted from January 1985 to December 1997, were reviewed. Survivors (Group I) and deceased (Group II) were separately analyzed, according to the following parameters: age, sex, race, clinical severity, electrocardiographic, echocardiographic and haemodynamic findings. Seven children were excluded from the study: six of them were lost to follow-up and one died from a surgical complication. Of the remaining 34 children, 20 were male (M) and 14 were female (F) (M/F: 1.4). Age range at diagnosis was 7 days to 14 years (median: 1.5 years), and follow-up time was from 18 days to 10.5 years (median: 2.5 years). Eleven (32.3%) children fully recovered, 13 (38.2%) survived with left ventricular dysfunction, and ten (29.4%) died, half of them within the first three months of follow-up. Mortality was 23.5% (8 out of 34 children) during the first year of follow-up and 29.4% (ten out of 34 children) at five years. Unfavorable prognosis was more frequently associated to: 1) clinical severity at the time of presentation; 2) lower mean left ventricular shortening fraction (10 +/- 7% in group II and 13 +/- 5% in group I); 3) occurrence of severe arrhythmia (40% in group II and 3.5% in group I). In this series a group of higher mortality risk was identified, based on some of the analyzed parameters, which should be considered as selection criteria for early heart transplantation.

[The sex distribution of congenital cardiopathies]. / Distribuição por sexos das cardiopatias congénitas.

For 17 years, 4150 infants and children under 13 years of age with final diagnosis of well defined congenital heart disease were studied and the pattern of sex differences are reported. In general, the sex distribution was equal: 2108 males (50.8%) and 2042 females (49.2%) with a male to female ratio of 1.03. Male sex predominance was marked for the following specific lesions: aortic stenosis (valvar and subvalvular) (70%), coarctation of the aorta (66%), transposition of the great arteries (60%), univentricular heart (76%), tricuspid atresia (63%), Ebstein anomaly (76%), hypoplastic left heart syndrome (85%), vascular rings (77%) and right midventricular stenosis (70%). Female sex predominance was marked for the following specific lesions: Persistent ductus arteriosus (72%), atrioventricular septal defects (62%) supravalvular aortic stenosis (71%) and isolated infundibular pulmonic stenosis (80%). A more balanced sex distribution was confirmed for cases of congenital rubella syndrome with persistent ductus arteriosus as isolated cardiovascular malformation (56%). The Importance to establish the sex dominance is emphasized as predictive of recurrence risk of congenital heart disease.

Down's syndrome: different distribution of congenital heart diseases between the sexes.

We have investigated the reasons why female patients with Down's syndrome prevail in our out-patient clinic for Paediatric Cardiology compared to the higher incidence of Down's syndrome among live born male children. We reviewed 277 cases of Down's syndrome, 119 males (42.96%) and 158 females (57.04%) from 1970 to 1987. A final diagnosis of the type of the congenital heart disease was accomplished among 210 cases, 85 males (40.47%) and 125 females (59.38%). This different distribution between the sexes was significant (P less than 0.01) when compared to that of the general population with congenital heart disease (4150 patients, 2108 males and 2042 females). The dominant lesion was atrioventricular septal defect, (130 cases; 46 males [54.11%] and 84 females [67.20%]). We found an identical incidence of this lesion among patients without Down's syndrome. In the studied population, we did not find any of the congenital heart diseases usually prevalent in males, such as aortic coarctation or stenosis and complete transposition. The molecular determinants of Down's syndrome seem to influence the preponderance of atrioventricular septal defect in females, increasing its incidence, while seeming to act in a negative way concerning congenital heart diseases usually showing male prevalence.