Cardiac hydatid cyst in a child.

Cardiac hydatid cyst is a rare disease, especially in children. An 11-year-old boy with a previous anaphylactic reaction and episodes of abdominal pain was admitted for workup of an acquired long systolic murmur. Echocardiographic investigation disclosed a tumor of the right ventricular anterior wall, with multiple loculations. Magnetic resonance imaging characterized it as a multilobular tumor with cyst formation and disclosed another cyst in the right pulmonary artery. With a positive ELISA reaction the child was admitted for surgery with the diagnosis of cardiac and pulmonary hydatid cysts. Cardiac surgery was performed with good results, followed by medical treatment with albendazole.

[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.

[Fetal cardiology. Bases for the prenatal diagnosis of congenital cardiopathies]. / Cardiologia fetal. Bases para o diagnóstico pré-natal das cardiopatias congénitas.

The bases for prenatal diagnosis of congenital heart disease are presented, in the line of a comprehensive program undertaken by paediatric cardiology and obstetrics. This program is designed to improve the screening of fetal heart diseases by obstetric echographists, particularly those working at peripheral high risk clinics. It includes the visit of paediatric cardiologists to district hospitals for joint meetings with local physicians, going over theoretical aspects, case presentation and four chamber view with outflow tract demonstrations using local ultrasound equipment. Referring protocols, handouts and support media exchange are contemplated. Support texts on the program divulged, on etiology of congenital heart disease, on abnormal fetal echocardiography and on fetal dysrhythmias are presented here in. The need for an organized national effort to increase the prenatal diagnosis of congenital heart disease is emphasized as well as the need to centralize some aspects related to diagnosis and treatment in order to improve the care of newborns with congenital heart disease.

[Fetal echocardiography. The results of a 3-year study]. / Ecocardiografia fetal. Resultado de três anos de estudo.

During the last three years (July 89 to July 92), 875 fetal echocardiographic studies were performed, at a mean gestational age of 26 +/- 6.5 weeks (12 to 40 weeks), with the following main referral indications: family history of congenital heart disease 23%; advanced maternal age 13%; fetal arrhythmia 10%; amniotic fluid abnormalities 8%; fetal malformations 7%; diabetes 6%; four chamber view changes 4%. There were 15 twin pregnancies, two of them thoracopagus. The referral patterns with higher percentage of cardiac pathology were: abnormal four chamber view 56%; fetal chromosomal anomalies 50%; omphalocele 33%; twin pregnancy 33%; IUGR 29%; arrhythmias 20%. The referral patterns with lower percentage of cardiac pathology were: congenital heart disease in the family 0.2%; maternal risk 2%; amniotic fluid volume alterations 2%. The diagnosis of congenital heart disease was made in 37 fetus (4.2%), with the following most frequent: 9 cases with the atrioventricular septal defect, 5 cases with ventricular septal defect, 5 cases with hypoplastic left heart syndrome and 5 cases with tricuspid valve abnormalities. From a total of 78 referred fetal arrhythmias, 25 were confirmed, 20 being ectopic beats without structural heart disease, 3 with tachycardia, and 2 with bradycardia. One newborn with tachycardia and Ebstein's disease died, and the fetus with AV block and left atrial isomerism died in utero. The treatment and follow up of the remaining cases with heart disease are described. The whole mortality in the cases with heart pathology was 65%.(ABSTRACT TRUNCATED AT 250 WORDS)

[Endocarditis caused by Candida parapsilosis after total correction of tetralogy of Fallot. Medico-surgical treatment]. / Endocardite por Cândida parapsilosis após correcção total de tetralogia de Fallot. Tratamento médico-cirúrgico.

Three children with Candida Parapsilosis endocarditis after total surgical correction for Tetralogy of Fallot, are reported. Blood cultures became negative in one child with amphotericin-B and in the other two with Ketoconazole, before reoperation for septal patch replacement. Oral therapy with Ketoconazole continued for a total of 24 months, and during a mean follow-up of 36 months no side effects or reinfection occurred. Medical and surgical conjoined therapy, with surgical timing decided on clinical and laboratorial grounds, were important for good results and cure.

[Evolution of the detection of interventricular septal defect]. / Evolução do rastreio da comunicação interventricular.

From 24.10.1969 to 31.12.1986, seventeen years, 1274 infants and children with proven isolated ventricular septal defect were observed and followed in Pediatric Cardiology of Santa Marta Hospital. There were 659 males and 615 females, ranging from 3 days to 12 years, born in portuguese territory and without syndromes. As early detection and referring is crucial to prevent pulmonary vascular disease with fixed pulmonary arterial resistance, the annual variation of the referring age was studied. The results improved since 1972 with a steady faster improvement during the last 7 years of the study.

[Interatrial communication associated with dilated cardiomyopathy]. / Communicação interauricular associada à cardiomiopatia dilatada.

A case of atrial septal defect in association with dilated cardiomyopathy is presented. Evolutive features are mainly assessed by echocardiography. On initial presentation the findings on physical examination pointed to the diagnosis of atrial septal defect with severe congestive heart failure. In addition to the demonstration of the congenital heart disease, echocardiography showed very dilated right atrial and ventricular cavities and later appearance of right atrium intracavitary thrombi. Left cavities were initially normal but, after surgical repair of the atrial septal defect, they rapidly increased. Endomyocardial biopsy confirmed the diagnosis of cardiomyopathy, which clinically seemed to only affect the right ventricle, but actually shown to be a diffuse myocardial disease.

[Percutaneous transluminal pulmonary valvuloplasty in children --5 years' experience]. / Valvuloplastia pulmonar transluminal percutânea em crianças--cinco anos de experiência.

OBJECTIVE: Assessment of the results of percutaneous transluminal balloon pulmonary valvuloplasty (BPV) in children. DESIGN OF THE STUDY: Retrospective analysis of the methodology and results between October 1985 and October 1990. SITE OF THE STUDY: Pediatric Cardiology Service, Santa Marta Hospital, Lisbon. PATIENTS: 43 consecutive children with pulmonary valve stenosis. 4 children with Noonan's Syndrome were excluded from the study and the results of 45 valvuloplasties in 39 patients are analysed. METHODS: Full diagnostic catheterization and right ventricular cineangiography were performed. One or two balloon dilatation catheters were inserted percutaneously in one or both femoral veins and positioned across the pulmonary valve under fluoroscopic control. The balloons were then inflated to their maximum pressure with diluted contrast medium. In 18 children follow up cardiac catheterization was performed 3 to 12 months after valvuloplasty (mean 6.7 /+- 2.7). RESULTS: A satisfactory dilatation was achieved in 35 patients (89%) with a substantial decrease in right ventricular systolic pressure (RVP) and in transvalvar peak systolic gradient (GR) (p less than 0.0001). A further significant spontaneous reduction in RVP and GR was recorded on follow up catheterization (p less than 0.01). A repeat BVP was performed in 6 children and was successful in 5. In cases a satisfactory relief of the stenosis was not achieved and 3 of them were submitted to surgery. There were no deaths. One child had cardiac arrest leading to successful surgical valvotomy and with no sequelae. Another child had cerebral embolism with hemiparesis. Ligation of a femoral vein was required in one case for haemorrhage. Mild pulmonary insufficiency resulted in 15 children. CONCLUSIONS: Our experience confirms that BPV is the method of choice for the treatment of children with pulmonary valve stenosis either isolated or associated to small ventricular septal defect. It proved to be a safe and effective technique for reduction of GR. Mortality, morbidity, costs of the treatment and hospitalisation time are lower for BPV than for surgery with comparable short term results.

[Continuing medical education: a call and a challenge]. / Educação médica contínua: um apelo e um desafio.

Professional responsibility of physicians requires continuous Medical Education. The Accrediation Council for Continuing Medical Education has developed in the U.S.A. a perfect program based on the Essentials and guidelines for accreditation. It is urgent to create a national trusty and dynamic structure to make possible the organization and coordination of CME and respective evaluation.

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.

[Congenital cardiopathy and cerebral abscess]. / Cardiopatia congénita e abcesso cerebral.

UNLABELLED: During 1986 the authors came across two cases of brain abscess among children with congenital heart disease followed at the Pediatric Cardiology Service and decided to evaluate their global experience on the subject. In a retrospective study of 860 infants and children with cyanotic congenital heart disease and final diagnosis, there were four cases complicated with brain abscess. The following items were evaluated: prevalence of the complication, type of congenital heart disease, date and age at the diagnosis of brain abscess, diagnostic methods, neurosurgical treatment and results. The main findings were: all patients were above two years of age and had noncorrected cyanotic congenital heart disease belonging to the classic high risk group; the first two cases had been treated in other institutions and only scanty information was available; two recent cases had early diagnosis on CAT scan followed by neurosurgical treatment. All children survived. CONCLUSIONS: brain abscess is a rare but severe complication occurring in patients with noncorrected cyanotic congenital heart disease above two years of age; whenever prevention turns impossible, early diagnosis and treatment provide good short term and long term results. A multidisciplinar approach with full cooperation is advocated.