Cleusa Lapa Santos

QT Dispersion predicting acute rheumatic carditis.

OBJECTIVE To investigate QT dispersion in the surface electrocardiogram of children with rheumatic carditis. METHODS QT dispersion was quantitatively evaluated in 33 children with acute rheumatic carditis. As a control group, we studied 33 healthy children free of any disease. The children were eligible for participation if the following criteria were met: diagnosis of acute rheumatic fever based on the revised Jones criteria and suffering from their first attack of carditis. The echo Doppler cardiogram was performed in all children, within 48-72 hours of hospitalisation. RESULTS Patients with carditis had a greater QT dispersion than the control group. When we analyse the QT dispersion according to the severity of the carditis, we observed that the dispersion tended to be greater in those with more severe valvar lesion. The sensitivity and specificity of the measurements of the QT dispersion in predicting acute carditis were estimated by using receiver operating characteristic curves. A QT dispersion greater than 40 milliseconds had a sensitivity of 63.6% and a specificity of 93.9% in predicting acute rheumatic carditis. CONCLUSION The lengthening of QT dispersion may reflect on cardiac involvement in rheumatic fever and be a new important parameter in the diagnosis and therapeutic decision for rheumatic carditis.

Aortic-right atrial tunnel.

We describe an asymptomatic 1-year-old boy who presented a continuous murmur, and was found to have a tunnel between the right atrium and the aorta. The definitive diagnosis was established by both echocardiography and aortography, and surgical interruption of the tunnel was successful. We emphasise the rarity of this condition, and discuss its major features.

Endomyocardial fibrosis in children.

We describe 10 children with endomyocardial fibrosis who underwent surgical treatment between 1978 and 1999. Seven were male and 3 female, with an age range from 4 to 15 years, having a mean age of 11 years. All were in the final stage of heart failure. Three had biventricular disease, 6 had involvement of the right ventricle alone, and one had endomyocardial fibrosis confined to the left ventricle. There were 3 deaths (30%) in the postoperative period due to low cardiac output. The 7 survivors were followed up for a period ranging from 12 to 168 months, with a mean of 72 months. Two late deaths have occurred resulting from heart failure and infectious endocarditis. Five (50%) children are still alive. Two required 3 reoperations for dysfunction of the inserted valvar prosthesis. One patient is in functional Class IV, and 4 are in Class II to III, despite intensive medical treatment. It is concluded that surgery for endomyocardial fibrosis is an essentially palliative procedure and, especially in children, the results of surgical treatment leave much to be desired.

The prolongation of the QT interval in the acute rheumatic carditis: an enigma

Correspondencia: lurildo R. Saraiva • Avenida Prof. Moraes Rego, SN – Hospital das Clinicas – Cidade Universitaria – 50670-901 – Recife, PE E-mail: lurildocleano@hotmail.com Artigo recebido em 18/04/06; revisado recebido em 18/05/06; aceito em 18/05/06. Palavras-chave Febre reumatica, miocardite, sindrome do QT longo, doencas das valvas cardiacas. (PRi = 180 ms) e sinais de sobrecarga atrial esquerda, percebese o QTc alongado (valor medio em D2 = 0,441, maximo para a idade e sexo, 0,4201) e, mais que isso, a ampliacao desse alongamento apos extra-sistoles, com ondas T esmaecidas. Valores tao elevados para o limite superior de normalidade do QTc, como 0,450, admitidos por autores ingleses2, nao constituem, de modo algum, um fato consensual entre nos. Na sua tese de Catedra, “A sistole eletrica ventricular”, de 1949, o Professor Luiz V. Decourt1, no momento em que contemplou a apreciacao do intervalo QT na molestia reumatica, emitiu uma serie de conclusoes, apos minuciosa analise do eletrocardiograma (ECG) de 60 casos de individuos portadores de reumatismo ativo, entre eles, 35 com cardite reumatica “primitiva”. Utilizando a formula de Bazett no calculo do QT Sindrome caracterizada pelo historico de episodios de sincope e um intervalo QT longo, algumas vezes, levando a morte subita, devido a arritmia ventricular paroxistica corrigido para a frequencia cardiaca (QTc), verificou que:

Correção intracardíaca da tetralogia de Fallot no primeiro ano de vida: resultados a curto e médio prazos

Objective: To evaluate short and medium term results of elective intracardiac correction of tetralogy of Fallot in the first year of life.

Congenital aneurysm of the right atrium

Congenital aneurysm of the right atrium is described in a 1-year-old girl who presented with cardiomegaly and symptoms of paroxysmal supraventricular tachycardia. Echocardiography and cineangiography both established the definitive diagnosis and surgical resection was successful. The rarity of this condition is pointed out and its main features outlined.

Macular and peripapillary retinal nerve fibre layer thickness in patients with cyanotic congenital heart disease

PurposeTo evaluate macular and retinal nerve fibre layer (RNFL) measurements in patients with cyanotic congenital heart disease (cCHD) using spectral domain optical coherence tomography (OCT).Patients and methodsThirty patients with cCHD (18 females and 12 males, mean age 10.9 years) and 60 healthy controls (35 females and 25 males, mean age 11.2 years) underwent complete ophthalmologic examination and OCT measurements of macular and peripapillary RNFL thickness.ResultsPatients with cCHD had significantly thinner measurements in all macular subfields compared with healthy controls (P<0.001). There was no significant difference in peripapillary RNFL thickness between the two groups, with the exception of the upper quadrant, for which thickness measurements were higher in patients with cCHD (P=0.021).ConclusionsPatients with cCHD showed a significant decrease in macular thickness and a thickened superior quadrant RNFL thickness when compared with healthy controls. This may represent the damage caused by the effect of hypoxia.

Clinical and functional characterisation of rheumatic mitral regurgitation in children and adolescents including the brain natriuretic peptide

Rheumatic fever is a public health problem of universal distribution, predominantly affecting individuals in developing countries. In individuals less than 20 years of age, pure mitral regurgitation is the most commonly found condition in chronic rheumatic valve disease. In the present study, rheumatic mitral regurgitation was assessed in children and adolescents, addressing its clinical (duration of the disease, symptoms, use of benzathine penicillin, and number of outbreaks of the acute phase of rheumatic fever), electrocardiographic (left atrium abnormality and/or left ventricle hypertrophy) and echocardiographic characteristics (left atrium and ventricle measurements, ejection fraction and pulmonary artery pressure), as well as plasma dose of N-terminal portion of the brain natriuretic peptide through electrochemiluminescence immunoassay. Fifty-three patients were studied. The patients had moderate (41.5%) or severe (58.5%) rheumatic mitral regurgitation; had not undergone surgery; were not in the acute phase of the disease; and were being treated at a paediatric cardiology reference hospital in Northeastern Brazil. Mean patient age was 10.6 years (minimum of 3 and maximum of 19 years). With the exception of the ejection fraction, the echocardiographic variables had a significant correlation to the natriuretic peptide, demonstrating that this hormone reflects the haemodynamic consequences of mitral regurgitation. It was concluded that cardiac remodelling that occurs in rheumatic mitral regurgitation in children and adolescents leads to the production of the brain natriuretic peptide, which could be used as a complementary diagnostic tool in the follow-up of such patients.

Tratamento cirúrgico da tetralogía de Fallot no adulto

Twenty-nine cyanotic patients with tetralogy of Fallot ranging in age from 16 to 43 years (mean, 21 yrs) have undergone total correction. Only two (6.8%) patients had previous Blalock-Taussig shunt. The established intracardiac technique for total repair was used throughout. In four (13.75) patients, reconstruction of the pulmonary outflow tract was required. The hospital mortality was 13.7% (4 of 29 patients). All deaths occurred in the beginning of the experience (1967-1977), when disposable oxygenators and myocardial protection were not used. The follow-up for the surviving patients was 1560 patient-months (mean, 62 months). There was one late death, not cardiac related. All but two survivors had good clinical results. These two patients were successfully reoperated for correction of residual defects at six months and 11 years after the initial procedure, respectively. We believe that advanced age is not a contraindication for total repair of tetralogy of Fallot since patients with this anomaly surviving to adulthood usually show favourable morphology.

Coarctação da aorta: resultados da cirurgia e análise crítica de diversas técnicas

Seventy patients ranging in age from 14 days to 49 years (mean 7.5 yrs.) were submitted to surgical repair of coarctation of the aorta. Twenty-six (37.1%) were in the first year of life. Surgical techniques performed included patch aortoplasty in 30 cases, subclavian flap angioplasty in 28, resection and end-to-end anastomosis in 9, resection and interposition of a Dacron tube graft in 2 and subclavian aortoplasty with preservation of arterial blood flow to the left arm in 1. There were 6 (8.5%) early and 2 (2.8%) late deaths, not related to the type of repair. Early mortality was clearly related to young age, associated anomalies and severe pre-operative heart failure. All surviving patients present good long-term results and no instance of recoarctation has occurred. No late aneurysm formation was seen in the group of patients submitted to patch aortoplasty probably due to the use of biological patchs in the majority of cases. At present, the authors try to individualize the operation, performing the more suitable technique for each case. Therefore, it is tried to use sublavian flap aortoplasty as much as possible in children below the age of 5 and end-to-end anastomosis or patch aortoplasty in older patients.