Coarctação Isolada da Aorta: Experiência em 100 Doentes Consecutivos

INTRODUCTION: Coarctation of the aorta (CoA) is a stenosis usually located in the descending aorta. Treatment consists of surgical or percutaneous removal of the obstruction and presents excellent immediate results but significant residual problems often persist. OBJECTIVES: To describe the presentation, treatment and long-term evolution of a population of 100 unselected consecutive patients with isolated CoA in a single pediatric cardiology center. METHODS: This was a retrospective study of all patients with isolated CoA treated during4 the last 21 years (1987-2008). RESULTS: The patients (n=100, 68.3% male) were diagnosed at a median age of 94 days (1 day to 16 years). The clinical presentation differed between patients aged less or more than one year, the former presenting with heart failure and the latter being asymptomatic with evidence of hypertension (88 and 63%, respectively; p < 0.01). Treatment, a median of 8 days after diagnosis, was surgical in 79 cases (20 end-to-end anastomosis, 31 subclavian flap, 28 patch) and percutaneous in the remaining 21 (15 balloon angioplasty, 6 with stenting). The mean age of surgical patients was younger than in those treated percutaneously (3.4 vs. 7.5 years; p < 0.01). Immediate mortality was 2% and occurred in the surgical group. There was no late mortality, in a mean follow-up of 7.2 +/- 5.4 years. Recoarctation occurred in 8 patients (6 surgical, 2 percutaneous). There are 46 patients who currently have hypertension (19 at rest, 27 with effort), their median age at diagnosis being older than the others (23 vs. 995 days; p < 0.01). CONCLUSIONS: Isolated CoA has an excellent short-term prognosis but a significant incidence of long-term complications, and should thus no longer be seen as a simple obstruction in the descending aorta, but rather as a complex pathology that requires careful follow-up after treatment. Its potentially insidious presentation requires a high level of clinical suspicion, femoral pulse palpation during physical examination of newborns and older children being particularly important. Delay in treatment has an impact on late morbidity and mortality. Taking into account the data currently available on late and immediate results, the final choice of therapeutic technique depends on the patient's age, associated lesions and the experience of the medical-surgical team. Hypertension should be closely monitored in the follow-up of these patients, as well as its risk factors and complications.

Utilidade do Doppler Tecidular na Predição de Eventos Arrítmicos em Adultos com Tetralogia de Fallot Corrigida

INTRODUCTION: Adults with repaired tetralogy of Fallot (TOF) may be at risk for progressive right ventricular (RV) dilatation and dysfunction, which is commonly associated with arrhythmic events. In frequently volume-overloaded patients with congenital heart disease, tissue Doppler imaging (TDI) is particularly useful for assessing RV function. However, it is not known whether RV TDI can predict outcome in this population. OBJECTIVE: To evaluate whether RV TDI parameters are associated with supraventricular arrhythmic events in adults with repaired TOF. METHODS: We studied 40 consecutive patients with repaired TOF (mean age 35 +/- 11 years, 62% male) referred for routine echocardiographic exam between 2007 and 2008. The following echocardiographic measurements were obtained: left ventricular (LV) ejection fraction, LV end-systolic volume, LV end-diastolic volume, RV fractional area change, RV end-systolic area, RV end-diastolic area, left and right atrial volumes, mitral E and A velocities, RV myocardial performance index (Tei index), tricuspid annular plane systolic excursion (TAPSE), myocardial isovolumic acceleration (IVA), pulmonary regurgitation color flow area, TDI basal lateral, septal and RV lateral peak diastolic and systolic annular velocities (E' 1, A' 1, S' 1, E' s, A' s, S' s, E' rv, A' rv, S' rv), strain, strain rate and tissue tracking of the same segments. QRS duration on resting ECG, total duration of Bruce treadmill exercise stress test and presence of exercise-induced arrhythmias were also analyzed. The patients were subsequently divided into two groups: Group 1--12 patients with previous documented supraventricular arrhythmias (atrial tachycardia, fibrillation or flutter) and Group 2 (control group)--28 patients with no previous arrhythmic events. Univariate and multivariate analysis was used to assess the statistical association between the studied parameters and arrhythmic events. RESULTS: Patients with previous events were older (41 +/- 14 vs. 31 +/- 6 years, p = 0.005), had wider QRS (173 +/- 20 vs. 140 +/- 32 ms, p = 0.01) and lower maximum heart rate on treadmill stress testing (69 +/- 35 vs. 92 +/- 9%, p = 0.03). All patients were in NYHA class I or II. Clinical characteristics including age at corrective surgery, previous palliative surgery and residual defects did not differ significantly between the two groups. Left and right cardiac chamber dimensions and ventricular and valvular function as evaluated by conventional Doppler parameters were also not significantly different. Right ventricular strain and strain rate were similar between the groups. However, right ventricular myocardial TDI systolic (Sa: 5.4+2 vs. 8.5 +/- 3, p = 0.004) and diastolic indices and velocities (Ea, Aa, septal E/Ea, and RV free wall tissue tracking) were significantly reduced in patients with arrhythmias compared to the control group. Multivariate linear regression analysis identified RV early diastolic velocity as the sole variable independently associated with arrhythmic history (RV Ea: 4.5 +/- 1 vs. 6.7 +/- 2 cm/s, p = 0.01). A cut-off for RV Ea of < 6.1 cm/s identified patients in the arrhythmic group with 86% sensitivity and 59% specificity (AUC = 0.8). CONCLUSIONS: Our results suggest that TDI may detect RV dysfunction in patients with apparently normal function as assessed by conventional echocardiographic parameters. Reduction in RV early diastolic velocity appears to be an early abnormality and is associated with occurrence of arrhythmic events. TDI may be useful in risk stratification of patients with repaired tetralogy of Fallot.

Alterações da Actividade Autonómica Durante o Teste de Inclinação em Doentes com Fibrilhação Auricular Paroxística: Análise com Wavelets

The autonomic nervous system (ANS) is known to be an important modulator in the pathogenesis of paroxysmal atrial fibrillation (PAF). Changes in ANS control of heart rate variability (HRV) occur during orthostatism to maintain cardiovascular homeostasis. Wavelet transform has emerged as a useful tool that provides time-frequency decomposition of the signal under investigation, enabling intermittent components of transient phenomena to be analyzed. AIM: To study HRV during head-up tilt (HUT) with wavelet transform analysis in PAF patients and healthy individuals (normals). METHODS: Twenty-one patients with PAF (8 men; age 58 +/- 14 yrs) were examined and compared with 21 normals (7 men, age 48 +/- 12 yrs). After a supine resting period, all subjects underwent passive HUT (60 degrees) while in sinus rhythm. Continuous monitoring of ECG and blood pressure was carried out (Task Force Monitor, CNSystems). Acute changes in RR-intervals were assessed by wavelet analysis and low-frequency power (LF: 0.04-0.15 Hz), high-frequency power (HF: 0.15-0.60 Hz) and LF/HF (sympathovagal) were calculated for 1) the last 2 min of the supine period; 2) the 15 sec of tilting movement (TM); and 3) the 1st (TT1) and 2nd (TT2) min of HUT. Data are expressed as means +/- SEM. RESULTS: Baseline and HUT RR-intervals were similar for the two groups. Supine basal blood pressure was also similar for the two groups, with a sustained increase in PAF patients, and a decrease followed by an increase and then recovery in normals. Basal LF, HF and LF/ HF values in PAF patients were 632 +/- 162 ms2, 534 +/- 231 ms2 and 1.95 +/- 0.39 respectively, and 1058 +/- 223 ms2, 789 +/- 244 ms2 and 2.4 +/- 0.36 respectively in normals (p = NS). During TM, LF, HF and LF/HF values for PAF patients were 747 +/- 277 ms2, 387 +/- 94 ms2 and 2.9 +/- 0.6 respectively, and 1316 +/- 315 ms2, 698 +/- 148 ms2 and 2.8 +/- 0.6 respectively in normals (p < 0.05 for LF and HF). During TF1, LF, HF and LF/ HF values for PAF patients were 1243 +/- 432 ms2, 302 +/- 88 ms2 and 7.7 +/- 2.4 respectively, and 1992 +/- 398 ms2, 333 +/- 76 ms2 and 7.8 +/- 0.98 respectively for normals (p < 0.05 for LF). During TF2, LF, HF and LF/HF values for PAF patients were 871 +/- 256 ms2, 242 +/- 51 ms2 and 4.7 +/- 0.9 respectively, and 1263 +/- 335 ms2, 317 +/- 108 ms2 and 8.6 +/- 0.68 respectively for normals (p < 0.05 for LF/HF). The dynamic profile of HRV showed that LF and HF values in PAF patients did not change significantly during TM or TT2, and LF/HF did not change during TM but increased in TT1 and TT2. CONCLUSION: Patients with PAF present alterations in HRV during orthostatism, with decreased LF and HF power during TM, without significant variations during the first minutes of HUT. These findings suggest that wavelet transform analysis may provide new insights when assessing autonomic heart regulation and highlight the presence of ANS disturbances in PAF.

Ecocardiografia em Medicina Interna

A abertura da ecocardiografia à especialidade de Medicina Interna exige, por parte dos seus executantes, a aplicação de regras e metodologias já estabelecidas, para que, num futuro próximo, se alcance representatividade creditada nesta área.

Perinatal Outcome and Change in Body Mass Index in Mothers of Dichorionic Twins: a Longitudinal Cohort Study

We used a prospective cohort to analyze the effect of change in BMI rather than change in weight, in mothers carrying dichorionic twins from a population that did not receive any dietary intervention. A total of 269 mothers (150 nulliparas and 119 multiparas) were evaluated. The average change (%) from the pre-gravid BMI was 7.2+/-6.1, 17.4+/-8.2, and 28.7+/-10.8, at 12-14, 22-25, and 30-34 weeks, respectively, without difference between nulliparas and multiparas. The comparison between maternities below or above the average change from the pregravid BMI failed to demonstrate an advantage (in terms of total twin birthweight and gestational age) of an above average change from the pregravid BMI, even when the lower versus upper quartiles were compared. Our observations reached different conclusions regarding the recommended universal dietary intervention in twin gestations. A cautious approach is advocated towards seemingly harmless excess weight gain, as normal weight women may turn overweight, or even obese, by the end of pregnancy, and be exposed to the untoward effects of obesity on future health and body image.

Factores de Risco para Asma Activa em Idade Escolar: Estudo Prospectivo com Oito Anos de Duração

A sibilância recorrente na infância é uma entidade clínica prevalente e heterogénea do pontode vista da história natural e do prognóstico. Efectuou-se um estudo prospectivo com 8 anos de duração, com o objectivo de relacionar a evolução clínica da sibilância recorrente nos primeiros anos de vida, com factores de prognóstico associados com a persistência da sintomatologia. Uma coorte de 308 crianças com sibilância recorrente, com idade ≤6 anos, foi incluída no estudo em 1993. Foi aplicado um questionário clínico, realizados testes cutâneos por prick e efectuado doseamento sérico de IgE total. Em 1996 procedeu-se a uma primeira reavaliação sistemática destas crianças. Em 2001 foi efectuada nova reavaliação sistemática, possível em 81% destas crianças (n=249), com repetição dos testes cutâneos e realização de avaliação funcional respiratória,em período intercrise, com espirometria com prova de broncodilatação (BD). As crianças reavaliadas apresentavam média etária de 11 anos (8-14 anos) e relação sexo M/F de 1.7/1. Permaneciam sintomáticas em 61% dos casos. A prevalência de atopia foi de 48% em 1993, 65% em 1996 e 75% em 2001. Pela realização de um modelo de regressão logística múltiplo foram identificados como factores de risco para asma activa em idade escolar: história pessoal de rinite alérgica (OR=15.8, IC95%=6.1-40.8; p<0.001), asma paterna (OR=7.2, IC95%=1.7-29.7; p=0.007), história pessoal de eczema atópico (OR=5.9, IC95%=2.2-15.7; p<0.001), asma materna (OR=5.4, IC95%=1.7-17.1; p=0.004), evidência de sensibilização alergénica (OR=3.4, IC95%=1.2-10.4;p=0.03) e início dos sintomas ≥2 anos de idade (OR=2.1, IC95%=1.1-4.8; p=0.04); a frequência de infantário antes dos 12 meses de idade foi identificada como factor protector (OR=0.4, IC95%=0.2- 0.9; p=0.04). Desenvolveram sensibilização alergénica de novo (ácaros do pó >80%) 66 das 128 crianças não atópicas em 1993 (52%). Apresentavam obstrução brônquica 36% das crianças: 47% das sintomáticas e 18% das assintomáticas (p<0.001). A prova de BD foi positiva em 35%: 47% nos sintomáticos e 13% nos assintomáticos (p<0.001). Concluindo, foram identificados como factores de mau prognóstico, antecedentes pessoais de doença alérgica, história parental de asma, presença de sensibilização alergénica e início dos sintomas na segunda infância. Os sintomas clínicos podem preceder em anos a sensibilização alergénica, realçando a importância da instituição precoce de medidas de controlo ambiental. Alterações nas provas funcionais respiratórias, mais frequentes nas asmas activas, estavam também presentes em crianças actualmente sem clínica, reforçando a necessidade de valorizar marcadores objectivos nesta cada vez mais prevalente doença respiratória crónica.

Conexão Venosa Pulmonar Anómala. Estudo de Vinte e Quatro Crianças com Angiografia de Subtracção Digital

Objectivo: Avaliar a acuidade da angiografia de subtracção digital (ASD) no diagnóstico morfológico da conexão venosa pulmonar anómala (CVPA) em crianças. Concepção do estudo: Estudo prospectivo de doentes consecutivos entre Janeiro de 1989 e Julho de 1992. Tipo de Atendimento: Serviço de Cardiologia Pediátrica de um Hospital Central. População: Vinte e quatro doentes com CVPA. Métodos: Todos os doentes fizeram avaliação clínica e ecocardiográfica completa (modo M, bidimensional e Doppler) antes da realização do exame hemodinâmico. Em todos os casos se fizeram, de modo sistemático, injecções selectivas de contraste de baixa osmolaridade (0,5-1 ml/kg; dose total <6 mi/kg) no tronco e ramos da artéria pulmonar com registo em angiografia com subtracção digital (ASD). As imagens colhidas foram trabalhadas, selecciona das e armazenadas em video-cassetes e películas fotográficas (câmara multiformato). Resultados: Dezasseis doentes tinham CVPA total (CVPAT): onze à veia cava superior (VCS), dois ao seio coronário e três infradiafragmáticos (dois à veia cava inferior (VCI) e um à veia porta). Oito crianças tinham CVPA parcial (CVPAP): três à VCS, uma à aurícula direita (AD), três à VCI (síndroma da cimitarra) e num caso a CVPA era mista. Em oito doentes (seis com CVPAT e dois com CVPAP), a ASD contribuiu significativamente para o diagnóstico final tendo completado ou corrigido a informação obtida por ecocardiografia. Nos dezoito doentes submetidos a cirurgia cardíaca foi confirmado o diagnóstico obtido por ASD. Conclusões: A ASD é um método muito útil para o diagnóstico anatómico de doentes com CVPA. Na nossa experiência foi particularmente informativa a análise de registos em «video». A ASD está indicada nos casos em que os achados clínicos e ecocardiográficos não sejam típicos.

Mutilação Genital Feminina: o que Sabem os Profissionais de Saúde?

Portugal is considered by the World Health Organization (WHO) a risk country for the practice of Female Genital Mutilation (FGM). Objectives: To evaluate the knowledge that health professionals from Maternity Dr. Alfredo da Costa (MAC) have regarding FGM. Population and Methods: Analysis of surveys delivered to health professionals from MAC (a hospital dedicated to reproductive health), between April and June 2008, addressing issues related to the knowledge about FGM. Results: Authors collected 112 valid surveys involving 38 doctors, 48 nurses and 26 medical auxiliaries/administrative personnel. From the respondents, 106 (95%) had heard about FMG practice before, the media being the most reported source of information; 59 (53%) replied they could be able to recognize FGM cases in their clinical practice; however, only 31 (28%) claimed to know the FGM type classiication and 32 (29%) admitted to be prepared to recognize and manage these situations in their own clinical practice; 9 had been consulted explicitly by a FGM practice complication and 1 doctor had admitted having been asked to perform/execute FGM; 13 (12%) recognized that the Portuguese legislation its this practice. Regarding the practice of FGM, 100 (89%) of respondentes stated that it should not be maintained and 97 (87%) stated that it should not be tolerated. However, 42 (38%) considered that if these practices were a reality, then they should be medical assisted. Discussion: Health professionals can play an important role in eliminating the practice of FGM, not only by the proper clinical management of this situation, but also by preventing those communities at risk to resort to FGM. Most health professionals are not prepared to deal with FGM in their clinical practice. It is important to promote a better knowledge on the subject and to create protocols for proper clinical management.

Restrição do Crescimento Fetal - Casuística de 4 Anos

Overview and aims: Fetal growth restriction (FGR) affects 15% of pregnancies and is associated with both increased perinatal and neonatal morbidity and mortality and long-term effects in adult life. Our aim was to describe cases and outcomes of FGR from a tertiary perinatal care centre and identify the predictors of neonatal morbidity and mortality. Study design: retrospective cohort. Population: pregnancies with early or late FGR caused by placental factors followed from 2006 to 2009 in a tertiary perinatal care centre. Methods: we collected data from clinical records on demographics, clinical history and fetal ultrasound parameters. Perinatal and neonatal outcomes were stratiied according to gestational age (above or below 28 weeks) and we used bivariate analysis to identify any associations with clinical and imaging indings. Results: we included 246 pregnancies; hypertension was the most prevalent maternal risk factor (16%). There were 15 cases of early FGR, 11 of which had cesarean delivery due to deterioration of fetal Doppler parameters. Outcomes in this group included one fetal and three neonatal deaths. Of 231 cases of late FGR, 64% were delivered early given a non-reassuring fetal status i.e. due to changes in Doppler evaluation or altered Manning biophysical proile. There were four cases of perinatal death in this group, three of which delivered at 28 weeks. Neonatal morbidity was associated with lower gestational age, lower birthweight and progressive placental dysfunction (p<0.01). Conclusion: there was an association between neonatal morbidity and gestational age, birthweight and Doppler deterioration, particularly for deliveries below 28 weeks. The assessment of vascular changes through Doppler analysis allows anticipation of fetal deterioration and is a helpful tool in deciding the optimum timing of delivery.

Uma Complicação Grave da Embolização das Artérias Uterinas

Uterine artery embolisation has been used as a therapeutic alternative for symptomatic uterine myomas. It is considered a safe and effective procedure, with very few cases published involving complications. The authors present a case of a 35-year-old nulliparous woman with an intramural myoma with 161x143x85mm, submitted to an uterine artery embolisation complicated by uterine necrosis. A hysterectomy was performed. This casereport reinforces the idea that artery embolization is not a riskfree procedure and serious complications may occur. Therefore, patients should be carefully selected.

Cateterismo da Veia Subclávia em Cuidados Intensivos Pediátricos - Análise Prospectiva de 3 Anos

Apresentam-se os resultados do estudo prospectivo dos primeiros 145 cateterismos da veia subclávia efectuados na Unidade de Cuidados Intensivos Pediátricos (U.C.I.P.) do Hospital de Dona Estefânia. Foram submetidos a cateterismo 131 doentes com idades compreendidas entre 1 mês e 15 anos. A técnica utilizada foi a de Seldinger (abordagem infraclavicular). A taxa de sucesso foi de 90.0% e o número médio de tentativas de 2.7 ± 2.4. A duração média da cateterização foi de 7.5 ± 7.2 dias. Houve 22 complicações «major». A incidência de sépsis de cateter foi de 6.9%. A remoção dos cateteres foi electiva em 91 (62.8%) casos. Não houve mortalidade atribuível ao cateterismo. Os resultados obtidos estão em conformidade com os divulgados na literatura; a aprendizagem e execução da técnica são relativamente fáceis e a sua aplicação tem alta taxa de sucesso e baixa incidência de complicações.

Perinatal Ischemic Stroke: a Five-Year Retrospective Study in a Level-III Maternity

Objective To study the incidence, clinical presentation, risk factors, imaging diagnosis, and clinical outcome of perinatal stroke. Methods Data was retrospectively collected from full-term newborns admitted to the neonatal unit of a level III maternity in Lisbon with cerebral stroke, from January 2007 to December 2011. Results There were 11 cases of stroke: nine were arterial ischemic stroke and two were cerebral venous sinus thrombosis. We estimated an incidence of arterial ischemic stroke of 1.6/5,000 births and of cerebral venous sinus thrombosis of 7.2/100,000 births. There were two cases of recurrent stroke. Eight patients presented with symptoms while the remaining three were asymptomatic and incidentally diagnosed. The most frequently registered symptoms (8/11) were seizures; in that, generalized clonic (3/8) and focal clonic (5/8). Strokes were more commonly left-sided (9/11), and the most affected artery was the left middle cerebral artery (8/11). Transfontanelle ultrasound was positive in most of the patients (10/11), and stroke was confirmed by cerebral magnetic resonance in all patients. Electroencephalographic recordings were carried out in five patients and were abnormal in three (focal abnormalities n=2, burst-suppression pattern n=1). Eight patients had previously identified risk factors for neonatal stroke which included obstetric and neonatal causes. Ten patients were followed up at outpatients setting; four patients developed motor deficits and one presented with epilepsy. Conclusions Although a modest and heterogeneous sample, this study emphasizes the need for a high level of suspicion when it comes to neonatal stroke, primarily in the presence of risk factors. The prevalence of neurological sequelae in our series supports the need of long-term follow-up and early intervention strategies.

Predição de Pré-Eclâmpsia no Primeiro Trimestre em Gravidezes de Baixo Risco: Determinação do Cut-Off numa Amostra da População Portuguesa

Objective:We aimed to identify the cut-off for risk of pre-eclampsia (PE) in Portuguese population by applying the first trimester prediction model from Fetal Medicine Foundation (FMF) in a prospective enrolled cohort of low risk pregnant women. Population and methods: A prospective cohort of low risk singleton pregnancies underwent routine first-trimester scree - ning from 2011 through 2013. Maternal characteristics, blood pressure, uterine artery Doppler, levels of pregnancy-associated plasma protein-A (PAPP-A) and free b-human chorionic gonadotropin were evaluated. The prediction of PE in first trimester was calculated through software Astraia, the outcome obtained from medical records and the cutoff value was subse quently calculated. Results:Of the 273 enrolled patients, 7 (2.6%) developed PE. In first trimester women who developed PE presented higher uterine arteries resistance, represented by higher values of lowest and mean uterine pulsatility index, p <0.005. There was no statistical significance among the remaining maternal characteristics, body mass index, blood pressure and PAPP-A. Using the FMF first trimester PE algorithm, an ideal cut-off of 0.045 (1/22) would correctly detect 71% women who developed PE for a 12% false positive rate and a likelihood ratio of 12.98 (area under the curve: 0.69; confidence interval 95%: 0.39-0.99). By applying the reported cutoff to our cohort, we would obtain 71.4% true positives, 88.3% true negatives, 11.4% false positives and 28.6% false negatives. Conclusion: By applying a first trimester PE prediction model to low risk pregnancies derived from a Portuguese population, a significant proportion of patients would have been predicted as high risk. New larger studies are required to confirm the present findings.