15 research outputs found
A Papillary Fibroelastoma of the Tricuspid Valve
Primary cardiac tumours are rare in children. Of these, papillary fibroelastomas are unusual but benign, usually being found in adults. There are only sporadic cases reported in children. We diagnosed such a papillary fibroelastoma involving the tricuspid valve in an asymptomatic child during a routine cardiac investigation
Percutaneous Treatment of Pulmonary Atresia with Intact Ventricular Septum: Pulmonary Valve Perforation Using Radiofrequency Energy and Arterial Duct Stenting
A atrésia pulmonar com septo interventricular intacto (AtrP-SI) é uma
cardiopatia congénita rara e de prognóstico
reservado. Apresenta grande variabilidade
anatómica, com diversos graus de hipoplasia do ventrículo direito (VD) o que
condiciona a abordagem terapêutica.
Idealmente, o objectivo é a reconstituição
de uma circulação de tipo biventricular.
Para o efeito, dispomos de técnicas
cirúrgicas e percutâneas. A perfuração da
válvula pulmonar com energia de
radiofrequência (RF) é um método válido
para doentes com atresia de tipo
membranoso, VD sem hipoplasia marcada
(bipartido ou tripartido) e circulação
coronária não dependente do VD. Por
vezes, há necessidade de suplementar a
circulação pulmonar implantando um stent
no canal arterial. Desta forma é possível
tratar alguns doentes com técnicas
exclusivamente percutâneas. Relatamos o
primeiro caso conhecido em Portugal de um
recém-nascido com AtrP-SI submetido a
perfuração com radiofrequência e, num
segundo tempo, implantação de stent no
canal arterial
Octreotide - Additional Conservative Therapy for Postoperative Chylothorax in Congenital Heart Disease
INTRODUCTION: Chylothorax is a rare but serious postoperative condition in children with congenital heart disease. Conventional medical treatment consists of specific long-term dietary modification, and surgical reintervention, such as lymphatic duct ligation, may be indicated in refractory cases. In recent years, an additional conservative treatment, octreotide, a synthetic analog of somatostatin, has been used in management of congenital and postoperative chylothorax.
METHODS: The objective of this work was to analyze the efficacy and safety of this treatment for chylothorax after congenital heart surgery. We reviewed the records of sixteen patients with chylothorax after surgery for congenital heart disease between January 1999 and December 2007, and collected the following data: demographic information; type of surgical procedure; onset, duration and management of chylothorax and treatment; and duration of hospital stay. To analyze efficacy we compared these parameters in children receiving conventional treatment only with those receiving octreotide. To analyze safety we compared the adverse effects of both treatments. Octreotide was administered at a dose of 4 to 10 microg/kg/hour, with monitoring of side effects.
RESULTS: The incidence of chylothorax in our population was 1.6%. It occurred more often after Glenn and Fontan procedures (8 patients). Octreotide was begun three days after diagnosis of chylothorax and continued for a median of seventeen days (ranging from 4 to 26 days), until complete resolution. Side effects were frequent (in 3 of the 8 patients) but of no clinical relevance. All patients responded to the therapy and there was no indication for further surgical intervention.
DISCUSSION AND CONCLUSIONS: Octreotide is safe and effective in the treatment of postoperative chylothorax in children with congenital heart disease. It is a useful adjunctive therapy to the conventional treatment of this complication
Aortopulmonary Window. Experience of Eleven Cases
A janela aorto-pulmonar consiste numa
comunicação entre a aorta ascendente e o
tronco da artéria pulmonar, na presença das
duas válvulas arteriais separadas. É uma
anomalia rara que ocorre em cerca de 0,1% a
0,2% de todas as cardiopatias congénitas.
O objectivo deste trabalho é avaliar a forma de apresentação clínica, o tratamento cirúrgico e a evolução dos doentes com janela aorto-pulmonar assistidos num centro terciário de cardiologia pediátrica num período de 30 anos.
Identificaram-se onze crianças consecutivas
com o diagnóstico de janela aorto-pulmonar.
Dez crianças tinham o defeito do tipo proximal e uma do tipo distal. A idade na primeira avaliação variou entre três dias e 13 anos(média=44,5±63,3 meses; mediana=três
meses). A ecocardiografia permitiu fazer o
diagnóstico correcto nos quatro doentes mais
recentes.
Sete doentes foram submetidos a encerramento
da janela aorto-pulmonar por via trans-aórtica e três doentes foram submetidos a laqueação da janela aorto-pulmonar. Simultaneamente, foram corrigidas anomalias associadas significativas em três doentes: correcção de interrupção do arco aórtico em dois doentes; encerramento de comunicação interventricular e alargamento do tracto de saída do ventrículo direito com remendo de Dacron, num doente.
Um doente teve seguimento médico por
apresentar hipertensão pulmonar fixa.
A mortalidade operatória foi de 10% (1/10) e
não se verificou mortalidade tardia.
O tempo médio de seguimento foi de 10±4,9
anos. Actualmente os nove doentes operados
sobreviventes estão assintomáticos, sem qualquer medicação, sem defeitos residuais e
sem evidência de hipertensão pulmonar.
Em conclusão, a evolução das técnicas de
diagnóstico e de tratamento cirúrgico da janela aorto-pulmonar nos últimos anos permite uma intervenção precoce com bons resultados clínicos
The Role of Propranolol in the Treatment of Infantile Hemangioma
INTRODUCTION: Infantile hemangioma (IH) is one of the most common childhood tumors. There are various medical or surgical therapeutic options, all with suboptimal results. Recently, the successful use of propranolol for involution of IH was described. We report the results of a single-center experience with this therapeutic option.
OBJECTIVE: To prospectively assess the efficacy and safety of propranolol in children with infantile hemangioma.
METHODS: We performed a prospective analysis of clinical data of all patients with IH referred to a pediatric cardiology center for baseline cardiovascular assessment prior to propranolol therapy. Propranolol was given at a starting dose of 1 mg/kg/day and titrated to a target dose of 2-3 mg/kg/day according to clinical response. Efficacy was assessed through a photograph-based severity scoring scale. Safety was assessed by collecting data regarding significant side effects.
RESULTS: Starting in 2010, 30 patients (15 female) were referred for propranolol treatment of IH, at a median age of six months (1-63 months). The mean target propranolol dose was 2.8 mg/kg/day, with a mean duration of therapy of 12 months. All patients experienced significant reduction of IH size and volume. There were no side effects.
CONCLUSIONS: In our experience propranolol appears to be a useful and safe treatment option for severe or complicated IH, achieving a rapid and significant reduction in their size. No adverse effects were observed, although until larger clinical trials are completed, potential adverse events should be borne in mind and consultation with local specialists is recommended prior to initiating treatment
Isolated Aortic Coarctation: Experience in 100 Consecutive Patients
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
Percutaneous Closure of Atrial Septal Defects: a Decade of Experience at a Reference Center
INTRODUCTION: Atrial septal defects (ASD) are among the most common congenital anomalies and account for 10% of congenital heart disease in the pediatric age-group and 30% in adults. Closure is indicated when there is evidence of hemodynamic significance or after a paradoxical embolic event. Ten years ago, percutaneous closure became the treatment of choice in our center for all patients with a clear indication and favorable anatomy. In this paper we report the experience of this first decade.
OBJECTIVE: To assess the short- and long-term results of our ten-year experience with percutaneous closure of atrial septal defects.
METHODS: We studied retrospectively all patients with ASD treated with a percutaneous approach between November 1998 and December 2008. The pediatric age-group consisted of patients younger than 19 years old. Demographic data, clinical indications, minor and major complication rates, success rate and long-term outcome were assessed.
RESULTS: In the first ten years of experience 510 patients, of whom 166 were in the pediatric group, were treated in our center by a team of adult and pediatric cardiologists. The overall success rate of the procedure was 98% (97.5% in ASD and 99.5% in patent foramen ovale (PFO). The minor complication rate was 3% (3.4% in ASD and 2% in PFO). The most frequent complication was supraventricular tachycardia. The major complication rate was 1.2% (0.6% in ASD and 2% in PFO). Two patients developed cardiac tamponade due to hemopericardium that was resolved by pericardiocentesis, without need for surgery. One patient had an arterial pseudoaneurysm corrected by vascular surgery. There was no device embolization and no need for urgent surgery in this population. During follow-up two patients had recurrence of ischemic stroke, one had a transient ischemic attack and another had a hemorrhagic stroke. Mortality was 0.6% (0.6% in ASD and 0.5% in PFO). There were no in-hospital deaths. During follow-up there were two deaths, both in the adult group.
DISCUSSION AND CONCLUSION: In this population the success rate was high and most of the complications were minor. The results of this collaboration between adult and pediatric cardiologists in the first ten years of activity confirm the safety and efficacy of percutaneous closure of septal defects, when there is careful patient selection and a standardized technique
Cardiac Tumors: the 17-Year Experience of Pediatric Cardiology Department
Introdução: Os tumores cardíacos primários
podem ser benignos ou malignos, ter origem
no endocárdio, miocárdio ou pericárdio.
São raros em crianças, com uma prevalência
descrita em séries de autópsia de
0,0017 a 0,28%; a maioria são benignos, representando os malignos cerca de 10% do total.
Material e Métodos: Com o objectivo de
avaliar a apresentação clínica e a evolução
das crianças assistidas num centro de
Cardiologia Pediátrica com este diagnóstico,
procedeu-se à revisão retrospectiva dos
processos clínicos dos doentes com tumores
cardíacos primários observados nos últimos
17 anos (1989-2006). Determinou-se a forma
de apresentação, diagnóstico morfológico,
complicações e evolução ao longo do período
de seguimento.
Resultados: Identificaram-se 12 doentes, cuja
idade média à data do diagnóstico foi de 23
meses. Em dois casos o diagnóstico foi
pré-natal. A causa mais frequente de
referenciação foi a esclerose tuberosa, seguida de sopro cardíaco. O ecocardiograma e a ressonância magnética permitiram o
diagnóstico em todos. Todos os doentes
realizaram electrocardiograma e registo Holter e o achado mais frequente foram as alterações inespecíficas da repolarização. O tumor mais frequente foi o rabdomioma (67%), na maioria associado a esclerose tuberosa; seguido do fibroma em (17%) e do fibroelastoma (8%).
Apesar da biópsia ser o exame de eleição
para a confirmação diagnóstica, realizou-se
apenas em dois doentes. A excisão cirúrgica
do tumor foi efectuada num doente
(fibroelastoma), por risco de embolia
pulmonar.
Discussão e Conclusões: Na população
estudada o tumor cardíaco mais frequente foi
o rabdomioma associado à esclerose tuberosa.
A maioria dos doentes não apresentava
sintomas cardiovasculares, sendo o seu
diagnóstico geralmente efectuado em
observações de rotina para esclarecimento de
sopro cardíaco ou devido ao diagnóstico de
esclerose tuberosa. O exame histológico
justifica-se apenas nos tumores mais raros
Long-Term Evaluation of Endothelial Function in Kawasaki Disease Patients
BACKGROUND:
Kawasaki disease is an acute systemic vasculitis. Cardiac complications are frequent and include endothelial dysfunction in patients with coronary anomalies. So far, the presence of endothelial dysfunction in patients with no coronary lesions has not been demonstrated. Peripheral arterial tonometry (Endo-PAT) measures the microvascular function in response to local ischaemia and has been validated in adult population, but its use in children is scarce. Aim To evaluate endothelial dysfunction in children as a long-term complication after Kawasaki disease using Endo-PAT.
METHODS:
We evaluated two groups of subjects: (1) Kawasaki disease patients over 11 years of age, diagnosed for >5 years, with no coronary lesions, or any other risk factors for cardiovascular disease; (2) control group of individuals without cardiovascular risk factors. Patients and controls were clinically accessed. Endo-PAT was performed to determine reactive hyperaemia index and augmentation index.
RESULTS:
A total of 35 individuals (21 males, age 21 ± 6 years) were evaluated (group 1: 19; controls: 16). Kawasaki disease patients presented significant lower reactive hyperaemia index (1.68 ± 0.49 versus 2.31 ± 0.53; p = 0.001). Augmentation index was similar in both groups (-10 ± 7 versus -11 ± 5; p > 0.005). Most patients with Kawasaki disease disclosed endothelial dysfunction (68%) compared with only 12% in controls.
CONCLUSIONS:
Endo-PAT is feasible and reproducible in the child population. Endothelial dysfunction is a frequent long-term complication in patients after Kawasaki disease with normal appearing coronary arteries. However, these results need validation in a larger population.info:eu-repo/semantics/publishedVersio
Quadricuspid Aortic Valve. 10-Year Case Series and Literature Review
Introdução: A válvula aórtica quadricúspide é uma malformação rara, com uma incidência estimada de 0,003 a 0,043% de todas as cardiopatias congénitas. Surge habitualmente como uma anomalia congénita isolada, podendo igualmente estar associada a outras malformações, sendo as mais frequentes as anomalias das artérias coronárias. A tecnologia actual permite o diagnóstico
não invasivo na grande maioria das situações. A sua história natural é a evolução para a insuficiência, rara antes da idade adulta.
Objectivos: Revisão dos casos de válvula aórtica quadricúspide diagnosticados nos últimos 10 anos num centro terciário de Cardiologia Pediátrica.
Material e Métodos: Revisão retrospectiva do processo clínico dos doentes aos quais foi detectada uma válvula aórtica quadricúspide, entre Janeiro de 2000 e Dezembro de 2009.
Resultados: Nos últimos 10 anos, foram diagnosticados quatro casos de válvula aórtica quadricúspide, em crianças com idades compreendidas entre os 6 meses e os 8 anos, duas do sexo masculino. Em três casos, os quatro folhetos eram de dimensões semelhantes, que é o achado mais frequente. Duas das válvulas eram normofuncionantes e duas apresentavam insuficiência mínima. Todos os doentes apresentavam outras malformações cardíacas associadas
(uma comunicação interauricular, duas comunicações interventriculares, uma estenoseçupravalvular aórtica e uma válvula pulmonar quadricúspide). Um doente tinha também o diagnóstico de Síndrome de Williams. Com um tempo de seguimento mediano de 2 anos [0 --- 9], todos os doentes se mantiveram assintomáticos e não requereram tratamento médico ou cirúrgico para
a válvula aórtica.
Conclusão: O diagnóstico de válvula aórtica quadricúspide é raro, sobretudo em idade
pediátrica, quando a maioria dos doentes são assintomáticos e apresentam válvulas normofuncionantes.
Nesta casuística, metade apresentava insuficiência aórtica mínima. Ao contrário
do que está descrito na literatura, todos os doentes apresentavam malformações cardíacas
concomitantes. Descrevemos pela primeira vez a associação com a Síndrome de Williams. Estes doentes deverão manter seguimento em ambulatório, de forma a detectar atempadamente o aparecimento ou agravamento de alterações funcionais e permitir uma intervenção terapêutica oportuna