Initial Use of Endothelial Progenitor Cells Capturing Stents in Paediatric Congenital Heart Disease

INTRODUCTION: Stenosis, mediated by neointimal hyperplasia and thrombosis, is a major limiting factor in successful stent implantation. The introduction of a stent, coated in its endoluminal surface by antihuman CD34 antibodies with endothelial progenitor cell-capturing properties, opens the possibility of promoting a rapid and normal functioning coverage by endothelium and thus avoids both an excessive cell proliferation within stent and the need for long-term dual antiplatelet therapy. These stents, developed for adult coronary artery disease, have not yet been implanted in children or in those with congenital heart disease. OBJECTIVE AND METHODS: In this paper, we describe the implantation of Genous® stents in three children with cyanotic congenital heart disease and obstructed systemic-to-pulmonary shunts. We describe the use of this stent and address its potential feasibility in paediatric congenital heart disease. RESULTS: To maintain the patency of two modified Blalock-Taussig shunts and one ductus arteriosus, four Genous® stents were implanted in three infants with cyanotic heart disease. All procedures were immediately successful, with resolution of stenosis and improvement in transcutaneous oxygen saturation from 66% ± 3.6% to 92% ± 2.6%. In the follow-up, one stent had no occlusion; however, the remaining two had partial occlusion after 5 and 5.5 months, which were successfully managed with balloon dilatation preceding elective definitive surgical correction. CONCLUSION: In our preliminary experience, we demonstrated that Genous® stent implantation was feasible in infants with complex congenital heart disease. Additional studies with larger samples and longer follow-up are required to confirm the potential benefits of this technology in this clinical setting.info:eu-repo/semantics/publishedVersio

Exercise-Induced Intraventricular Obstruction in a Child with Near Syncope and Chest Pain During Exercise

We report the case of a 10-year-old girl with two episodes of light-headedness and chest pain during exercise. She had an unremarkable clinical record, physical examination, ECG, and echocardiogram. Noninvasive ischemia tests were positive, but coronary angiography was normal. Exercise stress echocardiogram revealed an exercise-induced intra-left-ventricular obstruction with a peak gradient of 78 mmHg and replicated her symptoms. After starting beta-blocker therapy her clinical status improved and no residual obstruction was detected. The authors review this unsuspected clinical condition, seldom reported in the adult population and, to our knowledge, never before in a child

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

A Rare but Real Necessity: Case Report of Coronary Artery Stenting in an Infant

Percutaneous coronary intervention (PCI) is an extremely common and well-established procedure in adults which is rarely performed in children. We present a case of a successful left main coronary artery stenting in a small infant with a congenital coronary artery anomaly. We highlight the technical challenges of performing a PCI in a small patient, the risks of antithrombotic prophylaxis in this age group, and the importance of the combined work of the adult and pediatric interventional cardiologist.info:eu-repo/semantics/publishedVersio

Pulmonary Asymmetry on Chest X-Ray

A ausência unilateral de uma artéria pulmonar é uma anomalia congénita rara. Os autores descrevem o caso de um rapaz de dois anos, sem antecedentes patológicos prévios e que é referenciado para avaliação após a detecção na telerradiografia de tórax de assimetria dos campos pulmonares com desvio do mediastino para a direita. A tomografia axial computorizada e a cintigrafia de perfusão pulmonar entretanto efectuadas, indicavam para a ausência da artéria pulmonar direita que foi comprovada no cateterismo cardíaco e em ressonância magnética. Esta é uma patologia relevante pois o seu diagnóstico precoce e a sua correcção atempada podem evitar morbilidades no futuro. Dada a idade e o facto de o doente estar de momento assintomático, optou-se por uma atitude conservadora e vigilância em ambulatório

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

Stenosis of the Branches of the Neopulmonary Artery after the Arterial Switch Operation: a Cardiac Magnetic Resonance Imaging Study

Background : The neonatal arterial switch operation (ASO) is now the standard of care for children born with transposition of the great arteries. Stenosis of the neopulmonary artery on long‑term follow up is a known complication. Methods : We performed a retrospective analysis of eleven patients who underwent a cardiac magnetic resonance imaging (MRI) due to echocardiographic evidence suggestive of stenosis of the neopulmonary artery or its branches (mean estimated Doppler gradient 48 mmHg, min 30 mmHg, max 70 mmHg). A comprehensive evaluation of anatomy and perfusion was done by cardiac MRI. Results : The branches of the neopulmonary artery (neo PA) showed decreased caliber in three patients unilaterally and in two patients, bilaterally. Magnetic resonance (MR) perfusion studies showed concomitant decreased flow, with discrepancy between the two lungs of 35/65% or worse, only in the three patients with unilateral obstruction, by two different MR perfusion methods. Conclusions : Cardiac MR can be used as a comprehensive non‑invasive imaging technique to diagnose stenosis of the branches of the neopulmonary after the ASO, allowing evaluation of anatomy and function of the neoPA, its branches, and the differential perfusion to each lung, thus facilitating clinical decision making

An Estimation of the Entomological Inoculation Rate for Ifakara: A Semi-Urban Area in a Region of Intense Malaria Transmission in Tanzania.

An entomological study on vectors of malaria and their relative contribution to Plasmodium falciparum transmission in the semi-urban area of Ifakara, south-eastern Tanzania, was conducted. A total of 32 houses were randomly sampled from the area and light trap catches (LTC) performed in one room in each house every 2 weeks for 1 year. A total of 147 448 mosquitoes were caught from 789 LTC; 26 134 Anopheles gambiae s.l., 615 A. funestus, 718 other anophelines and 119 981 culicines. More than 60% of the total A. gambiae s.l. were found in five (0.6%) LTCs, with a maximum of 5889 caught in a single trap. Of 505 A. gambiae s.l. speciated by polymerase chain reaction, 91.5% were found to be A. arabiensis. Plasmodium falciparum sporozoite enzyme-linked immunosorbent assay tests were performed on 10 108 anopheles mosquitoes and 39 (0.38%) were positive. Entomological inoculation rate (EIR) estimates were generated using a standard method and an alternative method that allows the calculation of confidence intervals based on a negative binomial distribution of sporozoite positive mosquitoes. Overall EIR estimates were similar; 31 vs. 29 [95% confidence interval (CI): 19, 44] infectious bites per annum, respectively. The EIR ranged from 4 (95% CI: 1, 17) in the cool season to 108 (95% CI: 69, 170) in the wet season and from 54 (95% CI: 30, 97) in the east of the town to 15 (95% CI: 8, 30) in the town centre. These estimates show large variations over short distances in time and space. They are all markedly lower than those reported from nearby rural areas and for other parts of Tanzania